Intrahepatic Cystic Lesions in Children with Biliary Atresia After Kasai Procedure
Intrahepatic cystic lesion (ICL) is a common complication for biliary atresia post-Kasai portoenterostomy. The purpose of this study was to review the cases in our hospital and assess the correlation between characteristics of ICL and clinical outcomes.
Matrix metalloproteinase-7 (MMP-7) shows"good accuracy" in diagnosing biliary atresia (BA) and"holds great promise" as a marker to differentiate BA from non-BA, a single-center validation study in Shanghai reveals.Reuters Health Information
AbstractThoracoscopic repair (TR) of esophageal atresia (EA) has been performed with increasing frequency over the last two decades, with the expectation of improved outcomes by avoiding thoracotomy. To understand the current practice and outcomes of TR of EA, we reviewed the relevant literature, including 15 case series, 7 comparative studies, and 3 meta-analysis comparing TR with conventional open repair (COR). Most of the studies had a retrospective design and small numbers of patients. Although the evidence level is low because of the lack of prospective studies, this review found that TR is as safe as COR, with compar...
The objective of this study is to present an anatomical defect of duodenal atresia using a rabbit model and evaluate the preliminary experience for the training of surgical skills with pediatric surgeons. Adult white New Zealand male rabbits weighing 3.0 to 4.5 kg were used to create the defect. To simulate the bottom of the dilated blind pouch, the gastric antrum of the rabbit was obliterated using a 2-0 Prolene suture, and the cecal appendix was dissected to simulate the continuation of the duodenum. Participants performed laparoscopic duodenal atresia repair in this animal model using the iPhone trainer. Thirteen pediat...
Conclusion In free carnitine, DB, and CBMS/TBMS tests, blood concentrations are elevated in all infants with BA. However, they may not be elevated while they are newborns. These tests will result in high false negatives or positives. Thus, they should not be used as newborn screening tests for BA due to their lower sensitivity and specificity. [...] Georg Thieme Verlag KG Stuttgart · New YorkArticle in Thieme eJournals: Table of contents | Abstract | Full text
CONCLUSION: LDLT is a safe procedure in a selected group of BA patients with PH, however, further long-term clinical investigations and mechanical researches are needed. PMID: 31588186 [PubMed - in process]
Cell Death &Differentiation, Published online: 07 October 2019; doi:10.1038/s41418-019-0428-0Retraction Note: Interactions between Th1 cells and Tregs affect regulation of hepatic fibrosis in biliary atresia through the IFN-γ/STAT1 pathway
Condition: Duodenal Atresia Intervention: Procedure: Performance of laparoscopic duodenal atresia repair Sponsor: Boston Children’s Hospital Not yet recruiting
Plectin is a giant multifunctional cytolinker protein (500 kDa) expressed in several tissues with essential roles in striated and smooth muscles, epithelia and nerve. It is a component of hemidesmosomes, desmosomes and focal adhesion contacts where it interacts with actin and intermediate filaments. Pathogenic variants in the PLEC1 gene lead to a group of diseases including epidermolysis bullosa (simplex ogna with or without muscular dystrophy, myasthenic syndrome, or pyloric atresia) and isolated limb-girdle muscular dystrophy.