A Case of Liposarcoma With Peritonitis Due to Jejunal Perforation

A 21-year-old man, who had been treated for congenital dilatation of the bile duct 13 years previously, presented with an acute abdomen. The physical examination suggested peritonitis, and an emergent laparotomy was performed. A perforation was foundin the jejunum approximately 100 cm distal to the ligament of Treitz, followed by resection of a 60-cm jejunal segment. No tumorous lesions were found during the operation, and the resected jejunal segment showed only focal myxomatous thickening of the serosa. Despite intensive therapy, he died of uncontrollable septic shock 2 days after the operation. Unexpectedly, however, histological examination revealed a liposarcoma, showing an unclassifiable histology. From the distribution of the lesion and the histological findings, it is thought that a primary lesion was somewhere else, covered by severe adhesions due to the previous operation, and that the tumor cells spreading from it could have caused the jejunal perforation through vascular involvement. Although extremely rare, liposarcomas in the abdomen can cause intestinal perforation. It is important for both clinicians andpathologists to carefully investigate the cause of an unusual clinical presentation such as intestinal perforation

Fish mesonephric model of polycystic kidney disease in medaka (Oryzias latipes) pc mutant

Fish mesonephric model of polycystic kidney disease in medaka (Oryzias latipes) pc mutant.BackgroundPolycystic kidney disease (PKD) is a common hereditary disease. A number of murine and zebrafish mutants have been generated and used for the study of PKD as metanephric and pronephric models, respectively. Here, we report a medaka (Oryzias latipes) mutant that develops numerous cysts in the kidney in adulthood fish in an autosomal-recessive manner as a mesonephric model of PKD.MethodsThe phenotypes of the medaka pc mutant were described in terms of morphologic, histologic, and ultrastructural features. The pc see-through stock was produced by crossing a pc mutant and a fish from the see-through stock and used for observing the kidney through the transparent body wall of a live fish.ResultsThe mutant developed bilateral massive enlargement of the kidney in adulthood. They sexually matured normally within 2 months of age and died within 6 months of age. The affected kidney was occupied by numerous, fluid-filled cysts, which were lined by attenuated squamous epithelial cells. Developmentally, cystic formation began in the pronephros in 10-day-old fry and in the mesonephros in 20-day-old fry at the microscopic level. The pc see-through stock was useful in observing disease progression in live fish.ConclusionThe kidney disorder that develops in the medaka pc mutant is a mesonephric counterpart of PKD, particularly an autosomal-dominant PKD, based on its morphologic, histologic, and ultrastructural features, and slow progression

Polycystic Kidney Disease in the Medaka (Oryzias latipes) pc Mutant Caused by a Mutation in the Gli-Similar3 (glis3) Gene

Polycystic kidney disease (PKD) is a common hereditary disease in humans. Recent studies have shown an increasing number of ciliary genes that are involved in the pathogenesis of PKD. In this study, the Gli-similar3 (glis3) gene was identified as the causal gene of the medaka pc mutant, a model of PKD. In the pc mutant, a transposon was found to be inserted into the fourth intron of the pc/glis3 gene, causing aberrant splicing of the pc/glis3 mRNA and thus a putatively truncated protein with a defective zinc finger domain. pc/glis3 mRNA is expressed in the epithelial cells of the renal tubules and ducts of the pronephros and mesonephros, and also in the pancreas. Antisense oligonucleotide-mediated knockdown of pc/glis3 resulted in cyst formation in the pronephric tubules of medaka fry. Although three other glis family members, glis1a, glis1b and glis2, were found in the medaka genome, none were expressed in the embryonic or larval kidney. In the pc mutant, the urine flow rate in the pronephros was significantly reduced, which was considered to be a direct cause of renal cyst formation. The cilia on the surface of the renal tubular epithelium were significantly shorter in the pc mutant than in wild-type, suggesting that shortened cilia resulted in a decrease in driving force and, in turn, a reduction in urine flow rate. Most importantly, EGFP-tagged pc/glis3 protein localized in primary cilia as well as in the nucleus when expressed in mouse renal epithelial cells, indicating a strong connection between pc/glis3 and ciliary function. Unlike human patients with GLIS3 mutations, the medaka pc mutant shows none of the symptoms of a pancreatic phenotype, such as impaired insulin expression and/or diabetes, suggesting that the pc mutant may be suitable for use as a kidney-specific model for human GLIS3 patients

Study of stress in dairy cattle during student practical training on a farm

We investigated changes in cortisol (COR) concentration, which is well known as an index of stress in the serum of dairy cattle. The COR concentrations in serum obtained from dairy cattle were collected during practical training of first-year students on a farm attached to the Nippon Veterinary and Life Science University. Mean COR concentration in serum determined after practical training was significantly higher (P < 0.001) than that in serum collected before training. Discriminant analysis was used to classify the relation between COR concentration of serum collected before and after practical training. In conclusion, the data was bipartite according to the percentage of rise (rise rate) of COR concentration. Although the percentage of the rise was more than 300% in the high-rise-rate group, there was a significant negative correlation (P < 0.05) between age and COR concertation. It was thought that the high-rise-rate group has a chance to decrease stress after more experience. In contrast, the low-rise-rate group included 3 cattle indicating high COR concentration before and after practical training. Those 3 cattle were thought to be stressed easily. It is suggested that there was individual difference to stress