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CLINICAL AND GENETIC RISK FACTORS FOR CYSTIC FIBROSIS-RELATED LIVER DISEASE IN EGYPTIAN CF CHILDREN: A SINGLE-CENTER EXPERIENCE
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  • Samya Nasr,
  • Amel El-Faramawy,
  • Mohamed Al Emam MS,
  • Heba Moustafa Hamza
Samya Nasr
University of Michigan Department of Pediatrics

Corresponding Author:[email protected]

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Amel El-Faramawy
Ain Shams University Faculty of Medicine
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Mohamed Al Emam MS
Ain Shams University Faculty of Medicine
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Heba Moustafa Hamza
Ain Shams University Faculty of Medicine
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Abstract

Background: Cystic fibrosis (CF) is an autosomal recessive disease affecting multiple organ systems, including the liver, leading to cystic fibrosis-related liver disease (CFLD). It was noted that CFLD in Egyptian children with CF is more common than in non-Egyptian people with CF (pwCF). The present 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 and the comparison of CFLD in Egyptian and non-Egyptian CF patients will be done. Methods: The current cross-sectional study included 50 CF cases 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. All patients’ caregivers provided informed consent. Additionally, all subjects underwent detailed history taking, laboratory investigations, clinical assessment, and pelviabdominal ultrasound for evaluation of hepatic involvement. Results: Male sex, severe genetic mutation (class I and II), long duration, and early onset of the disease were independent risk factors for CFLD development. In addition, pancreatic insufficiency, as well as meconium ileus history, were predictors of CFLD. Diabetes mellitus and severe lung disease were proven to significantly elevate the risk of CFLD development. Conclusion: CFLD is not uncommon in Egyptian CF patients as one-third of the patients were found to have liver affection. CF patients with multiple risk factors are at increased risk of developing liver disease.
21 Nov 2022Submitted to Pediatric Pulmonology
22 Nov 2022Assigned to Editor
22 Nov 2022Submission Checks Completed
22 Nov 2022Review(s) Completed, Editorial Evaluation Pending
27 Nov 2022Reviewer(s) Assigned
07 Jan 2023Editorial Decision: Revise Major
25 Feb 20231st Revision Received
29 Mar 2023Assigned to Editor
29 Mar 2023Review(s) Completed, Editorial Evaluation Pending
29 Mar 2023Submission Checks Completed
29 Mar 2023Reviewer(s) Assigned
10 May 2023Editorial Decision: Revise Minor
14 Jun 20232nd Revision Received
15 Jun 2023Submission Checks Completed
15 Jun 2023Assigned to Editor
15 Jun 2023Review(s) Completed, Editorial Evaluation Pending
15 Jun 2023Reviewer(s) Assigned
26 Jul 2023Editorial Decision: Revise Minor
30 Jul 20233rd Revision Received
31 Jul 2023Assigned to Editor
31 Jul 2023Review(s) Completed, Editorial Evaluation Pending
31 Jul 2023Submission Checks Completed
31 Jul 2023Reviewer(s) Assigned
23 Oct 2023Editorial Decision: Revise Minor
27 Oct 20234th Revision Received
27 Oct 2023Review(s) Completed, Editorial Evaluation Pending
27 Oct 2023Submission Checks Completed
27 Oct 2023Assigned to Editor
27 Oct 2023Reviewer(s) Assigned
31 Oct 2023Editorial Decision: Revise Minor
01 Nov 20235th Revision Received
03 Nov 2023Assigned to Editor
03 Nov 2023Submission Checks Completed
03 Nov 2023Review(s) Completed, Editorial Evaluation Pending
06 Nov 2023Editorial Decision: Revise Minor
10 Nov 20236th Revision Received
13 Nov 2023Review(s) Completed, Editorial Evaluation Pending
13 Nov 2023Submission Checks Completed
13 Nov 2023Assigned to Editor
13 Nov 2023Editorial Decision: Revise Minor
16 Nov 20237th Revision Received
16 Nov 2023Submission Checks Completed
16 Nov 2023Assigned to Editor
16 Nov 2023Review(s) Completed, Editorial Evaluation Pending
17 Nov 2023Editorial Decision: Accept