ObjectiveTo explore the clinical characteristics, treatment and prognosis of severe liver damage in children.MethodsClinical data of 55 children with severe liver damage were retrospectively analyzed.Results In 55 children (31 boys and 24 girls) aged from 28 days to 12 years, forty-five children had acute liver injury mainly caused by infectious diseases (21 cases, 53.3%), blood tumor diseases (5 cases, 11.1%), hereditary metabolic diseases (4 cases, 8.9%), and unexplained diseases (10 cases, 22.2%), ten children had chronic liver injury with decompensated cir-rhosis. Most of severe liver damage in children was caused by antipyretic drugs, traditional Chinese medicine and cold medicine, including 31 cases of acute liver injury and 4 cases of chronic liver injury. In children with acute liver injury, clinical symptoms included gastrointestinal symptoms (32 cases, 71.1%), jaundice (26 cases, 57.8%), hemorrhage (9 cases, 20.0%), multiple organ dysfunction (13 cases, 28.9%) and hepatic encephalopathy (6 cases, 13.3%). In children with chronic liver damage, clinical symptoms included abdominal distension and ascites (10 cases), jaundice (9 cases), gastrointestinal bleeding (7 cases), hepatic encephalopathy (3 cases) and multiple organ dysfunction (1 case). In 55 chil-dren, 39 children were died and the total mortality was 70.91%. In 14 cases of multiple organs dysfunction syndromes, 13 cases (92.9%) were died. All three cases of hepatic encephalopathy were died.ConclusionsInfectious diseases are the leading cause of sever liver damage in children. The most common inciting factors are antipyretic drugs, traditional Chinese medicine and cold medicine. Children with severe liver damage have a high mortality. Rational use of medicine and the concept of the prevention first should been strengthened.

Objective To assess the safety, effectiveness and predictive factors of endoscopic balloon dilatation for the treatment of esophageal stricture and esophageal achalasia in children. Methods 28 patients with esophageal stricture and esophageal achalasia treated by endoscopic balloon dilatation from January 2012 to November 2014 were included. All the patients were divided into two groups, 22 in group A (esophageal stricture) and 6 in group B (esophageal achalasia). All procedures were performed under tracheal intubation and intravenous anesthesia using the 3rd grade controlled radial expansion (CRE) balloon with gastroscope. Outcomes, including success, complications and recurrence data were recorded, and predictors for outcomes were analyzed. Results A total of EBD 57 sessions (1 to 5 per patient, 2.00 ± 1.15) were performed on 28 patients in this study. 22 patients were diagnosed with esophageal stricture (78.57%) and 6 with esophageal achalasia (21.43%). The median age was 25 months (range 0 ~ 150), and female/ male ratio was 12/16. EBD was successful in all the 28 cases. The total success rate was 100.00%. Complications occurred in 6 patients during the dilatation, and no complication in 22 patients. Completely remission of symptoms was seen in 82.14% cases (n = 23), relief in 14.28% (n = 4), non-response in 3.57% (n = 1), and recurrence in 3.57% (n = 1). The stricture diameter before EBD was (6.28 ± 1.77) mm (range 3.0 ~ 10.0 mm), and it was (10.85 ± 2.51) (range 6 ~ 15 mm) after the last EBD. The difference was significant (P < 0.01). There was no significant difference in success, effectiveness, complications and recurrence among the two groups (P > 0.05). The effectiveness of EBD was significantly associated with the diameter and number of strictures (P < 0.05), more complications were seen in the patients with multiple and/or smaller strictures (P < 0.05). In group A, the longer interval between surgery and the first EBD was related to more dilatation in the patients with anastomotic esophageal strictures (P < 0.05). The age and the interval between symptom onset and the first EBD were not the predicting factors for treatment in group B (P > 0.05). Conclusions The results of this study indicated that EBD under general anesthesia was an effective primary treatment in children with esophageal stricture and esophageal achalasia. The diameter and number of stricture were the most important predictive factors for successful clinical outcomes, while the interval between surgery and the first EBD was the most risk factor for EBD sessions in the patients with anastomotic esophageal strictures.

Objective To learn about the etiology , clinical characteristics and prognosis of infants with cholestasis jaundice. Methods The clinical data of 175 cholestatic patients were retrospectively analyzed , then the prognosis was followed-up with telephone. Results After analyzing the etiology , we found that among 175 patients , there were 42 with biliary atresia , of which 19 infants died , 4 recovered well after liver transplanta-tion , 8 had liver cirrhosis waiting for transplantation , 5 recovered well after Kasai Portoenterostomy and 6 lost contact. There were 2 patients with Bile duct dysplasia and 2 with congenital cholangiectasis and they had posi-tive outcomes. And 29 patients with Cytomegalovirus infection also had positive outcome. There were 16 patients with Heredity metabolic diseases , among which 13 patients were with Citrin protein deficiency; 10 had positive outcomes; 2 lost contact and 1 died. There were 3 patients with tyrosinemia , of which one had positive outcome;one lost contact and another got liver cirohosis waiting for liver transplantation. Four patients with TPN-related cholestasis all had positive outcomes. There were still 80 cases with unkown etiology , but 79 had positive out-comes and 1 case lost. The clinical characteristics showed that the infants with cholestatic jaundice often accom-panied by stool color changed , liver and spleen enlargement and so on , and often complicated with pneumonia , hypoalbuminemia and coagulation dysfunction and so on. Conclusion There are many etiologies for infants with cholestatic jaundice. Early diagnosis and early treatment would benefit the prognosis.

Objective To explore fecal bacteria transplantation for the treatment of severe gastrointestinal disease caused by food allergy. Method The therapeutic process of fecal bacteria transplantation for treatment of severe food allergy gastrointestinal disease was retrospectively analyzed, and the related literature was reviewed. Results A 2-year-old boy had onset of intestinal infection and diarrhea was persistent even though he had received adequate anti-infection therapy and supportive treatment. Finally, the patient received the treatment of fecal bacteria transplantation and the symptoms were then improved. No adverse reactions were observed in 2 months of follow-up. In foreign literature, fecal bacteria transplantation in children is mainly applied to clostridium difficile infection (CDI) and inflammatory bowel disease (IBD), with efficiency of 90%- 100% and 55.6% - 100%, respectively. While in the domestic literature, fecal bacteria transplantation in children is mainly used in CDI and antibiotic associated diarrhea, and the effective rate is 100%. No serious adverse reactions were found in all the researches. Conclusion Fecal transplantation is safe and effective in the treatment of children with severe gastrointestinal disease caused by food allergy, but its application in children is not yet mature and needs more in-depth researches.

OBJECTIVETo explore the etiology and clinical characteristics of hypoxic hepatitis (HH) in children.

METHODClinical data of 7 patients with HH in Shenzhen Children's Hospital from January 2011 to March 2014 were retrospectively reviewed.

RESULTSeven cases diagnosed as HH, age from 4 months to 11 years, were admitted to pediatric intensive care unit (PICU), and accounted for 0.32% of patients in PICU during the same period. The primary causes of HH were respiratory failure and cardiac shock caused by severe hand-foot-and-mouth disease, fulminant myocarditis, infant muggy syndrome . Serologic tests for hepatitis B virus, hepatitis C virus, as well as serum antibody and DNA for Epstein-Barr virus and cytomegalovirus were all negative. There was an increase of alanine aminotransferase (ALT) (≥20 time supper limit of normal (ULN), the highest ALT was more than 130 times ULN in all the patients, which was decreased to 2 times ULN from peak within 10 days. There was a significant relationship between ALT and aspartate aminotransferase(AST)in 3 cases(r=1.000, 1.000, and 0.833, respectively, P<0.05), ALT and lactate dehydrogenase (LDH)in 2 cases(r=1.000 and 0.886, respectively, P<0.05), ALT and blood urea nitrogen(BUN)in 1 case(r=1.000, P<0.05), and ALT and creatine kinase(CK)in 1 case(r=0.964, P<0.05). The ALT, AST and LDH returned to normal soon after the primary diseases were controlled.

CONCLUSIONSevere heart failure, hypoxemia, shock, etc. are the leading primary diseases causing HH. The sharp increase in ALT, AST and LDH is the typical laboratory manifestion in HH after the onset, which may decline to normal shortly after the treatment, sometimes complicated with reversible change in BUN or CK.

Alanine Transaminase ; Animals ; Aspartate Aminotransferases ; Child ; Child, Preschool ; Creatine Kinase ; Heart Failure ; Hepatitis ; Herpesvirus 4, Human ; Humans ; Hypoxia ; Infant ; L-Lactate Dehydrogenase ; Respiratory Insufficiency ; Retrospective Studies

To explore the operation methods and indications of the duodenoscopic papillotomy (IEST) with endoscopic nasobiliary drainage (IENBD) for the treatment of duodenal papilla stenosis during the course of common bile duct operation.The clinical data of 219 cases of cholecystolithiasis with choledocholith and the stenosis of papillary underwent endoscopic sphincterotomy (IEST) plus endoscopic nasobiliary drainage (IENBD) in the Second People's Hospital of Chengdu were retrospectively analyzed.It was successful in 198 cases who had the gallbladder and common bile duct stones removed,and endoscopic papillary dissection was performed and the nasobiliary tube was successfully inserted.Nasobiliary drainage was successful in 186 cases (93.9％) of 198 cases.No liquid outflow was observed in nasobiliary drainage in 7 cases (3.5％).Nasal bile duct slipped early in 5 case (2.5％).Primary closure of bile duct incision was completed in 198 cases.It failed in 4 cases (2.0％) who had the bile leakage with primary closure of duct incision.Mild pancreatitis after operation occurred in 3 cases (1.5％).Nose bile duct ligation was performed in 1 case (0.5％).The overall postoperative complication rate was 4.0％ (8/198).IEST + IENBD in open laparotomy was successful in 21 cases.No perforation of intestine and bile duct,bleeding,severe pancreatitis and other complications and death were detected postoperatively in two groups.During the course of laparoscopy and open laparotomy,IEST + IENBD in treating cholecystolithiasis with choledocholith and the stenosis of papillary and primary closure of duct incision after the endoscopic nasobiliary drainage is safe and effective.

Objective To evaluate laparoscopic balloon nasobiliary biliary drainage (LBNBD),vs ureteral catheter drainage in one stage laparoscopy,choledochoscopy and duodenoscopy choledocholithotomy and primary closure of the small calibered common bile duct (diameter 0.3-0.8 cm).Methods During the period of Apr 2010 to Nov 2016 102 cases were enrolled including 50 cases receiving LBNBD and 52 cases using ureteral catheter drainage.Results Between the two groups,LBNBD was superior to ureteral catheter drainage in all the following parameters:the operation time,intraoperative blood loss,postoperative liver function,blood amylase and other laboratory indicators,gastrointestinal function recovery time,gastrointestinal symptoms and electrolyte imbalance,postoperative hospital stay,and bile duct drainage time with all differences statistically significant (P ＜ 0.05).Bile drainage differences during the postoperative first 3 days (averagely 200-400 ml a day) were not statistically different (P ＞ 0.05).Postoperative pancreatitis,bile leakage,and hemobilia were not statistically different (P ＞ 0.05).Conclusions The use of LBNBD is safe and effective in endoscopic choledocholithotomy in cases of small calibered common bile duct.

OBJECTIVETo summarize the clinical features, biochemical change and genetic mutations of a neonate with congenital bile acid synthesis disorder type 2.

METHODSClinical features, blood biochemical index, gene analysis and treatment of the patient were reviewed.

RESULTSThe patient presented with the symptoms of jaundice 3 days after birth but without skin itching. Pale stool was noted. Subsequently, he presented with hepatomegaly, blood coagulation disorders, left cochlear nerve damage, liver cirrhosis and remarkable growth retardation. Serum biochemistries showed that bilirubin and transaminase were elevated, while γ -GT and total bile acid was normal. Abdominal ultrasonography indicated decline of gallbladder contraction. Cholangiography showed normal extra- and intrahepatic bile ducts and patent biliary tract. Liver biopsy showed intrahepatic cholestasis. Gene testing has identified a homozygous mutation in AKR1D1 gene.

CONCLUSIONCongenital bile acid synthesis disorder should be suspected when a neonate has presented with jaundice, elevated bilirubin and transaminase, normal or reduced TBA and γ -GT. Genetic testing and urine mass spectrometry analysis can diagnose congenital bile acid synthesis disorder. Early therapy is crucial to patients with congenital bile acid synthesis disorder.

OBJECTIVE: To construct short hairpin RNA interfering expression vector of TDRG1,and detect the specific interfering effect of TDRG1-shRNA expression vector on NTERA-2 cells. METHODS: Oligos for short hairpin RNA targefing for TDRG1 were designed and connected to the expression vector pGPU6/GFP/Neo to construct the TDRG1 shRNA expression vector. The recombinant plasmid TDRG1-shRNA486, TDRG1-shRNA738, TDRG1-shRNA921 and lipofectamine ™2000 were used to generate and transfect shRNA into NTERA-2 cells. Expression of TDRG1 mRNA was assayed by RT-PCR. RESULTS: TDRG1-shRNA expression vector was successfully constructed. TDRG1-shRNA486 was more effective in the suppression of TDRG1 with significant reduction of TDRG1 mRNA. CONCLUSION TDRG1-shRNA can interfere the expression of TDRG1 in NTERA-2 cells.
Cell Line, Tumor ; Genetic Vectors ; Humans ; RNA Interference ; RNA, Messenger ; RNA, Small Interfering ; Transfection

Objective To analyze our experience in primary closure of common bile duct after laparoscopic bile duct exploration.Methods From June 1992 to March 2018,2 740 patients underwent primary closure of common bile duct after laparoscopic common bile duct exploration in the Second People's Hospital of Chengdu.Results The operations were successfully carried out in 2 534 (92.4％) out of 2 740 patients,of whom 15 patients (0.6％) were converted to open common bile duct exploration.Bile leakage occurred in 113 patients (4.1％).Residual stones were found in 29 patients (1.1％).One patient (0.1％) who had a pancreatic carcinoma died on postoperation day 15.48 patients (1.8％) developed other complications.The total postoperative complication rate was 7.0％ (191/2 740).Conclusion In suitable patients,laparoscopic bile duct exploration with primary closure was feasible,safe and effective.