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Analysis of etiology and clinical features of 36 children with cirrhosis
ZOU Biao , LIU Sheng-hui, HE Jia-yi, et al
2022, 37(7):
516-522.
DOI: 10.19538/j.ek2022070610
Objective To explore the etiology and clinical features of pediatric cirrhosis，to understand its prognosis，and to improve the levels of diagnosis and treatment of it.Methods The clinical data of 36 cases of childhood cirrhosis treated in Tongji Hospital，Tongji Medical College，Huazhong University of Science and Technology from October 2016 to December 2020 were studied.The clinical data included，gender，age，past history，etiology，clinical manifestations，complications，nutritional status at diagnosis，laboratory test results，genetic results，post-treatment follow-up.Compare the related indicators between the good prognosis group and the death group.Results Among the 36 patients with cirrhosis，18 were male and 18 were female with，the age was 51.7（5，84）months；13 cases had biliary atresia，6 were Wilson diseases，Carolis disease had 2 cases，progressive familial intrahepatic cholestasis（PFIC）2 cases；there were 7 patients with cryptogenic cirrhosis，one hereditary hemorrhagic telangiectasia（HHT），one case of Niemann-Pick disease，one case of congenital bile acid synthesis defect，case of hepatitis B cirrhosis，case of neonatal intrahepatic cholestasis（NICCD），and one case of Shwachman-Diamond syndrome（SDS）.The main clinical features were splenomegaly in 18 cases（50%），skin jaundice in 16 cases（44.4%），fever in 10 cases（27.7%），abdominal distension in 10 cases（27.7%），gastrointestinal bleeding in 6 cases（16.6%）and vomiting in 4 cases（11.1%），etc.At the time of diagnosis of cirrhosis，the number of children with underweight and growth retardation was 8（22.2%）and 12（33.3%）respectively.Ultrasonography was performed in all 36 cases to diagnose cirrhosis，abdominal CT examination showed cirrhosis in 14 cases，and 15 patients were diagnosed through histopathology.The main complications included esophagogastric fundus varicose veins in 9 cases（26.4%），hypersplenia and/or cytopenia in 8 cases（23.5%），and ascites in 6 cases（16.6%），etc.Genetic tests were performed in 10 children，and pathogenic genes were detected in 6 cases，including NPC1，ABCB4，SADM4，ATP7B，SBDS and SLC25A13.All the children were treated according to etiology，and the symptomatic supportive treatment such as liver protection and antixanthosis was added.Among them，27（75%）were in good general condition，7（19.4%）died，8 had been treated with liver transplantation，and 2 were currently awaiting liver transplantation.There was no significant difference in alanine aminotransferase（ALT），aspartate aminotransferase（AST），γ -glutamyl transpeptidase（GGT），prothrombin time（PT），platelet or total bilirubin（TBIL）between the good prognosis group and the death group（P＞0.05）.There was statistically significant difference in age，albumin（ALB）and direct bilirubin（DBIL）at the time of diagnosis（P＜0.05）.Conclusion The etiology of cirrhosis in children is very different from that in adults. biliary atresia is the most common etiology；B-ultrasonography is the most common examination method for its diagnosis；malnutrition is easy to occur，so it is suggested to strengthen nutritional supplements；pediatric cirrhosis with younger age，lower albumin and higher direct bilirubin has poor prognosis and high mortality.
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