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[Objective] To investigate the significance of liver biopsy in differential diagnosis and prognosis of congenital biliary atresia (CBA) and infant hepatitis syndrome (IHS).[Methods] Totally 77 children with congenital biliary atresia and 48 infants with hepatitis syndrome treated in Guangdong Women and Children Hospital from December 2012 to December 2016 were examined by liver biopsy and follow-up.Combined with immunohistochemistry and PAS staining,reticular fiber staining,Masson staining techniques,we make comparative analysis of both histopathological features and prognosis.[Results] The liver fibrosis grade,hepatic lobule inflammation activity staging,the degree of bile duct hyperplasia and the prognosis of CBA and IHS infants were statistically significant (P＜0.05).S2-S3-based liver fibrosis grading in infants with CBA,mainly in G2-G3 hepatic lobule inflammation staging,bile duct hyperplasia significantly;IHS infants with liver fibrosis grading as S0-S1,liver Slice inflammatory activity stage to G1-G2-based.The prognosis of infants with CBA was significantly worse than IHS,and the difference was statistically significant (P＜0.05).[Conclusion] The early liver biopsy of infants with congenital biliary atresia and infant hepatitis syndrome,combined with immunohistochemistry and PAS staining,reticular fiber staining,Masson staining techniques has important clinical significance to the differential diagnosis and prognosis of both.
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Objective To investigate the change of plasma vitamin D, A and E levels in infant with infantile hepatitis syndrome (IHS) and to provide reliable basis for clinical treatment. Methods Seventy-three infant with IHS (IHS group) from January 2015 to May 2016 and 82 cases of physical examination infant (control group) were enrolled in this study.The levels of plasma vitamin D, A and E were tested and compared between two groups. Results The levels of vitamin D, A and E in IHS group were significantly lower than those in control group:(37.871 ± 20.111) nmol/L vs. (97.708 ± 28.827) nmol/L, (1.082 ± 0.657)μmol/L vs. (1.810 ± 0.517)μmol/L, (21.252 ± 7.596)μmol/L vs. (26.647 ± 6.495)μmol/L, P<0.01 or <0.05. The nutritional status of vitamin D, A and E in two groups had significant differences (P<0.01). Conclusions There have obvious deficiency in fat-soluble vitamin D, A and E in infant with HIS , and vitamin D deficiency is the most obvious. The levels of vitamin D, A and E are the dynamic monitoring indicators of treatment, nutritional status evaluation and long-term complications development.
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Objective To investigate the expression and the possible clinical significance of serum Golgi protein(GP73)in infantile hepatitis syndrome(IHS)by different causes.Methods Totally 79 patients with IHS in Guangzhou Women and Children's Medical Center from February 2012 to December 2012 were enrolled in this study,including 15 cases with biliary atresia(BA)group,29 cases with infection(infection group),5 cases with neonatal intrahepatic cholestasis caused by citrin deficiency(NICCD group),and 30 cases with unknown etiology(idiopathic infantile hepatitis group).At the same time,30 healthy infants were enrolled as healthy control group.The serum levels of GP73 were determined by quantitative enzyme-linked immunosorbent assay(ELISA),and the children's liver function[alanine aminotransferase(ALT),aspartate aminotransferase(AST),total bilirubin(TBIL),direct bilirubin(DBIL),alkaline phosphatase(ALP),γ-glutamyl trans-peptidase(γ-GT),total bile acid(TBA)and albumin(ALB)] were measured by turbidimetric inhibition immuno assay.Then,the corresponding data were statistically analyzed.Results Serum GP73 in BA group,infection group,NICCD group,idiopathic infantile hepatitis group and the healthy control group were(296.6±67.5)μg/L,(185.1±66.4)μg/L,(199.2±87.1)μg/L,(181.7±74.2)μg/L and(65.3±17.0)μg/L,respectively.Serum γ-GT levels in BA group,infection group,NICCD group,idiopathic infantile hepatitis group and healthy control group were(764.7±775.8)U/L,(448.2±352.7)U/L,(239.4±88.7)U/L,(283.3±377.2)U/L and(54.0±72.6)U/L,respectively.The levels of GP73 and γ-GT were significantly higher in infants with IHS,and the levels of GP73 and γ-GT in infants with BA were the highest(F=46.775,9.238,all P0.05).The receiver operating characteristic curve(ROC)constructed with GP73 showed a sensitivity of 80.0％and specificity of 82.8％with an area under the receiver(AUC)of 0.872 for diagnosis of BA,comparatively,a sensitivity of 66.7％and specificity of 71.9％were showed with a AUC of 0.731 when performed with γ-GT.Conclusions Serum GP73 concentration significantly increased in all liver disease groups,regardless of the etiology.Serum GP73 expression is significantly higher in infants with BA.Serum GP73 shows a superior sensitivity and specificity to γ-GT for diagnosis of BA,which might be useful for early diagnosis of BA and IHS with different causes.
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[ ABSTRACT] AIM:To observe the effect of ursodeoxycholic acid ( UDCA) on the treatment of infantile hepatitis syndrome ( HIS) and to investigate its mechanism.METHODS:The children with infantile hepatitis syndrome were divid-ed into conventional treatment group and the UDCA treatment group.Twenty healthy children were selected as normal con-trol.The children in conventional therapy group were given antiviral and hepatoprotective treatments.The children in UD-CA treatment group were given ursodeoxycholic acid (10 mg? kg-1? d-1 ) in addition to the conventional treatment group for 2 to 3 weeks.The levels of total bilirubin (TBIL), direct bilirubin (DBIL), alanine aminotransferase (ALT), glu-tamyltransferase ( GGT) , total bile acids ( TBA) and TNF-α, IL-6 were detected before admission and 2 weeks later.RE-SULTS:The levels of TNF-αand IL-6 were significantly higher in the children with IHS than those in the normal control (P<0.01).The levels of TBIL, DBIL, ALT, GGT, TBA, TNF-αand IL-6 in conventional treatment group were reduced after therapy (P<0.01).All the above index in UDCA treatment group were decreased compared with conventional treat-ment group (P<0.01).CONCLUSION:On the basis of conventional therapy, ursodeoxycholic acid effectively alleviates the systemic inflammatory response in the children with IHS, reduces the liver damages.
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Purpose To study the values of liver histopathological assessment grading score in differential diagnosis between biliary atresia ( BA) and infantile hepatitis ( IHS) . Methods Thirty four cases of BA and sixteen cases of IHS were analyzed retrospectively, which were diagnosed by biopsy. A hepatic histopathological assessment grading score was developed. This consisted of eight features such as cholestasis, hepatocellular damage, bile duct proliferation, portal edema, portal inflammation, portal fibrosis, extramedullary hemopoiesis and multinucleated giant hepatocytes. The total scores were 24 points. All the cases were assessed one by one. Results The total scores of BA were significantly higher than that of IHS (P<0. 001). The frequencies of bile duct proliferation, portal fibrosis and portal edema were significantly higher in BA than that in IHS group, while the frequency of multinucleated giant hepatocytes was significantly higher in IHS than that in BA group. Conclusions This scoring system is helpful in differentiating BA from IHS.
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Objective To investigate the value of hepatobiliary scintigraphy combined with total bile acid (TBA) and γ-glutamyhransferase(γ-GT) detection in the differential diagnosis of persistent jaundice induced by infantile hepatitis syndrome(IHS) and congenital extrahepatic biliary atresia(EHBA).Methods A retrospective analysis of 60 infants with persistent jaundice undertaking 99Tcm-diethylacetanilide iminodiacetic acid (EHIDA) hepatobiliary scintigraphy was done in Nanfang Hospital by single photon emission computed tomography(SPECT).Meanwhile,these infants' sera were collected and separately detected by AU5431 automatic biochemical assay;the sensitivity,specificity and accuracy of hepatobiliary scintigraphy with TBA and γ-GT were evaluated.Results The sensitivity to 99Tcm-EHIDA hepatobiliary scintigraphy in the diagnosis of IHS and EHBA were 100.00％ (17/17 cases) and 67.57％ (25/37 cases),the specificity was 67.57％ (25/37 cases) and 100.00％ (17/17 cases),and the accuracy was 77.78％ (42/54cases) and 77.78％ (42/54 cases),respectively.The levels of TBA and γ-GT were higher in infants with EHBA than those with IHS(U =209.0,19.5,all P ＜0.05),and ROC curve analysis indicated that TBA in the IHS group and γ-GT in EHBA group had some diagnostic value[area under curve (AUC) =0.736,0.968,respectively].99Tcm-EHIDA hepatobiliary scintigraphy combined with TBA and γ-GT analysis suggested when intestinal non-radioactive imaging was shown,TBA was 98.5 μmol/L and γ-GT was 298 U/L,the sensitivity,specificity and accuracy of diagnosis of EHBA were 100.00.00％ (17/17 cases),100.00％ (37/37 cases) and 100.00％ (54/54 cases) in a serial test.Conclusions Hepatobiliary scintigraphy combined with TBA and γ-GT examination can effectively identify EHBA and IHS earlier,noninvasively and safely,which have important role in further treatment in infants with persistent jaundice.
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Objective To explore the correlation between human cytomegalovirus(HCMV) infection with infantile hepatitis syn‐drome(IHS) and hepatic function damage .Methods The real‐time fluorescent quantitative polymerase chain reaction(PCR) was a‐dopted to test the urine HCMV DNA in 236 infants with IHS and 236 healthy infants ,respectively .The hepatic functions in 254 in‐fants with HCMV infection were analyzed retrospectively .Results Among these 236 cases of IHS ,the positive rates of HCMV DNA in urine sample was 62 .7% (148/236) .The positive rates of HCMV DNA and HCMV IgM in the IHS group were significant‐ly higher than those in the control group with statistical difference(P< 0 .01) .The liver function indexes in 254 infants with HCMV infection showed that the serum concentrations of total bilirubin (TBIL ) ,gamma glutamyl transpeptidase (GGT ) ,total bile acid (TBA) ,aspartate aminotransferase(AST ) and alanine aminotransferase(ALT ) were higher than the normal reference ranges ,and the differences were statistically significant(P < 0 .01) .Conclusion The detection rate of HCMV infection is high among the in‐fants with IHS in Guangxi area and HCMV is an important pathogen of IHS .HCMV may lead to hepatic function damage .
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Objective To investigate the value of ultrasound-guided liver biopsy for infantile hepatitis syndrome regarding diagnosis,treatment and prognosis.Methods Fifty children with infantile hepatitis syndrome hospitalized in Guiyang Maternal and Child Hospital during Aug.2010 to May 2012 were involved in this study.Ultrasoundguided liver biopsies were performed to evaluate the inflammation grade and fibrosis stage.Immunohistochemical staining was used for pathogen diagnosis.The clinical outcomes were followed-up.Results Thirty-four cases (68％)were CMV infection,6 cases(12％) were vanishing bile duct syndrome,4 cases(8％) were chronic intrauterine infection,4 cases(8％) were congenital anomaly of bilirubin metabolism,and 2 cases (4％) were obstructive cholangitis.All 50 cases showed mild inflammation at portal area(G1-G2 grade).All 50 cases exhibited liver fibrosis.Sixteen cases were S1 stage,20 cases were S2 stage,8 cases were S3 stage and 6 cases were S4 stage.Pathogen analysis:all 50 cases showed intrahepatic cholestasis:38 cases were diffuse cholestasis,and 12 cases were moderate cholestasis.Treatment:all cases were treated using 2-week heteropathy; antivirus was used for CMV infected cases,thus 39 cases were finally cured,9 cases were relieved,and 2 cases were ineffective.Conclusion Liver biopsy is valuable for diagnosis,treatment and prognosis infantile hepatitis syndrome.