Multiple Endocrine Neoplasia Type 1 with Functional Parathyroid Cysts.

A 51-year-old woman was admitted because of hypercalcemia. Neck ultrasonography and computed tomography revealed the presence of parathyroid cysts on both sides. After primary hyperparathyroidism was diagnosed by technetium-99m-methoxyisobutylisonitrile scintigraphy, the patient was successfully treated with total parathyroidectomy and autotransplantation. She also had a non-functioning pancreatic neuroendocrine tumor, prolactinoma, and adrenal tumors with subclinical Cushing's syndrome. Given these clinical features and her family history, multiple endocrine neoplasia type 1 (MEN1) was suspected, and germline DNA sequencing revealed a missense mutation (c.1013T>C, [corrected] p.Leu338Pro) in exon 7 of MEN1. This case demonstrates the phenotypic and genetic diversity of MEN1.

Insulin-derived amyloidosis and poor glycemic control: a case series.

OBJECTIVES: Insulin-derived amyloidosis is a rare skin-related complication of insulin therapy. The purpose of this study was to show the effects of insulin-derived amyloidosis on blood glucose levels, insulin dose requirements, and insulin absorption. METHODS: Seven patients were found to have insulin-derived amyloidosis at the Tokyo Medical University Ibaraki Medical Center. The clinical characteristics and insulin therapy of the 7 patients were investigated. Insulin absorption was studied by comparing the serum insulin levels after insulin injections into insulin-derived amyloidosis sites versus injections into normal sites in 4 patients. RESULTS: When the insulin-derived amyloidosis was discovered, the mean hemoglobin A1c level was 9.3%, and the mean daily insulin dose was 57 units. After changing the injection sites to avoid the insulin-derived amyloidosis, the blood glucose concentrations improved, and the mean daily insulin dose could be reduced to 27 units (P = .035; 53% reduction). The insulin absorption at insulin-derived amyloidosis sites was 34% of that at normal sites (P = .030). CONCLUSIONS: Insulin-derived amyloidosis caused poor glycemic control and increased insulin dose requirements because of impairments in insulin absorption.

[A case of MPO-ANCA positive vasculitis associated with multiple pulmonary nodules following propylthiouracil treatment].

A 34-year-old woman who had been treated with propylthiouracil (PTU) for hyperthyroidism, was admitted because of bloody sputum and pyrexia. The chest CT scan showed some nodules in both lung fields. The serum level of myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) was high, but proteinase-3 antineutrophil cytoplasmic antibody (PR-3 ANCA) was negative. A limited form of Wegener's granulomatosis without PR-3 ANCA was ruled out, because of the absence of abnormalities in the upper airway and kidney. No lesions other than the multiple pulmonary nodules of the lung were detected. We diagnosed MPO-ANCA associated vasculitis induced by PTU. After the termination of PTU, bloody sputum, pyrexia, and pulmonary nodules improved spontaneously and the serum level of MPO-ANCA returned to normal gradually. The case of MPO-ANCA positive vasculitis associated with multiple pulmonary nodules following propylthiouracil treatment is very rare.

[A case of posterior mediastinal mature teratoma].

The chest radiograph of a 57-year-old man, complaining of paroxysmal dyspnea, suggested the probably of a tumor. Chest CT showed a tumor containing calcification, behind the left crus of the diaphragm. Chest MRI suggested lipid components and a cystic lesion within the tumor. Their findings were clinically compatible with posterior mediastinal teratoma. The pathological diagnosis of the surgically resected tumor was mature teratoma with neither malignant components nor thymic tissue. Study of past case reports suggests that posterior mediastinal teratomas should have less malignant characteristics than anterior mediastainal teratomas. Our case is the fifteenth case report in the Japanese literature, and accumulation of more cases is required.

Effect of prostaglandin E2 on urokinase-type plasminogen activator production by human lung fibroblasts.

Human lung fibroblasts are components of stromal tissue and produce various proteins as occasion demands, such as extracellular matrix (ECM) components and proteases. Pulmonary tumour cells produce high levels of prostaglandin E(2) (PGE(2)), which regulates tumour growth and metastasis. Urokinase-type plasminogen activator (uPA) is essential in the degradation of peritumour ECM. Furthermore, uPA is an important protease believed responsible for several tumour characteristics through its activation of certain proteases and growth factors. We hypothesized that the PGE(2) overexpression from tumour cells would have some effect on uPA expression in lung fibroblasts. In this study, the influence of PGE(2) on uPA expression in human lung fibroblasts was investigated using two lines of such fibroblasts. Although the cell surface uPA level was comparable to that of PGE(2) untreated cells, the expression of uPA mRNA and production was increased by the addition of PGE(2) in both lines of fibroblasts. These fibroblasts expressed both the EP(2) and EP(4) PGE(2) receptor mRNAs. Pretreatment with EP(2) and/or EP(4) receptor antagonists reduced the intercellular and cell surface uPA expression of the human lung fibroblasts. These results indicated that there is a relationship between the PGE(2) system and uPA production in human lung fibroblasts operating through EP(2) and/or EP(4) receptor signalling. uPA induced by PGE(2) from stromal fibroblasts surrounding lung tumour thus appears to play an important role through these EP receptors. Inhibition of EPs in tumour tissue might be a useful strategy for anti-metastasis therapy.