{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,11]],"date-time":"2026-03-11T00:33:52Z","timestamp":1773189232527,"version":"3.50.1"},"reference-count":42,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2023,12,1]],"date-time":"2023-12-01T00:00:00Z","timestamp":1701388800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2024,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Cystic fibrosis (CF) is an autosomal recessive disease. It affects multiple organ systems, including the liver, leading to CF\u2010related liver disease (CFLD). It was noted that CFLD in Egyptian children with CF is more common than in non\u2010Egyptian people with CF (pwCF). This study aimed to determine the incidence of CFLD and the potential risk factors for developing CFLD in Egyptian children. The correlation between CFLD and the various genotypes prevalent in Egyptian CF children will be discussed. In addition, comparison of CFLD in Egyptian and non\u2010Egyptian CF patients will be presented.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This cross\u2010sectional study included 50 pwCF from Ain Sham University's Pediatric Pulmonology Clinic in Children's Hospital, Cairo, Egypt. The sweat chloride test and genetic studies were done at the time of diagnosis. Additionally, all subjects underwent detailed history taking, laboratory investigations, clinical assessment, and pelvic abdominal ultrasound for evaluation of hepatic involvement.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>One\u2010third of the Egyptian children with CF were found to have liver disease. The following independent risk factors for developing CFLD were identified as: male sex, severe genetic mutation (class I and II), long duration of CF disease, early onset of the CF, pancreatic insufficiency, as well as history of meconium ileus. In addition, diabetes mellitus and severe lung disease were proven to significantly increase the risk of developing CFLD.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>CFLD is common in Egyptian pwCF. CFLD's risk factors are similar to other\u00a0reported research from other countries in the region.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.26775","type":"journal-article","created":{"date-parts":[[2023,12,1]],"date-time":"2023-12-01T13:16:09Z","timestamp":1701436569000},"page":"465-471","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Clinical and genetic risk factors for cystic fibrosis\u2010related liver disease in Egyptian CF children: A single\u2010center experience"],"prefix":"10.1002","volume":"59","author":[{"given":"Amel","family":"El\u2010Faramawy","sequence":"first","affiliation":[{"name":"Pediatrics Department, Hepatology Unit, Children's Hospital, Faculty of Medicine Ain Shams University Cairo Egypt"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4124-316X","authenticated-orcid":false,"given":"Samya Z.","family":"Nasr","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Pediatric Pulmonology University of Michigan, Michigan Medicine Ann Arbor Michigan USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mohamed M. S. Al","family":"Emam","sequence":"additional","affiliation":[{"name":"Pediatrics Department, Hepatology Unit, Children's Hospital, Faculty of Medicine Ain Shams University Cairo Egypt"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7338-2722","authenticated-orcid":false,"given":"Heba M. H.","family":"Fahmy","sequence":"additional","affiliation":[{"name":"Pediatrics Department, Pulmonology Unit, Children's Hospital, Faculty of Medicine Ain Shams University Cairo Egypt"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2023,12]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2006.04.004"},{"key":"e_1_2_11_3_1","first-page":"313","article-title":"Cystic fibrosis in Egyptian children: neonatal screening and high risk groups","volume":"4","author":"Abdel\u2010Salam E","year":"1993","journal-title":"JAC"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.5114\/pg.2014.43574"},{"key":"e_1_2_11_5_1","volume-title":"Cystic Fibrosis And Liver Disease","author":"Betapudi B","year":"2020"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.prrv.2017.03.001"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00120910"},{"key":"e_1_2_11_8_1","unstructured":"Cystic Fibrosis Foundation Patient Registry. 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