原 普二夫

It is difficult to discriminate between choledochal cust with obstructive jaundice and biliary atresia with a cyst at the porta hepatis in neonates or young infants. This review evaluates whether it is possible to differentiate between these two diseases. We here also provide an overview of our experience with type I cyst biliary atresia patients. Among all the biliary atresia infants who we treated, the infants who were diagnosed with type I cyst biliary atresia were identified and reviewed for their management and outcome. The clinical course and management in different reports were reviewed and compared to the cases presented to our institution. Among the 220 biliary atresia cases, 11 (5 %; male/female: 4/7) were diagnosed to be type I cyst biliary atresia. Two received hepaticoenterostomy and nine received hepatic portoenteros. Three patients had severe late complications; overall, nine (81.8 %) were alive with their native liver and without jaundice. Patient with choledochal cust are likely to represent larger cysts and inversely, smaller, static, anechoic cysts are more likely to represent cystic biliary atresia. However, exceptional cases were yet presented, and a definitive diagnosis may not be reached. Thus a complete differentiation between choledochal cust from type I cyst biliary atresia is yet hard to reach.

Main indications for liver transplantation in the pediatric population include biliary atresia and inherited metabolic diseases. The present study evaluated whether there are differences between pediatric patients undergoing living-related liver transplantation due to the two diseases in terms of their oxidative and immunological status Pduring their regular outpatient follow-up visits. A clinical outpatient study measuring serum oxidative stress index (calculated as serum oxidant/antioxidant ratio, in the form of serum total hydroperoxide/serum biological antioxidative potential), serum terminal complement component 5a, as an indicator of complement activity and immunological status, and transforming growth factor-β1, as a marker of liver fibrosis, in 16 patients (6 males and 10 females, 2.5-15 years old) who received living-related liver transplantation due to inherited metabolic diseases (n=6 in the form of propionic acidemia [n=1], methylmalonic acidemia [n=1], arginase deficiency [n=1], tyrosinemia [n=2], and glycogen storage disease type 1b [n=1], with an age range of 2.4-14.6 years old) and due to biliary atresia ([n=10], with an age range of 2.9-14.5 years old). Serum oxidative stress index, complement component-5a, and transforming growth factor-β1 were significantly higher in the inherited metabolic diseases group than in the biliary atresia group. In all patients, serum oxidative stress index correlated positively with complement component-5a and transforming growth factor-β1. Patients who receive living-related liver transplantation due to inherited metabolic diseases are prone to higher oxidative stress, complement activity, and serum transforming growth factor-β1.

Throughout the history of surgery for pectus excavatum (PE), the Nuss procedure and open repair have been performed with many modifications, with most of these procedures using a metal bar. However, the use of a metal bar has several drawbacks. Thus, we aimed to develop a procedure that did not require a metal bar. Through our experience of 426 pediatric cases that underwent various procedures for open repair of PE at Nagoya City University, we arrived at the current procedure that we describe herein. We have evaluated this procedure by review of clinical results and deformity indices (Haller's, steepness, excavation volume, and asymmetry index). The latest and current procedure that supports the sternum with a bridge constructed by the 4th or 5th costal cartilages is associated with fewer complications, a lower re-operation rate, and striking improvement in the indices examined. Our current open-repair procedure that does not require a metal bar is recommended for correction of deformities of PE in children.

ABO-incompatible liver transplantation (LTx) is becoming more common in response to the paucity of liver allografts. Several studies have expressed concern about the effect of ABO compatibility on graft survival. To evaluate the differences in serum cytokine levels between ABO-incompatible (ABO-i) and ABO-compatible (ABO-c; includes ABO-compatible and identical) pediatric LTx recipients during regular outpatient follow-up. Note that, in the field of organ transplantation, transplants are categorized as incompatible, compatible or identical; accordingly, these are the terms we use in the paper. A clinical outpatient study measuring serum transforming growth factor (TGF)-beta 1, interferon (IFN)-gamma, interleukin (IL)-2 and IL-10 in 43 living related liver transplantation (LRLT) recipients, of whom 36 received ABO-c LRLT (34 were ABO-identical and 2 were non-identical) and 7 ABO-i LRLT. Serum glutamic pyruvic transaminase, glutamic oxaloacetic transaminase, gamma-glutamyl transpeptidase, alkaline phosphatase, lactate dehydrogenase and bilirubin were measured as part of the patients' regular follow-up visits. There were no differences between the ABO-c and ABO-i groups in terms of recipient's age [mean 12.6 vs. 11.1 years (y)], post-LTx duration (mean 7.3 vs. 7.3 y), donor's age (mean 35.5 vs. 34.6 y), body weight (28.9 +/- A 2.9 vs. 27.9 +/- A 6.9 kg), or gender (19 female and 17 male vs. 4 female and 3 male). Serum TGF-beta 1, IFN-gamma and IL-2 were significantly higher in the ABO-i group than in the ABO-c group. IL-10, however, did not differ between the two groups. There was a tendency toward higher gamma GTP levels in the ABO-i group, but this difference did not reach significance. ABO-incompatible LRLTx patients have higher serum TGF-beta 1, IFN-gamma and IL-2 levels as measured at regular outpatient visits. As a result, they face a higher risk of T-helper 1 cell polarization, which could make graft rejection more likely.

Background Oxidative stress has been suspected to influence graft survival and prognosis in pediatric recipients of living related liver transplantation (LRLT). Purpose We determined the oxidative status of pediatric LRLT recipients during their regular outpatient follow-up visits, and looked for a relationship between oxidative status and post-liver transplantation (post-LTx) duration. Patients The study included 43 patients (20 males and 23 females) between the ages of 1.6 and 25.1 years (median 10.7 years) who had undergone LRLT from 5 months to 17.5 years (median 7 years) prior to the study, between the ages of 1.2 and 14.4 years (median 3.5 years). Methods Serum glutamic pyruvic transaminase (GPT), glutamic oxaloacetic transaminase (GOT), gamma-glutamyl transpeptidase (gamma-GTP), alkaline phosphatase (ALP), lactate dehydrogenase (LDH), direct bilirubin and choline-esterase were measured as part of the patients' regular follow-up visits. Serum total hydroperoxide (TH) and biological anti-oxidative potential (BAP) were measured using the free radical analytic system which requires 20 mu l of serum and 10 min of processing time for each sample. Oxidative stress index (OSI) was calculated as the ratio of TH to BAP. Results Serum OSI correlated positively with serum levels of GOT, GPT, LDH, ALP, gamma-GTP and direct bilirubin. Serum OSI, TH, LDH, ALP and GOT correlated negatively with post-LTx duration. Serum BAP correlated positively with post-LTx duration. Serum TH correlated positively with serum GOT and gamma-GTP, but negatively with serum BAP. Conclusions (1) The OSI, which can be calculated based on data acquired through a simple outpatient procedure, can serve as an index of our patients' laboratory results and oxidative status. (2) The LRLT recipients in our study were at risk for oxidative stress early in the post-operative period, but this risk subsided with time.