The Etiology Of 142 Cases Of Infant Cholestasis And Evaluation Of Several Clinically Common Used Methods For Diagnosis

Objective: This study was designed to investigate the etiology of infant cholestasis in local area,which comparatively analyzed the clinical manifestations,laboratory results,imaging results,and clinical outcomes of Biliary Atresia(BA)and Infant Intrahepatic Cholestasis(IHC).Discussions were made in the clinical diagnosis and treatment of infant cholestasis to provide reference in improving diagnostic and therapeutic level.Method: 142 hospitalized infants were included in this study with the diagnosis of infant cholestasis in the pediatric department of the First Affiliated Hospital of Fujian Medical University during January 1st 2012 to November 30 st 2016.The clinical manifestations,laboratory results,imaging results and clinical diagnosis of the patients were retrospectively analyzed,clinical outcomes were followed by telephone follow-up.Result: 99 of the 142 infant cholestasis(69.7%)had obvious causes of disease,the most common causes of which were BA,Cytomegalovirus Hepatitis,Citrin protein deficiency.Among the other 43 cases,37 were diagnosed with Idiopathic Infant Hepatitis(26.1%),6 were of unknown cause(4.2%).BA was defined by surgery(or)telephone follow-up.Compared with IHC cases,the incidences of acholic stools,hepatomegaly,and the liver size were significantly higher in BA,the incidence between the two groups was significant(P<0.01).The sensitivity,specificity and accuracy for diagnosis of BA was 86.8%,63.4% and 69.8% by acholic stool,while liver size?3cm was 71.0%,64.4%,66.2% respectively.The serum TBIL and DBIL levels during one month~,two month~,three month~,and the serum ?-GT levels during each month were significantly higher in BA group than those in IHC groups(P<0.01).When a serum ?-GT level greater than 300U/L was used as a diagnostic criterion for BA,the diagnostic sensitivity,specificity and accuracy were 65.2%,91.8%,83.3%,?respectively.The incidences of absent or small gallbladder on ultrasonography were significantly higher in BA groups than those in IHC groups(P<0.01).The sensitivity,specificity,and accuracy of absent or small gallbladder on ultrasonography for diagnosis of BA were 68.8%,90.8% and 84.9%.Investigation with hepatobiliary scintigraphy showed that 95% infants who had BA had no gut excretion,and 50% infants who had IHC had no gut excretion,showing a diagnostic sensitivity of 95% with 50% specificity and 68.0% accuracy.73.5% of patients died in BA group,while 26.5% improved,while early Kasai surgery and postoperative Liver Transplantation improved outcomes significantly.4.1% of patients died in IHC group,while 7.2% developed,and 88.7% cured.Conclusion: 99 of the 142 infant cholestasis(69.7%)had obvious causes of disease,the most common causes of which were BA,Cytomegalovirus Hepatitis,Citrin protein deficiency,successively.26.1% were diagnosed for Idiopathic Infant Hepatitis.In the infantile cholestasis cases who presented with acholic stools,liver size?3cm,a marked elevation in serum ?-GT(?300U/L),either absent or small gallbladder on ultrasonography,and no gut excretion on hepatobiliary scintigraphy,the most likely diagnosis was BA,but those were of low sensitivity and specificity,synthetic judgment and further invasive methods like laparoscopic examination and intraoperative cholangiography were required to make a clear diagnosis as soon as possible.Etiology was the key factor to determine the clinical outcomes,early diagnosis and treatment are of great importance to the outcomes of this disease.