| Objective:By collecting the case data of infants with cholestasis hepatopathy,the clinical characteristics were summarized in order to provide help for etiology and prognosis of ICH.Methods:The clinical data of infants with cholestatic hepatopathy whose age of onset within 3 months and hospitalized in our hospital from January 2016 to December 2018 were collected and analyzed retrospectively.Results:(1)A total of 310 infants with ICH were included in this study,including 192 males(61.94%)and 118 females(38.06%),with a male-to-female ratio of 1.63.There were 241 cases(77.74%)in less than 1 month,49 cases(15.81%)between 1 month and 2 months.,and 20 cases(6.45%)between 2 months and 3 months.(2)Etiological distribution of 310 infants : 74 cases are infectious diseases(23.87%),including 67 cases of CMV infection(21.61%);36 cases were hereditary hepatopathy(11.61%),including 21 cases of NICCD(6.77%),8 cases of ALGS(2.58%),3 cases of PFIC(0.97%),1 case of NTCP deficiency disease(0.32%),1 case of MMA(0.32%),1 case of GSD(0.32%),1 case of chromosomal disease(0.32%),and 9 cases of PNAC(2.90%).Another 191 cases(61.61%)were not clearly diagnosed when they were discharged from hospital.(3)By April 2020,168 cases(89.36%)of the 188 follow-up infants were improved,5 cases(2.67%)were not cured,and 15 cases(7.98%)died.The uncured infants were 1 case of PFIC1(20%),1 case of PFIC5(20%)and 3 cases of ALGS(60%).Among the dead cases,there were 2 cases of ALGS(13.33%),2 cases of CMV infection(13.33%),1 case of septicemia(6.67%),7 cases suspected of hereditary hepatopathy(46.67%),and 3 cases of unknown etiology(20%)(4)Among the 152 infancts with definite time of prognosis,the improvement rates of 4-month-old,6-month-old,1-year-old and 1~4 year-old were 37.5%,63.16%,83.55% and 92.11%,respectively.(5)The follow-up of liver function in 72 outpatients showed that total bilirubin,glutamic pyruvic transaminase,glutamic oxaloacetic transaminase,alkaline phosphatase,γ-glutamyl transpeptidase and total bile acid decreased with age.(6)Compared with the survival group,the levels of bilirubin in the death group were higher(P < 0.05),the concentration of albumin was lower(P = 0.003 < 0.05),the serum potassium concentration was lower(P=0.003 < 0.05).Conclusions:Most of the infants with ICH began within 1 month,and there were more males than females.At present,infectious diseases are the most common causes,mainly cytomegalovirus infection,followed by hereditary liver diseases,which NICCD and ALGS are relatively more;PFIC,NTCP deficiency disease,MMA,GSD and chromatosis are rare.Parenteral nutrition associated cholestasis is also an important cause of ICH.The cause of the disease is still unknown in more than half of the infants.After 1 to 4 years of follow-up,most of the infants can gradually improve,a few do not recover or die.The prognosis of infectious disease and PNAC are good.In hereditary liver diseases,the prognosis of NICCD is good,while that of PFIC and ALGS is poor. |
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