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Objective Early detection is the most effective way to improve the clinical outcome of biliary atresia(BA).Emerging metabolomics provides a powerful platform for discovering novel biomarkers and biochemical pathways to improve early diagnosis.The aim of this study is to find the potential biomarkers to distinguish BA from neonatal hepatitis syndrome(NHS) by using a metabolomics method.Methods We comprehensively analyzed the serum metabolites in a total of 26 blood samples from patients with BA or neonatal hepatitis syndrome(NHS) and from normal individuals using advanced metabolomic approaches.Results The levels of propanoic acid,hexadecanoic acid,eicosanoic acid,octadecenoic acid and cholesterol significantly increased in the BA group.Conclusion The levels of L-Tyrosine(Tyr)were reduced in the BA group compared to those in the NHS group,but still higher than the normal controls.The levels of L-Proline(Pro) in the NHS group were significantly elevated compared to those in the BA group.And at the same time,we find 5 patients with cirin deficiency.This study demonstrates the possibility of metabolomics as non-invasive biomarkers for the early detection of BA and also provides new insight into pathophysiologic mechanisms for BA.
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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 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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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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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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[ 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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Objective To compare the curative effect of hepatocyte growth factor(HGF) in treatment of infant hepatitis syndrome (IHS) caused by cytomegalovirus with ganciclovir.Methods Sixty-eight cases with infant hepatitis syndrome caused by cytomegalovirus from neonatology department of our hospital from September 2010 to September 2014 were randomly divided into two groups, the observation group (32 cases)took HGF and ganciclovir therapy and the control group (36 cases) only received ganciclovir treatment Both groups were given conventional guard liver therapy.After one month of treatment, liver function, cytomegalovirus DNA content,extinction time of jaundice, treatment efficiency and the adverse reaction rate were evaluated for two groups.Results Liver function of children in observation group improved significantly after treatment compared with the control group, CMV-DNA quantitatively lower than the control group, the extinction time of jaundice was earlier than the latter,and the overall treatment efficiency was higher in observation group (P ＜0.05);The adverse reaction has no significant difference between two groups (P ＞ 0.05).Conclusion HGF combined with ganciclovir treatment can significantly improve liver function in patients of IHS caused by cytomegalovirus infection, and can collaboratively reduce viral load.Symptoms subside quickly with fewer adverse reactions, which is worthy of clinical promotion.
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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 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 retrospectively evaluate different methods (laboratory tests,liver / gallbladder ultrasound and magnetic resonance cholangiopancreatography)in differentiating biliary atresia from infant hepatitis syndrome.Methods Seventy infants with cholestatic jaundice,50 cases of biliary atresia and 20 cases of infant hepatitis syndrome were studied prospectively from January 2010 to December 2012.All cases underwent abdominal ultrasound and magnetic resonance cholangiopancreatography.The accuracy,sensitivity,specificity and predictive values of these various methods were compared.Also the laboratory parameters were statistically analyzed and compared between groups.Results Patients with BA had significantly higher GGT values at presentation [(743.5 ± 564) IU/L] compared with infants with IHS [(198.8 ± 197.8) IU/L],showing statistically significant difference (P ＜ 0.05).The sensitivity,specificity,and accuracy of the ultrasound in diagnosis of BA were 84.0％ 、100.0％ 、88.6％ respectively(P ＜ 0.05).The values for magnetic resonance cholangiopancreatography were 82.0％ 、80.0％ 、81.4％ (P ＜ 0.05).Conclusion Currently,the method of ultrasound is more reliable than MRCP for differentiating biliary atresia from infant hepatitis syndrome.
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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.
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Objective To summarize the etiologies of infant hepatitis syndrome,clinical presentation,treatment and prognosis.Methods The clinical symptoms of 42 cases with infantile hepatitis syndrome were observed and the blood cytomegalovirus,hepatitis B virus,cell culture and a series of related biochemical parameters were examined.Results All of patients' liver have been damaged and have jaundice,blood CMV infection accounted for 48%( 20/42 ),hepatitis B virus,accounting for 14% (6/42),blood cultures have staphylococcus up to 7% ( 3/42 ).After combining Chinese and Western medicine treatment,the sufferers restore to health,cure rate up to 95% (37/39),3cases were lost.Conclusion The infant hepatitis syndrome originate from the infections of the blood cytomegalovirus,hepatitis B virus and bacterial infection,whose clinical manifestations are infant liver damaged and jaundice.We have a better prognosis on infantile hepatitis syndrome and the result of combining Chinese and Western medicine treatment is good.
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Objective To evaluate the value of laparoscopic-assisted cholangiography in diagnosing prolonged jaundice in infants.Methods Through an umbilical troear,an laparoscope was placed into the abdominal cavity to detect the gallbladder and liver.After confirming that the choleeyst is normal,we pulled out the fundus of the gallbladder through the right subcostal trocar,and then inserted a catheter into the gallbladder for cholangiography.If the fundus could not be exteriorized because of gallbladder atresia, the patient would be converted to an open surgery.Results Cholangiography showed infant hepatitis syndrome or cholestasis in 8 cases,biliary hypoplasia in 2,and biliary atresia in 2.In 5 patients,the gallbladder was dissected from the liver bed before cholangiography,2 of them had biliary hypoplasia and 3 showed biliary atresia.Cholangiography was given up in 21 children because of liver cirrhosis.These patients were diagnosed with biliary atresia and then were treated by Kasai portoenterostomy. Conclusions Laparoscopic-assisted cholangiography is a simple,accurate,and safe method in the diagnosis of prolonged jaundice in infants.By using the procedure,the whole biliary tree can be shown clearly without leading to serious injuries.
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Objective To investigate the changes of serum and bile from victims attacked by infantile hepatitis syndrome(IHS).Methods The constituents from 42 IHS subjects and 16 controls,including total bilirubin(TB),direct bilirubin(DB),alanine aminotransferase(ALT),glutamyltranspeptidase(?-GT),total bile acid(TBA),interleukin 6(IL-6) and tumor necrosis factor ?(TNF-? )both in bile and serum,were assayed by fully-auto chemistry analyzer and ELISA,respectively.The subjects of IHS were divided into cholestasis group and hepatitis group.Results Of IHS group,the values of serumal TB,DB,ALT,?-GT,TBA,IL-6 and TNF-? were higher than those of control(P_a
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Objective To explore the clinical efficacy and side effect of hepatocyte growth - promoting factor in treatment of infant hepatitis syndrome. Methods Sixty one cases of infant hepatitis syndrome were chased as the treatment group who hospitalized in our hospital from March 2002 to Feb 2003,and 54 cases of infant hepatitis syndrome as control group who hospitalized during March 2001 to Feb 2002. The treatment group were administrated with hepatocyte growth - promoting factor for 2 weeks. We obser-ved the recovery of patient's liver function (TBIL.ALT, AST) and the side effect of hepatocyte growth- promoting factor after two weeks of the treatment. Results After the treatment,TBIL and ALT decreased significantly in the treatment group of infant hepatitis syndrome. The treatment group was superior to the control group (P
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The clinical manifestation, complication, treatment and clinical outcome etc. of 63 in- fants with hepatitis syndrome admitted from 1987 to 1990 are analysed in this article. The in- fantile hepatitis syndrome has been regarded as a group of the clinical manifeststions. The causal diagnosis is of great importance. The abdominal B ultrosound investigntion, which should be regarded as a routinal screen method, is very helpful to the differentiation between hepatitis and biliary atresia. Corticosteroid is also an effective drug for treating the syndrome.