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Introduction: Pancreaticobiliary maljunction (PBM) is a congenital anomaly, which can be defined as a union of the pancreatic and biliary ducts located outside off the duodenal wall. We herein investigate clinical features of PBM including as the 2nd report of a Japanese nationwide survey. Patients and methods: During a period of 18 years (from 1990 to 2007), 2,561 patients with PBM were registered at 141 medical institutions in Japan. Among them, eligible patients ( n = 2,529) were divided into two groups: adult ( n = 1,511) and pediatric patients ( n = 1,018). Comparisons of clinical features including associated biliary cancers were performed according to the biliary dilatation (BD), age factor, and time era. Results: Only one case in pediatric patients with BD combined with a bile duct cancer (0.1 %). In adult patients, the bile duct cancer and the gallbladder cancer was seen in 6.9 and 13.4 % patients with BD and in 3.1 and 37.4 % patients without BD, respectively. In adult patients with BD, the occurrence rates of biliary cancers were increased in latter period (00'-07') compared with former period (90'-99'). The ratio of biliary cancer localization was changed between former and latter period, and the bile duct cancer was increased in latter period (from 5.5 to 9.3 %). Conclusions: The largest series of PBM were evaluated to clarify the clinical features including the associated biliary cancer in this Japan-nationwide survey. This report could be widely used in the future as a reference data for diagnosis and treatment of PBM. [ABSTRACT FROM AUTHOR]

We describe here a patient with relapsed hepatoblastoma after LDLT who developed heart failure, which was treated with irinotecan hydrochloride (CPT-11). His native liver was replaced by a liver graft from his mother at 26 months from the onset. However, LDLT failed to induce complete remission and he was diagnosed as relapsed hepatoblastoma six months after LDLT. We again administered cisplatin and doxorubicin. After six courses of chemotherapy, he developed congestive heart failure because of anthracycline toxicity. The chemotherapy regimen was therefore switched to irinotecan at 35 mg/m2 daily for three days/wk for two consecutive weeks, and repeated every 28 days. After four courses of irinotecan, metastatic lesions were remarkably reduced in size, and the serum level of AFP decreased from 0.7 million to 927 ng/mL. No severe side effects were documented and congestive heart failure improved. These results suggest that irinotecan may be safely given to a patient with relapsed hepatoblastoma after LDLT without serious side effects and may contribute to prolonging the survival. [ABSTRACT FROM AUTHOR]

Presents a pediatric case of endobranchial tuberculosis with a branchial polyp observed with fiberoptic bronchoscopy. Causes of chronic morbidity; Description of endobranchial involvements due to tuberculosis; Cause of polyp formation.

To evaluate the possible involvement of vascular endothelial growth factor (VEGF) in the pathogenesis of Castleman's disease, we studied VEGF levels in sera and supernatants of cultured lymph nodes from two patients with the plasma cell type of Castleman's disease, and analysed the expression of VEGF immunohistochemically in the lymph nodes. Clinically, one patient was classified as the localized type and the other as the multicentric type. Histologically, mature plasma cells and hyalinized vessels were prominent in the interfollicular region. The VEGF levels of the sera and the supernatants of cultured lymph nodes of both patients were higher than those of normal controls. VEGF was strongly expressed in plasma cells in the interfollicular region of the lymph nodes of both patients, but rarely in normal lymph nodes. Our results suggest that VEGF may be involved in the marked vascular proliferation in the interfollicular region of the lymph nodes of the plasma cell type of Castleman's disease. [ABSTRACT FROM AUTHOR]