Clinical features of neonatal intra-hepatic cholestasis caused by citrin defects and observation on therapeutic effect of Linda mixture for treatment of the disease / 中国中西医结合急救杂志

ABSTRACT

Objective To observe the clinical efficacy of Linda mixture self made by combination of disease with syndrome pattern for treatment of neonatal intrahepatic cholestasis disease caused by Citrin defects (NICCD) and to provide an experience in early diagnosis and treatment of NICCD.Methods The clinical features of 20 patients with NICCD was confirmed by NICCD common genetic loci detection during hospitalization admitted to the Department of Integrated Traditional and Western Medicine in Wuhan Children Hospital from June 2012 to June 2015 were retrospectively analysed.The clinical efficacy of Chinese medicine Linda mixture for treatment of NICCD,before and after treatment,the index changes of liver function and coagulation function were observed and Color Doppler ultrasound was used to detect the situation of hepatosplenomegaly in the patients.Results There were 20 cases all their pathological jaundice occurring within 3 months old,after treatment,15 cases of them obtained clinical recovery and 5 cases were getting better,the average length of stay in hospital was (14.67 ± 1.56) days.After the treatment,the levels of serum total bilirubin (TBil),direct bilirubin (DBil),alanine aminotransferase (ALT),total bile acid (TBA) were markedly lower compared with those before treatment [TBil (μmol/L)64.0 ± 39.5 vs.173.5 ± 54.1,DBil (μmol/L)37.7±24.8 vs.80.9±46.4,ALT (U/L)42.1±25.8 vs.55.0±32.2,TBA (μnol/L)67.5±20.3 vs.195.0±61.3,all P ＜ 0.05],albumin (Alb) and total albumin (GLB) were significantly higher compared with those before treatment [Alb (U/L)37.9 ± 4.7 vs.33.1 ± 4.7,GLB (g/L)17.3 ± 4.0 vs.14.6 ± 2.8,both P ＜ 0.05],activated partial thromboplastin time (AP'TT) of coagulation function was significantly reduced (s38.2± 8.3 vs.63.1±24.0,P ＜ 0.05).Color Doppler ultrasound examination showed that hepatosplenomegaly was shrunk after treatment [hepatomegaly (cm)2.12 ± 0.70 vs.3.04 ± 0.25,splenomegaly (cm)0.25 ± 0.03 vs.0.58 ± 0.32,both P ＜ 0.05].After 1 month of follow up,the infants' body weights were almost normal,the average increase in weight being (1.01±0.32) kg,the color and frequency of stool were distinctly better compared with those before treatment,the stool color in 15 cases was golden,pale yellow in 5 cases and no one,pottery clay in color.The stool frequencies of 18 cases were kept under 5 times,and fatty diarrhea situation was ameliorated.Conclusion Using Chinese medicine for treatment of NICCD can obtain significant clinical effects.