Notch1 Has an Important Role in β-Cell Mass Determination and Development of Diabetes

BackgroundNotch signaling pathway plays an important role in regulating pancreatic endocrine and exocrine cell fate during pancreas development. Notch signaling is also expressed in adult pancreas. There are few studies on the effect of Notch on adult pancreas. Here, we investigated the role of Notch in islet mass and glucose homeostasis in adult pancreas using Notch1 antisense transgenic (NAS).MethodsWestern blot analysis was performed for the liver of 8-week-old male NAS mice. We also conducted an intraperitoneal glucose tolerance test (IPGTT) and intraperitoneal insulin tolerance test in 8-week-old male NAS mice and male C57BL/6 mice (control). Morphologic observation of pancreatic islet and β-cell was conducted in two groups. Insulin secretion capacity in islets was measured by glucose-stimulated insulin secretion (GSIS) and perifusion.ResultsNAS mice showed higher glucose levels and lower insulin secretion in IPGTT than the control mice. There was no significant difference in insulin resistance. Total islet and β-cell masses were decreased in NAS mice. The number of large islets (≥250 µm) decreased while that of small islets (ConclusionOur study provides that Notch1 inhibition decreased insulin secretion and decreased islet and β-cell masses. It is thought that Notch1 inhibition suppresses islet proliferation and induces differentiation of small islets. In conclusion, Notch signaling pathway may play an important role in β-cell mass determination and diabetes.

Neovaginoplasty Using Sigmoid Colon in Patient with Male Pseudo-hermaphroditism / 대한비뇨기과학회지

Gender assignment is the most important step in treating a child with ambiguous genitalia. Once an appropriate sex assignment has been made, the next critical step is the performance of a reconstructive procedure in a timely fashion. The aim of vaginoplasty should be the creation of a neovagina that will have a satisfying appearance, function and feeling, with a low rate of morbidity. Different forms of vaginal reconstruction have been described, and include the use of split-thickness or full-thickness grafts, amnion, peritoneum or bowel. Of these, vaginal reconstruction, with isolated bowel segments, provides a cosmetic, self-lubricating neovagina, with low rates of failure and revision, and without the need for routine dilation. Sigmoid vaginoplasty especially seems to have a clear advantage, with an excellent surgical success rate, and low complication rates. We report our initial experience of a neovaginoplasty, using sigmoid colon, in a 10-year old patient with an incomplete form of androgen insensitivity syndrome (Reifenstein syndrome).

Paraganglioma of the Bladder / 대한비뇨기과학회지

Paragangliomas are rare tumors of a neural crest origin, and belong to the amine- precursor-uptake decarboxylation (APUD) system. The great majority of paraganglioma are in the adrenal medulla. The majority of extraadrenal sites arise near the adrenal or along the nerve plexuses. Paragangliomas are only occasionally found in the wall of the bladder. Their preoperative diagnosis is suspected from a clinical history and an estimation of catecholamines, or their metabolic by-products, in a twenty-four-hour urine sample. The correct diagnosis is not made in the majority of cases because the tumors are frequently silent with regard to hormonal activity. Therefore, an excretory urogram, arteriography and cystoscopy, including biopsy, are important procedures for their preoperative diagnosis. We report a case of paraganglioma arising in the bladder wall, with brief review of literatures.

Retroperitoneal Malignant Schwannoma / 대한비뇨기과학회지

Schwannoma, also known as a neurilemmoma, neurinoma, and perineural fibroblastoma, can be either a benign or malignant tumor, arising from the associated nerve sheath. A retroperitoneal malignant schwannoma is a rare tumor. Immunohistochemical staining for S-100, leucine-7 and the myelin basic protein is useful for diagnosis. Here we report a case of retroperitoneal malignant schwannoma with a brief review of the literatures.

Adenomatoid Tumor of the Epididymis / 대한비뇨기과학회지

We report a rare case of an epididymal adenomatoid tumor in a 31-year-old man who had a slowly growing painless left scrotal mass for approximately two years. Physical examination revealed a rubbery, non-tender, 2x2cm sized mass located in the tail of the epididymis. Scrotal ultrasonography showed a 1.7x1.7cm sized isoechogenic mass inferior to the left testicle. Left epididymectomy done. The diagnosis was confirmed by a surgical excision and a subsequent histological analysis.

Ureteropelvic Junction Avulsion Due to Blunt Abdominal Trauma / 대한비뇨기과학회지

Ureteropelvic junction avulsion caused by blunt trauma is a rare condition which is often diagnosed after some delay. Herein, we report a case of traumatic ureteropelvic junction avulsion which has been treated successfully by end to end pyeloureterostomy.