[Clinical features of different clinical forms of childhood congenital hepatic fibrosis].
OBJECTIVE
To compare the clinical features of children with different clinical forms of congenital hepatic fibrosis (CHF), and provides a description of the characteristics of childhood CHF.


METHODS
Sixty children with CHF between January 2002 and June 2015 were enrolled, including 26 children with portal hypertensive CHF (PH CHF), 3 children with cholangitic CHF, 30 children with combined portal hypertensive and cholangitic CHF (mixed CHF), and 1 child with latent forms of CHF. The medical data of 26 children with PH CHF and 30 children with mixed CHF, including gender, age, clinical manifestations, physical signs, laboratory tests and imaging characteristics, were retrospectively studied.


RESULTS
Fever, jaundice and hepatomegaly were more frequently noted in children with mixed CHF than in those with PH CHF (P<0.05). Splenomegaly and liver cirrhosis occurred more often in children with CHF, but there was no significant difference in the incidences of splenomegaly and liver cirrhosis between the children with PH CHF and mixed CHF. The plasma prothrombin activity, white blood cell counts, platelet counts, mean platelet volume, serum levels of alanine transaminase, aspartate transaminase, alkaline phosphatase, γ-glutamyl transferase, leucine aminopeptidase, and total bile acids in children with mixed CHF were higher than in those with PH CHF (P<0.05). The decreased international normalized ratio and lower serum albumin levels were more frequently observed in children with mixed CHF than in those with PH CHF (P<0.05).


CONCLUSIONS
PH and mixed CHF are common forms in childhood CHF. The children with the two forms of PH usually manifest portal hypertension such as cirrhosis and hepatosplenomegaly. The liver damage may be common in children with mixed CHF.
Department of Traditional Chinese Medicine, Beijing Shijitan Hospital Affiliated to Capital Medical University, Beijing 100038, China. jhwux2@163.com.
Xin
X
Wu
Xiao-Rang
XR
DU
Jin-Fang
JF
Ding
Meng-Jin
MJ
Wu
Sheng-Qiang
SQ
Luo
Xing-Zhong
XZ
Feng
D000293:Adolescent; D000469:Alkaline Phosphatase / Q000097:blood; D002648:Child; D005260:Female; D030342:Genetic Diseases, Inborn / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D008103:Liver Cirrhosis / Q000150:complications / Q000175:diagnosis*; D008297:Male; D013163:Splenomegaly / Q000209:etiology
D000469:Alkaline Phosphatase