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Journal articles on the topic 'Cystic fibrosis Patients Biography'

Author: Grafiati

Published: 4 June 2021

Last updated: 25 January 2023

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1

Radlovic, Nedeljko. "Cystic fibrosis." Srpski arhiv za celokupno lekarstvo 140, no. 3-4 (2012): 244–49. http://dx.doi.org/10.2298/sarh1204244r.

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Cystic fibrosis (CF) is a multisystemic autosomal recessive disease caused by a defect in the expression of CFTR protein, i.e. chloride channel present in the apical membrane of respiratory, digestive, reproductive and sweat glands epithelium. It primarily occurs in the Caucasians, while being considerably or exceptionally rare in persons of other races. Absence, deficit or structural and functional abnormalities of CFTR protein lead to mucosal hyperconcentration in the respiratory, digestive and reproductive systems and malabsorption of chloride and sodium in the sweat glands. Thus, the clinical features of patients? with CF are predominated by respiratory, digestive and reproductive disorders, as well as the tendency to dehydration in the condition of increased sweating. Beside genotype variations, the degree of disease manifestation is also essentially influenced by various exogenous factors, such as the frequency and severity of respiratory infections, the level of aero-pollution, quality of immunoprophylaxis, patients? nutritional condition and other. Chloride concentration of over 60 mmol/L in sweat, a high level of immunoreactive chymotrypsinogen in blood and the verification of homozygous mutation of CFTR gene are the basic methods in the diagnostics of the disease. CF belongs to the group of severe and complex chronic diseases, and therefore requires multidisciplinary therapeutic approach. Owing to the improvement of healthcare provision, most patients with CF now survive into adulthood. In addition, their quality of life is also considerably improved.

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2

Hadj Fredj, Sondess, Slaheddine Fattoum, Abdelraouf Chabchoub, and Taieb Messaoud. "First report of cystic fibrosis mutations in Libyan cystic fibrosis patients." Annals of Human Biology 38, no. 5 (February 18, 2011): 561–63. http://dx.doi.org/10.3109/03014460.2011.557090.

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3

Onay, Tuncer, Julian Zielenski, Ozlem Topaloglu, Nalan Gokgoz, Hulya Kayserili, Memnune Yuksel Apak, Yildiz Camcioglu, et al. "Cystic Fibrosis Mutation and Associated Haplotypes in Turkish Cystic Fibrosis Patients." Human Biology 73, no. 2 (2001): 191–203. http://dx.doi.org/10.1353/hub.2001.0022.

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4

Standen, Jen. "Cystic fibrosis." InnovAiT: Education and inspiration for general practice 13, no. 1 (November 27, 2019): 39–46. http://dx.doi.org/10.1177/1755738019883322.

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In the UK over 10 000 people live with cystic fibrosis (CF), with 1-in-25 people being carriers of the disease. Multidisciplinary care is provided by tertiary care CF centres, with or without local secondary service shared care agreements. There are still, however, several reasons why CF sufferers or their families present to their GPs. This article aims to provide a brief overview of CF and its management. It also gives the information needed to guide patients about genetic testing and neonatal screening for the disease.

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5

Westwood, A. T. R. "Splenectomy in cystic fibrosis patients." Archives of Disease in Childhood 89, no. 11 (November 1, 2004): 1078. http://dx.doi.org/10.1136/adc.2004.051508.

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6

Pozler, O., H. Vanicek, D. Neumann, and J. Bures. "ELECTROGASTROGRAPHY IN CYSTIC FIBROSIS PATIENTS." Journal of Pediatric Gastroenterology and Nutrition 32 (April 2001): S54. http://dx.doi.org/10.1097/00005176-200104001-00043.

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7

Grody, Wayne W. "Cystic Fibrosis." Archives of Pathology & Laboratory Medicine 123, no. 11 (November 1, 1999): 1041–46. http://dx.doi.org/10.5858/1999-123-1041-cf.

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Abstract Objective.—To review the current status of scientific knowledge and opinion regarding molecular genetic testing of mutations in the CFTR gene for purposes of diagnosis and population carrier screening of cystic fibrosis (CF). Data Sources.—Published research findings on the nature of the CFTR gene, pilot population screening studies in the United States and Europe, and ongoing deliberations of professional and governmental agencies considering implementation of widespread testing. Study Selection.—Findings relevant to the molecular heterogeneity of CFTR mutations and its implications for population carrier screening were considered. Data Extraction.—Information was extracted from studies published by us and others, as made available to recent consensus panels and professional committees. Data Synthesis.—These data were reevaluated in light of recent movements in professional and public policy regarding acceptability and desirability of widespread CF mutation testing. Effects to date of such testing on patient outcomes is reported. Conclusions.—The ability to test for CFTR mutations at the molecular level has already improved the diagnosis of symptomatic patients and expanded the reproductive options of family members of CF patients. The same technology also holds promise of identifying asymptomatic carriers and at-risk couples without family history in the general population so that they too might be offered prenatal diagnosis or other options. However, a number of key questions remain to be worked out before a widespread national screening program can be put into practice. These include the target population to be offered testing (the entire population vs high-risk ethnic groups), the size and nature of the mutation test panel (universal vs ethnic specific), the inclusion or exclusion of CFTR variants that do not cause classical CF, the optimal testing technology, appropriate standards for laboratory quality assurance, and the development of sufficient educational materials and genetic counseling resources for test delivery, reporting, and interpretation. The answers to these questions will be relevant not only to CF testing but also to many other large-scale molecular genetic screening programs being considered in the future.

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8

Balanetchi, L., R. Selevestru, A. Cotoman, D. Rotaru-Cojocari, and S. Sciuca. "P099 Evaluation by plethismography of cystic fibrosis and non-cystic fibrosis patients." Journal of Cystic Fibrosis 20 (2021): S69. http://dx.doi.org/10.1016/s1569-1993(21)01125-5.

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9

Bar-Yoseph, Ronen, Anat Ilivitzki, Dan M. Cooper, Michal Gur, Gur Mainzer, Fahed Hakim, Galit Livnat, et al. "Exercise capacity in patients with cystic fibrosis vs. non-cystic fibrosis bronchiectasis." PLOS ONE 14, no. 6 (June 13, 2019): e0217491. http://dx.doi.org/10.1371/journal.pone.0217491.

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10

Bingol, A., M. G. Ertosun, R. Artan, A. Yilmaz, E. Mihci, B. N. Guzel, M. Erman Akar, et al. "35 Cystic fibrosis transmembrane regulator mutations in Turkish patients with cystic fibrosis." Journal of Cystic Fibrosis 13 (June 2014): S55. http://dx.doi.org/10.1016/s1569-1993(14)60172-7.

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11

Nikolova, M., B. Gospodinova, M. Baycheva, K. Ketev, N. Guenkova, V. Nedkova, I. Hristov, M. Georgieva, P. Perenovska, and G. Petrova. "P241 Cystic fibrosis-associated liver disease prevalence in Bulgarian cystic fibrosis patients." Journal of Cystic Fibrosis 19 (June 2020): S124. http://dx.doi.org/10.1016/s1569-1993(20)30574-9.

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12

Kannekens, A. L., J. M. van den Berg, H. Pijl, C. K. Van der Ent, and H. G. Heijerman. "205 Microalbuminuria in patients with cystic fibrosis: diabetic or cystic fibrosis-related?" Journal of Cystic Fibrosis 6 (June 2007): S50. http://dx.doi.org/10.1016/s1569-1993(07)60188-x.

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13

Furgeri, Daniela Tenório, Fernando Augusto Lima Marson, Cyntia Arivabeni Araújo Correia, José Dirceu Ribeiro, and Carmen Sílvia Bertuzzo. "Cystic fibrosis transmembrane regulator haplotypes in households of patients with cystic fibrosis." Gene 641 (January 2018): 137–43. http://dx.doi.org/10.1016/j.gene.2017.10.052.

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14

Thomas, Caroline S., and Rebekah F. Brown. "Sleep screening for cystic fibrosis patients: A survey of cystic fibrosis programs." Pediatric Pulmonology 55, no. 12 (September 11, 2020): 3358–63. http://dx.doi.org/10.1002/ppul.25051.

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15

Kusuma Yana, I. Gusti Agung Ari. "Cystic Fibrosis: Review." Jurnal Sains dan Kesehatan 3, no. 1 (February 28, 2021): 79–87. http://dx.doi.org/10.25026/jsk.v3i1.231.

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Cystic fibrosis (CF) is a reversed autosomal genetic disease that originates from some white or caucasian races. This condition is caused by mutations in the CFTR gene, especially the CFTR Phe508del. If CFTR damage or error occurs then normal function will be disrupted and have a clinical impact on other organs or multiorgan. Complications such as the pancreas, liver, digestive tract, bone, genital, and respiratory tract that can cause most deaths in patients with cystic fibrosis. Malabsorption, inflammation, infection and lung obstruction are signs of cystic fibrosis. Therapy for cystic fibrosis is limited to the improvement of the airway mokus, recovery of infections maintained with antibiotics, improve physical health and nutrition of patients

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16

Ozcelik, Nazire, Richard Shell, Melissa Holtzlander, and Clifford Cua. "Decreased Right Ventricular Function in Healthy Pediatric Cystic Fibrosis Patients Versus Non-Cystic Fibrosis Patients." Pediatric Cardiology 34, no. 1 (June 10, 2012): 159–64. http://dx.doi.org/10.1007/s00246-012-0407-4.

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17

Coltrera, Marc D., Susan M. Mathison, Tracy A. Goodpaster, and Allen M. Gown. "Abnormal Expression of the Cystic Fibrosis Transmembrane Regulator in Chronic Sinusitis in Cystic Fibrosis and Non-Cystic Fibrosis Patients." Annals of Otology, Rhinology & Laryngology 108, no. 6 (June 1999): 576–81. http://dx.doi.org/10.1177/000348949910800609.

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18

Ren, Clement L., Rebecca L. Morgan, Christopher Oermann, Helaine E. Resnick, Cynthia Brady, Annette Campbell, Richard DeNagel, et al. "Cystic Fibrosis Foundation Pulmonary Guidelines. Use of Cystic Fibrosis Transmembrane Conductance Regulator Modulator Therapy in Patients with Cystic Fibrosis." Annals of the American Thoracic Society 15, no. 3 (March 2018): 271–80. http://dx.doi.org/10.1513/annalsats.201707-539ot.

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19

Kasmi, I., E. Vevecka, G. Kasmi, and B. Saraci. "P166 Prevalence of cystic fibrosis-associated liver disease in Albanian cystic fibrosis patients." Journal of Cystic Fibrosis 20 (2021): S89—S90. http://dx.doi.org/10.1016/s1569-1993(21)01192-9.

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20

Faro, Albert, Christopher Goss, Elizabeth Cromwell, Alex Elbert, Anne W. Brown, and Bruce C. Marshall. "When is cystic fibrosis not cystic fibrosis? The importance of appropriately classifying patients." Respiratory Medicine 193 (March 2022): 106727. http://dx.doi.org/10.1016/j.rmed.2021.106727.

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21

Alyasin, Soheila, Mozhgan Moghtaderi, Shirin Farjadian, Maryam Babaei, and Saeed Hosseini Teshnizi. "Allergic bronchopulmonary aspergillosis in patients with cystic fibrosis and non-cystic fibrosis bronchiectasis." Electronic Physician 10, no. 1 (January 25, 2018): 6273–78. http://dx.doi.org/10.19082/6273.

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22

Olveira Fuster, G., A. Dorado Galindo, C. Olveira Fuster, F. Espildora Hernandez, N. Porras Perez, E. García Fuentes, I. Gaspar, R. Jimeno, J. L. De la Cruz, and M. Murri. "146 Oxidative stress biomarkers in cystic fibrosis and non-cystic fibrosis bronchiectasis patients." Journal of Cystic Fibrosis 11 (June 2012): S94. http://dx.doi.org/10.1016/s1569-1993(12)60316-6.

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23

Fluge, G., H. V. Olesen, M. Gilljam, P. Meyer, T. Pressler, O. T. Storrösten, F. Karpati, and L. Hjelte. "Co-morbidity of cystic fibrosis and celiac disease in Scandinavian cystic fibrosis patients." Journal of Cystic Fibrosis 8, no. 3 (May 2009): 198–202. http://dx.doi.org/10.1016/j.jcf.2009.02.002.

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24

Lamb, Harriet M., and Karen L. Goa. "Management of Patients with Cystic Fibrosis." Disease Management and Health Outcomes 6, no. 2 (1999): 93–108. http://dx.doi.org/10.2165/00115677-199906020-00005.

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25

Kondratyeva, E. I., S. A. Krasovsky, N. Yu Kashirskaya, E. L. Amelina, E. K. Zhekaite, V. D. Sherman, O. I. Simonova, et al. "COVID-19 in cystic fibrosis patients." PULMONOLOGIYA 30, no. 5 (October 26, 2020): 544–52. http://dx.doi.org/10.18093/0869-0189-2020-30-5-544-552.

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Since the beginning of the COVID-19 epidemic, the European cystic fibrosis society (ECFS) has decided to launch a special ECFS-COVID-19 program to collect information on the of COVID-19 characteristics in the patients with cystic fibrosis (CF). The results of the program should help timely and efficiently provide the patients with CF with the necessary care. Initially, it was assumed that COVID-19 would be severe in CF patients. The aim. Тo assess the prevalence and characteristics of COVID-19 in patients with cystic fibrosis (CF) in the Russian Federation (RF). Methods. 6 cases (4 children and 2 adults) of COVID-19 in Russian CF patients were analyzed. Results. There are 405,843 infected with SARS-CoV-2 in Russia, the incidence of coronavirus infection in Russia was 1.4 cases per 1 thousand people. According to the Ministry of Health of the RF, as of December 2019, there were 3,931 patients with CF (2,823 children and 1,108 adults). The incidence of COVID-19 was 1.5 per 1000 patients with CF (1.4 : 1,000 for children and 1.8 : 1,000 for adults). The incidence was not higher than in the General population. The diagnosis of COVID-19 was confirmed in 4 boys and 2 women, 3 of the patients were infected with Pseudomonas aeruginosa and 2 – with Achromobacter spp. Mild disease was seen in 5 patients including all the children. Pneumonia was registered in 3 patients. One child with COVID-19 had abdominal syndrome. 2 patients – 1 adult and 1 child – needed in-patient care. Additional antibiotics were given to 4 patients, 2 of them received i/v antibiotics. One adult patient was on the lung transplantation waiting list. This woman had long-term oxygen therapy and BiPAP noninvasive respiratory support before the infection with SARS-CoV-2, FEV1 was 24 %pred. Conclusion. Despite the fact that patients with CF are at risk of severe COVID-19, to date, in the described cases, COVID-19 infection has not led to a significant deterioration of the symptoms of CF. Not a single fatal outcome in Russian patients with CF has been recorded.

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26

Symoens, Françoise, Gerhard Haase, Marc Pihet, Jacqueline Carrere, Hugues Beguin, Nicolas Degand, Laurent Mely, and Jean-Philippe Bouchara. "UnusualAspergillusspecies in patients with cystic fibrosis." Medical Mycology 48, O1 (November 2010): S10—S16. http://dx.doi.org/10.3109/13693786.2010.501345.

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27

Ziegler, B., A. K. Fernandes, P. R. S. Sanches, D. P. Silva Junior, P. R. O. Thomé, and P. T. R. Dalcin. "Dyspnea perception in cystic fibrosis patients." Brazilian Journal of Medical and Biological Research 46, no. 10 (October 2013): 897–903. http://dx.doi.org/10.1590/1414-431x20133139.

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28

Kalnins, Daina, Peter R. Durie, and Paul Pencharz. "Nutritional management of cystic fibrosis patients." Current Opinion in Clinical Nutrition and Metabolic Care 10, no. 3 (May 2007): 348–54. http://dx.doi.org/10.1097/mco.0b013e3280a94f80.

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29

Wainwright, Claire E. "Ivacaftor for patients with cystic fibrosis." Expert Review of Respiratory Medicine 8, no. 5 (August 22, 2014): 533–38. http://dx.doi.org/10.1586/17476348.2014.951333.

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30

HILMAN, BETTINA C., MOIRA L. AITKEN, and MICHAEL CONSTANTINESCU. "Pregnancy in Patients With Cystic Fibrosis." Clinical Obstetrics and Gynecology 39, no. 1 (March 1996): 70–86. http://dx.doi.org/10.1097/00003081-199603000-00008.

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31

Egan, J. J., K. Webb, and A. A. Woodcock. "Lung donors for cystic fibrosis patients." Thorax 51, no. 8 (August 1, 1996): 873. http://dx.doi.org/10.1136/thx.51.8.873.

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32

Della Rocca, Giorgio. "Anaesthesia in patients with cystic fibrosis." Current Opinion in Anaesthesiology 15, no. 1 (February 2002): 95–101. http://dx.doi.org/10.1097/00001503-200202000-00014.

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33

Wine, J., and C. Solc. "Chloride channels in cystic fibrosis patients." Science 247, no. 4939 (January 12, 1990): 222. http://dx.doi.org/10.1126/science.2152986.

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34

Van Aalderen, W. M. C., G. P. M. Mannes, E. S. Bosma, R. J. Roorda, and H. S. A. Heymans. "Home care in cystic fibrosis patients." European Respiratory Journal 8, no. 1 (January 1, 1995): 172–75. http://dx.doi.org/10.1183/09031936.95.08010172.

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35

Hebestreit, Helge, Susi Kriemler, and Thomas Radtke. "Exercise for all cystic fibrosis patients." Current Opinion in Pulmonary Medicine 21, no. 6 (November 2015): 591–95. http://dx.doi.org/10.1097/mcp.0000000000000214.

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36

Strandvik, Birgitta. "Care of Patients with Cystic Fibrosis." Annales Nestlé (English ed.) 64, no. 3 (2006): 131–40. http://dx.doi.org/10.1159/000095377.

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37

Billard, Lisa, Rozenn Le Berre, Léa Pilorgé, Christopher Payan, Geneviève Héry-Arnaud, and Sophie Vallet. "Viruses in cystic fibrosis patients’ airways." Critical Reviews in Microbiology 43, no. 6 (March 24, 2017): 690–708. http://dx.doi.org/10.1080/1040841x.2017.1297763.

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38

Lamoureux, Claudie, Charles-Antoine Guilloux, Clémence Beauruelle, Anne Jolivet-Gougeon, and Geneviève Héry-Arnaud. "Anaerobes in cystic fibrosis patients’ airways." Critical Reviews in Microbiology 45, no. 1 (January 2, 2019): 103–17. http://dx.doi.org/10.1080/1040841x.2018.1549019.

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39

Lind-Ayres, M., W. Thomas, B. Holme, M. Mauer, M. L. Caramori, and A. Moran. "Microalbuminuria in Patients With Cystic Fibrosis." Diabetes Care 34, no. 7 (May 11, 2011): 1526–28. http://dx.doi.org/10.2337/dc10-2231.

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40

Wilson, D. C., P. R. Durie, L. Ellis, M. Corey, M. R. Knowles, L. C. Tsui, E. Tullis, W. Ip, H. Levison, and J. Zielenski. "PATIENTS PREVIOUSLY DIAGNOSED WITH CYSTIC FIBROSIS." Journal of Pediatric Gastroenterology and Nutrition 21, no. 3 (October 1995): 359. http://dx.doi.org/10.1097/00005176-199510000-00151.

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41

Winklhofer-Roob, B. M. "Antioxidant status in cystic fibrosis patients." American Journal of Clinical Nutrition 63, no. 1 (January 1, 1996): 138–39. http://dx.doi.org/10.1093/ajcn/63.1.138a.

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42

Murphy, Jane, Kirsi Laiho, and Steve Wootton. "Fat malabsorption in cystic fibrosis patients." American Journal of Clinical Nutrition 70, no. 5 (November 1, 1999): 943–44. http://dx.doi.org/10.1093/ajcn/70.5.943a.

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43

ESPINOZA, M. "32. Cystic fibrosis in adult patients." Netherlands Journal of Medicine 54 (June 1999): S31. http://dx.doi.org/10.1016/s0300-2977(99)90084-0.

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44

VANIEK, H. "162. Electrogastrography in cystic fibrosis patients." Netherlands Journal of Medicine 54 (June 1999): S65. http://dx.doi.org/10.1016/s0300-2977(99)90214-0.

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45

Sheikh, Shahid I., Karen McCoy, Nancy A. Ryan-Wenger, Alpa Patel, and Stephen Kirkby. "Lobectomy in Patients with Cystic Fibrosis." Canadian Respiratory Journal 21, no. 4 (2014): e63-e66. http://dx.doi.org/10.1155/2014/709671.

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BACKGROUND: Some patients with cystic fibrosis (CF) develop severe but localized lung disease or recurrent hemoptysis/pneumothorax refractory to conventional medical therapies.METHODS: The outcomes of lung resection in patients with CF and worsening localized lung disease or recurrent hemoptysis/pneumothorax refractory to conventional therapy (n=15) were evaluated by reviewing the medical records of all patients with CF followed at the CF Center at Nationwide Children’s Hospital (Columbus, Ohio, USA), who underwent lobectomy over a 15-year period (1998 to 2012).RESULTS: The median age of the 15 patients (93% Caucasian) was 20 years (range two to 41 years) and their mean forced expiratory volume in 1 s (FEV1) was 59.5% of predicted one year before surgery. Three patients died within two years after lobectomy; all three deaths occurred in patients with an FEV1≤40% of predicted before surgery. There were no significant changes in mean height, weight, body mass index, hospital admissions or antibiotic use over time. The mean FEV1decreased over time. Compared with at surgery, decline in FEV1in the year before surgery was −5.4% (P=0.024) and decline in the year after surgery was −1.3% (P=0.513); however, the difference in the rate of decline was not statistically significant.CONCLUSION: In patients with CF and localized worsening bronchiectasis and/or recurrent hemoptysis/pneumothorax, lobectomy carried a significant risk of mortality, especially in patients with FEV1≤40% of predicted, and should only be considered when all other measures fail.

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46

Horrevorts, A. M., J. de Witte, J. E. Degener, G. Dzoljic-Danilovic, W. C. J. Hop, O. Driessen, M. F. Michel, and K. F. Kerrebijn. "Tobramycin in Patients with Cystic Fibrosis." Chest 92, no. 5 (November 1987): 844–48. http://dx.doi.org/10.1378/chest.92.5.844.

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47

Lanng, S. "Glucose intolerance in cystic fibrosis patients." Paediatric Respiratory Reviews 2, no. 3 (September 2001): 253–59. http://dx.doi.org/10.1053/prrv.2001.0148.

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48

Oermann, Christopher M. "Fertility in Patients With Cystic Fibrosis." Chest 118, no. 4 (October 2000): 893–94. http://dx.doi.org/10.1378/chest.118.4.893.

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49

Brown-Guttovz, Heather. "Helping patients cope with cystic fibrosis." Nursing Made Incredibly Easy! 6, no. 6 (November 2008): 48–54. http://dx.doi.org/10.1097/01.nme.0000341177.36358.a0.

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50

Sojo, A., C. Bousoño, M. D. Garcia-Novo, S. Heredia, J. Manzanares, F. Baranda, and C. Vazquez. "194 Pancreatitis in cystic fibrosis patients." Journal of Cystic Fibrosis 6 (June 2007): S47. http://dx.doi.org/10.1016/s1569-1993(07)60177-5.

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