ABSTRACT
Biliary atresia is an infant disease characterized by obstruction of the internal and ex-ternal bile duct and progressive fibrosing process of the liver. Its etiology and pathogenesis remains un-clear and most infants would die in one year if thereˊs no medical intervention. In this article,we reviewed recent research progress of liver fibrosis in biliary atresia in infants and animal models,which proposed some unsettled problems and research direction in the future.
ABSTRACT
Biliary atresia ( BA) is a pediatric liver disease characterized by progressive inflammation and fi-brosis of both the extrahepatic and intrahepatic bile ducts. Even though Kasai portoenterostomy increases the survival of children with BA, 80% of patients with BA will eventually require liver transplantation, making this condition the leading indication for pediatric liver transplantation worldwide. Currently,the causes of this disease are largely unde-fined and theories of pathogenesis include viral infection,autoimmune-mediated bile duct destruction,and abnormalities in bile duct development.
ABSTRACT
The morbidity of Hirschsprung's disease (HD) is 1/5 000,and HD is the second common congenital digestive tract malformation.The main treatment of HD is surgery.In 1948,Swenson designed a pull-through colorectal resection procedure,which was followed by Duhamel,Soave and Rehbein procedures.All of these procedures are all trans-abdominal approach,severe complications are common.With the development of laparoscopy,it is gradually applied to HD surgery.In 1998,Torre proposed the one-stage endorectal pull-through procedure,which further reduced the operation wound.Subsequently,natural orifice transluminal endoscopic surgery(NOTES) and transumbilical laparoscopy emerged.All the developments are for the purpose of minimal invasion,with the advantages of less pain,shorter hospital stay,a better cosmetic result and at the same time,surgical effect guaranteed.
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Objective To investigate the correlation between the remnants of extrahepatic bilie duct with the prognosis of biliary atresia after Kasai operation.Methods From Sep.2005 to Sep.2011,93 cases of biliary atresia [51 boys and 42 girls,ages at operation:61 days(40-121 days)] in Tongji Hospital Affiliated of Tongji Medical College of Huazhong University of Science and Technology were operated with Kasai procedure,and the diagnosis of type Ⅲ biliary atresia was based on the cholangiography and the intraoperative findings.According to whether there was fibrotic remnant plates of porta hepatis or not,type Ⅲ biliary atresia cases were grouped into 2 subtypes:subtype A,in which there was fibrotic remnant plates of porta hepatis (n =80) ; subtype B,in which there was no fibrotic remnant plates,but solid cords structure like hepatic duct was observed(n =13).Specimens of the remnants were colleted in operation and pathologic section detected the degree of bile ductular proliferation in the remnants.Postoperatively,all of the patients were followed up.The contents included the cholangitis occurrence rate,the jaundice disappearance rate and survival rate during postoperative 2 years.Results The patients had more bile ductular proliferation in subtype A (x2 =18.49,P ＜0.05).There was no significant difference in incidence of cholangitis in postoperative 1 month between the 2 groups (x2 =0.01,P ＞ 0.05).The jaundice disappearance rate in postoperative 6 months of subtype A was higher than the rate of subtype B(x2 =9.19,P ＜ 0.05).The survival rate during postoperative 2 years of subtype A was higher than the rate of subtype B(x2 =4.49,P ＜ 0.05).Conclusions There are higher jaundice disappearance rate and 2 years survival rate in type Ⅲ biliary atresia with the fibrotic remnant plates of porta hepatis,which suggest that there is more bile ductular proliferation in the extrahepatic remnants which is good for biliary drainage in subtype A after Kasai operation,and it can slow down hepatic injury and have a long-term good life quality.
ABSTRACT
Intestinal obstruction in children is a common acute abdomen in pediatric surgery.The causes of obstruction are complex.Intestinal obstruction not only impacts on the intestine both in anatomy and function but also causes systemic physiological disorder.So accurate and timely diagnosis of intestinal obstruction is obviously favorable for the treatment.And it is also critical in rescuing serious state and avoiding death.
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Objective: To evaluate the omrphological and histochemical properties of cartilage matrix of children with pectus excavatum(PE). Methods: The collagen of cartialge matrix of 19 PE and of 14 age-matched children was examined by transmission electron microscopy(TEM) and immunohistochemical staining examination. Meanwhile, the proteoglycan was studied by PAS and Safranin-O staining examination. All of the results of stanining were also analysed by the GT-2 model of image analysis software. Results: Compared with control group, although the intensity of type II collagen was intact, unequally distribution of collagen had been found by the TEM and immunohistochemical staining examination. The intensity and distribution of proteoglycan of PE group remained normal found by the PAS and Safranin-O staining examination. Conclusion: Although the intensity and distribution of proteoglycan was intact, as well as intensity of collagen, the distribution of collagen in PE was abnormal, and this change may possibly be related to the defectiveness of costal cartialge of PE.
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Objective To evaluate the diagnosis and treatment of intestinal neuronal dysplasia type B (IND) in childhood. MethodsForty-five patients underwent preoperative barium enema examination, 23 patients underwent electromanometry, and mucosal biopsy and immunohistochemical staining for S100 protein were performed in 17 cases. All 45 patients underwent resection of the invalid segment with coloproctostomy. Whole layer was sampled on several sites of the resected segment and examined by two independent pathologists. All patients were followed up from 3 months to 9 years (mean 4.6 years).ResultsTwenty eight patients were complicated with Hirschsprung′s disease, one patient with hypogangliosis, and isolated IND was diagnosed in the other 16 children. The narrowed distal segment with proximal dilatation was merely noted in 4 children with isolated IND. Internal sphincter relaxations were missing in 6 children with isolated IND. The indicative diagnosis might be merely gained in 7 patients by the mucosa biopsy. The correct diagnosis can be established by whole layer biopsy of the resected segment. Three children with enterocolitis after operation were cured by conservative treatment. One patient suffering from postoperative sluice syndrome underwent second resection. Postoperative continence was achived in all patients. ConclusionThe correct diagnosis of IND can be obtained by biopsy of whole layer, and resection of invalid bowel segment with coloproctostomy is the choice of therapy.
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Objective To evaluate the long-term results of partial internal sphincterectomy for the treatment of internal anal sphincter achalasia in childhood. Methods The clinical,radiographic,manometrical and histochemical data of 6 cases were reviewed retrospectively. All patients had received partial internal sphincterectomy and were followed-up for 2 to 8 years. Results All patients presented with severe constipation with or without soiling. No stenosis zone of intestine could be noted in 3 patients by barium enema examination. The rectoanal inhibition reflex on rectal balloon inflation was absent in all patients. The normal acetylcholinesterase activity on rectal biopsies was demonstrated by histochemical staining. Ganglion cells within internal anal sphincter was noted in all cases. On follow-up,all patients regained regular bowel habits and are not on any laxatives. Conclusion The long term results of partial internal sphincterectomy for the treatment of internal anal sphincter achalasia in childhood are satisfactory.