ArticleJournal of clinical medicine2019
Hepatobiliary Complications in Children with Sickle Cell Disease: A Retrospective Review of Medical Records from 616 Patients.
Article in Journal of clinical medicine, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers.
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Who cites it
25 citing papers in PubMed, 60 citations in OpenAlex.
- Pharmacokinetics of Voxelotor in Patients With Renal and Hepatic Impairment.Journal of clinical pharmacology · 2021Trial
- Multimodal imaging features to diagnose abdominal complications of sickle cell disease.Abdominal radiology (New York) · 2026Review
- Hepatic Enzyme Abnormalities and Their Association With Hematological Parameters in Sickle Cell Disease: A Case-Control Study in Cameroon.Health science reports · 2026Article
- A Rare Case of Giant Cell Hepatitis Superimposed on Sickle Cell Hepatopathy.ACG case reports journal · 2026Article
- Multimodal imaging features to diagnose abdominal complications of sickle cell disease.Abdominal radiology (New York) · 2026Review
- Article
- Sickle Cell Hepatopathy With Acute Hepatic Sequestration and Extreme Hyperbilirubinemia.Cureus · 2025Article
- Hepatobiliary complications in patients with sickle cell disease: A 30-year review of 1009 patients.Hepatology communications · 2025Article
- Article
- Epistaxis: A Rare Presentation of Sickle Cell Intrahepatic Cholestasis.Case reports in hematology · 2025Article
- Jaundice in a Child with Sickle Cell Anemia: A Case Based Approach.Indian journal of pediatrics · 2024Article
- Long-Term L-Glutamine Treatment Reduces Hemolysis without Ameliorating Hepatic Vaso-Occlusion and Liver Fibrosis in a Mouse Model of Sickle Cell Disease.Biomedicines · 2023Article
- Liver Disease and Sickle Cell Disease: Auto-Immune Hepatitis more than a Coincidence; A Systematic Review of the Literature.Mediterranean journal of hematology and infectious diseases · 2023Review
- Genotypic and Phenotypic Composition of Sickle Cell Disease in the Arab Population - A Systematic Review.Pharmacogenomics and personalized medicine · 2023Review
- Article
- Article
- Ultrasound Imaging in Subjects with Sickle Cell Disease: The Saudi Arabia Experiences.International journal of general medicine · 2023Article
- Clinical Vignettes Part I.Hematology/oncology clinics of North America · 2022Review
- Hyperbilirubinemia Following Retroperitoneal Mass Resection in a Patient with Sickle Cell Anemia.Urology · 2022Article
- Acute Cholelithiasis With Acute Pancreatic Calcifications: A Unique Presentation of Sickle Cell Crisis.Cureus · 2022Article
Corrections and comments
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Authors and funding
9 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Hepatobiliary complications in children with sickle cell disease (SCD) are rarely reported but can be life-threatening. We retrospectively assessed their prevalence in a cohort of 616 children followed in a French university-hospital SCD reference center. Eligibility criteria were the following: age <18 years, seen at least twice with an interval of more than 6 months from January 2008 to December 2017, with all genotypes of SCD. Patients with hepatobiliary complications were identified via the local data warehouse and medical files were thoroughly reviewed. At least one hepatobiliary complication was reported in 37% of the children. The most frequent was cholelithiasis, in 25% of cases, which led to systematic screening and elective cholecystectomy in the case of gallstones. Overall, 6% of the children experienced acute sickle cell hepatic crisis, sickle cell intra-hepatic cholestasis, or acute hepatic sequestration, with severity ranging from mild liver pain and increased jaundice to multiple organ failure and death. Emergency treatment was exchange transfusion, which led to normalization of liver tests in most cases. Five children had chronic cholangiopathy, associated with auto-immune hepatitis in two cases. One needed liver transplantation, having a good outcome but with many complications. Transfusion iron load and infectious hepatitis cases were mild. Hepatotoxicity of an iron chelator was suspected to contribute to abnormal liver test results in five patients. We propose recommendations to prevent, explore, and treat hepatobiliary complications in SCD children. We underline the need for emergency exchange transfusion when acute liver failure develops and warn against liver biopsy and transplantation in this condition.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.