Klotho coreceptors inhibit signaling by paracrine fibroblast growth factor 8 subfamily ligands.

It has been recently established that Klotho coreceptors associate with fibroblast growth factor (FGF) receptor tyrosine kinases (FGFRs) to enable signaling by endocrine-acting FGFs. However, the molecular interactions leading to FGF-FGFR-Klotho ternary complex formation remain incompletely understood. Here, we show that in contrast to αKlotho, βKlotho binds its cognate endocrine FGF ligand (FGF19 or FGF21) and FGFR independently through two distinct binding sites.

Dietary and genetic evidence for enhancing glucose metabolism and reducing obesity by inhibiting klotho functions.

Klotho is a multifunctional protein involved in numerous biological functions, ranging from mineral ion metabolism to signaling activities. Recent studies have identified klotho as a target gene for peroxisome proliferator-activated receptor-γ (PPAR-γ), a master regulator of adipocyte differentiation, and an adipogenesis-promoting factor. In a similar line of observation, eliminating klotho function from mice resulted in the generation of lean mice with almost no detectable fat tissue.

Long-term outcomes and the quality of life of Hirschsprung disease in adolescents who have reached 18 years or older--a 47-year single-institute experience.

Background And Aims: Hirschsprung disease is considered to be curable when performing definitive surgery. However, the postoperative bowel function is not always satisfactory. The long-term postoperative function of bowel evacuation and the quality of life of such patients are considered to be important.

Effectiveness of basic endoscopic surgical skill training for pediatric surgeons.

Purpose: Pediatric surgeons require highly advanced skills when performing endoscopic surgery, but their experience with such cases tend to be limited in comparison to general surgeons. The aim of this study was to evaluate the effectiveness of basic endoscopic surgery training for less-experienced young pediatric surgeons and then compare their skills with those of general surgeons.

Methods: The surgeons (n = 477) subjected to this study underwent a 2-day endoscopic skill training program, consisting of lectures, box training, VR simulator training, tissue training, and live tissue training.

Clinical features of Hirschsprung's disease associated with Down syndrome: a 30-year retrospective nationwide survey in Japan.

Purpose: To identify the clinical features in diagnosis and treatment of Hirschsprung's disease (HD) associated with Down syndrome (DS), the authors retrospectively analyzed data for patients with DS from the past 3 nationwide surveys in Japan. This survey was already approved by the Japanese Society of Pediatric Surgeons.

Methods: Patient data were collected in 3 phases-phase I (1978-1982), n = 47; phase II (1988-1992), n = 79; and phase III (1998-2002), n = 90.

Direct vasodilative effect of FK506 on porcine mesenteric artery in small bowel transplantation.

Background: Tacrolimus (FK506) is widely used as an immunosuppressive drug in small bowel transplantation. However, its precise effects on the vascular tone of the transplanted organ have not been studied. This study aimed to clarify the effects of FK506 on the porcine mesenteric artery.

Mechanism of Rho-kinase-mediated Ca2+-independent contraction in aganglionic smooth muscle in a rat model of Hirschsprung's disease.

Purpose: Lack of ganglion cells is the main cause of bowel movement disorder in Hirschsprung's disease. Because smooth muscle is the primary organ, the properties of intestinal smooth muscle need to be investigated. We therefore investigated the reactivity of the contractile system and the mechanism of contraction in aganglionic intestinal smooth muscle.

Total colonic aganglionosis with or without small bowel involvement: a 30-year retrospective nationwide survey in Japan.

Purpose: We conducted a nationwide survey in Japan, to identify recent trends in the diagnosis and treatment of patients who had total colonic aganglionosis with or without small bowel involvement (TCSA). These data were compared with 2 similar studies conducted within the last 30 years.

Methods: Patient data were collected in 3 phases; phase I included 135 patients between 1978 and 1982; phase II, 107 patients between 1988 and 1992; and phase III, 101 patients between 1998 and 2002.

Intracellular calcium mobilization of the aganglionic intestine in the endothelin B receptor gene-deficient rat.

Background And Aim: Up to now, numerous reports have analyzed the pathogenesis of Hirschsprung's disease (HD) by means of physiologic, pathologic, or molecular biologic methods. However, very little is still known about the smooth muscle cell itself. The endothelin B receptor gene-deficient (EDNRB(-/-)) rat, which is suitable for research of HD, has an aganglionic segment of the total colon.

Morphological and physiological changes of interstitial cells of Cajal after small bowel transplantation in rats.

Intestinal dysmotility has been reported to be associated with a decreased number of interstitial cells of Cajal (ICCs). However, the chronological changes in ICCs after small bowel transplantation (SBT) have not yet been elucidated. In this study, we aimed to evaluate the chronological change of ICCs after SBT.

New classification of hypoganglionosis: congenital and acquired hypoganglionosis.

Aim: Hypoganglionosis has been associated with fewer intestinal ganglion cells. However, current reports questioned the validity of this clinical entity. The aim of this study is to demonstrate the existence of hypoganglionosis.