Waisbourd-Zinman Orith, Frenklak Rachel, Hakakian Odelia, Hilmara Didja, Lin Henry
Department of Gastroenterology, Hepatology and Nutrition, Schneider Children's Medical Center of Israel, Petach Tikva.
Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
J Pediatr Hematol Oncol. 2021 Oct 1;43(7):254-257. doi: 10.1097/MPH.0000000000001985.
Hepatic and biliary tract diseases are common in sickle cell disease (SCD) patients, likely due to sickling, hemosiderosis, viral hepatitis, or cholelithiasis. Literature is lacking on associations between SCD, autoimmune hepatitis (AIH), and/or sclerosing cholangitis (SC)-together, autoimmune liver disease (AILD). We aimed to better understand the relationship of these diseases in pediatric patients.
A retrospective analysis of patients with SCD and AILD at the Children's Hospital of Philadelphia (January 2008 to August 2015).
Seven patients, ages 8 to 23 years (3 males), were identified. Three had AIH, 2 SC, and 2 AIH/SC overlap, known as autoimmune SC. All patients with AIH treated with azathioprine significantly improved their liver enzymes. One patient with SC and inflammatory bowel disease underwent successful bone marrow transplant. Two SC patients died from SCD complications.
In this cohort, there seems to be an association between SCD and AILD; SC in this population was severe. Physicians should be aware of this and evaluate patients with SCD and elevated liver enzymes for AILD.
肝脏和胆道疾病在镰状细胞病（SCD）患者中很常见，可能是由于镰状化、含铁血黄素沉着症、病毒性肝炎或胆石症所致。关于SCD、自身免疫性肝炎（AIH）和/或硬化性胆管炎（SC）——统称为自身免疫性肝病（AILD）——之间的关联，目前缺乏相关文献。我们旨在更好地了解这些疾病在儿科患者中的关系。
对费城儿童医院2008年1月至2015年8月期间患有SCD和AILD的患者进行回顾性分析。
共确定了7名患者，年龄在8至23岁之间（3名男性）。其中3例患有AIH，2例患有SC，2例为AIH/SC重叠，即自身免疫性SC。所有接受硫唑嘌呤治疗的AIH患者的肝酶均显著改善。1例患有SC和炎症性肠病的患者成功接受了骨髓移植。2例SC患者死于SCD并发症。
在这个队列中，SCD与AILD之间似乎存在关联；该人群中的SC病情严重。医生应意识到这一点，并对SCD和肝酶升高的患者进行AILD评估。
