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N-Methyl-2-methoxymethylanilines 1bearing various 5-substituted-pyrimidin-2-yl groups were prepared, and their rotational behaviors were explored in detail. It was revealed that the rotational barriers around two N–Ar bonds increase in proportion to the electron-withdrawing ability of substituents X at the 5-position.

The addition of methanesulfonic acid to N-(2,6-dimethylpyridin-4-yl)-N-methyl-2-iso-propylaniline led to the selective protonation of the pyridine nitrogen atom, resulting in a significant deceleration of the rotation rates around both N-pyridyl and N-(i-Pr)phenyl bonds through a relayed brake mechanism.

We report here two cases of Kasai type IIIa biliary atresia (BA), associated with pancreaticobiliary maljunction (PBM). Both cases were referred to our hospital because of hyperbilirubinemia and diagnosed with BA preoperatively by abdominal ultrasonography and biliary scintigraphy. They underwent Kasai portojejunostomy at 49 and 37 days old, respectively. Intraoperative cholangiographies performed from their gallbladders clarified Kasai type IIIa BA with PBM. Laboratory test of colorless fluid from the gallbladder of one patient showed high level of lipase (460 U/L). Reported cases of BA associated with PBM are rare and this is a second report showed elevated level of a pancreatic enzyme in bile. The findings in our cases indicate the pathogenesis of biliary anomaly in association with PBM.

Congenital urethroperineal fistula is an extremely rare anomaly in which a second channel rises from the prostatic urethra and opens onto the perineum. This entity resembles the hypospadiac type of urethral duplication, but resection of the ventral channel is simple and curative.

Epigastric heteropagus (EH) refers to asymmetrically conjoined twins in whom an incomplete parasite is attached to the autosite's epigastric region from the xiphisternum to the umbilicus. We herein report an extremely rare case of EH in which unexpected rapid recurrence occurred 22 months after excision of the parasite. The recurrent mass comprised the intestinal wall with peristalsis-like movement, urogenital tissues, and hepatic tissue of the parasite. The recurrence was caused by a remnant of the parasite that extended from the autosite's xiphisternum to intra-abdominal cavity.

•Herlyn–Werner–Wunderlich syndrome is a rare, congenital Müllerian anomaly.•The trilogy is uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis.•Presentation is usually cyclical abdominal pain and a vaginal mass after menarche.•Resection of hymenal or vaginal septum at an early stage can relieve acute symptoms.

•The cyst is located in the neck, and suprasternal, presternal, and scapular areas.•The cyst is covered with pseudostratified, ciliated, columnar epithelium.•Smooth muscle bundles, mucinous glands, or cartilage is present.

•A splenic abscess ruptured spontaneously during conservative treatment.•A surgical procedure for a splenic abscess should be considered at an early stage.•Bacteroides fragiliswas isolated from the ascitic fluid.

Low-grade fibromyxoid sarcoma (LGFMS) is an extremely rare neoplasm classified as a fibroblastic soft tissue tumor. It has been described by its characteristic deceptively benign histological appearance with metastatic potential during long-term follow-up. We herein describe an 8-year-old boy with LGFMS located in the internal oblique muscle. Histological findings of the resected specimen included contrasting fibrous and myxoid areas with a swirling, whorled growth pattern; bland, benign-appearing fibroblastic spindle cells; and large collagen rosettes. Immunohistologic examination revealed mucin 4 positivity. The surgical margin of the tumor was obscure; therefore, we performed an additional resection 2 months later with a 1 cm margin from the edge of the tumor. No recurrence or metastasis was identified throughout 1 year of follow-up.

Adult Hirschsprung's disease is a rare motor disorder of the gut that is frequently misdiagnosed as refractory constipation. We describe a 30-year-old patient with adult Hirschsprung's disease with a history of chronic constipation requiring daily enema. Barium enema and rectal biopsy showed short segment-type Hirschsprung's disease with a grossly distended sigmoid colon with fecal retention. Staged operations, including the Duhamel-GIA method as a definitive surgery, completely resolved the patient's symptoms. There were no complications by the end of a 7-year follow-up. Adult Hirschsprung's disease should be considered in the differential diagnosis of cases where adult patients present with chronic constipation. Despite technical difficulty of the modified Duhamel-GIA procedure due to chronic fibrous changes of the rectum, it is a feasible surgical option for treating cases of adult Hirschsprung's disease.

Congenital prepubic sinus (CPS) is an extremely rare anomaly, which is often associated with purulent discharge from a midline opening overlying the pubis. CPS was first described by Campbell et al. in 1987 and they suggested that it might represent a variation in normal embryological development. Several theories have been proposed regarding the pathogenesis of CPS. However, the etiology of CPS is still unclear because the anatomical and pathological features of CPS often differ from each other. We report a case of CPS and review the literature to improve the global understanding of CPS.

Light and electron microscopic studies were made of the renal glomeruli of 11 control Chinese hamsters, 4 siblings of diabetic hamsters, and 6 with spontaneous diabetes of varying age of onset, duration, and severity. Aging alone was associated with an increase in mesangial matrix and some degree of capillary dilatation and thickening of the basement membrane. In severe diabetes of early-onset, there was a marked change in mesangial cells, consisting of an increase in vesicles, multivesicular bodies, vacuoles, ghost-like appearance of the cytoplasm, and mitochondrial disorganization. Only in the more advanced stages was there an increase in the mesangial matrix. Initially, the basement membrane was thin, particularly in dilated capillaries. Measurements of the basement membrane in the later stages indicated that there were both thick and thin areas; increased thickness alone was not characteristic. Although similar findings developed progressively with age, mesangial cell alterations, coalescence and cystic dilatation and fusion of capillary loops in the glomeruli of young hamsters were characteristic findings of diabetic glomerulopathy. These findings were characteristic and disproportionately marked in any later stage of the disease. Coalescence of capillaries may have explained the decrease in the number of loops. The foot processes of the epithelial cells were well-preserved in all glomeruli and nodular lesions were not seen. There was no evidence in this series that litter-mates of diabetic animals developed such glomerular lesions in the absence of overt diabetes. An analogy was suggested between the dilatation and coalescence of glomerular capillaries associated with mesangial cell changes and the dilatation of retinal capillaries associated with degeneration of the supporting mural cells in man. Nous avons étudié aux microscopes optique et électronique les glomérules rénaux de 11 hamsters chinois non-diabétiques, de 4 hamsters non-diabétiques mais provenant de nichées comportant des animaux diabétiques, et de 6 animaux ayant souffert d'un diabète de durée et de sévérité variables. Le vieillissement en soi est associé à une augmentation du mésangium et à une dilatation des capillaires avec léger épaississement de la membrane basale. Chez les diabétiques sévères de type juvénile, nous avons observé des altérations profondes des cellules mésangiales, avec la formation de vésicules, de corps multivésiculaires, de vacuoles et une apparence lavée du cytoplasme avec désorganisation mitochondriale. Ce n'est qu'aux stades les plus avancés qu'on observe une augmentation de la matrice mésangiale. Aux premiers stades, les membranes basales sont minces, particulièrement pour les capillaires dilatés. A des stades plus avancés, on trouve des régions épaisses et des régions minces de la membrane basale; certainement pas d'épaississement généralisé. Bien que des altérations semblables eussent été observées avec le vieillissement simple, les altérations des cellules mésangiales, la coalescence, la dilatation cystique et la fusion des anses capillaires des glomérules sont caractéristiques d'une glomérulopathie diabétique chez les animaux jeunes. Les altérations sont beaucoup plus marquées à des stades plus avancés de l'état diabétique. La diminution du nombre des anses capillaires est probablement le résultat de la coalescence des capillaires. Les cellules épithéliales sont bien préservées dans tous les glomérules et il n'y a pas de lésions nodulaires. Nous n'avons pas observé de lésions glomérulaires semblables chez les frères et soeurs des animaux diabétiques ne souffrant pas eux-mêmes d'un diabète patent. Il semblerait y avoir une analogie entre la dilatation et la coalescence des capillaires du glomérule en présence d'altérations des cellules mésangiales et la dilatation des capillaires rétiniens associée à la dégénérescence des cellules murales, ou péricytes, chez l'homme. Wir haben im Licht- und im Elektronen-Mikroskop die renalen Glomeruli von 11 Kontrolltieren, von 4 nicht-diabetischen Tieren aus gleichem Wurf wie diabetische Hamster, und von 6 Tieren mit Spontan-Diabetes verschiedenen Alters, sowie mit unterschiedlicher Dauer und Schwere des Krankheitsbildes, untersucht. Zunehmendes Alter führte zur Zunahme der mesangialen Matrix mit einiger Erweiterung der Kapillaren und Verdickung der Basalmembran. Bei früh einsetzendem schweren Diabetes wurden schwere Veränderungen der Mesangialzellen beobachtet, bestehend aus Vermehrung der vesikulären Bläschen, Entstehung von multi-vesikulären Strukturen, Vacuolen, und einem verwaschenen Aussehen des Zytoplasmas, sowie mitochondrialer Desorganisation. Bei vorgerücktem Krankheitsbild bestand eine deutliche Zunahme der mesangialen Matrix. Anfänglich war die Basalmembran dünn, besonders in den erweiterten Kapillaren. Später, wies die Basalmembran sowohl verdickte wie verdünnte Stellen auf. Obwohl ähnliche Veränderungen auch mit zunehmendem Alter zu sehen waren, ließ die Beobachtung von Veränderungen der mesangialen Zellen, von zystischer Erweiterung und Verschmelzen von Kapillarschlingen beijungenHamstern sicher auf diabetische Glomerulopathie schließen. Diese Veränderungen waren denn auch verhältnismäßig noch deutlicher und besonders charakteristisch in späteren Stadien der Krankheit. Die Fusion von Kapillaren erklärt vielleicht die Abnahme der Zahl der Kapillarschlingen. Die Ausläufer der Epithelialzellen blieben in allen Glomeruli wohl erhalten und es waren keine noduläre Läsionen zu sehen. In den von uns beobachteten Tieren waren solche Veränderungen der Glomeruli bei nicht-diabetischen Tieren nicht zu sehen. Es wird darauf hingewiesen, daß zwischen der Erweiterung und der Fusion von Kapillarschlingen, im Zusammenhang mit Veränderung der mesangialen Zellen, und der Erweiterung von Kapillaren der Netzhaut, im Zusammenhang mit Degeneration deren Perizyten beim Menschen, eine Analogie besteht.

Congenital biliary dilatation (CBD) associated with intestinal malrotation (IM) has rarely been reported. We herein report three cases of pediatric CBD associated with IM that was incidentally found at surgery. The patients underwent total resection of the extrahepatic bile duct followed by hepaticoduodenostomy at the hepatic hilum or hepaticoduodenostomy as the definitive surgery for CBD. Ladd's procedure was simultaneously performed for asymptomatic IM. Postoperative adhesive intestinal obstruction occurred in all three patients, and conservative treatment was successful. Duodenogastric regurgitation was also observed as a biliary reconstruction-related complication, and conversion to hepaticojejunostomy at the hepatic hilum was performed in two of the three patients. We herein present the clinical course of these three patients and discuss the clinical significance of the association between CBD and IM in terms of the surgical strategy and in comparison with 12 previously reported cases.