Samanta Tryambak, Basu Rajarshi, Purkait Radheshyam, Kar Sudipta, Das Debasis, Ganguly Sutapa
Department of Medical Gastroenterology Medical College Kolkata India.
Department of Pediatrics Nil Ratan Sircar Medical College Kolkata India.
JGH Open. 2020 Oct 30;5(1):20-27. doi: 10.1002/jgh3.12441. eCollection 2021 Jan.
Limited data exist regarding the etiological spectrum of the subset of chronic liver diseases (CLDs) diagnosed in noncirrhotic states in children. Our primary objective was to study the clinicoetiological profile of CLDs detected in noncirrhotic stages in children younger than 12 years of age. The secondary objective was to find the hepatic histological correlation of provisional diagnosis by different ranks of doctors.
This was an observational epidemiological study, cross-sectional in design, conducted in a tertiary-care setting over a 2-year period.
Thirty-seven cases were enrolled, with a mean ± SD age of 8 ± 4.1 years and a male:female ratio of 1.8:1. Etiologies noted were Wilson disease ( = 8), autoimmune hepatitis ( = 4), secondary hemochromatosis ( = 4), chronic hepatitis B ( = 3), chronic hepatitis C ( = 2), non-alcoholic steatohepatitis ( = 2), progressive familial intrahepatic cholestasis ( = 2), extrahepatic biliary atresia ( = 2), Alagille syndrome ( = 1), galactosemia ( = 1), Gaucher disease ( = 1), Niemann-Pick disease ( = 1), and Budd-Chiari syndrome ( = 1), with an inconclusive diagnosis in five children. Relevant investigations were ordered more frequently by the specialist consultant (SC) and super specialist (SS) combined in comparison with the senior resident (SR) and junior resident (JR) together. ( = 0.0013). Irrelevance of the tests ordered was significantly higher in the junior tier (JR and SR; SR > JR) in contrast to the senior tier of doctors (SC and SS) ( < 0.01). The clinicohistological correlation of an etiological diagnosis significantly differed between the junior and senior ranks of physicians. We noted that an ideal clinical acumen could help to avoid liver biopsy for etiological diagnosis in 78.3% (29/37) of the study population.
Interpretation of clinical presentation by the senior set of doctors is preferable, which could obviate the need for liver biopsy regarding diagnosis in a proportion of pediatric CLD patients.
关于儿童非肝硬化状态下诊断出的慢性肝病（CLD）子集的病因谱，现有数据有限。我们的主要目标是研究12岁以下儿童非肝硬化阶段检测到的CLD的临床病因学特征。次要目标是找出不同级别医生初步诊断的肝脏组织学相关性。
这是一项观察性流行病学研究，采用横断面设计，在三级医疗机构进行，为期2年。
共纳入37例病例，平均年龄±标准差为8±4.1岁，男女比例为1.8:1。记录的病因包括威尔逊病（8例）、自身免疫性肝炎（4例）、继发性血色素沉着症（4例）、慢性乙型肝炎（3例）、慢性丙型肝炎（2例）、非酒精性脂肪性肝炎（2例）、进行性家族性肝内胆汁淤积症（2例）、肝外胆道闭锁（2例）、阿拉吉尔综合征（1例）、半乳糖血症（1例）、戈谢病（1例）、尼曼-匹克病（1例）和布加综合征（1例），5名儿童诊断不明确。与住院医师（SR）和实习医师（JR）相比，专科顾问医师（SC）和特级专科医师（SS）联合开具相关检查的频率更高（P = 0.0013）。与高级别医生（SC和SS）相比，初级医生（JR和SR；SR＞JR）开具的无关检查显著更多（P＜0.01）。病因诊断的临床组织学相关性在初级和高级医生之间存在显著差异。我们注意到，理想的临床敏锐度有助于在研究人群的78.3%（29/37）中避免因病因诊断进行肝活检。
高级别医生对临床表现的解读更可取，这可以避免一部分儿童CLD患者诊断时进行肝活检的必要性。
