Complementary Indicators for Diagnosis of Hepatic Vein Stenosis After Pediatric Living-donor Liver Transplantation.

INTRODUCTION: Although hepatic vein stenosis after liver transplantation is a rare complication, the complication rate of 1% to 6% is higher in pediatric living-donor liver transplantation than that in other liver transplantation cases. Diagnosis is very important because this complication can cause hepatic congestion that develops to liver cirrhosis, graft loss, and patient loss. However, this is unlikely in cases where there are no ascites or hypoalbuminemia. OBJECTIVES: Eleven of 167 patients who had undergone pediatric living-donor liver transplantation were identified in the outpatient clinic at Jichi Medical University as having suffered from hepatic vein stenosis, and were enrolled in the study. METHODS: We conducted a retrospective study in which we reviewed historical patient records to investigate the parameters for diagnosis and examine treatment methods and outcomes. RESULTS: The 11 patients were treated with 16 episodes of balloon dilatation. Three among these received retransplantation and another 2 cases required the placement of a metallic stent at the stenosis. Histological examination revealed severe fibrosis in four of nine patients who had a liver biopsy, with mild fibrosis revealed in the other five grafts. Furthermore, hepatomegaly and splenomegaly diagnosed by computed tomography, elevated levels of hyarulonic acid, and/or a decrease in calcineurin inhibitor clearance were found to be pathognomonic at diagnosis, and tended to improve after treatment. CONCLUSIONS: Diagnosis of hepatic vein stenosis after liver transplantation can be difficult, so careful observation is crucial to avoid the risk of acute liver dysfunction. Comprehensive assessment using volumetry of the liver and spleen and monitoring of hyarulonic acid levels and/or calcineurin inhibitor clearance, in addition to some form of imaging examination, is important for diagnosis and evaluation of the effectiveness of therapy.

Iron overload after pediatric liver transplantation: a case report.

Iron is an essential nutrient for living cells; however, an excessive accumulation of iron leads to organ damage and directly affects systemic immunity. Iron overload is clinically classified as hereditary or secondary. Most of secondary iron overload is caused by frequent blood transfusions because there is no active mechanism to excrete iron from the body. As recommended in various guidelines, chelation therapy is effective for reducing iron burden and improving organ function. There have been few reports on iron overload through blood transfusion during the perioperative period of liver transplantation. This report presents a case of iron overload due to repeated transfusions after pediatric liver transplantation managed by chelation therapy. The patient, an 11-month-old female with biliary atresia, underwent living donor liver transplantation. She revealed refractory anemia and required frequent blood transfusion. Both serum ferritin and transferrin saturation tended to increase after repeated transfusions, leading to secondary iron overload. Iron chelation therapy was started to prevent progression to organ failure and infection due to iron overload, and yielded a favorable outcome. It is crucial to consider the possibility of secondary iron overload and to achieve early detection and treatment to avoid progression to irreversible organ damage.

Effect of fat supplementation on the maintenance of gut integrity in elemental diet-fed rats.

AIMS: The aims of this study were to investigate the mechanism of atrophic change in ileal mucosa supplied with an elemental diet (ED) and to assess the value of supplemented fat emulsion in the prevention of atrophic change. MATERIALS AND METHODS: In experiment 1, 25 male Wistar rats with a body weight of 160-180 g were divided into 3 groups. The first group was fed regular rat chow (Control group, n=5). The second group was given ED containing 0.6% long-chain triglycerides (ED group, n=10). The third group was provided with fat-enriched ED (FED) containing 3.5% long-chain triglycerides (FED group, n=10). Each group received an isocaloric diet (300 kcal/kg/day). 4 weeks later, after euthanization, ileal samples were taken for light and electron microscopic examinations. The morphological changes of the intestinal mucosa and the crypt cell proliferation rate (CCPR) were determined. In experiment 2, to determine the site of fat absorption, 9 rats were fed ED for 1 week. After 24 h of food deprivation, all rats were given 2 ml of FED through a gastric tube. Then 1, 2, and 3 h(s) later, groups of 3 rats each were euthanized, and the total small intestine was obtained from each rat. The proximal and distal jejunum and distal ileum were stained with oil red O. RESULTS: In experiment 1, the samples had almost the same morphological appearance irrespective of the type of feeding. The CCPR was significantly diminished in the ED group compared with the Control group, while there was no statistical difference between the FED and Control groups. In experiment 2, the oil red O stain was positive in the proximal and distal jejunum, but was completely negative in the distal ileum. CONCLUSIONS: The introduction of ED does not soon result in an atrophic morphological change of the ileum but will decrease the CCPR. An additive fat emulsion which was rapidly absorbed by the distal jejunum could play a role in maintaining ileal mucosa integrity through some mechanism independent of absorption.

Meningoencephalocele associated with Tripterygium wilfordii treatment.

We treated a male infant with occipital meningoencephalocele associated with the taking of Tripterygium wilfordii. The infant was delivered normally at 38 weeks of gestation with a huge cystic mass protruding from the occiput. He was diagnosed with occipital meningoencephalocele and cerebellar agenesis. His mother had taken T. wilfordii for rheumatoid arthritis early in her pregnancy. T. wilfordii is a herbal medicine used for rheumatoid arthritis and male contraception. Since its toxicity is high and its use during pregnancy is restricted, it is the most likely cause of this infant's anomalies.

The effect of carnitine on the metabolism of valproic acid in epileptic patients.

The half-life of valproic acid (VPA) was studied in 8 epileptic and severely mental retarded patients before and after one month of carnitine supplementation. Serum carnitine concentration was significantly decreased and VPA half-life was prolonged especially in adult patients before carnitine supplementation. After the treatment with carnitine, serum carnitine concentration was increased, and prolonged half-lives of VPA were corrected near to the normal range (from 12.2 +/- 4.2 hr to 9.7 +/- 2.2 hr; p < 0.05). Controlled state of epilepsy was unchanged during the short period of observation.

[Total parenteral nutrition in surgical treatment of infants: recent progress and problems].

The frequency of TPN in surgical infants was 242/686 (37.8%) in neonates and 260/2693 (9.7%) in older infants for the past 15 years. The frequent indications of TPN were intestinal atresia, Hirschsprung's disease and esophageal atresia in the neonatal period, and Hirschsprung's disease, pyloric stenosis and biliary atresia in infancy. The effectiveness of TPN was impressively indicated by better survival rate in the infants with massive intestinal resection received TPN. An amino acid solution (N1-2) was newly devised, based on the analysis of plasma aminograms in 36 infants received TPN with a commercial amino acid solution (Proteamin), and theoretical considerations. It was more useful solution for TPN in 12 young infants. Urinary phosphorus was a considerable parameter for the administration of Vitamin D in TPN. The incidence of TPN-induced hepatic dysfunction was significantly referred to the amount of amino acid in TPN. Closed infusion system with a soft bag and triple bacterial filters in the line was effective for the prevention of sepsis caused by central venous catheter. The nutritional care was important in the treatment of infants with biliary atresia, because generally they had some nutritional defects such as essential fatty acid deficiency, insufficient amino acid metabolism and zinc deficiency. The nutritional care in infants with advanced neuroblastoma favorably altered the course of the disease.