撒哈拉以南非洲一家转诊医院小儿自身免疫性肝炎的临床特征。
Clinical characteristics of paediatric autoimmune hepatitis at a referral hospital in Sub Saharan Africa.
机构信息
Department of Child Health, Medical School, College of Health Sciences, University of Ghana, Accra, Ghana.
Department of Paediatrics and Child Health, University of Health and Allied Sciences, Ho, Ghana.
出版信息
PLoS One. 2020 Dec 23;15(12):e0239964. doi: 10.1371/journal.pone.0239964. eCollection 2020.
BACKGROUND
Autoimmune hepatitis (AIH) is a progressive inflammatory liver disease of unknown aetiology. The number of reported AIH cases is increasing in the developed countries but the same cannot be said about sub Saharan Africa (SSA). Paediatric AIH diagnosis is usually missed and patients present with decompensated liver disease. Our study highlights the clinical profile of paediatric AIH cases at a referral hospital in Ghana.
METHODS
This is a retrospective review of all cases of children diagnosed with autoimmune hepatitis at the gastroenterology clinic in Korle Bu Teaching Hospital, Accra, Ghana. Data was extracted from the patients' records from April 2016 to October 2019. These children were diagnosed based on the presence of autoantibodies, elevated immunoglobulin G and histologic presence of interphase hepatitis with the exclusion of hepatitis A, B, C and E depending on their clinical presentation, Wilson's disease, HIV, Schistosomiasis and sickle cell disease.
RESULTS
Thirteen patients aged between 5 years to 13 years with a mean age of 10 years were diagnosed with AIH. All the patients had type 1 AIH with majority 8 (61.5%) being females. Most of the children presented with advanced liver disease with complications. Three patients had other associated autoimmune diseases. The patients were treated with prednisolone with or without azathioprine depending on the severity of the liver disease.
CONCLUSION
Majority of paediatric AIH presents with advanced liver disease. There is the need for early detection to change the natural history of AIH in SSA.
背景
自身免疫性肝炎（AIH）是一种病因不明的进行性炎症性肝病。在发达国家，报告的 AIH 病例数量正在增加，但撒哈拉以南非洲（SSA）却并非如此。儿科 AIH 的诊断通常被忽视，患者表现为肝功能失代偿。我们的研究强调了加纳一家转诊医院儿科 AIH 病例的临床特征。
方法
这是对 2016 年 4 月至 2019 年 10 月在加纳科勒布教学医院消化科就诊的所有自身免疫性肝炎儿童病例进行的回顾性研究。从患者记录中提取数据。这些儿童是根据自身抗体、免疫球蛋白 G 升高和组织学存在界面肝炎而诊断的，根据其临床表现、威尔逊病、HIV、血吸虫病和镰状细胞病排除了甲型、乙型、丙型和戊型肝炎。
结果
13 名年龄在 5 至 13 岁之间的患者被诊断为 AIH，平均年龄为 10 岁。所有患者均为 1 型 AIH，其中 8 例（61.5%）为女性。大多数儿童出现晚期肝病并伴有并发症。有 3 例患儿伴有其他自身免疫性疾病。根据肝病的严重程度，患者接受泼尼松龙联合或不联合硫唑嘌呤治疗。
结论
大多数儿科 AIH 表现为晚期肝病。需要早期发现，以改变 SSA 中 AIH 的自然病程。
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