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Zgoba, Kristen M., Wayne R. Sager, and Philip H. Witt. "Evaluation of New Jersey's Sex Offender Treatment Program at the Adult Diagnostic and Treatment Center: Preliminary Results." Journal of Psychiatry & Law 31, no. 2 (2003): 133–64. http://dx.doi.org/10.1177/009318530303100202.
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This study examined 10-year sexual and non-sexual offense recidivism for sex offenders released from New Jersey's general prison system and from the Adult Diagnostic and Treatment Center (ADTC), New Jersey's correctional facility and treatment center for repetitive-compulsive sexual offenders. The study found that sexual offenders released from the ADTC had significantly lower rates of committing both non-sexual offenses and any offense, compared with the general prison population of sex offenders. For both groups, the 10-year sexual offense reconviction rates were relatively low, 8.6% for the ADTC offenders and 12.7% for the general prison sexual offenders, while reoffense rates for non-sexual offenses were 25.8% and 44.1% for ADTC and general prison sex offenders, respectively.
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Egeli, B., and S. Ugurlu. "POS1369 ADULT-ONSET STILL’S DISEASE: A SINGLE-CENTER EXPERIENCE." Annals of the Rheumatic Diseases 80, Suppl 1 (2021): 966.2–966. http://dx.doi.org/10.1136/annrheumdis-2021-eular.3280.
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Background:Adult-Onset Still’s disease (AOSD) is an autoinflammatory condition characterized by fever, rash, and arthritis. The diagnosis of AOSD is made by excluding common causes of fever of unknown origin which are infections, malignancies, autoimmune conditions and medication adverse effects. As it is a diagnostic challenge, further data on highlighting clinical and laboratory findings are necessary on guiding clinicians.Objectives:Our main objective is to present our single tertiary center experience of patients diagnosed with AOSD.Methods:This retrospective study was conducted at a tertiary rheumatology center. Patients were diagnosed with AOSD using Yamaguchi’s criteria and followed between 2007 and 2020. Demographic, clinical and laboratory information was retrieved from the patient chars. Treatment-related and prognostic information were also noted with additional information from phone call interviews.Results:The study includes 69 patients (23 M, 46 F). The mean age of diagnosis was 33.86±14.3. The presenting signs and symptoms of the patients are shown in Figure 1. The laboratory findings supporting the diagnosis at initial encounter are summarized in Table 1. The mean corticosteroid dose at initial diagnosis was 29.7±18 mg. In addition to corticosteroid treatment these patients were followed with different glucocorticoid-sparing agents. Methotrexate was the choice of treatment in 54 patients with the mean dose of 14.5±3.43 mg. Eight patients were treated with leflunomide, seven with anti-TNF agents, seven with tocilizumab, nineteen with anakinra and four with canakinumab.Figure 1.The presenting signs and symptoms of the patientsConclusion:In conclusion, the most common presenting symptoms in our AOSD cohort were fever and salmon-colored rash. In the differential diagnosis of fever of unknown origin especially with rash, AOSD should be considered. Corticosteroid was the main treatment modality. In patients who are unresponsive to conventional immunosuppressive treatment, biologic agents can be an alternative.Table 1.The laboratory findings at initial encounterMean ± Standard DeviationFerritin (ng/mL)3179.46±6503.56ESR (mm/h)77.43±28.47CRP (mg/L)102.29±70.39Leukocyte Count (cells/L)13147.3±4640.9ESR (mm/h)80±28.48CRP (mg/L)105.15±54.67Leukocyte Count (cells/L)12427.14±6530.43Disclosure of Interests:None declared
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Dzhordzhikiya, R. K., I. I. Vagizov, G. I. Kharitonov, I. V. Bilalov, and M. N. Mukharyamov. "Changes in tactics and treatment results in congenital heart diseases in adults during the last 15 years (investigation based on the data of cardio-surgical departments of the Republic of Tatarstan)." Kazan medical journal 96, no. 4 (2015): 623–28. http://dx.doi.org/10.17750/kmj2015-623.
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Aim. To summarize and to distinguish the features of diagnosis and treatment of congenital heart diseases in adults characteristic for the contemporary cardio surgery, by a retrospective study of case reports.
 Methods. The study analyzed the medical charts of 245 adults with congenital heart diseases treated in the Cardio surgical department №2 of the Interregional Clinical and Diagnostic Center between 2007 and 2015 years. The comparison group included 701 adult patients with congenital heart diseases treated in the Kazan Center of Cardiovascular surgery between 1987 and 1997.
 Results. The prevalence of congenital heart diseases in adults remains significant. In recent years, the share of operations for these diseases is 5.4% of all cardiac interventions. The most frequent pathology is atrial septal defects. The most frequent complications of septal defects are tricuspidal valve insufficiency (83%) and pulmonary hypertension (77%). Comorbidities include: hypertensive heart disease (36%), cardiac arrhythmias (31%) and ischemic heart disease (33%). The number surrendered surgeries decreased from 28.8% in 1997 to 3.7% in 2014. Postoperative mortality has decreased from 3.3% to 1%. Nowadays a significant amount of operations (56%) is transcatheter occlusion. There is an experience of such surgeries in defects of secondary atrial septum and patent ductus arteriosus.
 Conclusion. Congenital heart diseases in adults are characterized by severe impairment of haemodynamics, leading to arterial pulmonary hypertension and tricuspid insufficiency, therefore, require early diagnostics and timely treatment before the onset of complications. 68% of such adult patients suffer from comorbidities that increase the risk of surgical interventions. Endovascular surgeries are low invasive, have minimal complication rate and produce good immediate and long-term results.
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Petryaykina, E. E., I. O. Shchederkina, I. P. Vitkovskaya, et al. "Primary pediatric stroke center in the multidisciplinary pediatric hospital. New reality in pediatrics." City Healthcare 1, no. 1 (2020): 15–30. http://dx.doi.org/10.47619/2713-2617.zm.2020.v1i1;15-30.
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Background. The increase in the number of diagnosed strokes in children, significant distinctive features and complexity of diagnosis, treatment and prevention of cerebrovascular diseases in children compared with adults, the impossibility of direct extrapolation of therapeutic recommendations from adult practice to pediatrics required the creation of specialized primary centers of pediatric stroke in Russia. Purpose. The purpose of this article is to describe the experience of organizing a Center for the treatment of children and adolescents with cerebrovascular diseases (hereinafter referred to as the Center) on the basis of the multidisciplinary pediatric hospital of the Morozovskaya Children's City Clinical Hospital of Moscow Healthcare Department, the introduction of modern methods of diagnosis and treatment of strokes in pediatrics, the organization of preventive measures, maintaining the city register of children's stroke, coordinating the provision of medical care to children with cerebrovascular diseases at various levels in the city of Moscow. Materials and methods. The presented experience of organizing and operating the Center covers the period from 2014 to 2019. Researchers used descriptive, statistical, and comparative analysis to demonstrate the Center's performance and justify proposed improvements in diagnostics, management of pediatric patients with cerebrovascular disease, and relapse prevention. Results. In the course of the organization and operation of the Center, there was assessed the frequency of children's strokes in the city: in 2015 - 6.59 cases per 100 thousand of the child population of Moscow; in 2016 - 6.51 per 100 thousand; in 2017 - 6.43 per 100 thousand and in 2018 - 5.86 per 100 thousand. There were improved: the diagnostics of cerebrovascular pathology and its algorithm, modern reperfusion methods of treatment (thrombolysis, thromboextraction) were introduced into practice, outpatient observation. The equipment and trained specialists concentration on the basis of the Center allowed the creation of the Center "full cycle". Maintaining the city register of pediatric stroke made it possible to compare Russian data with those available in the literature and to establish international cooperation with the International Pediatric Stroke Organization. Conclusion. The establishment of the Center is an important example of interdisciplinary interaction in pediatrics. The City Register of Pediatric Stroke will make it possible to assess the problem of childhood stroke in Moscow. The accumulated organizational, medical and diagnostic, scientific, international and educational experience of the Center can be introduced in other regions of the Russian Federation to improve the provision of medical care to children and to solve the most important problem - preserving the health of the country's child population.
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Vlasova, A. V., T. A. Tenovskaya, L. V. Dymnova, et al. "Primary pediatric stroke center in the multidisciplinary pediatric hospital. New reality in pediatrics." City Healthcare 1, no. 1 (2020): 51–59. http://dx.doi.org/10.47619/2713-2617.zm.2020.v1i1;51-59.
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Background. The increase in the number of diagnosed strokes in children, significant distinctive features and complexity of diagnosis, treatment and prevention of cerebrovascular diseases in children compared with adults, the impossibility of direct extrapolation of therapeutic recommendations from adult practice to pediatrics required the creation of specialized primary centers of pediatric stroke in Russia. Purpose. The purpose of this article is to describe the experience of organizing a Center for the treatment of children and adolescents with cerebrovascular diseases (hereinafter referred to as the Center) on the basis of the multidisciplinary pediatric hospital of the Morozovskaya Children’s City Clinical Hospital of Moscow Healthcare Department, the introduction of modern methods of diagnosis and treatment of strokes in pediatrics, the organization of preventive measures, maintaining the city register of children’s stroke, coordinating the provision of medical care to children with cerebrovascular diseases at various levels in the city of Moscow. Materials and methods. The presented experience of organizing and operating the Center covers the period from 2014 to 2019. Researchers used descriptive, statistical, and comparative analysis to demonstrate the Center’s performance and justify proposed improvements in diagnostics, management of pediatric patients with cerebrovascular disease, and relapse prevention. Results. In the course of the organization and operation of the Center, there was assessed the frequency of children’s strokes in the city: in 2015 - 6.59 cases per 100 thousand of the child population of Moscow; in 2016 - 6.51 per 100 thousand; in 2017 - 6.43 per 100 thousand and in 2018 - 5.86 per 100 thousand. There were improved: the diagnostics of cerebrovascular pathology and its algorithm, modern reperfusion methods of treatment (thrombolysis, thromboextraction) were introduced into practice, outpatient observation. The equipment and trained specialists concentration on the basis of the Center allowed the creation of the Center «full cycle». Maintaining the city register of pediatric stroke made it possible to compare Russian data with those available in the literature and to establish international cooperation with the International Pediatric Stroke Organization. Conclusion. The establishment of the Center is an important example of interdisciplinary interaction in pediatrics. The City Register of Pediatric Stroke will make it possible to assess the problem of childhood stroke in Moscow. The accumulated organizational, medical and diagnostic, scientific, international and educational experience of the Center can be introduced in other regions of the Russian Federation to improve the provision of medical care to children and to solve the most important problem - preserving the health of the country’s child population.
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Thomas, John, Prasanth Thangavel, Wei Yan Peh, et al. "Automated Adult Epilepsy Diagnostic Tool Based on Interictal Scalp Electroencephalogram Characteristics: A Six-Center Study." International Journal of Neural Systems 31, no. 05 (2021): 2050074. http://dx.doi.org/10.1142/s0129065720500744.
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The diagnosis of epilepsy often relies on a reading of routine scalp electroencephalograms (EEGs). Since seizures are highly unlikely to be detected in a routine scalp EEG, the primary diagnosis depends heavily on the visual evaluation of Interictal Epileptiform Discharges (IEDs). This process is tedious, expert-centered, and delays the treatment plan. Consequently, the development of an automated, fast, and reliable epileptic EEG diagnostic system is essential. In this study, we propose a system to classify EEG as epileptic or normal based on multiple modalities extracted from the interictal EEG. The ensemble system consists of three components: a Convolutional Neural Network (CNN)-based IED detector, a Template Matching (TM)-based IED detector, and a spectral feature-based classifier. We evaluate the system on datasets from six centers from the USA, Singapore, and India. The system yields a mean Leave-One-Institution-Out (LOIO) cross-validation (CV) area under curve (AUC) of 0.826 (balanced accuracy (BAC) of 76.1%) and Leave-One-Subject-Out (LOSO) CV AUC of 0.812 (BAC of 74.8%). The LOIO results are found to be similar to the interrater agreement (IRA) reported in the literature for epileptic EEG classification. Moreover, as the proposed system can process routine EEGs in a few seconds, it may aid the clinicians in diagnosing epilepsy efficiently.
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Rosenberg, Anna G. W., Minke R. A. Pater, Kirsten Davidse, et al. "What Every Internist Should Know About Rare Genetic Syndromes in Order to Prevent Needless Diagnostics, Missed Diagnoses and Medical Complications: Five-Year Experience of Internal Medicine for Complex Rare Genetic Syndromes." Journal of the Endocrine Society 5, Supplement_1 (2021): A513—A514. http://dx.doi.org/10.1210/jendso/bvab048.1050.
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Abstract Introduction: Patients with complex rare genetic syndromes (CRGS) have, by definition, combined medical problems affecting multiple organ systems. Intellectual disability (ID) is often part of the syndrome. During childhood, most patients with CRGS receive multidisciplinary (MD) and specialized pediatric care in tertiary centers. As improvement of medical care has improved life expectancy, more and more patients are now reaching adult age. While the complexity of the syndromes persist into adulthood, adequate multidisciplinary syndrome-specific care is rarely available for adults with CRGS. Although multiple organ systems are usually affected, internists are rarely involved. Pediatricians have expressed the urgent need for adequate, syndrome-specific, MD tertiary healthcare for adults with CRGS. Methods: In 2015 we have launched the Center for Adults with CRGS, a specialized MD outpatient clinic (MOPC) within the Endocrinology unit of the department of Internal Medicine. As adult manifestations are unknown for most CRGS, all CRGS patients who visit our MOPC undergo a systematic health screening (followed by treatment, if indicated). Before visiting the MOPC, caregivers fill out a medical questionnaire. We gathered the physical complaints, medication use and missed diagnoses of 726 adults with CRGS. Results: Between 2015 and 2020, 256 males and 470 females with over 60 syndromes visited the Center for Adults with CRGS. The main features of this population, as compared with general internal medicine patients, were intellectual disability, polypharmacy and use of psychotropic drugs. Missed diagnoses were common and many patients had undergone extensive diagnostic tests for symptoms that could actually be explained by their syndrome. Fatigue (52%), abdominal discomfort (23%) and hypertension (10%) were among the most frequent reasons for referral to Internal Medicine. Based on the literature and our clinical findings, 73% of the syndromes was associated with endocrine problems. We provide an algorithm for the clinical approach to CRGS adults, in order to prevent unnecessary diagnostics as well as missed diagnoses. Conclusion: Our overview of 726 adults with CRGS shows that missed diagnosed and needless invasive tests are common in this patient population. As more and more CRGS patients are now reaching adult age and transfer to Internal Medicine, internists and endocrinologists should be aware of the special needs of adults with CRGS and of the medical pitfalls. Knowledge about syndrome-specific health problems and multidisciplinary expert care is crucial to prevent the personal and financial burden of unnecessary diagnostics and under- and overtreatment.
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Esaulenko, Elena Vladimirovna, and Anastasiya Aleksandrovna Sukhoruk. "Liver transplantation in children and adults." Pediatrician (St. Petersburg) 6, no. 3 (2015): 98–103. http://dx.doi.org/10.17816/ped6398-103.
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The article presents an analysis of modern domestic and foreign literature on the problems of liver transplantation in children and adults. Liver transplantation is performed for more than 50 years and it has become a reliable, rational way, and sometimes the only life-saving treatment for children and adults suffering from diffuse liver disease or unresectable tumors. Currently, the Russian Federation opened 14 centers for liver transplantation (in Moscow, St. Petersburg, Belgorod, Krasnodar, Irkutsk, Novosibirsk, Yekaterinburg and Nizhniy Novgorod), which is performed annually 200-240 operations. Estimated demand for the provision of this service is much higher: 52-64 center, and 2000-2500 operations per year. However, liver transplantation in children performs only in two centers in Moscow. Possibility of timely planning of liver transplantation in children of any age has now become a reality, but with a very limited capacity. Recently there has been improvement in diagnostic capabilities to identify various congenital and acquired diseases of the liver in children requiring liver transplantation. We can already predict the increase in demand for this type of surgery. In the North-Western Federal District is a center in which possible liver transplantation in adults, while the children’s opportunities for transplantation in the region do not have. The reasons leading to liver transplantation in children and adults are different. In adult practice dominated by cirrhosis in the outcome of chronic viral hepatitis B and C, whereas in children biliary atresia and congenital metabolic disorders.
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Gallucci, Fernando, Ilaria Ronga, Francesco Di Pietto, Gerardino Amato, Rosario Buono, and Generoso Uomo. "Adult bacterial myositis: report of a single-center series of 26 cases." Italian Journal of Medicine 10, no. 3 (2016): 226. http://dx.doi.org/10.4081/itjm.2016.667.
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Bacterial infections involving muscle are quite uncommon and generally require specific predisposing factors. Bacterial myositis is more rarely described in the typical kind of patients observed in Internal Medicine (presence of multiple co-morbidities, partial/limited immune-deficiency, advanced age). Twenty-six patients suffering from bacterial myositis (8 women and 18 men; mean age 58.5 years, range 27-82) observed in a single Internal Medicine Unit were reported. Muscles involved were ileopsoas, thigh, paravertebral, gluteus, calf, forearm and rectus abdomen. Simultaneous presence of arthritis was registered in 17 patients and all patients presented relevant comorbidity. Main cultured bacteria were <em>Staphylococcus aureus, Escherichia coli</em>, other Gram-negative bacteria, <em>Streptococcus spp.</em> Multi-drug-resistance was observed in 14 out 26 (53.8%). Computed tomography, ultrasound and magnetic resonance imaging were utilized for diagnostic purposes. Antibiotic treatment was administered to all patients. Surgical debridement and drainage were performed in 12 patients; 7 patients were treated with percutaneous aspiration and drainage. At discharge, relevant functional impairment was present in 17 patients (65.3%). Four patients died (in-hospital mortality 7.6%, global mortality at three months 15.3%). Management of bacterial myositis is difficult and its prognosis is poor. In the near future, this demanding infection will be more frequently observed in Internal Medicine setting as comorbidity, which is very often the main characteristic of these patients.
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Janssen, Nico A. F., Roger J. M. Brüggemann, Monique H. Reijers, et al. "A Multidisciplinary Approach to Fungal Infections: One-Year Experiences of a Center of Expertise in Mycology." Journal of Fungi 6, no. 4 (2020): 274. http://dx.doi.org/10.3390/jof6040274.
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Invasive fungal diseases (IFDs) often represent complicated infections in complex patient populations. The Center of Expertise in Mycology Radboudumc/CWZ (EMRC) organizes a biweekly multidisciplinary mycology meeting to discuss patients with severe fungal infections and to provide comprehensive advice regarding diagnosis and treatment. Here, we describe the patient population discussed at these meetings during a one-year period with regards to their past medical history, diagnosis, microbiological and other diagnostic test results and antifungal therapy. The majority of patients discussed were adults (83.1%), 62.5% of whom suffered from pulmonary infections or signs/symptoms, 10.9% from otorhinolaryngeal infections and/or oesophagitis, 9.4% from systemic infections and 9.4% from central nervous system infections. Among children, 53.8% had pulmonary infections or signs/symptoms, 23.1% systemic fungal infections and 23.1% other, miscellaneous fungal infections. 52.5% of adult patients with pulmonary infections/symptoms fulfilled diagnostic criteria for chronic pulmonary aspergillosis (CPA). Culture or polymerase chain reaction (PCR) demonstrated fungal pathogens in 81.8% of patients, most commonly Aspergillus. A multidisciplinary mycology meeting can be a useful addition to the care for patients with (I)FDs and can potentially aid in identifying healthcare and research needs regarding the field of fungal infections. The majority of patients discussed at the multidisciplinary meetings suffered from pulmonary infections, predominantly CPA.
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Arney, Luke, Ryan Leib, Louise Schneider, et al. "QIM19-117: Reducing Diagnostic Delays: Impact of a Novel Cancer Diagnostic Service." Journal of the National Comprehensive Cancer Network 17, no. 3.5 (2019): QIM19–117. http://dx.doi.org/10.6004/jnccn.2018.7222.
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Background: Although timeliness of care was one of the aims of quality improvement in the Institute of Medicine’s 2001 “Crossing the Quality Chasm” report, a significant proportion of patients with cancer still experience delays in diagnosis and treatment. For example, in a study of 3,831 older adults diagnosed with myeloma in the United States, the median time between the first myeloma-related symptom and diagnosis was 99 days (Friese CR, Leukemia & Lymphoma 2009). Such delays are associated with substantial anxiety, poor patient-reported outcomes, and increased cost. Methods: A novel adult cancer diagnostic service (CDS) was established by the Dana-Farber/Brigham and Women’s Cancer Center in October 2017. The clinic is embedded in the Department of Medicine at the Brigham and Women’s Hospital, with the aim of expediting the cancer diagnostic work-up and treatment for clinically complex patients with symptoms concerning for cancer, but for whom the next diagnostic steps are unclear. This clinic is comprised of an internist, a physician assistant, and a practice assistant. The clinic staff conduct a weekly phone conference with a multidisciplinary team—including a solid tumor oncologist, a hematologic oncologist, and a radiologist—to discuss the work-up for each patient. For every patient evaluated who receives a cancer diagnosis, we measure the diagnostic interval (days from CDS referral to diagnosis date). We define diagnosis date as the date the pathologic report is signed. We also measure the interval between CDS referral and first oncology appointment. Results: From the inception of the CDS to October 1, 2018, 221 patients were seen in the clinic and 91 (41.2%) were diagnosed with cancer. The top 3 cancer diagnoses were lymphoma (31%), gastrointestinal cancers (20%), and lung cancer (19%; Figure 1). The median number of days from CDS referral to diagnosis was 14 days (interquartile range [IQR], 10, 21; Table 1). Finally, the median time between referral to CDS and first oncology appointment was 20 days (IQR, 14, 27). Conclusion: This novel cancer diagnostic service substantially shorted the diagnostic trajectory (∼2 weeks) compared to existing literature with median diagnostic intervals often lasting more than 3 months (Friese CR, Leukemia & Lymphoma 2009). Our findings suggest that a cancer diagnostic care model, grounded in internal medicine, with engagement of oncologists and radiologists, has significant potential to improve delays in cancer diagnostic care.
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Hur, Joon Young, Kang Kook Lee, Jun Ho Jang, et al. "Clinical Relevance of Diagnostic Criteria for Hemophagocytic Lymphohistiocytosis (HLH) and Survival Outcome of Adult HLH Patients: A Single-Center Prospective Cohort Study." Blood 132, Supplement 1 (2018): 3718. http://dx.doi.org/10.1182/blood-2018-99-114858.
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Abstract Introduction Hemophagocytic lymphohistiocytosis (HLH) is an immune-mediated life-threatening condition in which activated macrophages phagocytize hematopoietic cells in various clinical situations from physiologic reactions to pathologic conditions such as malignancy. The diagnosis of HLH is currently done according to the HLH-2004 criteria which were mainly based on pediatric experiences even though underlying causes and clinical features of adult patients with HLH are different from pediatric patients. However, there is little information about the value of each item and its clinical relevance in the diagnosis of adult patients. Thus, we analyzed the diagnostic value of HLH-2004 criteria in adult patients who had symptoms or signs suspicious of hemophagocytosis. Methods We have conducted a prospective cohort study for adult HLH since January of 2017 (Prospective cohort for adult hemophagocytosis: NCT03117010). Adult patients older than 18 years having at least one of the following problems could be enrolled onto this prospective cohort: 1) Pathologically confirmed hemophagocytosis in bone marrow or lymph nodes; 2) Presence of at least 3 conditions among 8 items of HLH-2004 diagnostic criteria. After obtaining written informed consent, we performed laboratory tests for the diagnosis of HLH including natural killer (NK)-cell activity and soluble CD25 in blood. The primary objective of this study was to explore the feasibility and clinical usefulness of HLH-2004 criteria in adult patients, and secondary objective was to analyze underlying causes and outcomes of adult HLH. Results At the time of analysis, 44 patients were enrolled in the cohort, and their median age was 51.5 years (range, 19-85 years). Male (n=27) was more common than female (n=17). All patients had fever (body temperature ≥ 38.5°C) and splenomegaly was found in 29 patients (66%, Figure 1). Although cytopenia such as neutropenia and thrombocytopenia was commonly found, only 20 patients had bi-lineage cytopenia (45%). Hypofibrinogenemia and/or hypertriglyceridemia were also less common findings (n=18, 41%). Hemophagocytosis in bone marrow or lymph nodes was pathologically confirmed in 27 patients (61%). Elevated serum level of ferritin (≥ 500 mg/L) and soluble CD25 (≥ 2400 U/mL) were 42 (95%) and 35 patients (80%), respectively. Among 33 patients whose NK-cell activity was evaluated, 22 patients had low or absent NK-cell activity (67%). According to diagnostic criteria, 28 patients having at least five conditions were diagnosed with HLH, and the remaining patients including 7 patients with bone marrow hemophagocytosis could not be diagnosed. Among 28 patients with HLH, T/NK-cell (n=8) and B-cell lymphomas (n=7) were the most common underlying disorders (n=15, 54%). Infection including EBV (n=6) and rheumatologic disorders (n=3) accounted for non-malignancy associated HLH whereas the remaining four patients' cause was not identified. At the time of analysis, 14 patients died due to uncontrolled HLH or underlying disorders or infectious complications during treatment. The median overall survival of all patients was 13.9 months (95% CI: 10.9 - 16.9), and patients with HLH was not significantly different from patients without HLH (p=0.655). The survival outcome of patients with lymphoma was significantly worse than patients without it regardless of HLH diagnosis (p<0.001). Conclusions Fever and elevated level of serum ferritin were the most frequent condition in patients having suspicious symptoms of HLH. Low or absent NK-cell activity and increased level of soluble CD25 could have additional diagnostic value for timely diagnosis of adult HLH. Considering lymphoma is frequently associated with adult HLH, immediate evaluation for lymphoma should be done in adult patients suspicious of HLH. Figure. Figure. Disclosures No relevant conflicts of interest to declare.
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Liu, Yao, Jun Rao, Jiali Li, et al. "Tandem autologous hematopoietic stem cell transplantation for treatment of adult T-cell lymphoblastic lymphoma: a multiple center prospective study in China." Haematologica 106, no. 1 (2019): 163–72. http://dx.doi.org/10.3324/haematol.2019.226985.
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T-cell lymphoblastic lymphoma (T-LBL) is a highly aggressive form of lymphoma with poor clinical outcomes and lacks of a standard treatment regimen. In this study, we assessed the safety and efficacy of tandem autologous hematopoietic stem cell transplantation (auto-HSCT) strategy for adult T-LBL and evaluated prognostic factors affecting survival. 181 Newly-diagnosed adult T-LBL patients were enrolled, 89 patients were treated with chemotherapy alone, 46 patients were allocated to single auto-HSCT group, 46 patients were treated with tandem auto-HSCT. The median follow-up time was 37 months, the 3-year progression/relapse rate of the tandem auto-HSCT group was significantly lower than that of the single auto-HSCT group and chemotherapy group (26.5% vs 53.1% and 54.8%). The 3-year PFS and OS rate of the tandem auto-HSCT group (73.5% and 76.3%) were significantly higher than those of the single auto-HSCT group (46.9% and 58.3%) and the chemotherapy group (45.1% and 57.1%). In the tandem auto-HSCT group, age and disease status after the first transplantation impacted the OS and PFS. Multivariate analysis identified that disease status after the first transplantation was the only independent prognostic factor for patients treated with tandem-HSCT. In addition, diagnostic models of the initial CD8+CD28+/CD8+CD28- T cell ratio in predicting the disease status were found to be significant. Taken together, tandem auto-HSCT can be considered an optimal strategy for adult T-LBL patients (ChiCTR-ONN-16008480).
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Climans, Seth Andrew, Ronald Cepe Ramos, Normand Laperriere, Mark Bernstein, and Warren P. Mason. "Outcomes of presumed malignant glioma treated without pathological confirmation: a retrospective, single-center analysis." Neuro-Oncology Practice 7, no. 4 (2020): 446–52. http://dx.doi.org/10.1093/nop/npaa009.
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Abstract Background Tissue diagnosis is essential in the usual management of high-grade glioma. In rare circumstances, due to patient preference, performance status, comorbidities, or tumor location, biopsy is not feasible. Sometimes a biopsy is nondiagnostic. Many neuro-oncology clinics have patients like this, but these patients’ outcomes and responses to treatment are not known. Methods We retrospectively reviewed records from adult patients diagnosed with presumed high-grade glioma of the brain without definitive pathology, diagnosed between 2004 and 2016. We recorded several clinical variables including date of first diagnostic imaging and date of death. Results We identified 61 patients and subclassified them to brainstem glioma (n = 32), supratentorial presumed glioblastoma (n = 24), presumed thalamic diffuse midline glioma (n = 2), gliomatosis cerebri (n = 2), and cerebellar glioma (n = 1). Most brainstem glioma patients had no biopsy because of tumor location. Supratentorial presumed glioblastoma patients had no biopsy predominantly because of comorbidities. Median survival, from first diagnostic imaging, was 3.2 months (95% CI: 2.9 to 6.3 months) in the supratentorial glioblastoma group and 18.5 months (95% CI: 13.0 to 44.1 months) in the brainstem group. Treatment with radiation or chemotherapy did not alter the median survival of the supratentorial glioblastoma group (hazard ratio 1.41, uncorrected P = .5). Conclusions Patients with imaging diagnoses of high-grade glioma have similar, if not worse, survival than those with pathological confirmation. Based on these uncontrolled data, it is unclear how effective radiation or chemotherapy is in this population.
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Gennaro, Nicolò, Andrea Marrari, Salvatore Lorenzo Renne, et al. "Multimodality imaging of adult rhabdomyosarcoma: the added value of hybrid imaging." British Journal of Radiology 93, no. 1112 (2020): 20200250. http://dx.doi.org/10.1259/bjr.20200250.
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Rhabdomyosarcoma (RMS) represents more than 50% of paediatric soft tissue tumours. Conversely, it is extremely rare among adults, where it shows peculiar biological and clinical features that are still poorly investigated. RMS patients should be referred to a Sarcoma Centre, where the contribution of experienced radiologists plays a relevant role in the diagnostic assessment of the disease, including precise localisation, staging, image-guided biopsy, response evaluation after treatment and follow-up. Besides CT and MRI, hybrid imaging including positron emission tomography (PET)/CT and PET/MRI are giving an increasing contribution to provide functional insights about tumour biology and to improve the diagnostic accuracy of the imaging work-up. This review paper provides a revision of the pathology, clinical and radiological features of adult RMS, with a particular focus on the growing role of hybrid PET-based imaging.
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Nuzhny, Valentin, Ruckshanda Majid, Komal Imtiaz, and Reeba Mathew. "425 Investigating the Utility of Routine Carbon Dioxide Measurements During Polysomnography in the Evaluation of Obese Adult Patients." Sleep 44, Supplement_2 (2021): A168. http://dx.doi.org/10.1093/sleep/zsab072.424.
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Abstract Introduction With the increasing prevalence of obesity, the diagnosis of obstructive sleep apnea (OSA) and obesity hypoventilation syndrome (OHS) have also increased. Adding routine transcutaneous carbon dioxide (TcCO2) or end-tidal carbon dioxide sensors (EtCO2) may add beneficial information to the polysomnogram (PSG) and expand the diagnostic and treatment capabilities in this population. Our study looks at the use of this parameter in obese adults on whom CO2monitoring has been used. Methods We performed a retrospective chart review of obese adult patients (body mass index [BMI] &gt;30) undergoing a PSG. We documented the EtCO2 values at baseline (supine awake) and during sleep. Correlations between the EtCO2 readings and BMI were reviewed. We excluded patients that had poor EtCO2 waveforms and patients with known preexisting hypoventilation syndromes, such a COPD. Results Fifty patients were identified between January and November 2020 at the Memorial Hermann Sleep Center. 54% were female and 46% were male with an average age of 55.3 years (range 26–73) and an average BMI for the cohort of 40.1 (SD +/-9.5). The average AHI on the diagnostic study (CMS criteria) was 30.9 events/hour (SD +/- 43) and the average oxygen desaturation nadir was 79%. Sixteen patients (32%) met diagnostic criteria for OHS based on the baseline awake EtCO2 which would have otherwise been missed without CO2 monitoring. When comparing the mean values of the ETCO2 between Group 1 whose BMI was less than 40 kg/m2 (39.9 mmHg) to Group 2 whose BMI was greater than 40 kg/m2 (45.9 mm Hg), the difference was statistically significant with a p-value is 0.001. Conclusion OHS is reported to have greater mortality when compared to OSA. CO2 monitoring is currently only routinely required in pediatric PSGs. Our review suggests a higher diagnostic yield of OHS in adults with the use of CO2 monitoring especially when morbidly obese. Given the alarming trend towards obesity in the US, this advocates for the routine use of CO2 monitoring in adult obese patients. Although more research is needed, we may draw a conclusion that there is meaningful data to support the use of routine ETCO2 monitoring in this adult patient population. Support (if any):
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Parikh, Sameer A., Prashant Kapoor, Shaji K. Kumar, Louis Letendre, and Alexandra Wolanskyj. "Outcome of Adult Patients with Hemophagocytic Syndrome: A Tertiary Care Center Experience." Blood 120, no. 21 (2012): 1040. http://dx.doi.org/10.1182/blood.v120.21.1040.1040.
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Abstract Abstract 1040 Introduction: Hemophagocytic syndrome (HPS) is a rare disorder characterized by fever, splenomegaly, cytopenias, hypofibrinogenemia, hyperferritinemia and hemophagocytosis in the bone marrow or lymph nodes. It is caused by pathologic activation of the immune system, and manifests clinically with signs and symptoms related to extreme inflammation. Primary HPS or familial hemophagocytic lymphohistiocytosis (HLH) occurs predominantly in children (<18 years) due to inherited defects in natural killer and cytotoxic T-cell function. Secondary HPS generally occurs in adults (>18 years) and is precipitated by autoimmune disorders, malignancy and a variety of infections. The results of HLH-94 trial, the largest prospective study in pediatric patients with HLH, reported an estimated 5-year survival of 54%. There is a paucity of information about HPS in adult patients. Methods: Records of all adult (≥ 18 years) patients with a diagnosis of “hemophagocytosis“ or “hemophagocytic syndrome” presenting from January 1996 through December 2011 at Mayo Clinic were reviewed. Our hematopathology database was also analyzed for patients bearing a diagnosis of HPS. Out of a total of 127 patients identified in the database, 50 adult patients were diagnosed to have HPS as per the diagnostic criteria of the HLH-1994 trial (for patients seen prior to 2004), and HLH-2004 trial (for patients seen after 2004). This study was approved by the Institutional Review Board at Mayo Clinic. Results: Baseline characteristics of all patients are shown in Table 1. The median time to diagnosis from the onset of symptoms was 38 days (4–158 days). Secondary HPS was diagnosed in 46 (92%) patients. The causes of secondary HPS are shown in Table 2. Twenty (40%) patients were treated with etoposide containing regimen, 26 (52%) were treated with therapeutic agents directed specifically to the underlying precipitating disease, and 4 (8%) patients did not receive any treatment. No patient in our study underwent a stem cell transplant (SCT). After a median follow-up of 43 months, 36 (72%) patients have died. The causes of death were infection (47%), malignancy (42%), bleeding (8%) and unknown (3%). The median overall survival (OS) for the entire cohort was 1.5 months (95% CI, 0.7–4.5 months). The median OS for patients with malignancy- related HPS was 0.8 months (95% CI, 0.4–2.4 months) compared to 11 months (95% CI, 0.7-not reached) for patients with non-malignancy related HPS (p-value:0.006; Figure). On univariableanalysis, factors associated with a worse survival were serum albumin <2 g/dL (hazard ratio, 8.3), malignancy-related HPS (hazard ratio, 7.3) and serum creatinine >1.3 mg/dL (hazard ratio, 3.8). Conclusion: In this large series of adult HPS patients treated at a single tertiary care center, most cases are secondary, and are associated with a worse survival compared to historical outcomes reported for children enrolled on the HLH-94 trial. This is likely related to a marked delay in referral to our institution, and high early mortality precluding consideration of SCT. Adult patients with malignancy- related HPS in this study had a markedly inferior outcome compared to patients with non-malignancy related HPS. Disclosures: No relevant conflicts of interest to declare.
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Ardianto, Bambang, Rina Triasih, Eddy Supriyadi, et al. "#23: Survival Analysis of Childhood Acute Myeloblastic Leukemia in a Limited Resource Center: Do Infection and Lymphocyte-Mediated Immune Response Play a Role?" Journal of the Pediatric Infectious Diseases Society 10, Supplement_1 (2021): S18. http://dx.doi.org/10.1093/jpids/piaa170.056.
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Abstract Background Acute myeloblastic leukemia (AML) is the second most prevalent cancer among Indonesian children. In contrast to those of acute lymphoblastic leukemia (ALL), a number of studies reported unfavorable outcomes following the initiation of cytostatic protocols for childhood AML. In addition, our previous study showed that Gram-negative bacterial infection constitutes the major cause of treatment-related mortality in children with AML. These findings raised a question on the prognostic role of diagnostic lymphopenia, which represents the immunosuppressive state, as demonstrated in adult patients with solid tumors. Methods A retrospective cohort study, which involved children younger than 18 years of age with the newly diagnosed, non-M3 AML, was conducted. These children were admitted to Dr. Sardjito General Hospital, Yogyakarta, Indonesia, during the period of 2011–2019 and received National Pilot Protocol (2011–2016), Pediatric AML Protocol (2016–2018), and International Society of Pediatric Oncology (SIOP) Pediatric Oncology in Developing Countries (PODC) Protocol with prephase (2018–2019). One-year overall survival (OS) was analyzed, using diagnostic absolute lymphocyte counts (ALC0) of more than 4.5 × 109/L and less than 4.5 × 109/L as the determinants, in accordance with those reported in a number of studies on adult AML. Results Sixty-five children, which consisted of 41 (63%) children with ALC0 of more than 4.5 × 109/L and 24 (37%) children with ALC0 of less than 4.5 × 109/L, were eligible for this study. In a multivariate logistic regression analysis, compared with those of diagnostic white blood cell and peripheral blood myeloblast counts, treatment-related pneumonia, gastrointestinal tract infection, and sepsis were the most responsible factors for the occurrence of death within the first year of treatment [relative risk (RR), 2.82; 95% confidence interval (95% CI), 0.99–8.04; P = 0.05]. OS analysis showed that 43% and 16% of children with ALC0 of more than 4.5 × 109/L and less than 4.5 × 109/L, respectively, were alive within the first year of treatment. Cox-regression analysis, however, failed to reach statistical significance [hazard ratio (HR), 0.63; 95% CI, 0.33–1.18; P = 0.15]. Conclusion Despite lacked statistical significance, our study showed the superior one-year OS in children with ALC0 of more than 4.5 × 109/L. Our findings, therefore, might indicate the prognostic role of infection and lymphocyte-mediated immune response in childhood AML.
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Travina, M. L., A. G. Popov, S. A. Popov, and E. V. Kulikova. "Cystic changes in the mammary gland structure in different age periods." HEALTH OF WOMAN, no. 1(117) (February 28, 2017): 37–43. http://dx.doi.org/10.15574/hw.2017.117.37.
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The article is devoted to problems of diagnosis of cystic disease of breast at different ages including children and teens. Analyzed various options for classification of cystic changes. The main method of detecting liquid formation is breast ultrasound, then, as a final verification is possible after thorough laboratory studies education (cytological, biochemical and histological method). Therapeutic and diagnostic puncture cysts do not affect the continued operation of the glandular tissue and can be used at any age. Active sonirovanie cysts in children due to the high probability of occurrence of inflammation on the available background cystic cavity. In the adult diagnostic and treatment interventions have a diagnostic character and for reducing local pain symptoms associated with overexertion cysts. Analysis of the results carried out on the basis of examination and treatment of 22 381 patients aged 8 to 72 years, who applied to receive a specialized mammography department of the Federal State Autonomous Institution «National Health Research and Practical Center for Children» of the Ministry of Health of the Russian Federation for the period from 2008 to 2015. Key words: mammalogy, breast cysts, fibrocystic breast disease, breast ultrasound, puncture the cyst.
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Buckley, Sarah A., Daniel Egan, and Roland B. Walter. "Hemophagocytic Lymphohistiocytosis in Adults: A Case Series of 30 Patients At a U.S. Tertiary Care Center." Blood 120, no. 21 (2012): 4681. http://dx.doi.org/10.1182/blood.v120.21.4681.4681.
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Abstract Abstract 4681 Background: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder characterized by overwhelming immune activation that results in excessive inflammation, phagocytosis of blood cells by macrophages, and end-organ damage. Both familial forms (“primary HLH”), often presenting in childhood, and sporadic forms (“secondary HLH”), often presenting in adults, have been recognized. While pediatric HLH is well characterized, only limited data is available on the diagnosis and treatment of HLH in adults. This study, the largest single center case series of adult HLH to date, characterizes cases treated at the University of Washington since 2000 to further our understanding of the clinical characteristics and treatment outcomes of HLH in the adult population. Patients and Methods: Potential cases of HLH were identified by searching hospital billing records for the appropriate ICD-9 code (288.4). Information available from the electronic medical records was used for disease confirmation according to the HLH-2004 criteria, and to obtain relevant information on demographics as well as patient- and disease-related characteristics (including laboratory data, treatment, and outcome. Results: Among the 30 cases who met HLH-2004 diagnostic criteria, the median age was 42 (range: 19 – 76) years. The most common signs and symptoms were fever (100%), splenomegaly (80%), hepatomegaly (40%), lymphadenopathy (40%), and rash (37%). The most common laboratory abnormalities during hospitalization were hyperferritinemia (100%), hepatitis (100%), pancytopenia (93%), elevated creatinine (73%), elevated triglycerides (60%), and low fibrinogen (57%). The presumed causes of HLH were: 30% viral (EBV [n=9], HSV [n=1]), 23% malignancies (B-cell lymphoma [n=4], NK/T-cell lymphoma [n=2], acute lymphoblastic leukemia [n=1]), 20% rheumatologic (Still's disease [n=2], lupus-like syndrome [n=2], Stevens-Johnson syndrome [n=1], dermatomyositis [n=1]), 17% EBV-driven lymphoma (Hodgkin's disease [n=2], NK/T-cell lymphoma [n=1], post-transplant lymphoproliferative disorder-like syndrome [n=1]), 3% bacterial sepsis, and 6% idiopathic. Genetic testing was performed in only 4 patients, without identification of mutations. For treatment, corticosteroids were frequently used; fewer than half the patients received cyclosporine, etoposide, intravenous immunoglobulins, rituximab, interleukin-1 receptor antagonist, or intrathecal therapy. Two patients were retreated after relapse, and one received an allogeneic hematopoietic cell transplant. Two patients received no treatment: one died before therapy was initiated; in the other, treatment was withheld due to pregnancy, and symptoms resolved post-partum. The in-hospital mortality rate was 50%, with most patients dying of multi-organ failure. In univariate analysis, factors associated with in-hospital mortality were older age (49 vs 35 years; p=0.025), lower hemoglobin nadir (6.4 vs 7.3 g/dL; p=0.020), lower triglycerides (284 vs 447 mg/dL; p=0.027), and higher total bilirubin (15.0 vs 6.4 mg/dL; p=0.019); gender, ethnicity, number of co-morbid conditions, symptoms, latency to treatment, and treatment used were not significantly associated. The best outcomes were seen in patients with EBV-driven lymphoma (80% survival, n=5), while the worst outcomes were seen in patients with non-EBV-driven hematologic malignancies (29% survival, n= 7). Patients with EBV-driven disease were more likely to be treated with etoposide (75 vs 11%); those with malignancy were less likely to be treated with cyclosporine (25 vs 65%). Conclusion: A high index of suspicion for HLH should be maintained for adults who present with fever, splenomegaly, cytopenias, and hepatitis. HLH is associated with high mortality, particularly when occurring in older patients, those with anemia and hyperbilirubinemia, and those with non-EBV-driven hematologic malignancies. Further studies from this and other cohorts are needed to develop and refine prognostic criteria for outcomes in adult HLH. Disclosures: No relevant conflicts of interest to declare.
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Alsawah, Fares, Samuel A. Merrill, Paula L. Bockenstedt, et al. "Ruxolitinib Is Active and Well Tolerated in Adult Patients with Secondary Hemophagocytic Lymphohistiocytosis: Preliminary Results of an Ongoing, Open-Label, Single Center Study." Blood 132, Supplement 1 (2018): 1119. http://dx.doi.org/10.1182/blood-2018-99-116854.
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Abstract Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a devastating disorder with protean clinical manifestations caused by the overproduction of T-cell derived cytokines. Cytokine-dependent activation of the Janus family kinases (JAK) is a hallmark of the final common pathway in HLH pathogenesis. Therefore, we initiated a single center, open-label, investigator-initiated trial to assess the efficacy and safety of ruxolitinib in adult patients with secondary HLH. Methods: Adult patients (≥18 years) who fulfilled 5 of 8 diagnostic criteria were eligible. Patients with CNS involvement or an active malignancy were ineligible. Patients who had received any prior systemic therapy (excluding corticosteroids) within 7 days of treatment were ineligible. Patients with normal renal function received oral ruxolitinib 15 mg twice daily on a continuous, 28-day cycle. Dose reductions for renal insufficiency and toxicity are permitted. Treatment was continued indefinitely until disease progression, unacceptable toxicity, or any other conditions for treatment discontinuation were met. The first patient was enrolled in February, 2016 and enrollment is ongoing. The primary endpoint is overall survival at 2 months. Secondary endpoints include the response rate, duration of response, progression-free and overall survival. Adverse events were graded and attributed in accordance with the National Cancer Institute Guidelines for the Cancer Therapy Evaluation Program. Disease parameters evaluable for response included all quantifiable signs and laboratory abnormalities included in the diagnostic criteria for HLH. A complete response (CR) required normalization of all signs and laboratory abnormalities. At least 25% improvement in two or more signs/laboratory abnormalities was required for a partial response (PR). At least a 50% worsening in two or more signs/laboratory abnormalities was considered progressive disease (PD), while failure to fulfill any of these criteria was considered stable disease (SD). Results: A total of 4 patient have been enrolled, all of whom fulfilled at least 5 of 8 diagnostic criteria for HLH. Hemophagocytosis, a pathologic hallmark of HLH, was observed in every patient. At the time of treatment initiation, all patients were anemic [median hemoglobin 7.1 g/dL (range: 6.2-7.5 g/dL)] and thrombocytopenic [median platelet count 47 K/uL (range: 14-107 K/uL)]. Three patients were neutropenic [median ANC 0.95 K/uL (range: 0-1.9 K/uL)]. HLH was secondary to an autoimmune disorder (n=3), infection (n=1), and a homozygous mutation in the STXBP2 gene, recently identified as a causative defect in primary HLH, was observed in one patient. Cytopenias significantly improved within the first week of treatment in all patients. On day +7, the mean increase in hemoglobin was 1.88 g/dL (0.2-3 g/dL), in ANC was 1.53 K/uL (range 0.1-5.3 K/uL) and platelet count was 74 K/uL (range 17-116 K/uL). Neutropenia resolved by day +40 and thrombocytopenia resolved by day +47 in all patients. Three of four patients became transfusion independent within 2 days of treatment initiation. Three of four patients received corticosteroids prior to and after initiation of ruxolitinib. The mean corticosteroid (prednisone equivalent) dose prior to treatment initiation was 225 mg/day (range: 60-391 mg/day) and was 38 mg/day (range: 0-75 mg/day) on day +30. Within 47 days of treatment initiation, corticosteroids were discontinued in 2 patients, and were discontinued in all patients by day +54. Significant improvements in ferritin and sIL-2R were also observed with a median 92.6% decrease in ferritin by day +30 (range 86.4-92.6%) and 86% median decrease in sIL-2R by day +30 (range 66.6-92.7%). All patients achieved at least a PR, and 2 patients remain on treatment (>2 months, >8 months). Two patients discontinued treatment, one for progressive disease (due to recurrent symptoms), and another for drug intolerance (neuropathic foot pain). No severe adverse events have been observed. In addition to the patients enrolled in this study, two additional patients who met eligibility criteria were similarly treated (off study) at another institution. Both of these patients achieved at least a PR and remain on treatment (>8 months, >14 months). Conclusions: Ruxolitinib led to rapid and durable responses and was well tolerated in adult patients with secondary HLH. Disclosures Devata: Affimed: Research Funding. Phillips:Abbvie: Research Funding; Pharmacyclics: Consultancy, Research Funding; Genentech: Consultancy; Bayer: Consultancy; Seattle Genetics: Consultancy; Gilead: Consultancy. Sood:Bayer: Research Funding. Wilcox:Incyte, Corp: Research Funding.
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Rodriguez-Romo, Laura, Alberto Olaya Vargas, Sumit Gupta, et al. "Delivery of Pediatric Cancer Care in Mexico: A National Survey." Journal of Global Oncology, no. 4 (December 2018): 1–12. http://dx.doi.org/10.1200/jgo.17.00238.
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Purpose Limited data describe the delivery of pediatric cancer care in Mexico. We report a nationwide survey of pediatric cancer units. Methods An electronic survey was distributed to 74 pediatric cancer units in Mexico to describe case volumes; organization of care; and availability of medical/surgical specialists, supportive care, complex therapies, and diagnostic services. Centers were classified as low (< 30 new patients/year), medium (30 to 59/year) and high (≥ 60/year). Results Sixty-two centers completed the survey (response rate, 84%). The median annual new case volume per center was 50 (interquartile range [IQR], 23 to 81). Thirty-four percent (n = 21), 26% (n = 16), and 40% (n = 25) of units were low-, medium-, and high-volume centers, respectively. Treatment units reported a median of two pediatric oncologists (IQR, 2) and one pediatric hematologist (IQR, 1 to 2). Availability of medical and surgical subspecialists varied by center size, with substantially more specialist support at higher-volume centers ( P < .01). Multidisciplinary tumor boards are available at 29% (six of 21), 56% (nine of 16), and 76% (19 of 25) of low- to high-volume centers, respectively ( P = .005). Radiation and palliative care services are available at 42% (n = 26) and 63% (n = 36) of all centers, which did not vary by center volume. Educational support for hospitalized children and school reintegration programs are available at 56% (n = 36) and 58% (n = 36) of centers, respectively. One third (38% [n = 23]) of centers reported that at least one half of patients were lost to follow-up during the transition from pediatric to adult programs. Conclusion A large variation exists in annual case volumes across Mexican pediatric cancer centers. Additional efforts to increase access to multidisciplinary, supportive, and palliative care across all pediatric cancer units in Mexico are required.
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Hsiao, Susan Jean, Anthony Sireci, Danielle Pendrick, et al. "Clinical utility and reimbursement for expanded genomic panel testing in adult oncology." Journal of Clinical Oncology 37, no. 15_suppl (2019): 6593. http://dx.doi.org/10.1200/jco.2019.37.15_suppl.6593.
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6593 Background: The routine use of large next generation sequencing (NGS) cancer panels is required to identify the increasing number of, but often uncommon actionable alterations present across multiple tumor histologies to guide therapy. Inconsistent coverage and variable payment is hindering adoption of these tests into clinical practice. A review of clinical utility, coverage and reimbursement was conducted in a cohort of adult oncology patients who received expanded genomic panel testing as part of their clinical care. Methods: The Columbia Combined Cancer Panel (CCCP), a 467 gene panel designed to detect single nucleotide variants, indels, and copy number variations in solid and liquid tumors was performed in a CLIA-certified laboratory at Columbia University Irving Medical Center. Clinical utility categories included: immediate change in management; informed future treatment options; provided diagnostic/prognostic information; and other impact. Claims were submitted between 1/1/17 and 4/30/18. Carriers were categorized into commercial, managed-government, and government plans. Results: 300 tumors underwent NGS. Reimbursement data were available for 258 cases. 57% of testing was performed for a treatment-resistant, recurrent, or high stage cancer, or for a cancer of rare/mixed histology (21%). Findings were clinically actionable in 183 cases (61%). Results led to an immediate change in management (n = 6, 2%), informed future treatment options (n = 140, 47%), and provided diagnostic/prognostic information (n = 29, 10%). Only 57 tests (22%) received coverage. In 59% of denials (118/201), a clinically-actionable result was found. Commercial plans reimbursed 29/119 tests (24%) and managed-government plans reimbursed 28/54 tests (52%). Government plans provided no coverage for 85 tests. On average, insurers reimbursed 10% of the total CCCP service charges: 12.5% for commercial and 22% for managed-government plans. Conclusions: Expanded genomic testing identified clinically-impactful alterations in 61% of cases. Limited coverage and low reimbursement remain a barrier and broader reimbursement policies are needed to adopt expanded genomic testing that benefits patients into clinical practice.
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Athanazio, Rodrigo Abensur, Luiz Vicente Ribeiro Ferreira da Silva Filho, Alberto Andrade Vergara, et al. "Brazilian guidelines for the diagnosis and treatment of cystic fibrosis." Jornal Brasileiro de Pneumologia 43, no. 3 (2017): 219–45. http://dx.doi.org/10.1590/s1806-37562017000000065.
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ABSTRACT Cystic fibrosis (CF) is an autosomal recessive genetic disorder characterized by dysfunction of the CFTR gene. It is a multisystem disease that most often affects White individuals. In recent decades, various advances in the diagnosis and treatment of CF have drastically changed the scenario, resulting in a significant increase in survival and quality of life. In Brazil, the current neonatal screening program for CF has broad coverage, and most of the Brazilian states have referral centers for the follow-up of individuals with the disease. Previously, CF was limited to the pediatric age group. However, an increase in the number of adult CF patients has been observed, because of the greater number of individuals being diagnosed with atypical forms (with milder phenotypic expression) and because of the increase in life expectancy provided by the new treatments. However, there is still great heterogeneity among the different regions of Brazil in terms of the access of CF patients to diagnostic and therapeutic methods. The objective of these guidelines was to aggregate the main scientific evidence to guide the management of these patients. A group of 18 CF specialists devised 82 relevant clinical questions, divided into five categories: characteristics of a referral center; diagnosis; treatment of respiratory disease; gastrointestinal and nutritional treatment; and other aspects. Various professionals working in the area of CF in Brazil were invited to answer the questions devised by the coordinators. We used the PubMed database to search the available literature based on keywords, in order to find the best answers to these questions.
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Ojuawo, Olutobi Babatope, Ademola Emmanuel Fawibe, Olufemi Olumuyiwa Desalu, et al. "Clinical utility of serum procalcitonin in adult patients admitted with community-acquired pneumonia in Ilorin, Nigeria." Journal of the Pan African Thoracic Society 2 (May 25, 2021): 77–84. http://dx.doi.org/10.25259/jpats_1_2021.
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Objectives: The usefulness of biomarkers in community acquired pneumonia (CAP) has been under the research light with limited reports from Africa. This study aimed at evaluating the clinical usefulness of serum procalcitonin (PCT) in patients admitted with CAP in a tertiary hospital in Ilorin, Nigeria. Materials and Methods: This was prospective single center observational study of 102 admitted patients with clinical and radiologic features of CAP. All the patients had serum PCT assay, complete blood count, blood culture, sputum microbiology, and serological evaluation for atypical pathogens. Repeat PCT assay was done following 1 week of antibiotic therapy. The patients were classified into one of two diagnostic groups: Those with microbiologically confirmed bacterial CAP and those without bacterial CAP. Results: Over half (58/102; 56.8%) of the patients had microbiologically confirmed bacterial CAP. The baseline serum PCT concentrations were significantly higher in patients with bacterial CAP when compared to the non-bacterial CAP group (2.55 ± 0.14 vs. 0.94 ± 0.61 ng/ml; P < 0.001). There was also a statistically significant difference between the pre- and post-treatment serum PCT concentrations in the bacterial CAP group (P < 0.001) and the non-bacterial CAP group (P = 0.006). The area under the receiver operating characteristic (AUC) for pre-treatment PCT in diagnosing bacterial CAP was 0.795 (95% confidence level [CI]: 0.709–0.881) with a sensitivity of 67.2% and specificity of 79.5% at an optimal cutoff of 1.5 ng/ml. Overall, the biomarker was independently associated with white cell counts >10 × 109/L (AOR = 6.28; 95% CI: 1.30–30.32, P = 0.02). The baseline mean serum PCT levels were also significantly higher in patients admitted for 7 or more days (P = 0.010). Conclusion: Serum PCT had good diagnostic strength in patients admitted with bacterial CAP in Ilorin. The biomarker can also assist clinicians with predicting the pathogenic group and monitoring clinical progress of CAP.
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Doede, Tina, Anusha Viddam, Kathleen F. Villa, Alison L. Hannah, Charles H. Weaver, and William Tappe. "Diagnosis of Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome (VOD/SOS) with or without Multi-Organ Dysfunction (MOD) after Hematopoietic Stem Cell Transplantation (HSCT): Analysis of a Multicenter Chart Review." Blood 128, no. 22 (2016): 5756. http://dx.doi.org/10.1182/blood.v128.22.5756.5756.
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Abstract Background: VOD/SOS, a potentially life-threatening complication of HSCT conditioning, has historically been diagnosed using modified Seattle criteria (≥2 of: bilirubin >2 mg/dL, hepatomegaly, or >2% weight gain [sometimes >5%]) or Baltimore criteria (bilirubin ≥2 mg/dL and ≥2 of: hepatomegaly, ascites, ≥5% weight gain); the European Society for Blood and Marrow Transplantation proposed new adult criteria in 2016. VOD/SOS with multi-organ dysfunction (MOD) may be associated with >80% mortality. This chart review characterizes VOD/SOS diagnosis patterns. Methods: Physicians performed ≥5 transplants/year in accredited centers listed by the Center for International Blood and Marrow Transplant Research, and provided data from the most recent 1-3 patients with VOD/SOS (with or without MOD) post-HSCT diagnosed in the past 3 years. MOD was defined by renal failure (creatinine clearance 3´ baseline), pulmonary failure (mechanical ventilation, oxygen requirement, or saturation <90%), or heart failure. Results: 200 charts were identified, most at centers participating in a VOD/SOS drug-treatment protocol. 8 charts had missing data, leaving 192 (125 adults and 67 pediatric patients [≤17 years]). Prespecified VOD/SOS risk factors (most common: ≥2 chemotherapy regimens, Karnofsky score <85, second remission, and hepatic comorbidity) were generally more frequent in adults than pediatric patients. Most adult and pediatric patients were reported to have had myeloablative conditioning (MAC; 74% and 65%, respectively) vs reduced-intensity conditioning/autologous HSCT (RIC; 26% and 35%, respectively). In adults, diagnostic ultrasound was used in 90% and biopsy in 29%, typically on the day of VOD/SOS diagnosis. However, patients were diagnosed with VOD/SOS even though 31% and 19% of the respective tests did not confirm VOD/SOS. At diagnosis, 48% of adult and 51% of pediatric patients exceeded minimums for both ≥5% weight gain and ≥2 mg/dL bilirubin components of Baltimore criteria; by modified Seattle criteria, 62% of adults and 51% of pediatric patients exceeded >2% weight and >2 mg/dL bilirubin components (Figure). Adults had higher laboratory values at diagnosis, while pediatric patients generally had more clinical symptoms (Table). MOD was identified in 54% of adult and 69% of pediatric VOD/SOS patients, and these patients generally had greater clinical and laboratory burden than patients without MOD. MOD was more common among patients receiving RIC (65%) than MAC (57%). Bilirubin and weight gain at diagnosis and at peak were higher in patients with vs without MOD (Table). The increase in bilirubin level between the day before diagnosis and day of diagnosis was generally higher in patients with MOD, although rate of weight gain was similar between adult MOD and non-MOD groups. Conclusion: In this study, many patients' signs at diagnosis had progressed beyond VOD/SOS diagnostic criteria. Overall, adults were diagnosed later in disease progression than pediatric patients. Patients with MOD generally had earlier occurrence and higher, more rapidly rising levels of markers at diagnosis: an observation that may aid early identification of MOD. Interestingly, patients with VOD/SOS who received RIC vs MAC had higher rates of MOD. Together, these findings may help in prompt diagnosis of VOD/SOS, which may improve patient outcomes. Support: Jazz Pharmaceuticals Disclosures Doede: Jazz Pharmaceuticals: Employment, Equity Ownership. Viddam:Jazz Pharmaceuticals: Employment, Equity Ownership. Villa:Jazz Pharmaceuticals: Employment, Equity Ownership. Hannah:Jazz Pharmaceuticals: Consultancy. Weaver:Jazz Pharmaceuticals: Consultancy. Tappe:Jazz Pharmaceuticals, Inc.: Employment, Other: stock options exercisable for, and other stock awards of, ordinary shares of Jazz Pharmaceuticals plc.
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Douglass, Carolinda, and Charles Visconti. "Factors Influencing the Use of Adult Day Care by Individuals with Alzheimer's Disease: A Multivariate Examination of the California Alzheimer's Disease Diagnostic and Treatment Center Program." Home Health Care Services Quarterly 17, no. 2 (1998): 53–69. http://dx.doi.org/10.1300/j027v17n02_04.
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Shankar, Ananth, Jamshed Bomanji, and Harpreet Hyare. "Hybrid PET–MRI Imaging in Paediatric and TYA Brain Tumours: Clinical Applications and Challenges." Journal of Personalized Medicine 10, no. 4 (2020): 218. http://dx.doi.org/10.3390/jpm10040218.
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(1) Background: Standard magnetic resonance imaging (MRI) remains the gold standard for brain tumour imaging in paediatric and teenage and young adult (TYA) patients. Combining positron emission tomography (PET) with MRI offers an opportunity to improve diagnostic accuracy. (2) Method: Our single-centre experience of 18F-fluorocholine (FCho) and 18fluoro-L-phenylalanine (FDOPA) PET–MRI in paediatric/TYA neuro-oncology patients is presented. (3) Results: Hybrid PET–MRI shows promise in the evaluation of gliomas and germ cell tumours in (i) assessing early treatment response and (ii) discriminating tumour from treatment-related changes. (4) Conclusions: Combined PET–MRI shows promise for improved diagnostic and therapeutic assessment in paediatric and TYA brain tumours.
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Azim, Adnan, Clair Barber, Paddy Dennison, John Riley, and Peter Howarth. "Exhaled volatile organic compounds in adult asthma: a systematic review." European Respiratory Journal 54, no. 3 (2019): 1900056. http://dx.doi.org/10.1183/13993003.00056-2019.
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The search for biomarkers that can guide precision medicine in asthma, particularly those that can be translated to the clinic, has seen recent interest in exhaled volatile organic compounds (VOCs). Given the number of studies reporting “breathomics” findings and its growing integration in clinical trials, we performed a systematic review of the literature to summarise current evidence and understanding of breathomics technology in asthma.A PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses)-oriented systematic search was performed (CRD42017084145) of MEDLINE, Embase and the Cochrane databases to search for any reports that assessed exhaled VOCs in adult asthma patients, using the following terms (asthma AND (volatile organic compounds AND exhaled) OR breathomics).Two authors independently determined the eligibility of 2957 unique records, of which 66 underwent full-text review. Data extraction and risk of bias assessment was performed on the 22 studies deemed to fulfil the search criteria. The studies are described in terms of methodology and the evidence narratively summarised under the following clinical headings: diagnostics, phenotyping, treatment stratification, treatment monitoring and exacerbation prediction/assessment.Our review found that most studies were designed to assess diagnostic potential rather than focus on underlying biology or treatable traits. Results are generally limited by a lack of methodological standardisation and external validation and by insufficiently powered studies, but there is consistency across the literature that exhaled VOCs are sensitive to underlying inflammation. Modern studies are applying robust breath analysis workflows to large multi-centre study designs, which should unlock the full potential of measurement of exhaled volatile organic compounds in airways diseases such as asthma.
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Vincent, Robert N., John Moore, Robert H. Beekman, et al. "Procedural characteristics and adverse events in diagnostic and interventional catheterisations in paediatric and adult CHD: initial report from the IMPACT Registry." Cardiology in the Young 26, no. 1 (2015): 70–78. http://dx.doi.org/10.1017/s1047951114002637.
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AbstractObjectivesTo report procedural characteristics and adverse events on data collected in the registry.BackgroundThe IMPACT – IMproving Paediatric and Adult Congenital Treatment – Registry is a catheterisation registry of paediatric and adult patients with CHD undergoing diagnostic and interventional cardiac catheterisation. We are reporting the procedural characteristics and adverse events of patients undergoing diagnostic and interventional catheterisation procedures from January, 2011 to March, 2013.MethodsDemographic, clinical, procedural, and institutional data elements were collected at the participating centres and entered via either a web-based platform or software provided by American College of Cardiology-certified vendors, and were collected in a secure, centralised database. Centre participation was voluntary.ResultsDuring the time frame of data collection, 19,797 procedures were entered into the IMPACT Registry. Procedures were classified as diagnostic only (35.4%); one of six specific interventions (23.8%); other or multiple interventions (40.7%); and were further broken down into four age groups. Anaesthesia was used in 84.1% of diagnostic procedures and 87.8% of interventional ones. Adverse events occurred in 10.0% of diagnostic and 11.1% of interventional procedures.ConclusionsThe IMPACT Registry is gathering data to set national benchmarks for diagnostic and certain specific interventional procedures. We are seeing little differences in procedural characteristics or adverse events in diagnostic procedures compared with interventional procedures overall, but there is significant variation in adverse events amongst age categories. Risk stratification and patient acuity scores will be required for further analysis of these differences.
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Li Petri, Sergio, Salvatore Gruttadauria, Duilio Pagano, et al. "Surgical Management of Complex Liver Trauma: A Single Liver Transplant Center Experience." American Surgeon 78, no. 1 (2012): 20–25. http://dx.doi.org/10.1177/000313481207800113.
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Complex liver trauma often presents major diagnostic and management problems. Current operative management is mainly centered on packing, damage control, and early utilization of interventional radiology for angiography and embolization. In this retrospective observational study of patients admitted to the Mediterranean Institute for Transplantation and Advanced Specialized Therapies, Palermo, Italy, from 1999 to 2010, we included patients that underwent hepatic resection for complex liver injuries (grade I to Vaccording to the American Association for the Surgery of Trauma-Organ Injury Scale). Age, gender, mechanism of trauma, type of resection, surgical complications, length of hospital stay, and mortality were the variables analyzed. A total of 53 adult patients were admitted with liver injury and 29 underwent surgical treatment; the median age was 26.7 years. Mechanism was blunt in 52 patients. The overall morbidity was 30 per cent, morbidity related to liver resection was 15.3 per cent. Mortality was 2 per cent in the series of patients undergoing liver resection for complex hepatic injury, whereas in the nonoperative group, morbidity was 17 per cent and mortality 2 per cent. Liver resection should be considered a serious surgical option, as initial or delayed management, in patients with complex liver injury and can be accomplished with low mortality and liver-related morbidity when performed in specialized liver surgery/transplant centers.
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Madigan, Theresa, Nipunie Rajapakse, and Robin Patel. "1108. Diagnostic Yield of the BioFire FilmArray Gastrointestinal Panel in Hospitalized Children at an Academic Children’s Center." Open Forum Infectious Diseases 5, suppl_1 (2018): S332. http://dx.doi.org/10.1093/ofid/ofy210.942.
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Abstract Background The BioFire FilmArray Gastrointestinal Panel (BioFire Diagnostics) (GIP) is a multiplex stool PCR test that detects 22 organisms. Studies in adults suggest that the diagnostic yield of the GIP in hospitalized patients is low. The utility of the GIP among hospitalized pediatric patients and indications for diagnostic stewardship of this test are not well described. Methods We conducted a retrospective chart review of hospitalized pediatric patients who had a GIP ordered between October 2015 and October 2017. Demographic, clinical, and laboratory information was extracted from the medical record. Statistical analysis was completed using JMP Pro 13.0.0 (SAS Institute Inc.). Results Over the 2-year study period, 193 GIPs were obtained on 155 individual pediatric patients. The mean patient age was 8 years and 59% were male. Forty-four percent of patients were immunocompromised and 21% had inflammatory bowel disease. The pediatric infectious disease (PID) team was consulted in 15% of patients at the time the test was ordered. The overall positivity rate of the GIP for one or more pathogens was 42% (Figure 1), with 76% of GIPs positive for one, 23% for two, and 1% for three pathogens. No parasitic infections were diagnosed. The GIP was more likely to be positive if GI symptom onset was prior to admission (48% vs. 24%, P = 0.004), if GI symptoms had been present for &lt; 2 weeks vs. ≥2 weeks (52% vs. 20%, P = 0.0001), and if GI symptoms were the primary reason for the hospital admission (50% vs. 32%, P = 0.012). Only Clostridioides difficile or viral pathogens were detected in patients whose symptoms began in the hospital (Figure 2). Among patients with a positive test, 40% received treatment targeted at one or more of the detected pathogens (Figure 1). Enteropathogenic E.coli (EPEC) and Enteroaggregative E.coli (EAEC) were never treated (Figure 3). Conclusion The GIP was positive for one or more pathogens in 42% of hospitalized children for whom the test was ordered, and led to specific therapy in 40% of those with a positive test. EPEC and EAEC were not treated. The diagnostic yield of the GIP was higher if GI symptoms were present for &lt;2 weeks, began before hospitalization, and were the primary reason for admission. The GIP was frequently obtained without guidance from the PID team. Disclosures R. Patel, CD Diagnostics, BioFire, Curetis, Merck, Hutchison Biofilm Medical Solutions, Accelerate Diagnostics, Allergan, and The Medicines Company: Grant Investigator, Research grant – monies paid to Mayo Clinic. Curetis, Specific Technologies, Selux Dx, GenMark Diagnostics, PathoQuest and Genentech: Consultant and Scientific Advisor, Consulting fee – monies paid to Mayo Clinic. ASM and IDSA: Travel reimbursement and editor’s stipends, Travel reimbursement and editor’s stipends. NBME, Up-to-Date and the Infectious Diseases Board Review Course: Varies, Honoraria. Mayo Clinic: Employee, Salary.
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Ishii, Kazuhiro, Ib Leth Nielsen, and Karin Vargervik. "Characteristics of Jaw Growth in Cleidocranial Dysplasia." Cleft Palate-Craniofacial Journal 35, no. 2 (1998): 161–66. http://dx.doi.org/10.1597/1545-1569_1998_035_0161_cojgic_2.3.co_2.
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Objective The purpose of this pilot study was to assess craniofacial morphology in young and adult individuals with cleidocranial dysplasia (CCD). Design Craniofacial morphology in young individuals (primary dentition) and in young adults was compared with control data using ratios and angles obtained from lateral head films. Setting The CCD individuals were referred to the Center for Craniofacial Anomalies for diagnostic workup and treatment recommendations. Subjects The sample consisted of 14 Caucasians. The inclusion criterion for the young, prepubertal group (A) was complete primary dentition, and for the adult, postpubertal group (B), the eruption of all four first molars was required. Interventions No treatment other than extraction or surgical removal of selected primary or supernumerary teeth was performed. Results Both groups showed significantly smaller anterior upper face height compared with controls. Group B subjects demonstrated significantly smaller face height values than the controls in the A point-nasion-B point (ANB) angle, facial axis, mandibular plane angle, palatal plane/mandibular plane angle, and gonial angle. No significant differences were found between group A individuals and the controls for these measurements. The older group had shorter anterior lower face height compared with both anterior upper face height and posterior lower face height. Conclusions Whereas young CCD subjects showed relatively normal jaw proportions and morphology of the mandible, older CCD individuals tended to have short lower face height, acute gonial angle, anterior inclination of the mandible, and mandibular prognathism. These differences can be attributed to pronounced horizontal mandibular growth resulting from lack of vertical maxillary growth and impaired eruption of permanent teeth.
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Mazreku, A., A. Karaj, I. Avdia, and S. Bilali. "The presentation and management of presacral tumors." Acta chirurgica Iugoslavica 57, no. 2 (2010): 55–59. http://dx.doi.org/10.2298/aci1002055m.
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Objectives: Retrorectal masses are uncommon lesions. They remain a difficult diagnostic and therapeutic challenge despite the use of newer imaging modalities and improvement in preoperative care. Although useful for planning a resection, neither CT nor MRI can provide a definitive diagnosis. The objective was to remove the entire lesion as revealed by the radiological study to avoid any recurrence of disease and malignant degeneration, but with the least amount of consequential impairment. Methods: We present our experience with 9 (nine) presacral tumors during 10 year period 1998-2007. The files and charts of adults' patients who underwent treatment for presacral tumors were collected and retrospectively reviewed.Results: From January 1998 to December 2007, a total of 9 adult patients were evaluated for primary tumors originating in retrorectal space in Surgical Clinic of Oncology near University Hospital Center 'Mother Teresa'. Four patients were males and five were females. The diagnosis of these retrorectal tumors were, developmental tail gut cyst, teratoma, chordoma and chondrosarcoma. In six patients were achived good results during surgical treatment. Coccygeal resection in one case. In three cases we couldn't perform sarectomy. Conclusion: In conclusion, presacral tumors are uncommon. They can be congenital or acquired, benign or malignant, which forms the basis for a simple classification. Their treatment is always surgical. The good results were obtained in the benign lesions.
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Baluku, Joseph Baruch, Sylvia Nassozi, Brian Gyagenda, et al. "Prevalence of Malaria and TB Coinfection at a National Tuberculosis Treatment Centre in Uganda." Journal of Tropical Medicine 2019 (July 25, 2019): 1–7. http://dx.doi.org/10.1155/2019/3741294.
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The prevalence of malaria and tuberculosis (TB) coinfection is not well established in countries that are highly burdened for both diseases. Malaria could impair TB containment and increase mortality of TB patients. The objective of this study was to determine the prevalence of malaria/TB coinfection among bacteriologically confirmed adult TB patients at a national TB treatment centre in Uganda. Using a cross-sectional study design we enrolled 363 bacteriologically confirmed adult TB patients, and data on demographics and medical history was collected. Blood samples were tested for malaria blood smear, rapid malaria diagnostic test (RDT), complete blood count, haematological film analysis, HIV serology, and CD4+ and CD8+ cell counts. Malaria was defined as either a positive blood smear or RDT. The study participants were mostly male (61.4%), with a median age of 31 (interquartile range, IQR: 25-39) years, and 35.8% were HIV positive. The prevalence of malaria was 2.2% (8/363) on the overall and 5% (3/58) among participants with rifampicin resistance. A triple infection of HIV, malaria, and rifampicin resistant TB was observed in 3 participants. The prevalence of malaria among TB patients is low, and further evaluation of the epidemiological, clinical, and immunological interaction of the two diseases is warranted.
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Kluczynski, Lukasz, Magdalena Godlewska, Damian Rogozinski, et al. "Pituitary Stalk Lesions - Single Center Long Term Observation." Journal of the Endocrine Society 5, Supplement_1 (2021): A631—A632. http://dx.doi.org/10.1210/jendso/bvab048.1287.
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Abstract Background & Methods: Pituitary stalk lesions (PSL) are various changes located in the pituitary infundibulum. The underlying pathology and exact diagnosis are difficult to establish due to their unique anatomical locus. A retrospective observational analysis of 60 adult patients (34W/26M) with pituitary lesions was performed. The mean age of diagnosis was 33.8 years (SD 23.7). The etiologies were divided into 3 groups (congenital, inflammatory, neoplastic), classified as exact, probable or unknown and characterized hormonally. Aim: To present the etiological spectrum of pituitary stalk lesions and their clinical and hormonal characteristics on the basis of long term observation in the pediatric/adult endocrinology departments of our university. Results: The most common causes of PSL were neoplasms (20/60, 33.3%, 14W/6M); congenital malformations were detected in 17/60 (28.3%, 6W/11M), while inflammatory etiology was found in 15/60 (25.0%, 9W/6M) of patients. The exact diagnosis was established in 26/60 (43.3%) cases (16 congenital malformations, 6 adenomas, 1 pituitary cancer, 1 craniopharyngioma, 1 germinoma and 1 lymphocytic hypophysitis [LH]). The probable cause was suggested in 26/60 patients (43.3%) - 10 with the suspicion of LH, 4 with histiocytosis, 3 with a metastatic tumor from a disseminated cancer, 3 craniopharyngiomas, 1 posterior pituitary lobe ectopy, 1 prolactinoma, 1 granular cell tumor, 2 adenomas and 1 pituicytoma. The origin of 8/60 PSL (13.3%) remains unknown. During hormonal assessment the most common insufficiency concerned the gonadal axis found in 29/60 (48.3%) of patients, followed by thyroid (26/60, 43.4%), somatotropic (21/60, 35.0%) and adrenal axis (20/60, 33.3%) insufficiencies. Hyperprolactinemia was detected in 20/60 (33.3%) of patients, while diabetes insipidus was confirmed in 15/60 (25%) of cases. 45 patients presented at least 1 hormonal deficit, some of them were transient. In clinical aspect, symptoms associated with hormonal deficits led to the initiation of diagnostic work-up in 29 patients (48.3%; including 15 patients (25.0%) with growth retardation). Neurological symptoms such as headaches, visual disturbances and seizures were seen in 13 patients (21.7%). Polydipsia and polyuria were the primary presentation in 11 cases (18.3%), while 5 cases (8.3%) had a clinical manifestation of hormone overproduction. Incidental diagnosis was seen in 2 female patients (3.3%). Conclusions: The diagnosis, management and treatment of the pituitary stalk lesions remains challenging. Difficulties in establishing the exact diagnosis might also be related to the non-specific, transient characteristics of the symptoms and hormonal insufficiencies. Long term observations might help better the understanding of the disease and result in improvement of management.
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Ferguson, Glenn E., Roy J. Eidelson, and Philip H. Witt. "New Jersey's Sex Offender Risk Assessment Scale: Preliminary Validity Data." Journal of Psychiatry & Law 26, no. 3 (1998): 327–49. http://dx.doi.org/10.1177/009318539802600303.
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The Registrant Risk Assessment Scale (RRAS) was developed to aid New Jersey law enforcement representatives in assigning convicted sex offenders to risk tier classifications. The three risk tier classifications (low, moderate, and high risk) are linked to corresponding levels of community notification. The present study examined the scores of 574 adult males convicted of sex offenses in New Jersey on the seven RRAS items that assess static indicators of recidivism risk. Based on sentencing decisions, the sample included three groups of offenders: probationers, state prisoners, and those assigned to the Adult Diagnostic and Treatment Center (ADTC), an inpatient sex offender treatment facility for repetitive and compulsive offenders. An exploratory factor analysis of the RRAS items identified two important orthogonal factors: a forcible assault factor and a sexual deviance factor. One-way ANOVAs revealed significant differences among the three placement groups, with state prisoners tending to score highest on items reflecting antisocial orientation and forcible sexual assault and ADTC offenders scoring highest on items reflecting deviant sexual behavior. A discriminant analysis generated two distinct functions that classified well over half of the sample into their correct sentencing groups. Taken as a whole, the results provide preliminary support for the use of the RRAS in making sex offender risk determinations.
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Astakhov, Yury S., Vitaly O. Sokolov, Natalya V. Morozova, Yuriy V. Skorobogatov, Ekaterina N. Drobyshevskaya, and Mikhail A. Morozov. "Differential diagnosis of dirofilariasis of the lower lid: a clinical case from Ophthalmological diagnostic center no. 7 for children and adults, Saint Petersburg." Ophthalmology journal 9, no. 3 (2016): 45–54. http://dx.doi.org/10.17816/ov9345-54.
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We present a brief literature review on dirofilariasis and its incidence in Russia. The diagnosis and operative treatment of a patient with dirofilariasis caused by infection with Dirofilaria repens is described. The diagnostic criteria (epidemiological analysis, ultrasonographic, and laboratory-based and parasitological work-up) are presented. Ultrasonography plays an important role in the differential diagnosis of dirofilariasis and cavernous hemangiomas. Different non-surgical and surgical treatment approaches are discussed.
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Tambaro, Francesco, Dristhi Ragoonanan, Sajad Khazal, et al. "Sinusoidal Obstructive Syndrome Among Pediatric and Adolescent and Young Adult Patients: Analysis of Pediatric EBMT Diagnostic and Severity Criteria at MD Anderson." Blood 134, Supplement_1 (2019): 4495. http://dx.doi.org/10.1182/blood-2019-132085.
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Background: Sinusoidal obstructive syndrome (SOS) is a potentially catastrophic complication of stem cell transplantation (SCT) which disproportionately affects younger patients. The incidence of SOS may vary widely depending on the diagnostic criteria (Baltimore versus Seattle), and delays in initiation of definitive treatment remains the most important predictor of outcome. Recently proposed pediatric diagnostic and severity grading criteria by the European Society of Blood and Marrow Transplantation (EBMT) may offer increased sensitivity and allow for more prompt diagnosis, but the extent to which these have been adopted and/or validated by centers in the United States is unclear. Methods: This is a retrospective analysis of all patients undergoing SCT on the Pediatric service at MD Anderson Cancer Center from 2009-2019. Patient records were reviewed for diagnosis of SOS. Among patients identified with SOS, application of Baltimore, Seattle and pediatric EBMT (pEBMT) criteria were compared. Descriptive statistics were used for data analysis where appropriate. Results: A total of 248 patients received 268 transplants during the study period. Patient characteristics are summarized in Table 1. When Baltimore criteria was used the incidence of SOS was 4.5% (n=12) as compared to 8.2% (n=22) when the Seattle or pEBMT criteria were applied. At diagnosis, the majority of these patients had moderate to very severe disease according to pEBMT criteria. The median time to diagnosis of SOS varied according to diagnostic criteria used, with a trend to earlier diagnosis with pEBMT criteria (13 days post-SCT with pEBMT versus 15 days with Seattle/Baltimore) as shown in Table II. Prior exposure to inotuzumab and gemtuzumab were associated with high rates of SOS (75% and 86% respectively). Anicteric SOS, refractory thrombocytopenia and rising bilirubin from a baseline value on 3 consecutive days or bilirubin ≥2 mg/dL within 72 hours were present in 63.6%, 77.3% and 91% of patients with SOS, when pEBMT criteria were applied. Late-onset SOS (diagnosed after 21 days post-SCT) was present in 22.7% of patients when Seattle or pEBMT criteria were applied (n=5) and could not be diagnosed per Baltimore criteria. Severe SOS was eventually observed in 95% of patients. Severe ascites requiring paracentesis (23%), impaired coagulation (77%), respiratory distress requiring supplemental oxygen and/or invasive pulmonary ventilation ( 55%), delirium (32%), acute kidney injury/KDIGO Stage 3/requiring renal replacement therapy (50%) were observed. Defibrotide treatment was initiated in 55% of patients with a median duration of treatment of 26 (3-74) days and complete resolution of SOS was observed in 42% of these patients. For patients who were initiated on defibrotide on the day of diagnosis (n=7) versus those who were not (range 1-11 days) the median time to complete resolution of SOS was 35 and 20 days respectively (p=0.2). Conclusions: To our knowledge, this is the first reported retrospective analysis of SOS among pediatric and adolescent young adult SCT patients in the United States using pEBMT SOS diagnostic and severity criteria. Our study was limited by small sample size associated with single center analyses. Use of the pEBMT criteria showed a trend to earlier time to diagnosis by a median of two days. This is a potentially clinically significant finding, as a delay in initiation of defibrotide among children with severe SOS, has been associated with statistically significant decreased rates of complete resolution. Inherent flaws of the Baltimore and Seattle criteria are the time restriction and reliance of hyperbilirubinemia for the diagnosis in children and adolescents and young adults. Our retrospective analysis suggests that pEBMT criteria for the diagnosis of SOS among children and young adults may be appropriate, especially given the rates of anicteric and late-onset SOS seen in this population. Disclosures Petropoulos: Foundation for the Accreditation of Cellular Therapy (FACT): Membership on an entity's Board of Directors or advisory committees, Other: Foundation for the Accreditation of Cellular Therapy (FACT). Kebriaei:Amgen: Research Funding; Jazz: Consultancy; Pfizer: Honoraria; Kite: Honoraria. Wierda:Xencor: Research Funding; Janssen: Research Funding; Loxo Oncology Inc.: Research Funding; KITE pharma: Research Funding; Juno Therapeutics: Research Funding; Gilead Sciences: Research Funding; Acerta Pharma Inc: Research Funding; Pharmacyclics LLC: Research Funding; Genentech: Research Funding; GSK/Novartis: Research Funding; AbbVie: Research Funding; Cyclcel: Research Funding; Oncternal Therapeutics Inc.: Research Funding; Miragen: Research Funding; Sunesis: Research Funding. Mahadeo:Recipient of unrestricted medical education grant from Jazz: Research Funding; PI for ATARA EBV CTL Trials: Other: Other .
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Unger, Joseph M., and Mark Fleury. "Nationally representative estimates of the participation of cancer patients in clinical research studies according to the commission on cancer." Journal of Clinical Oncology 39, no. 28_suppl (2021): 74. http://dx.doi.org/10.1200/jco.2020.39.28_suppl.74.
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74 Background: The successful conduct of cancer clinical trials hinges on the willingness of patients to participate. The rate of adult clinical trial participation has been regarded as being < 5%. However, national estimates of trial participation are nearly two decades old, and no evidence based on original data sources has been examined for many years. Moreover, studies about trial participation have focused solely on enrollment to treatment trials, which does not reflect the willingness of patients to contribute to other key elements of clinical research, such as quality of life or biorepository studies. We determined inclusive, contemporary estimates of clinical trial participation for adults with cancer using a national sample of data from 1,200 institutions. Methods: The data were from the Commission on Cancer (CoC), a consortium of cancer-related organizations providing accreditation for both academic and community cancer care facilities across the U.S. CoC enrollment data represent 70% of all cases of cancer diagnosed each year. Deidentified, institution-level aggregate counts of annual enrollment to treatment, biorepository, diagnostic, economic, genetic, prevention, quality of life, registry, and screening studies were examined. Overall, study-type estimates for the period 2013-2017 were estimated. Multiple imputation by chained equations was used to account for missing data, with summary estimates calculated separately by type of program (e.g., NCI-designated cancer programs) and pooled. Results: Across the entire U.S. system, the estimated participation rate to cancer treatment trials was 6.3%. Enrollment to treatment trials was highest at NCI-designated comprehensive cancer centers (18.9%), while for community cancer programs (CCPs) and comprehensive CCPs, treatment trial rates were 4.4% and 3.6%, respectively. Nearly 1 in 7 patients participated in biorepository studies (13.4%), including 39.4% at NCI cancer centers. Patients participated in a wide variety of other study types, including registry (8.1%), prevention (6.4%), genetic (3.6%), quality of life (2.9%), economic (2.7%), diagnostic (2.7%), and screening studies (1.8%). At least 25.4% of adult cancer patients were estimated to participate in one or more cancer clinical research studies. Conclusions: In a first-time use of nationally representative enrollment data from the CoC, enrollment to cancer treatment trials was 6.3%, higher than historical estimates of < 5%. Patients participated in a diverse set of other study types, and taken together, at least one quarter of patients participated in a study. Contributions of adult patients with cancer to clinical research is much more comprehensive than previously understood.
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Elyamany, Ghaleb, Azzah Alzahrani, Huda Elfaraidi, et al. "Hemophagocytic Lymphohistiocytosis: Single-Center Series of 12 Cases from Saudi Arabia." Clinical Medicine Insights: Pediatrics 10 (January 2016): CMPed.S35853. http://dx.doi.org/10.4137/cmped.s35853.
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Background Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal disease that commonly appears in infancy, although it has been reported in adults. Chemoimmunotherapy-based treatments have improved the survival of patients with HLH; however, overall survival is still poor. We retrospectively analyzed the data of 12 HLH patients who were admitted between 2005 and 2014. All patients were Saudi Arabia in origin with a female predominance (75%) and a median age of onset of 9.5 months. The consanguinity rates were significantly high (75%) with a positive family history in 41% of cases. Of the 12 patients, nine were defined as primary HLH patients and three were confirmed to be secondary HLH patients. All patients fulfilled the 2004 diagnostic criteria for HLH and received HLH-2004 treatment. Six of these patients showed a good response to chemotherapy, while the remainder of the patients showed partial or no response to chemotherapy. Five patients in this cohort received stem cell transplant, and these patients are currently in remission. The mortality rate of this cohort is currently 50%. Genetic mutational analysis showed a positive STX11 mutation in five patients and a PRF1 ( perforin) mutation in two patients. To the best of our knowledge, this is the first case series of HLH from Saudi Arabia.
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Hussain, Taimoor, Ayodele Alaiya, Majed Dasouki, et al. "A Clinical, Genomic and Proteomic Approach for the Characterization of Fanconi Anemia in Adolescent and Young Adult (AYA) Patients : A Single Center Study of 55 Patients from a National Bone Marrow Failure Referral Center." Blood 132, Supplement 1 (2018): 2594. http://dx.doi.org/10.1182/blood-2018-99-114639.
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Abstract Introduction: Fanconi Anemia (FA) is an autosomal recessive disorder characterized by bone marrow failure (BMF), constitutional anomalies and high risk of developing cancer. Distinguishing FA from severe aplastic anemia (SAA) can be challenging especially in asyndromic patients. We undertook a clinical and laboratory cohort study of adolescent and young adult (AYA) patients with a diagnosis of FA treated at our institution to characterize the clinical features in our population, and conducted a prospective translational study to explore integration of a genomic and proteomic approach for improved diagnosis and molecular characterization of FA. Methods: Data on FA patients was obtained from an institutionally approved BMF database and hematopoietic stem cell transplant (HSCT) database. Further data was obtained from a register of chromosomal breakage (CB) analysis results. Index cases were identified if they were older than 14 years of age at the time of diagnosis or under the care of adult hematology with a clinical diagnosis of FA based on the presence of BMF and abnormal CB, or clinical phenotype with the presence of homozygous FA related genes. Family pedigrees were constructed based on history. In addition, patients presenting with BMF were enrolled onto an institutionally approved study investigating proteomic biomarkers and genomics of BMF syndromes. Consented peripheral blood samples and/or extracted DNA were subject to either panel based next generation sequencing (NGS) testing as part of the Saudi Genome Project or subjected to whole exome sequencing (WES) by external lab. For proteomic analysis, peripheral blood plasma (PBP) samples from 6 patients with FA, 10 SAA patients and 7 normal controls were subjected to expression proteomics using liquid chromatography tandem mass spectrometry (LC-MS/MS). Result: Patients and clinical features: 55 patients (26 M, 29 F) in 30 families were identified. While 18 patients (32%) were referred with a diagnosis/suspicion of FA, in 26 (47%) FA was diagnosed at our institution. The most frequent anomaly was short stature (14 patients, 25%), skin changes (7, 12%), urogenital abnormalities (7, 12%), dysmorphism/craniofacial abnormalities (7, 12%), hands anomalies (4, 7%); 12 (22%) had no recorded anomalies. 18 patients (33%) developed a malignancy either before or after diagnosis of FA: solid tumors in 5 (9%), AML and/or MDS in 15 (27%); 3 (5%) of these patients had both solid tumors and AML/MDS. Diagnostic Tests: 35 patients (63.6%) had a positive CB analysis with diepoxybutane (DEB) or mitomycin-C (MMC) testing; in 5 patients (9%) DEB testing was borderline and 3 (5%) had a normal CBA but had a diagnostic phenotype+/- family history and presence of a homozygous mutation in a known FA related gene. 14 patients had cytogenetic abnormalities and abnormalities involving chromosome 1 were the most frequent (50%). Mutation Analysis: Mutational analysis was available for 12 (22%) cases; homozygous mutations in FA genes were identified in 10 patients (18%) in 7 families (23% of families): FANCA (5 patients/3 families); BRIP1 (2/2); FANCP (1/1); FANCD2 (2/1). In one case, post matched sibling (HSCT) blood sample revealed a known pathogenic heterozygous c.2632G>C,p.Glu878Gln mutation in FANCA, suggesting a carrier donor. Proteomic analysis: Over 1650 unique PBP protein species were identified of which 605 were significantly differentially expressed (≥ 2 to ∞ - fold change & p < 0.001) between SAA /FA/ normal control subjects (Fig 1a). DNMT3A, Kinase Insert Domain Receptor (KDR) and TGFB-1 was found to be highly expressed in SAA versus FA, while ATM and APOB were highly expressed in FA versus SAA (Fig.1b). Treatment outcomes: 36 out of 55 patients (65%) received HSCT. Actuarial survival of HSCT (n=37) and non-HSCT (n=14) patients was 70% and 77%, respectively. Treatment details were not available on 6 (11%). CONCLUSION: We report the first characterization of AYA patients with FA in Saudi Arabia. Our report emphasizes the need for a high index of suspicion of a diagnosis of FA in BMFs. CB may be falsely negative in cases, and panel based and/or WES based NGS testing increases diagnostic accuracy; in this cohort, mutations in FANCA were the most frequent (50%). Occurrence of hematological and solid tumors is a significant risk in these AYA patients. We also report proteomic panels as potential biomarkers that distinguish FA from SAA and may provide mechanistic insights. Disclosures No relevant conflicts of interest to declare.
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Mulla-Ali, Dalal Hamza, Kevin H. M. Kuo, Amanda M. Brandow, Nancy M. Heddle, George A. Tomlinson, and Aisha Bruce. "Management of Osteonecrosis By Adult and Pediatric Sickle Cell Providers." Blood 126, no. 23 (2015): 4465. http://dx.doi.org/10.1182/blood.v126.23.4465.4465.
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Abstract Introduction: Osteonecrosis (ON) is a debilitating complication in 40% of adult sickle cell disease (SCD) patients. Almost all SCD ON will collapse, develop intractable pain or require surgery in 8 to 15 years from diagnosis. Majority of treatments have not been examined in a controlled setting, leading to variations in care, impeding research to verify the efficacy of existing therapies, and development of new treatments. The study's objective is to describe the screening, diagnostic and management patterns of ON in pediatric and adult SCD patients by US and Canadian providers. Materials and Methods: A cross-sectional survey of SCD providers from the Sickle Cell Adult Provider Network, the American Society of Pediatric Hematology/Oncology, and the Canadian Hemoglobinopathy Association was conducted from March to July 2015. The survey had 24 questions about provider demographics, characteristics of SCD population, ON diagnosis, staging and treatment. The survey was distributed electronically over 9 weeks with 2 reminders 3 weeks apart. Descriptive statistics were calculated and logistic regression was used to identify factors (specialty, location, years of practice, number of patients, adult vs. pediatric centers) that were associated with screening, diagnostic, staging and treatment approaches. Results: Of the 2,475 health care providers surveyed, 78 participants responded from 63 centers with 17,232 patients (17-23% of the estimated SCD population in US and Canada). Majority of respondents were located in urban teaching centers (93%). All (100%) respondents self-identified as SCD providers: 44 adult hematology/oncology (heme/onc) specialist; 3 internists; 24 pediatric heme/onc specialist; 5 nurse clinicians; 1 pharmacist; and 1 social worker. The median time in practice was 16 years (min 1, max 40). The estimated number of unique patients under the care of the respondents was 10,697, of which 1,946 (18%) had ON. More than half of the ON patients had joint collapse (n=1,050, 54%), and 72% had joint replacement. Only (13%) of the respondents screened for ON in asymptomatic patients, and screening measures include 50% by x-ray; 38% by physical, 12% by other modalities. All (100%) respondents screened for ON in symptomatic patients: 51% using x-ray and MRI, 41% x-ray, 7% MRI. The majority of respondents (72%) did not know the ON staging methods used. Frequencies of respondents using non-surgical and surgical treatments in pre-collapse and collapsed ON are shown in Table 1. Respondents' years in practice was the only factor significantly associated with the use of screening in asymptomatic SCD patients (OR 2.9/decade in practice, p=0.030). Respondents' years in practice was also associated with a reduced likelihood of hydorxyurea (HU) use in pre-collapse ON (OR 0.44/decade more in practice, p=0.011). Patients with collapsed ON under the care of pediatric heme/onc specialists were more likely to be treated with core decompression than adult heme/onc specialist or internist (OR 10.7, p=0.016). Discussion: Our survey suggests that the diagnosis, classification and management of ON is highly heterogeneous, despite the 2014 NIH expert panel report on the management of SCD prior to the distribution of the survey. The lower prevalence of ON in our study compared to that in cohort-wide screening may be due to the lack of screening by surveyed providers in asymptomatic SCD patients, suggesting ON may be underdiagnosed. Despite lack of evidence for their efficacy, HU and core decompression were used by majority of respondents in pre-collapse ON. A limitation of the study is difficulty in estimating the true response rate since the number of SCD providers in US and Canada is not known, potentially impacting the results' generalizability. The variations in practice and the high prevalence of collapsed joints requiring replacement in the surveyed population confirms the need to develop a uniformed approach in screening, diagnosis, and evidence-based therapies in SCD ON. Table 1. Non-surgical and surgical treatments used by respondents in pre-collapse and collapsed ON. Some respondents selected multiple responses Treatments Number of Respondents (%) Non-surgical Pre-collapse (n=54) Collapsed (n=53) Analgesia 53 (98%) 52 (98%) Physiotherapy 49 (91%) 37 (70%) Hydroxyurea 32 (59%) 28 (53%) Surgical Pre-collapse (n=38) Collapsed (n=45) Core decompression 33 (87%) 10 (22%) Hip replacement 9 (24%) 43 (95%) Disclosures Off Label Use: Hydroxyurea for treatment of osteonecrosis in sickle cell disease. Kuo:Novartis Canada: Honoraria, Other: Advisory Board; Alexion Pharmaceuticals: Honoraria, Other: Advisory Board.
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Bayramoğlu, Elvan, Selin Elmaogulları, Elif Sagsak, and Zehra Aycan. "Evaluation of long-term follow-up and methimazole therapy outcomes of pediatric Graves’ disease: a single-center experience." Journal of Pediatric Endocrinology and Metabolism 32, no. 4 (2019): 341–46. http://dx.doi.org/10.1515/jpem-2018-0495.
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Abstract Background The management options for Graves’ disease in children are limited and there is controversy regarding optimal treatment. Remission rate with anti-thyroid drug (ATD) treatment in children is said to be lower than in adults. Definitive treatments are effective, but they often result in permanent hypothyroidism. The objective of this study was to investigate the outcome of methimazole treatment, identify significant predictors of a remission and evaluate the adverse effects of methimazole in a pediatric population of GD patients. Methods Medical records of the patients who had been diagnosed with Graves’ disease were screened retrospectively. Diagnostic criteria included elevated free thyroxine (fT4) and total triiodothyronine (T3), suppressed thyroid-stimulating hormone (TSH) and either positive thyroid-stimulating immunoglobulin (TSI) or thyroid receptor antibodies (TRABs) or clinical signs suggestive of Graves’ disease, for example, exophthalmos. Remission was defined as maintenance of euthyroidism for more than 12 months after discontinuing methimazole treatment. Results Of the 48 patients, provisional remission was achieved in 21 patients. Of the 21 patients, 14 experienced a relapse (66.6%). Remission was achieved in seven (24.1%) of 29 patients who received methimazole treatment for more than 2 years. In patients who achieved long-term remission, the male sex ratio and fT4 levels at diagnosis were significantly lower than the relapsed and non-remission groups, whereas the free triiodothyronine (fT3)/fT4 ratio and duration of methimazole treatment were significantly higher than the relapse group. Conclusions Long-term methimazole treatment in pediatric Graves’ disease would be appropriate. High fT4 levels at the time of diagnosis and male sex were associated with a risk of relapse.
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Baryshevskaya, L. A., T. Yu Vladimirova, O. V. Zeleva, and E. V. Koldova. "CHRONIC INFLAMMATION OF THE TONSILS ASSOCIATED WITH EPSTEIN-BARR VIRUS." Science and Innovations in Medicine 3, no. 1 (2018): 6–10. http://dx.doi.org/10.35693/2500-1388-2018-0-1-6-10.
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Aim - to identify the prevalence of chronic tonsillitis in the Samara region and the causes of its recurrence. Materials and methods. The authors analyzed the frequency of occurrence of chronic tonsillitis among the patients of the Samara Diagnostic Center for the period from 2011 to 2016, the frequency of quinsy and paratonsillar abscesses. Middle-aged and elderly people with signs of chronic tonsillitis were examined for the presence of Epstein-Barr virus. Results. 56 adult and elderly patients aged from 44 to 75 years (18 men - 31% and 38 women - 65.5%) underwent additional examination of saliva and blood for the presence of Epstein-Barr virus. The analyses were taken on an empty stomach in the morning. When taking saliva, hygienic treatment of the oral cavity was not carried out. Saliva was investigated by a qualitative method of PCR diagnostics. We also performed serodiagnostic assay by ELISA. DNA of Epstein-Barr virus was discovered in 16 patients (27.6%), IgG antibodies to nuclear antigen (EBNA) - in 13 patients (22%), and 1 patient (1.7%) had IgM antibodies to capsid antigen (VCA). Thus, 1.7% of Epstein-Barr virus was diagnosed at an early stage, 22% of patients had infection in the past and 27.6% had recent infection. Conclusions. Presented evidence suggests that it is necessary to combine examination of the content of tonsillar lacunae with PCR and ELISA diagnostics for Epstein-Barr virus not only in young patients of working age with chronic tonsillitis, but also in middle-aged and elderly patients with chronic tonsillitis in order to improve the efficiency and quality of the treatment at the stage of outpatient visit.
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Freeman, Edith M., and Larry Dyer. "High-Risk Children and Adolescents: Family and Community Environments." Families in Society: The Journal of Contemporary Social Services 74, no. 7 (1993): 422–31. http://dx.doi.org/10.1177/104438949307400704.
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Developmental factors, family problems such as substance abuse, and other environmental variables, including violence, greatly affect the adjustment and coping of children and youth. Such variables can result in inaccurate assessments and diagnoses when young people are viewed in isolation from their situations and environments and when adult-oriented diagnostic tools are utilized. The authors describe the use of clinical interviews to provide more accurate diagnoses and strengths-oriented assessments for children and youth who receive case-management services from mental health centers. Implications for prevention and treatment are discussed.
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Klein, Michael, Mirza Khan, Jorge L. Salinas, and Rolando Sanchez. "Disseminated pulmonary histoplasmosis in immunocompetent patients: a common epidemiological exposure." BMJ Case Reports 12, no. 3 (2019): e227994. http://dx.doi.org/10.1136/bcr-2018-227994.
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We describe the initial presentation, diagnostic work-up and treatment of three adult immunocompetent men who presented within a short time frame of each other to an academic medical centre with acute respiratory distress syndrome. Their presentation was found to be secondary to a large inoculum of histoplasmosis from remodelling a building with bat droppings infestation. We discuss the pathophysiology of histoplasmosis and highlight the importance of exposure history in patients with acute respiratory failure and why patients with the occupational risk of exposure to fungal inoculum should wear protective respirator gear.
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Sparacino, Lucrezia, Luca Bruschini, Andrea De Vito, Giacomo Fiacchini, and Stefano Berrettini. "Surgical treatment in obstructive sleep apnea syndrome: an overview." International Surgery Journal 7, no. 8 (2020): 2807. http://dx.doi.org/10.18203/2349-2902.isj20203284.
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Obstructive sleep apnea syndrome is a common pathology affecting pediatric and adult population. It is caused by an increase upper airway resistance due to augmented collapsibility that cause alteration in ventilation and oxygen saturation. Sleep oxygen desaturations have multi-organ implications. Since many years, OSAS, has been recognized as a risk factors for comorbid cardiovascular disease. Well-known is the causal link with cognitive impairment and atherosclerosis. Healthcare professionals, should be trained to recognize signs and symptoms of the disease in order to send the patients forward for specialist care in centers dealing with sleep-disordered breathing. However treatment knowledge is of pivotal importance in the cultural baggage of all the physicians. Surgical and non-surgical therapies have undergone a great number of changes. Going along with the innovations in diagnostic evaluation and phenotyping of the patients, surgeries were heightened by new developments such as advances in tailoring procedures and technologies. The aim of this overview is to describe the evolution and main characteristics of encoded procedures classified into site of surgical correction.
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Parfenov, A. I., S. V. Bykova, E. A. Sabelnikova, et al. "All-Russian Consensus on Diagnosis and Treatment of Celiac Disease in Children and Adults." Terapevticheskii arkhiv 89, no. 3 (2017): 94–107. http://dx.doi.org/10.17116/terarkh201789394-107.
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1Moscow Clinical Research-and-Practical Center, Moscow Healthcare Department, Moscow; 2A.I. Evdokimov Moscow State University of Medicine and Dentistry, Ministry of Health of Russia, Moscow; 3Children’s Health Research Center, Ministry of Health of Russia, Moscow; 4I.I. Mechnikov North-Western State Medical University, Ministry of Health of Russia, Saint Petersburg; 5M.F. Vladimirsky Moscow Regional Research Clinical Institute, Moscow; 6Russian Medical Academy of Postgraduate Education, Ministry of Health of Russia, Moscow; 7Novosibirsk State Medical University, Ministry of Health of Russia, Novosibirsk; 8Russian Children’s Clinical Hospital, Moscow; 9Department of Pediatrics, Russian Medical Academy of Postgraduate Education, Ministry of Health of Russia, Moscow; 10I.P. Pavlov First Saint Petersburg State Medical University, Ministry of Health of Russia, Saint Petersburg; 11Acad. Yu.E. Veltishchev Research Clinical Institute of Pediatrics, N.I. Pirogov Russian National Research Medical University, Ministry of Health of Russia, Moscow; 12Clinic Four, Department of Pediatrics, I.P. Pavlov First Saint Petersburg State Medical University, Ministry of Health of Russia, Saint Petersburg; 13Childhood Diseases Department Two, N.I. Pirogov Russian National Research Medical University, Ministry of Health of Russia, Moscow; 14Research Laboratory of Surgical Gastroenterology and Endoscopy, N.I. Pirogov Russian National Research Medical University, Ministry of Health of Russia, Moscow; 15Department of Endoscopic Surgery, City Clinical Hospital Thirty-One, Moscow Healthcare Department, Moscow; 16A.N. Ryzhikh State Research Center of Coloproctology, Ministry of Health of Russia, Moscow; 17Department of Intermediate-Level Therapy, Tver State Medical University, Ministry of Health of Russia, Tver; 18Laboratory of Medical Genetics, Diagnostic Center of Medical Genetics, Saint Petersburg; 19HLA Typing Laboratory, Blood Transfusion Station, Moscow Healthcare Department, Moscow; 20S.P. Botkin City Clinical Hospital, Moscow Healthcare Department, Moscow; 21Department of Nervous System Diseases, I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia, Moscow; 22Department of Endocrinology and Diabetology, A.I. Evdokimov Moscow State University of Medicine and Dentistry, Ministry of Health of Russia, Moscow; 23Acad. V.I. Kulakov Research Center of Obstetrics, Gynecology, and Perinatology, Ministry of Health of Russia, Moscow; 24Department of Therapy, Kazan State Medical Academy, Ministry of Health of Russia, Kazan; 25Department of Intermediate-Level Therapy with Course of Occupational Diseases, Faculty of General Medicine, Omsk State Medical University, Ministry of Health of Russia, Omsk; 26Dmitry Rogachev Federal Research Clinical Center of Pediatric Hematology, Oncology, and Immunology, Ministry of Health of Russia, Moscow The paper presents the All-Russian consensus on the diagnosis and treatment of celiac disease in children and adults, which has been elaborated by leading experts, such as gastroenterologists and pediatricians of Russia on the basis of the existing Russian and international guidelines. The consensus approved at the 42nd Annual Scientific Session of the Central Research Institute of Gastroenterology on Principles of Evidence-Based Medicine into Clinical Practice (March 2—3, 2016). The consensus is intended for practitioners engaged in the management and treatment of patients with celiac disease. Evidence for the main provisions of the consensus was sought in electronic databases. In making recommendations, the main source was the publications included in the Cochrane Library, EMBASE, MEDLINE, and PubMed. The search depth was 10 years. Recommendations in the preliminary version were reviewed by independent experts. Voting was done by the Delphic polling system.
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Yorio, Florencia, Juan Bautista Blaquier, Bernadette Calabrese, Sebastián Cerrato, Blanca Diez, and Alejandro Muggeri. "LMD-17. Neoplastic meningitis in lung cancer: retrospective review of clinical features, diagnosis and outcome in adult patients." Neuro-Oncology Advances 3, Supplement_3 (2021): iii11. http://dx.doi.org/10.1093/noajnl/vdab071.042.
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Abstract Background Neoplastic Meningitis (NM) is a lethal complication of cancer. Its incidence is rising and in 10% of the cases NM is the first manifestation of the disease. Diagnosis relies on the clinical manifestations, neuroimages, and finding of malignant cells in cerebrospinal fluid (CSF). Diagnosis is often challenging due to the low sensitivity of the different diagnostic modalities. The aim of this study is to identify the clinical features, diagnosis, treatment and outcome of lung cancer patients with NM. Methods Clinical records from patients with diagnosis of lung cancer and NM between 2011–2021 were retrospectively reviewed at a tertiary neurological center in Buenos Aires, Argentina. Results Twenty-seven patients were included. Median age was 58 years (IQR 52–64). 17 (65%) were female. Twenty-four patients had non-small cell lung cancer (91% adenocarcinoma), two had neuroendocrine lung cancer and one small cell lung cancer. In 19 (70%), meningeal involvement was a result of progressive disease from previously diagnosed cancer. In 12 (44%) patients meningeal disease developed posterior to parenchymal brain metastases surgical approach, 5 (41%) with posterior fossa craniotomy. Headache was the most frequent symptom (53%). CSF analysis was abnormal in 13 (48%) patients, with positive cytology in 10 (37%). Meningeal enhancement was detected with magnetic resonance imaging of brain or spine in 24 (92%) patients. Twenty-one (77%) patients received oncological treatment, 14 (51%) with chemotherapy (8 systemic, 3 intrathecal and 3 intrathecal plus systemic). Thirteen (48%) patients underwent treatment with either immunotherapy or target-therapy. 11 patients underwent whole brain radiotherapy. Median overall survival was 7 months (CI 95%: 3.5–10.4). Conclusion Headache was the most frequent symptom. Ninety-two percent of patients had meningeal pathological enhancement in high-quality MRI with gadolinium contrast of brain and spine. Despite median survival was poor (7 months), small subsets of these patients (22%) survived more than 2 years.