儿童慢性肝胆疾病的临床概况：来自印度西部三级转诊中心的经验

Shah Ira, Bhatnagar Susmita

Department of Pediatric Medicine and Pediatric Surgery, Hepatobiliary Clinic, B. J. Wadia Hospital for Children, Parel, Mumbai-400012.

Trop Gastroenterol. 2010 Apr-Jun;31(2):108-10.

AIM

To determine etiological spectrum as well as clinical profile of chronic hepatobiliary disorders in children.

METHODS

45 children with chronic hepatobiliary disorders were evaluated in the study.out of 105 children with liver diseases referred to the clinic. All underwent detailed history and clinical examination. Clinical and laboratory features as well as causes of chronic hepatobiliary disorders were studied.

RESULTS

The common causes were biliary atresia in 11 (25%) patients, neonatal hepatitis and Wilson's disease in 6 (13%) patients each, glycogen storage disorder (GSD) and idiopathic hepatitis in 5 patients (11%) each, Hepatitis B in 2 (5%), Hepatitis C in 1 (2%), Hepatitis B and C in 1 (2%), Caroli's disease in 2 (5%), autoimmune hepatitis in 2 (5%); sclerosing cholangitis, viral hemophagocytosis and thalassemia major in 1 (2%) patient each. Common clinical presentations were jaundice in 32 (71%), dark urine in 19 (42%), fever in 13 (29%), failure to thrive in 7 (16%), splenomegaly in 21 (47%) and hepatomegaly in 32 (71%). Also children with neonatal cholestasis presented in 1s' year of life, those with idiopathic liver disease and GSD presented within 1st 5 years of life and those with Wilson's disease. Autoimmune hepatitis, Caroli's disease presented between 5-10 years of age and viral hepatitis was seen in 2nd decade of life (p < 0.001).

CONCLUSION

Commonest cause of chronic hepatobiliary disorders in children is neonatal hepatitis. Metabolic liver disease usually presents in 1st 5 years of life whereas chronic viral hepatitis has a presentation in adolescence.

目的

确定儿童慢性肝胆疾病的病因谱及临床特征。

方法

在本研究中，对105名转诊至门诊的肝病患儿中的45名患有慢性肝胆疾病的儿童进行了评估。所有患儿均接受了详细的病史询问和临床检查。对慢性肝胆疾病的临床和实验室特征以及病因进行了研究。

结果

常见病因包括11例（25%）患儿为胆道闭锁，6例（13%）患儿为新生儿肝炎和威尔逊病，5例（11%）患儿为糖原贮积病（GSD）和特发性肝炎，2例（5%）患儿为乙型肝炎，1例（2%）患儿为丙型肝炎，1例（2%）患儿为乙型和丙型肝炎，2例（5%）患儿为卡罗利病，2例（5%）患儿为自身免疫性肝炎；1例（2%）患儿为硬化性胆管炎、病毒噬血细胞综合征和重型地中海贫血。常见临床表现为黄疸32例（71%）、尿色深19例（42%）、发热13例（29%）、生长发育迟缓7例（16%）、脾肿大21例（47%）和肝肿大32例（71%）。新生儿胆汁淤积患儿在1岁以内发病，特发性肝病和GSD患儿在1至5岁内发病，威尔逊病、自身免疫性肝炎、卡罗利病患儿在5至10岁发病，病毒性肝炎在第二个十年发病（p<；0.001）。