Journal articles: 'Cystic fibrosis, juvenile literature'

1

Orsini, Sara Immacolata, Edoardo Marrani, Ilaria Pagnini, Giovanni Taccetti, Vito Terlizzi, and Gabriele Simonini. "Concomitant Use of Elexacaftor/Tezacaftor/Ivacaftor and Etanercept in a Cystic Fibrosis Patient with Juvenile Idiopathic Arthritis." Journal of Clinical Medicine 12, no. 5 (February 21, 2023): 1730. http://dx.doi.org/10.3390/jcm12051730.

Full text

Abstract:

Patients with cystic fibrosis often complain of joint manifestations. However, only a few studies have reported the association between cystic fibrosis and juvenile idiopathic arthritis and addressed the therapeutic challenges of these patients. We describe the first paediatric case of a patient affected by cystic fibrosis, Basedow’s disease and juvenile idiopathic arthritis who was contemporarily treated with elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) and anti-tumor necrosis factor α (anti-TNFα). This report seems to reassure regarding the potential side effects of these associations. Moreover, our experience suggests that anti-TNFα is an effective option in CF patients affected by juvenile idiopathic arthritis, and is even safe for children receiving a triple CFTR modulator.

APA, Harvard, Vancouver, ISO, and other styles

2

Krawczyk, Patrycja, Urszula Sioma-Markowska, Dominika Krówka, Mariola Machura, Sylwia Kubaszewska, Violetta Skrzypulec-Plinta, and Patrycja Sodowska. "Cystic fibrosis and pregnancy – literature review." Annales Academiae Medicae Silesiensis 70 (2016): 84–88. http://dx.doi.org/10.18794/aams/61771.

Full text

APA, Harvard, Vancouver, ISO, and other styles

3

Wassil, Sarah K., Kristie M. Fox, and James W. White. "Once Daily Dosing of Aminoglycosides in Pediatric Cystic Fibrosis Patients: A Review of the Literature." Journal of Pediatric Pharmacology and Therapeutics 13, no. 2 (January 1, 2008): 68–75. http://dx.doi.org/10.5863/1551-6776-13.2.68.

Full text

Abstract:

Patients with cystic fibrosis receive many courses of antibiotic therapy throughout their lifetime. Dosing aminoglycosides once daily has become common practice in many of these individuals. Due to ease of home administration, decreased nursing time, and improved quality of life, this regimen is being increasingly explored in the cystic fibrosis population. Because patients with cystic fibrosis have increased aminoglycoside clearance, once daily dosing may result in a prolonged time during the dosing interval when concentrations of the drug may be undetectable. This makes the use of once daily dosing of these antibiotics in patients with cystic fibrosis controversial. Although aminoglycosides exhibit a post antibiotic effect, the duration of this effect is unknown in humans; therefore, the development of resistance to the aminoglycoside is a concern. This manuscript will review the organisms most commonly associated with a pulmonary exacerbation of cystic fibrosis, the properties of the aminoglycoside that make once daily dosing feasible, the concept of once daily dosing in those with cystic fibrosis and the current literature regarding efficacy, monitoring, toxicity and concerns of resistance with once daily dosing in this population.

APA, Harvard, Vancouver, ISO, and other styles

4

Pinzaru, Anca Daniela, Cristina Maria Mihai, Tatiana Chisnoiu, Alexandru Cosmin Pantazi, Vasile Valeriu Lupu, Mustafa Ali Kassim Kassim, Ancuta Lupu, et al. "Oxidative Stress Biomarkers in Cystic Fibrosis and Cystic Fibrosis-Related Diabetes in Children: A Literature Review." Biomedicines 11, no. 10 (September 29, 2023): 2671. http://dx.doi.org/10.3390/biomedicines11102671.

Full text

Abstract:

The most common inherited condition that results in death, particularly in those of Caucasian heritage, is cystic fibrosis (CF). Of all the young adults diagnosed with cystic fibrosis, 20% will develop hyperglycemia as a complication, later classified as a disease associated with cystic fibrosis. Impaired insulin secretion and glucose intolerance represent the primary mechanisms associated with diabetes (type 1 or type 2) and cystic fibrosis. Oxidative stress represents the imbalance between oxygen-reactive species and antioxidant defense mechanisms. This pathogenic mechanism is vital in triggering other chronic diseases, including cystic fibrosis-related diabetes. It is essential to understand oxidative stress and the significant impact it has on CFRD. This way, therapies can be individually adjusted and tailored to each patient’s needs. This review aims to understand the connection between CFRD and oxidative stress. As a subsidiary element, we analyzed the effects of glycemic balance on complications and their evolution over time, providing insights into their potential benefits in mitigating oxidative stress-associated complications.

APA, Harvard, Vancouver, ISO, and other styles

5

Kutsev, S. I., V. L. Izhevskaya, and E. I. Kondratyeva. "Targeted therapy for cystic fibrosis." PULMONOLOGIYA 31, no. 2 (April 11, 2021): 226–36. http://dx.doi.org/10.18093/0869-0189-2021-31-2-226-236.

Full text

Abstract:

The basic therapy of cystic fibrosis is currently aimed at slowing down the pathological processes associated with a decrease in the CFTR protein activity (cystic fibrosis transmembrane conductance regulator) in the gastrointestinal tract and the respiratory system. The pancreatic insufficiency is well compensated by replacement therapy with microsphere enzyme preparations and a high-calorie diet rich in proteins and fat. Chronic treatment of cystic fibrosis-related lung disease aims to improve the clearance of the bronchial tree, suppressing chronic bacterial infection and local chronic inflammation. However, no therapy was available to correct the defect in the gene or its product until 2012.The aim was to analyze literature on CFTR modulators, including their efficacy and safety, and assess the potential for developing new modulators to treat cystic fibrosis.Materials. The review included literature data (45 publications) on the use of CFTR modulators and international websites’ data.Results. Since the discovery of the CFTR gene in 1989, more than 2000 mutations or variants of the CFTR gene (hereinafter referred to as genetic variants) have been described. They interfere with the synthesis of the CFTR protein, its transport to the apical membrane of the cell, or disrupt its function as a channel for chloride anions. Although it is currently not possible to completely replace the mutant gene with a normal copy, small molecules have been identified that can modify the mutant CFTR protein and amend its function. The potential therapeutic measures are determined by class of the mutation. In clinical practice, pharmacological modeling of ion transport is currently possible only with the use of CFTR modulators: correctors and potentiators. The review defines these groups of drugs and describes 4 licensed CFTR modulators, including molecules of ivacaftor, lumacaftor, tezacaftor, elexacaftor. The data on the promising emerging next generation modulators and the prospects for the personalized selection of drugs using the assays on intestinal organoids are presented.

APA, Harvard, Vancouver, ISO, and other styles

6

Eid, Nemr S., Lawrence R. Shoemaker, and Tamisha D. Samiec. "Vitamin A in Cystic Fibrosis." Journal of Pediatric Gastroenterology and Nutrition 10, no. 2 (February 1990): 265–69. http://dx.doi.org/10.1002/j.1536-4801.1990.tb09983.x.

Full text

Abstract:

Much has been learned about vitamin A physiology in the last 50 years, yet few changes have been made in therapy. Unfamiliarity with vitamin A bioavailability and distribution may inadvertently result in toxicity. A literature search demonstrates that hypovitaminosis A has rarely been reported in patients with cystic fibrosis, and may manifest very differently in children of different ages. Furthermore, hypervitaminosis A may present with similar features, and can result from correction of deficiency. We report such a case in a 4.5‐month‐old infant, newly diagnosed with cystic fibrosis, who suffered first from vitamin A deficiency and then vitamin A toxicity. A brief review of vitamin A physiology, deficiency, and toxicity is presented.

APA, Harvard, Vancouver, ISO, and other styles

7

Burlutskaya, A. V., V. Yu Brisin, A. V. Statova, O. Yu Zenkina, and J. V. Pisotskaya. "Targeted Therapy for Cystic Fibrosis in Children." Innovative Medicine of Kuban, no. 4 (November 17, 2023): 142–47. http://dx.doi.org/10.35401/2541-9897-2023-8-4-142-147.

Full text

Abstract:

A mutation in the CFTR gene causes the malfunctioning of the CFTR protein that is located on the apical membrane of epithelial cells and functions as a chloride channel. The discovery of CFTR modulators is an advance in cystic fibrosis treatment.Objective: To analyze modern Russian and foreign literature on targeted therapy for cystic fibrosis in children. We also describe the history of cystic fibrosis, evolution in its diagnosis and treatment, and mechanisms behind targeted therapy for cystic fibrosis.

APA, Harvard, Vancouver, ISO, and other styles

8

Rodrigues, Roberta, Carmen S. Gabetta, Karla P. Pedro, Fabio Valdetaro, Maria I. M. Fernandes, Patrícia K. R. Magalhães, José N. Januário, and Léa M. Z. Maciel. "Cystic fibrosis and neonatal screening." Cadernos de Saúde Pública 24, suppl 4 (2008): s475—s484. http://dx.doi.org/10.1590/s0102-311x2008001600002.

Full text

Abstract:

The clinical and diagnostic aspects of cystic fibrosis have been extensively reviewed, with an emphasis on neonatal screening. This systematic literature review involved a search for relevant contributions in the PubMed and SciELO databases. The first references to cystic fibrosis date to the Middle Ages. Cystic fibrosis is the most frequent autosomal recessive hereditary disease among Caucasians (1:2,000 to 3,500). More than 1,000 mutations lead to the disease, the most common being "F508, with 70% prevalence among Canadian, Northern European, and American Caucasians and 23 to 55% prevalence among Brazilians. The basic defect is in chloride ion secretion. Cystic fibrosis screening has long been controversial, and after almost three decades, there are few nationwide programs (most are regional or local). However, the U.S. Centers for Disease Control and Prevention (CDC) has concluded that screening for cystic fibrosis is justified. The lack of a specific screening test and the ethnic heterogeneity of the Brazilian population pose challenges for neonatal screening.

APA, Harvard, Vancouver, ISO, and other styles

9

Loyd, India, Nicole Papac, Jason Hirshburg, Jarad Levin, Jennifer Dannelley, Janislynn Dorris, Jason Stratton, and Nighat Mehdi. "If At First You Don't Succeed, Trikafta Again." Journal of Pediatric Pharmacology and Therapeutics 27, no. 5 (May 1, 2022): 467–69. http://dx.doi.org/10.5863/1551-6776-27.5.467.

Full text

Abstract:

Adverse reactions, including severe cutaneous reactions, to cystic fibrosis transmembrane conductance regulator (CFTR) modulators have been described in the literature. Herein we present a drug eruption in response to elexacaftor/tezcaftor/ivacaftor (brand name, Trikafta) in a 7-year-old male with cystic fibrosis, followed by desensitization and successful continuation. A review of the literature outlining similar cases is provided. Attempting to mitigate and manage drug reactions to CFTR modulators is essential because they represent vital and irreplaceable therapies for individuals with cystic fibrosis (CF).

APA, Harvard, Vancouver, ISO, and other styles

10

Buzzetti, R., D. Salvatore, E. Baldo, M. P. Forneris, V. Lucidi, D. Manunza, I. Marinelli, et al. "An overview of international literature from cystic fibrosis registries: 1. Mortality and survival studies in cystic fibrosis." Journal of Cystic Fibrosis 8, no. 4 (July 2009): 229–37. http://dx.doi.org/10.1016/j.jcf.2009.04.001.

Full text

APA, Harvard, Vancouver, ISO, and other styles

11

Daly, Conor, Philip Ruane, Karl O’Reilly, Louise Longworth, and Gabriela Vega-Hernandez. "Caregiver burden in cystic fibrosis: a systematic literature review." Therapeutic Advances in Respiratory Disease 16 (January 2022): 175346662210864. http://dx.doi.org/10.1177/17534666221086416.

Full text

Abstract:

Background: Cystic fibrosis (CF) affects more than 80,000 people worldwide, having a considerable impact on the quality of life of patients and their caregivers, who assist patients with time-consuming treatment regimens. Despite this, a review of the available evidence has not been previously undertaken. This systematic literature review aimed to identify the humanistic and economic burdens of CF on caregivers. Methods: A systematic literature review was conducted, in accordance with Preferred Reporting Items for Systematic reviews and Meta-Analyses guidelines. Publications reporting outcomes for the caregivers of people with CF, including utility data, health status, and occupational impact, were reviewed. Sources searched were Embase (OvidSP), Medline (PubMed), the Cochrane Database of Systematic Reviews, and the Epistemonikos database, from 2010 to March 2020. A subsequent search with updated terms identified articles up to April 2020. Electronic searches were supplemented by hand searches to capture all relevant literature. Results: A total of 889 articles reporting humanistic burden and 310 reporting economic burden were identified. Following full-text screening by two independent reviewers, 72 articles were included in the review, of which 65 and 17 reported data on humanistic and economic burdens, respectively, with 10 reporting on both. The reviewed literature covered several outcomes and identified multiple key findings: greater disease severity is associated with the reporting of greater caregiver burden and lower utility scores of quality of life; reduced patient lung function is associated with increased caregiver depression and anxiety; and caregiving causes significant occupational impact, with pulmonary exacerbations decreasing caregiver productivity by up to a third compared with the patient being in a ‘well’ state. Conclusion: Findings from this systematic literature review highlight the substantial humanistic and economic burdens borne by the caregivers of people with CF. Future research would help to further inform on the link between disease severity and caregiver burden.

APA, Harvard, Vancouver, ISO, and other styles

12

Herman, Katarzyna, Małgorzata Kowalczyk-Zając, and Tomasz Pytrus. "Oral cavity health among cystic fibrosis patients: Literature overview." Advances in Clinical and Experimental Medicine 26, no. 7 (October 31, 2017): 1147–53. http://dx.doi.org/10.17219/acem/64054.

Full text

APA, Harvard, Vancouver, ISO, and other styles

13

Salvatore, Donatello, Roberto Buzzetti, Ermanno Baldo, Maria Pia Forneris, Vincenzina Lucidi, Daniela Manunza, Italo Marinelli, et al. "An overview of international literature from cystic fibrosis registries." Journal of Cystic Fibrosis 9, no. 2 (March 2010): 75–83. http://dx.doi.org/10.1016/j.jcf.2009.11.002.

Full text

APA, Harvard, Vancouver, ISO, and other styles

14

Santi, Maristella, Gregorio P. Milani, Giacomo D. Simonetti, Emilio F. Fossali, Mario G. Bianchetti, and Sebastiano A. G. Lava. "Magnesium in cystic fibrosis-Systematic review of the literature." Pediatric Pulmonology 51, no. 2 (December 10, 2015): 196–202. http://dx.doi.org/10.1002/ppul.23356.

Full text

APA, Harvard, Vancouver, ISO, and other styles

15

Saiman, Lisa, and Jane Siegel. "Infection Control in Cystic Fibrosis." Clinical Microbiology Reviews 17, no. 1 (January 2004): 57–71. http://dx.doi.org/10.1128/cmr.17.1.57-71.2004.

Full text

Abstract:

SUMMARY Over the past 20 years there has been a greater interest in infection control in cystic fibrosis (CF) as patient-to-patient transmission of pathogens has been increasingly demonstrated in this unique patient population. The CF Foundation sponsored a consensus conference to craft recommendations for infection control practices for CF care providers. This review provides a summary of the literature addressing infection control in CF. Burkholderia cepacia complex, Pseudomonas aeruginosa, and Staphylococcus aureus have all been shown to spread between patients with CF. Standard precautions, transmission-based precautions including contact and droplet precautions, appropriate hand hygiene for health care workers, patients, and their families, and care of respiratory tract equipment to prevent the transmission of infectious agents serve as the foundations of infection control and prevent the acquisition of potential pathogens by patients with CF. The respiratory secretions of all CF patients potentially harbor clinically and epidemiologically important microorganisms, even if they have not yet been detected in cultures from the respiratory tract. CF patients should be educated to contain their secretions and maintain a distance of >3 ft from other CF patients to avoid the transmission of potential pathogens, even if culture results are unavailable or negative. To prevent the acquisition of pathogens from respiratory therapy equipment used in health care settings as well as in the home, such equipment should be cleaned and disinfected. It will be critical to measure the dissemination, implementation, and potential impact of these guidelines to monitor changes in practice and reduction in infections.

APA, Harvard, Vancouver, ISO, and other styles

16

Floto, R. Andres, Kenneth N. Olivier, Lisa Saiman, Charles L. Daley, Jean-Louis Herrmann, Jerry A. Nick, Peadar G. Noone, et al. "US Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis: executive summary." Thorax 71, no. 1 (December 16, 2015): 88–90. http://dx.doi.org/10.1136/thoraxjnl-2015-207983.

Full text

Abstract:

Non-tuberculous mycobacteria (NTM) are ubiquitous environmental organisms that can cause chronic pulmonary infection, particularly in individuals with pre-existing inflammatory lung disease, such as cystic fibrosis (CF). Pulmonary disease (PD) caused by NTM has emerged as a major threat to the health of individuals with CF, but remains difficult to diagnose and problematic to treat. In response to this challenge, the US Cystic Fibrosis Foundation (CFF) and the European Cystic Fibrosis Society (ECFS) convened a panel of 19 experts to develop consensus recommendations for the screening, investigation, diagnosis and management of NTM-PD in individuals with CF. PICO (population, intervention, comparison, outcome) methodology and systematic literature reviews were employed to inform draft recommendations, which were then modified to achieve consensus and subsequently circulated for public consultation within the USA and European CF communities. We have thus generated a series of pragmatic, evidence-based recommendations as an initial step in optimising management for this challenging condition.

APA, Harvard, Vancouver, ISO, and other styles

17

Ritivoiu, Mirela-Elena, Cristina Manuela Drăgoi, Dumitru Matei, Iustina Violeta Stan, Alina Crenguţa Nicolae, Mihai Craiu, Ion-Bogdan Dumitrescu, and Alina Angelica Ciolpan. "Current and Future Therapeutic Approaches of Exocrine Pancreatic Insufficiency in Children with Cystic Fibrosis in the Era of Personalized Medicine." Pharmaceutics 15, no. 1 (January 3, 2023): 162. http://dx.doi.org/10.3390/pharmaceutics15010162.

Full text

Abstract:

This review presents current updates of pancreatic enzyme replacement therapy in children with cystic fibrosis based on literature published in the last decade and some special considerations regarding pancreatic enzyme replacement therapy in the era of new therapies, such as cystic fibrosis transmembrane conductance regulator modulator therapies. Few articles evaluate the efficacy of pancreatic enzyme replacement therapy in the pediatric population, and most studies also included children and adults with cystic fibrosis. Approximately 85% of cystic fibrosis patients have exocrine pancreatic insufficiency and need pancreatic enzyme replacement therapy. Fecal elastase is the most commonly used diagnostic test for exocrine pancreatic insufficiency, although this value can fluctuate over time. While it is used as a diagnostic test, it cannot be used for monitoring the effectiveness of pancreatic enzyme replacement therapy and for adjusting doses. Pancreatic enzyme replacement therapy, the actual treatment for exocrine pancreatic insufficiency, is essential in children with cystic fibrosis to prevent malabsorption and malnutrition and needs to be urgently initiated. This therapy presents many considerations for physicians, patients, and their families, including types and timing of administration, dose monitoring, and therapy failures. Based on clinical trials, pancreatic enzyme replacement therapy is considered effective and well-tolerated in children with cystic fibrosis. An important key point in cystic fibrosis treatment is the recent hypothesis that cystic fibrosis transmembrane conductance regulator modulators could improve pancreatic function, further studies being essential. Pancreatic enzyme replacement therapy is addressed a complication of the disease (exocrine pancreatic insufficiency), while modulators target the defective cystic fibrosis transmembrane conductance regulator protein. Exocrine pancreatic insufficiency in cystic fibrosis remains an active area of research in this era of cystic fibrosis transmembrane conductance regulator modulator therapies. This new therapy could represent an example of personalized medicine in cystic fibrosis patients, with each class of modulators being addressed to patients with specific genetic mutations.

APA, Harvard, Vancouver, ISO, and other styles

18

Djaziri, Nabila, Cindy Burel, Lilia Abbad, Zeineb Bakey, Rémi Piedagnel, and Brigitte Lelongt. "Cleavage of periostin by MMP9 protects mice from kidney cystic disease." PLOS ONE 18, no. 12 (December 1, 2023): e0294922. http://dx.doi.org/10.1371/journal.pone.0294922.

Full text

Abstract:

The matrix metalloproteinase MMP9 influences cellular morphology and function, and plays important roles in organogenesis and disease. It exerts both protective and deleterious effects in renal pathology, depending upon its specific substrates. To explore new functions for MMP9 in kidney cysts formation and disease progression, we generated a mouse model by breeding juvenile cystic kidney (jck) mice with MMP9 deficient mice. Specifically, we provide evidence that MMP9 is overexpressed in cystic tissue where its enzymatic activity is increased 7-fold. MMP9 deficiency in cystic kidney worsen cystic kidney diseases by decreasing renal function, favoring cyst expansion and fibrosis. In addition, we find that periostin is a new critical substrate for MMP9 and in its absence periostin accumulates in cystic lining cells. As periostin promotes renal cyst growth and interstitial fibrosis in polycystic kidney diseases, we propose that the control of periostin by MMP9 and its associated intracellular signaling pathways including integrins, integrin-linked kinase and focal adhesion kinase confers to MMP9 a protective effect on the severity of the disease.

APA, Harvard, Vancouver, ISO, and other styles

19

Polgreen, Philip M., and Alejandro P. Comellas. "Clinical Phenotypes of Cystic Fibrosis Carriers." Annual Review of Medicine 73, no. 1 (January 27, 2022): 563–74. http://dx.doi.org/10.1146/annurev-med-042120-020148.

Full text

Abstract:

Cystic fibrosis (CF) is an autosomal recessive genetic disorder caused by mutations in CFTR, the cystic fibrosis transmembrane conductance regulator gene. People with CF experience a wide variety of medical conditions that affect the pulmonary, endocrine, gastrointestinal, pancreatic, biliary, and reproductive systems. Traditionally, CF carriers, with one defective copy of CFTR, were not thought to be at risk for CF-associated diseases. However, an emerging body of literature suggests that heterozygotes are at increased risk for many of the same conditions as homozygotes. For example, heterozygotes appear to be at increased risk for chronic pancreatitis, atypical mycobacterial infections, and bronchiectasis. In the United States alone, there are almost 10 million CF carriers. Universal newborn screening and prenatal genetic screening will identify more. Thus, there is a critical need to develop more precise estimates of health risks attributable to the CF carrier state across the lifespan.

APA, Harvard, Vancouver, ISO, and other styles

20

Sun, Xingshen, Alicia K. Olivier, Bo Liang, Yaling Yi, Hongshu Sui, Turan I. A. Evans, Yulong Zhang, et al. "Lung Phenotype of Juvenile and Adult Cystic Fibrosis Transmembrane Conductance Regulator–Knockout Ferrets." American Journal of Respiratory Cell and Molecular Biology 50, no. 3 (March 2014): 502–12. http://dx.doi.org/10.1165/rcmb.2013-0261oc.

Full text

APA, Harvard, Vancouver, ISO, and other styles

21

Yankina, G. N., E. I. Kondratieva, E. V. Loshkova, I. V. Doroshenko, M. V. Rebrienko, Yu S. Rafikova, E. A. Bozhenko, et al. "Cystic fibrosis: comorbidity with other serious diseases." Experimental and Clinical Gastroenterology, no. 3 (July 25, 2023): 98–111. http://dx.doi.org/10.31146/1682-8658-ecg-211-3-98-111.

Full text

Abstract:

Numerous epidemiological studies demonstrate that cystic fibrosis, the most common orphan disease in the world, can not occur in isolation, but can be combined with other serious diseases. The most common such combination in all populations is traditionally cystic fibrosis and celiac disease, which, on the one hand, differ in the leading mechanism of inflammation, in cystic fibrosis - chronic microbial inflammation, in celiac disease - autoimmune, on the other hand, these two diseases have a number of common pathogenesis links, in particular, realizing the syndrome of malabsorption, which only complicates the timely diagnosis of these combined pathological conditions. In addition, the authors of the literature review focus on clinical examples of late detection of autoimmune gluten intolerance against the background of cystic fibrosis. They also consider the combination of cystic fibrosis with other severe, disabling diseases (rheumatoid arthritis, phenylketonuria, oncological diseases), which sometimes require a serious change in therapeutic tactics.

APA, Harvard, Vancouver, ISO, and other styles

22

Quittner, Alexandra L., Janice Abbott, Anna M. Georgiopoulos, Lutz Goldbeck, Beth Smith, Sarah E. Hempstead, Bruce Marshall, Kathryn A. Sabadosa, and Stuart Elborn. "International Committee on Mental Health in Cystic Fibrosis: Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus statements for screening and treating depression and anxiety." Thorax 71, no. 1 (October 9, 2015): 26–34. http://dx.doi.org/10.1136/thoraxjnl-2015-207488.

Full text

Abstract:

Studies measuring psychological distress in individuals with cystic fibrosis (CF) have found high rates of both depression and anxiety. Psychological symptoms in both individuals with CF and parent caregivers have been associated with decreased lung function, lower body mass index, worse adherence, worse health-related quality of life, more frequent hospitalisations and increased healthcare costs. To identify and treat depression and anxiety in CF, the CF Foundation and the European CF Society invited a panel of experts, including physicians, psychologists, psychiatrists, nurses, social workers, a pharmacist, parents and an individual with CF, to develop consensus recommendations for clinical care. Over 18 months, this 22-member committee was divided into four workgroups: Screening; Psychological Interventions; Pharmacological Treatments and Implementation and Future Research, and used the Population, Intervention, Comparison, Outcome methodology to develop questions for literature search and review. Searches were conducted in PubMed, PsychINFO, ScienceDirect, Google Scholar, Psychiatry online and ABDATA by a methodologist at Dartmouth. The committee reviewed 344 articles, drafted statements and set an 80% acceptance for each recommendation statement as a consensus threshold prior to an anonymous voting process. Fifteen guideline recommendation statements for screening and treatment of depression and anxiety in individuals with CF and parent caregivers were finalised by vote. As these recommendations are implemented in CF centres internationally, the process of dissemination, implementation and resource provision should be closely monitored to assess barriers and concerns, validity and use.

APA, Harvard, Vancouver, ISO, and other styles

23

Liu, Xin, Jingxi Wang, Yanglu Liu, Shuang Luo, Gaowu Yan, Huaqi Yang, Lili Wan, and Guohua Huang. "High Intensity Focused Ultrasound Ablation for Juvenile Cystic Adenomyosis: Two Case Reports and Literature Review." Diagnostics 13, no. 9 (May 1, 2023): 1608. http://dx.doi.org/10.3390/diagnostics13091608.

Full text

Abstract:

Cystic adenomyosis is a rare type of uterine adenomyosis, mainly seen in young women, which is often characterized by severe dysmenorrhea. The quality of life and reproductive function of young women could be affected by misdiagnosis and delayed treatment. At present, there are no universal guidelines and consensus. We report two cases of patients with cystic adenomyosis in juveniles treated with high-intensity focused ultrasound (HIFU) ablation. In the first case, magnetic resonance imaging (MRI) indicated a cystic mass of 2.0 cm × 3.1 cm × 2.4 cm in the uterus. After she underwent HIFU treatment, her pelvic MRI showed a mass of 1.1 × 2.4 cm in size, and her dysmenorrhea symptoms gradually disappeared. In the second case, a pelvic MRI indicated a 5.1 cm × 3.3 cm × 4.7 cm cystic mass in the uterus. After she underwent HIFU and combined four consecutive cycles of GnRH-a treatment, the lesion shrunk 1.2 cm ×1.4 cm × 1.6 cm, without dysmenorrhea. Simultaneously, the report reviewed 14 cases of juvenile cystic adenomyosis over the last ten years. HIFU or HIFU-combined drugs were safe and effective in treating juvenile cystic adenomyosis, but multicenter and prospective studies may be necessary to validate this in the future.

APA, Harvard, Vancouver, ISO, and other styles

24

Kozlov, A. V., O. A. Gusyakova, A. A. Ereshchenko, and A. V. Khaliulin. "DIAGNOSTIC POSSIBILITIES OF MODERN BIOCHEMICAL STUDY OF SPUTUM FROM PATIENTS WITH CYSTIC FIBROSIS (LITERATURE REVIEW)." Russian Clinical Laboratory Diagnostics 64, no. 1 (April 29, 2019): 24–28. http://dx.doi.org/10.18821/0869-2084-2019-64-1-24-28.

Full text

Abstract:

The review presents the pathobiochemical and molecular mechanisms of sputum formation in patients with cystic fibrosis associated with the pathophysiological features of the disease. Statistical data on the prevalence of this pathology in the world and in the Russian Federation are presented. The mechanisms of sputum formation and disorders of the mucociliary apparatus, leading to the accumulation of viscous bronchopulmonary secret in cystic fibrosis, are considered. The principles of the relationship between the rheological properties of sputum and the formation of inflammation in the lungs with the addition of a concomitant specific microflora in the bronchopulmonary system in patients with cystic fibrosis are presented. Describes the opportunities for biochemical studies of sputum of patients with this pathology: determining the activity of enzymes (myeloperoxidase), the content of proteinase inhibitors (α2-macroglobulin and α1-antitrypsin) and proinflammatory cytokines (IL-8 and TNFa), concentrations of iron and ferriferous proteins (lactoferrin and ferritin), which makes biochemical studies of sputum available, non-invasive, quick and cost-effective method of diagnosis, which can be widely used as an auxiliary laboratory method and makes it possible to use these metabolites as diagnostic markers to assess the severity of inflammation and infection of the lower respiratory tract and predict the development of respiratory complications in patients with cystic fibrosis.

APA, Harvard, Vancouver, ISO, and other styles

25

Schwarz, C., P. N. Eschenhagen, J. G. Mainz, T. Schmidergall, H. Schuette, and E. Romanowska. "Pulmonary Aspergillosis in People with Cystic Fibrosis." Seminars in Respiratory and Critical Care Medicine 45, no. 01 (January 29, 2024): 128–40. http://dx.doi.org/10.1055/s-0043-1777267.

Full text

Abstract:

AbstractIn the last decade, fungal respiratory diseases have been increasingly investigated for their impact on the clinical course of people with cystic fibrosis (CF), with a particular focus on infections caused by Aspergillus spp. The most common organisms from this genus detected from respiratory cultures are Aspergillus fumigatus and Aspergillus terreus, followed by Aspergillus flavus, Aspergillus niger, and Aspergillus nidulans. These species have been identified to be both chronic colonizers and sources of active infection and may negatively impact lung function in people with CF. This review article discusses definitions of aspergillosis, challenges in clinical practice, and current literature available for laboratory findings, clinical diagnosis, and treatment options for pulmonary diseases caused by Aspergillus spp. in people with CF.

APA, Harvard, Vancouver, ISO, and other styles

26

Feuillet-Fieux, M. N., G. Lenoir, I. Sermet, C. Elie, J. Djadi-Prat, M. Ferrec, M. Magen, et al. "Nasal polyposis and cystic fibrosis(CF): review of the literature." Rhinology journal 49, no. 3 (August 1, 2011): 347–55. http://dx.doi.org/10.4193/rhino10.225.

Full text

Abstract:

The aim of this study was to address whether NP might be a predictive factor for severity of CF. The authors collected data from the literature on NP as a unique or associated sign in CF and reviewed the clinical and molecular aspects of CF associated with NP. CF genotypes and clinical severity in NP(+) vs. NP(-) patients were reviewed, taking into account pulmonary function, frequency of P. aeruginosa lung infection, frequency of allergy, nutritional status, and exocrine pancreatic function. The CFTR gene was also analyzed in a patient with isolated severe NP as the unique feature of CF. This review of the literature showed a `milder` phenotype in `NP+` vs. `NP-` CF patients, contrasting with a marked association between NP and `severe` CF mutations. In addition, a complex genotype was identified, associating four heterozygous variants, namely p.Q493X (a severe mutation) on the paternal allele, and p.V562I, p.A1006E, and (TG)11(T)5 (IVS8-5T) on the maternal allele, in a case of CF presenting as isolated NP. The authors speculate that genetic/environmental factors associated with NP might attenuate the functional impact of `severe` CF mutations. The overrepresentation of CF carriers among patients with isolated NP also advocates the need for CFTR molecular screening in such populations for genetic counselling purposes.

APA, Harvard, Vancouver, ISO, and other styles

27

Saldana, Pablo S., and Jamie L. Pomeranz. "Cystic fibrosis and the workplace: A review of the literature." Work 42, no. 2 (2012): 185–93. http://dx.doi.org/10.3233/wor-2012-1353.

Full text

APA, Harvard, Vancouver, ISO, and other styles

28

Scurati-Manzoni, Elisabetta, Emilio F. Fossali, Carlo Agostoni, Enrica Riva, Giacomo D. Simonetti, Maura Zanolari-Calderari, Mario G. Bianchetti, and Sebastiano A. G. Lava. "Electrolyte abnormalities in cystic fibrosis: systematic review of the literature." Pediatric Nephrology 29, no. 6 (December 11, 2013): 1015–23. http://dx.doi.org/10.1007/s00467-013-2712-4.

Full text

APA, Harvard, Vancouver, ISO, and other styles

29

Narayanan, Siva, Jochen G. Mainz, Smeet Gala, Harold Tabori, and Daniel Grossoehme. "Adherence to therapies in cystic fibrosis: a targeted literature review." Expert Review of Respiratory Medicine 11, no. 2 (January 20, 2017): 129–45. http://dx.doi.org/10.1080/17476348.2017.1280399.

Full text

APA, Harvard, Vancouver, ISO, and other styles

30

Zitsch, Robert P., and James S. Reilly. "Vocal Cord Paralysis Associated with Cystic Fibrosis." Annals of Otology, Rhinology & Laryngology 96, no. 6 (November 1987): 680–83. http://dx.doi.org/10.1177/000348948709600613.

Full text

Abstract:

The course of the left recurrent laryngeal nerve through the chest brings it in close proximity with the heart and great cardiac vessels. Diseases of the heart and the great vessels are known to cause vocal cord paralysis, probably by mechanical injury to the recurrent laryngeal nerve. Pulmonary artery hypertension and dilation occur in up to 80% of patients with cystic fibrosis. We report a case of a 23-year-old woman with cystic fibrosis and left vocal cord paralysis. We believe that sudden pulmonary artery expansion produced recurrent laryngeal nerve injury and vocal cord paralysis. This is only the second association of unilateral vocal cord paralysis and cystic fibrosis in the medical literature. The pathophysiology of the cardiovocal syndrome is discussed.

APA, Harvard, Vancouver, ISO, and other styles

31

Farooqui, Azhar, Susan Gamal Eldin, Muna Dawood Ali, Ali AlTalhi, and Ahmad AlDigheari. "Cystic Fibrosis in a Female Infant with Cardiac, Ocular, and Musculoskeletal Anomalies." Case Reports in Pediatrics 2015 (2015): 1–3. http://dx.doi.org/10.1155/2015/379018.

Full text

Abstract:

Cystic fibrosis (CF) remains the most common hereditary disease in the western population. Its concomitant presence with other congenital abnormalities is a rare phenomenon with very little documentation. In this case report we describe a case of cystic fibrosis in a female infant with cardiac, ocular, and musculoskeletal abnormalities. A brief literature review is also provided.

APA, Harvard, Vancouver, ISO, and other styles

32

Touw, DJ, RW Brimicombe, ME Hodson, HG Heijerman, and W. Bakker. "Inhalation of antibiotics in cystic fibrosis." European Respiratory Journal 8, no. 9 (September 1, 1995): 1594–604. http://dx.doi.org/10.1183/09031936.95.08091594.

Full text

Abstract:

Aerosol administration of antipseudomonal antibiotics is commonly used in cystic fibrosis. However, its contribution to the improvement of lung function, infection and quality of life is not well-established. All articles published from 1965 until the present time concerning the inhalation of antibiotics in cystic fibrosis were collected by computerized literature search and analysed. Effective aerosol delivery is compromised by nebulizers with limited capacity to produce particles in the respirable range. Twelve studies concerning maintenance treatment were published. Four uncontrolled studies evaluating antibiotic aerosol maintenance treatment in stable patients indicated a beneficial effect in terms of reducing the number of hospital admissions. Eight placebo-controlled studies were found; six of these showed a significant improvement of lung function in the treatment group. Four studies showed a reduction of the number of hospital admissions. In some studies, there was a considerable negative effect of the nebulized placebo solution on the outcome, probably due to the improper choice of its osmolarity. Studies with antibiotic aerosols as adjunct to intravenous therapy in cystic fibrosis patients with an acute exacerbation showed no enhancement of the clinical effects of the intravenous antibiotic by the aerosol; sputum colony counts, however, were lower. Toxicity studies carried out so far have shown no renal or ototoxicity; however, long-term toxicity studies still have to be performed using higher dosages. Introduction or selection of resistant bacteria is relatively rare, but remains a matter of concern. Aerosol maintenance treatment with an appropriate antibiotic in high enough dosage can be recommended for patients with cystic fibrosis chronically infected with P. aeruginosa, and may improve lung function and reduce the number of hospital admissions due to an acute exacerbation.

APA, Harvard, Vancouver, ISO, and other styles

33

Suardiani, Luh Adi Kusuma, Fauna Herawati, and Ni Made Suastini Suastini. "The Efficacy and Safety of Azithromycin for Patients with Cystic Fibrosis: A Systematic Review." Journal of Health Sciences 15, no. 02 (May 25, 2022): 98–106. http://dx.doi.org/10.33086/jhs.v15i02.2233.

Full text

Abstract:

Chronic inflammation of the lungs is a major cause of morbidity and mortality in patients with cystic fibrosis. One of the macrolides, azithromycin has antimicrobial, immunomodulatory, and anti-inflammatory effects that are useful in diseases with chronic inflammatory processes such as cystic fibrosis. This systematic review aimed to determine the efficacy and safety of azithromycin administration for cystic fibrosis patients in improving lung function. Pulmonary function was assessed by measuring the value of forced expiratory volume in one second (FEV1) in patients after intervention. The process of searching literature through PUBMED and Cochrane Library uses the keywords "Cystic Fibrosis" and "Azithromycin" with the Boolean operator "AND". There were seven studies selected, with criteria RCT studies, patients of all ages, patients with cystic fibrosis, and compared azithromycin with placebo. After reviewing seven studies, 71.4% of the studies stated that there was a significant increase of mean FEV1 value after being given azithromycin therapy. Other outcomes assessed were FVC values, pro-inflammatory indicators, exacerbations, changes in body weight, and quality of life. Azithromycin administration is considered relatively safe and well-tolerated by patients.

APA, Harvard, Vancouver, ISO, and other styles

34

Ahmed, Taha, Samra Haroon Lodhi, Michael I. Anstead, and Adrian W. Messerli. "Cerebral paradoxical embolisation in a patient with cystic fibrosis with patent foramen ovale: a comparative review of literature." BMJ Case Reports 14, no. 5 (May 2021): e242302. http://dx.doi.org/10.1136/bcr-2021-242302.

Full text

Abstract:

A 52-year-old woman with cystic fibrosis presented to the emergency department with expressive aphasia and right-sided hemiparesis. CT scan of the brain revealed a left middle cerebral artery territory infarct. A diagnosis of cerebral paradoxical embolisation associated with patent foramen ovale and a history of deep venous thrombosis was made. The patient underwent endovascular thrombectomy and percutaneous closure of patent foramen ovale. Current literature, including five published case reports, pertaining to the subject is discussed. The unique aspects of the case are highlighted, including the particular risk of cerebral paradoxical embolisation in patients with cystic fibrosis. The result of this case report, in context to previously reported literature, suggests that clinicians should be aware of paradoxical embolisation in patients with cystic fibrosis via an intracardiac shunt, particularly with implanted vascular access devices and a history of deep venous thrombosis.

APA, Harvard, Vancouver, ISO, and other styles

35

Deblaere, Lieselot, Wouter Froyman, Thierry Van den Bosch, Anne‐Sophie Van Rompuy, Jeroen Kaijser, Jan Deprest, and Dirk Timmerman. "Juvenile cystic adenomyosis: A case report and review of the literature." Australasian Journal of Ultrasound in Medicine 22, no. 4 (July 3, 2019): 295–300. http://dx.doi.org/10.1002/ajum.12171.

Full text

APA, Harvard, Vancouver, ISO, and other styles

36

Pattishall, Edward N. "Negative Clinical Trials in Cystic Fibrosis Research." Pediatrics 85, no. 3 (March 1, 1990): 277–81. http://dx.doi.org/10.1542/peds.85.3.277.

Full text

Abstract:

The statistical power of 61 negative clinical trials of therapeutic regimens in patients with cystic fibrosis published from 1977 through 1988 was reviewed and the ability of the investigations to detect small, medium, and large standardized differences was calculated. Small, medium, and large standardized differences were defined as ratios of 0.2, 0.5, and 0.8, respectively, of the observed difference compared with the standard deviation. The average numbers (±SD) of patients in the treatment and control groups were 14.3 ± 6.9 and 14.5 ± 7.9, respectively. None of the studies had 80% power to detect a small or medium standardized difference and only 4 of the reports had 80% statistical power to detect a large standardized difference. The variability of cystic fibrosis causes a decrease in the standardized difference, making it more difficult to demonstrate statistical significance. Statistical power of negative clinical trials reported in the literature deserves more attention from investigators as well as physicians who treat patients with cystic fibrosis.

APA, Harvard, Vancouver, ISO, and other styles

37

Djolai, Matilda, Tamara Boskovic, Aleksandra Levakov, Mihaela Mocko-Kacanski, Bojana Andrejic, and Sandra Trivunic. "Pathohistological changes in fetuses with cystic fibrosis." Medical review 65, no. 1-2 (2012): 65–67. http://dx.doi.org/10.2298/mpns1202065d.

Full text

Abstract:

Introduction. Cystic fibrosis or mucoviscidosis is a genetically caused disease. The intensity of disease and histopathological changes grow throughout the life. According to the literature, pathological changes characteristic of cystic fibrosis become noticeable around the sixth month of life. Case Report. After amniocentesis of a 5-lunar month-old fetus had been done, which confirmed cystic fibrosis, the Ethics Commission approved the preterm labor. The autopsy and histopathological analysis demonstrated the existence of typical histopathological changes in the pancreas and intestines. Discussion. In the late fetal period or during the period around the delivery, cystic fibrosis is usually manifested as meconial cap with or without obstruction of the intestinal lumen. Morphological changes in the exocrine glands usually develop only after birth. In this case, the existence of meconial obstruction, as well as the typical acidofil content in the secretory ducts and acini of the pancreas was confirmed, which is unusual for the fetal age of five months.

APA, Harvard, Vancouver, ISO, and other styles

38

Morais, Helio S. Autran de, Stephen P. DiBartola, and Dennis J. Chew. "Juvenile renal disease in Golden Retrievers: 12 cases (1984-1994)." Journal of the American Veterinary Medical Association 209, no. 4 (August 15, 1996): 792–97. http://dx.doi.org/10.2460/javma.1996.209.04.792.

Full text

Abstract:

Objective To evaluate clinical and pathologic findings in Golden Retrievers with renal dysplasia. Design Retrospective study. Animals 12 young Golden Retrievers with chronic renal disease. Procedure Medical records of affected dogs were evaluated on the basis of clinical findings, laboratory test results, and histologic findings. Results Common clinical findings were vomiting, anorexia, weight loss, polydipsia, and polyuria. Common laboratory findings were azotemia, hyperphosphatemia, hypercholesterolemia, isosthenuria, proteinuria, hypercalcemia, and nonregenerative anemia. Many affected dogs also had urinary tract infections, and some were hypertensive. Renal lesions consisted of moderate-to-severe interstitial fibrosis and mild-to-moderate lymphoplasmacytic interstitial inflammation. Cystic glomerular atrophy and periglomerular fibrosis were prominent features in most affected dogs. Fetal lobulation of glomeruli, adenomatoid hyperplasia of collecting tubule epithelium, and primitive mesenchymal connective tissue were histologic features suggestive of renal dysplasia. Clinical Implications Renal dysplasia should be suspected in Golden Retrievers < 3 years old with clinical findings and laboratory results indicative of renal disease. (J Am Vet Med Assoc 1996;209:792–797)

APA, Harvard, Vancouver, ISO, and other styles

39

Parisi, Giuseppe Fabio, Maria Papale, Lucia Tardino, Raffaella Nenna, Fabio Midulla, and Salvatore Leonardi. "Biomarkers in Pediatric Lung Diseases Including Cystic Fibrosis." Current Respiratory Medicine Reviews 15, no. 3 (January 1, 2020): 163–73. http://dx.doi.org/10.2174/1573398x15666190521112824.

Full text

Abstract:

In recent decades, scientific studies of chemical processes involving metabolites have been steadily increasing, indicating that we are well into the metabolomics era. This has resulted in numerous studies that explore the field of biomarkers. One of the medical areas most concerned with these innovations is certainly that of childhood respiratory disorders, including asthma and cystic fibrosis. This current study is a review of the literature about biomarkers used or studied in the field of pediatric pulmonology, including asthma and cystic fibrosis.

APA, Harvard, Vancouver, ISO, and other styles

40

Peptisor, Cristina-Laura, Ioan Anton Arghir, Irina Ion, Alexandra Floriana Nemes, Maria-Cristina Mihai, Tatiana Chisnoiu, Simona Mosescu, and Oana-Cristina Arghir. "Cystic Fibrosis Associated Liver Disease and Bronchiectasis in Puberty. Case Report and Literature Review." ARS Medica Tomitana 28, no. 3 (August 1, 2022): 108–12. http://dx.doi.org/10.2478/arsm-2022-0023.

Full text

Abstract:

Abstract Cystic fibrosis (CF) represents a multisystemic condition, due to the transmembrane conductance regulatory protein (CFTR gene), involving both gastrointestinal tract and lungs. The prevalence of CF associated liver disease is related with increased life expectancy and it is rare in childhood or puberty. Liver disease (LD) is often preceded by complications associated with pulmonary damage. We present a case of a 13 year- old girl, known with cystic fibrosis since the age of 4 months, diagnosed with cholecyst hypoplasia and cholestasis, severe malnutrition progressive extension of bronchiectasis, recurrent respiratory infections in childhood, and, later, in puberty, liver disease with third stage fibrosis (F3) revealed by Fibroscan. Hepatobiliary damage is a late consequence, which is precisely why not performing a liver biopsy, especially in infants and young children, can be justified. We highlight the value of Fibroscan, a non-invasive imaging technique capable to reveal liver fibrosis and to stage it.

APA, Harvard, Vancouver, ISO, and other styles

41

Davids, L. M. "The daily physiotherapy regimen in cystic fibrosis?" South African Journal of Physiotherapy 42, no. 3 (August 31, 1986): 73–76. http://dx.doi.org/10.4102/sajp.v42i3.804.

Full text

Abstract:

The aim of this paper is to question the accepted physiotherapy regimen for children with Fibrocystic Disease. Is it the most effective way of keeping the lungs clear of secretions? How much are we imposing added stress on an already stressed family? Is it not possible to design a programme which reduces stress and is socially acceptable?The traditional physiotherapy regimen of breathing exercises, postural drainage and percussion is described. Forced expiratory technique is described as well as various forms of nebulisation.The psychological impact on the family of a chronic, life-threatening disease, is discussed. Compliance with set physiotherapy regimens and the family's (especially the mother's) reaction to them is discussed.The most recent literature on the effectiveness of traditional physiotherapy techniques is reviewed.From this review as well as from personal experience, it is concluded that a rigid physiotherapy regimen is stressful and compliance is frequently poor. Suggestions are made on how to overcome this. It is stressed that this paper deals with the home programme, not with the hospital programme for acute exacerbations of the disease.

APA, Harvard, Vancouver, ISO, and other styles

42

Weber, Ronja, Nathan Perkins, Tobias Bruderer, Srdjan Micic, and Alexander Moeller. "Identification of Exhaled Metabolites in Children with Cystic Fibrosis." Metabolites 12, no. 10 (October 17, 2022): 980. http://dx.doi.org/10.3390/metabo12100980.

Full text

Abstract:

The early detection of inflammation and infection is important to prevent irreversible lung damage in cystic fibrosis. Novel and non-invasive monitoring tools would be of high benefit for the quality of life of patients. Our group previously detected over 100 exhaled mass-to-charge (m/z) features, using on-line secondary electrospray ionization high-resolution mass spectrometry (SESI-HRMS), which distinguish children with cystic fibrosis from healthy controls. The aim of this study was to annotate as many m/z features as possible with putative chemical structures. Compound identification was performed by applying a rigorous workflow, which included the analysis of on-line MS2 spectra and a literature comparison. A total of 49 discriminatory exhaled compounds were putatively identified. A group of compounds including glycolic acid, glyceric acid and xanthine were elevated in the cystic fibrosis group. A large group of acylcarnitines and aldehydes were found to be decreased in cystic fibrosis. The proposed compound identification workflow was used to identify signatures of volatile organic compounds that discriminate children with cystic fibrosis from healthy controls, which is the first step for future non-invasive and personalized applications.

APA, Harvard, Vancouver, ISO, and other styles

43

Kleshchenko, Elena I., Elena V. Shimchenko, Aleksander F. Komarov, and Valeria E. Kharchenko. "The Risk of Developing of Malnutrition and the Principles of Correction of Nutritional Status Disorders in Children with Cystic Fibrosis." Pediatric pharmacology 20, no. 5 (November 17, 2023): 515–22. http://dx.doi.org/10.15690/pf.v20i5.2632.

Full text

Abstract:

Cystic fibrosis is a severe hereditary disease with polysystemic manifestations and progressive course. Malnutrition in cystic fibrosis occurs as a result of exocrine insufficiency of the pancreas, an increase in energy losses in chronic inflammation in the bronchopulmonary system, manifested by increased stress on the respiratory system. The presented literature review highlights the modern principles of prevention and correction of malnutrition in children with cystic fibrosis, identifies the most promising methods for further development that correct nutritional status disorders. The review has shown that an active approach to nutrition at any age, the use of aggressive methods of nutritional support against the background of enzyme replacement therapy, timely and adequate therapy of respiratory tract pathology lead to an improvement in the indicators of nutritional status in cystic fibrosis. The most promising is the further development of targeted therapy, which allows, as a result of exposure to the etiopathogenetic mechanisms of the disease, to reduce the frequency and severity of bronchopulmonary exacerbations, partially restore the exocrine function of the pancreas, which is manifested in patients with cystic fibrosis by an increase in body weight and mass-growth index.

APA, Harvard, Vancouver, ISO, and other styles

44

Aksenova, M. S., E. N. Bocharova, S. G. Abbasova, A. S. Ponomarev, V. V. Loginova, M. V. Bolotnikova, N. V. Belskaya, et al. "Comparability of the Reference Drug Pulmozim and a Similar Drug Tigerase in Terms of their Pharmacodynamic, Toxicological and Pharmacokinetic Properties." Journal Biomed 19, no. 1 (April 7, 2023): 47–60. http://dx.doi.org/10.33647/2074-5982-19-1-47-60.

Full text

Abstract:

This work presents research studies into the comparability of Tigerase® (inhalation solution, manufactured by JSC «GENERIUM», Russia) to the reference drug Pulmozim® (inhalation solution, manufactured by Hoffmann-La Roche Ltd., Switzerland). Both drugs contain human recombinant deoxyribonuclease I – dornase alpha as an active substance and are intended for the treatment of cystic fibrosis with pulmonary manifestations (cystic fibrosis). The specific enzymatic activity of dornase alpha was studied in vitro and ex vivo using samples of patients’ purulent sputum. The pharmacokinetic parameters of the drugs were studied in the blood serum, bronchi and lungs. The main physiological parameters (body weight and temperature, the state of the cardiovascular, respiratory, excretory systems, hematological and biochemical blood parameters, pathomorphological changes in the internal organs, including the state of the cornea of the eyes, mortality rates) were assessed in comparative studies of subchronic toxicity in juvenile and sexually mature rats using 28-day inhalation in doses of 0.2 mg/kg to sexually mature and 0.26 mg/kg to juvenile animals (the dose was 6 times higher than the recommended dose for clinical use). It was concluded that the drugs were comparable in terms of their enzymatic, mucolytic (secretolytic) DNase activity, safety profile, and basic pharmacokinetic parameters.

APA, Harvard, Vancouver, ISO, and other styles

45

H. Goumghar and M. Sidqui. "Effects of cystic fibrosis on the oral cavity." Magna Scientia Advanced Research and Reviews 3, no. 2 (December 30, 2021): 061–72. http://dx.doi.org/10.30574/msarr.2021.3.2.0089.

Full text

Abstract:

Cystic fibrosis is a complex, lethal, multi-system autosomal recessive disease resulting from mutations on chromosome 7 that cause dysfunction of an ion channel located on epithelial surfaces. Pulmonary disease is the leading cause of morbidity and mortality in CF. The specific dental manifestations of the disease may result from the condition itself or from complications of treatment. This study presents the dental problems observed in patients with cystic fibrosis and reported in the literature. Particular attention was paid to dental caries, disturbances in the mineralization of hard dental tissues, gingivitis and changes in the content and properties of saliva.

APA, Harvard, Vancouver, ISO, and other styles

46

Simonova, Olga I., Yulia V. Gorinova, and Vera P. Chernevich. "CYSTIC FIBROSIS: A BREAKTHROUGH IN 21ST-CENTURY THERAPY." Russian Pediatric Journal 23, no. 1 (March 27, 2020): 35–41. http://dx.doi.org/10.18821/1560-9561-2020-23-1-35-41.

Full text

Abstract:

The review presents new data on the latest advances in the treatment of cystic fibrosis, a rare genetic disease. The methods used were literature search in the Scopus, Web of Science, and EMBASE databases. The importance of a number of drugs prescribed for anti-inflammatory purposes (ibuprofen, azithromycin) is discussed, data from multicenter studies of new drugs are presented. The role of mucolytic agents and the need to develop new antibacterial compounds are shown. Particular attention in the review is given to the development of new targeted therapies for cystic fibrosis. The data of studies of ivacaftor, lumacactor, tezacactor in this category of patients are presented, as well as a spectrum of mutations of the cystic fibrosis gene in which these molecules can be prescribed. The experience of the use of correctors in adult patients with cystic fibrosis in the Russian Federation is described, the effectiveness and safety of the long-term use of these drugs are described.

APA, Harvard, Vancouver, ISO, and other styles

47

Baer, S., and J. Davis. "Cystic hygroma presenting in adulthood." Journal of Laryngology & Otology 103, no. 10 (October 1989): 976–77. http://dx.doi.org/10.1017/s0022215100110679.

Full text

Abstract:

AbstractWe report a typical case of cystic hygroma presenting in an elderly female patient. A review of the literature on this condition reveals that although rare in adults, it should form part of the differential diagnosis of a cystic swelling in the neck in this group of patients as well as in the more commonly observed juvenile population. The embryology and pathogenesis of lymphangiomata are discussed as well as the recommended management of a cystic hygroma in an older patient.

APA, Harvard, Vancouver, ISO, and other styles

48

Pizzignacco, Tainá Maués Pelúcio, and Regina Aparecida Garcia de Lima. "Socialization of children and adolescents with cystic fibrosis: support for nursing care." Revista Latino-Americana de Enfermagem 14, no. 4 (August 2006): 569–77. http://dx.doi.org/10.1590/s0104-11692006000400015.

Full text

Abstract:

The aim of this paper is to identify the routine (school, work, free time activities and relationships with friends and family) of children and adolescents with Cystic Fibrosis (CF) through their experiences and identify situations that can affect these routines. The objects of analysis of this research are children and adolescents with CF who attend a teaching hospital in a city of the State of São Paulo - Brazil. It is a qualitative research, with data collection based on open interviews and patient charts. The data brought the following themes: misleading knowledge about the disease, concern with self-image, search for self-care and hope of improvement in the future. The results evidence the repercussion of CF in those patients' socialization process, evidencing the importance of health professionals knowing about these demands and incorporating them into the care plan, with a view to effective interventions to promote infant-juvenile growth and development.

APA, Harvard, Vancouver, ISO, and other styles

49

Kleštincová, Tereza, Eva Klásková, Lenka Bakaj Zbrožková, and Vladimír Mihál. "Influenza in a cystic fibrosis infant: case report with literature review." Pediatrie pro praxi 19, no. 6 (December 15, 2018): 349–53. http://dx.doi.org/10.36290/ped.2018.070.

Full text

APA, Harvard, Vancouver, ISO, and other styles

50

Feuillet-Fieux, M. N., G. Lenoir, I. Sermet, C. Elie, J. Djadi-Prat, M. Ferrec, M. Magen, et al. "Nasal polyposis and cystic fibrosis: review of literature and case report." Journal of Cystic Fibrosis 9 (June 2010): S4. http://dx.doi.org/10.1016/s1569-1993(10)60017-3.

Full text

APA, Harvard, Vancouver, ISO, and other styles

Journal articles on the topic 'Cystic fibrosis, juvenile literature'

Create a spot-on reference in APA, MLA, Chicago, Harvard, and other styles