Promoter methylation and protein expression of the E-cadherin gene in the clinicopathologic assessment of adenoid cystic carcinoma.

Adenoid cystic carcinoma, a relatively uncommon tumor of salivary glands, is characterized by a prolonged clinical course and a fatal outcome. The molecular events underlying their progression are unknown. In this study, we examined the methylation status of E-cadherin gene and its protein expression in 23 cases of adenoid cystic carcinoma and correlated the results with the clinicopathologic factors to determine its role in these tumors. We also analyzed the effect of 5-azacytidine on the re-expression in a methylated cell line of adenoid cystic carcinoma for this gene. In our study, E-cadherin immunoreactivity, although heterogeneous, showed a progressive reduction with high histological grade and in metastatic and recurrent lesions. Promoter methylation was detected in 16 of 23 cases (70%), but there was no correlation with the histological grade or patient prognosis. Microdissection of immuno-negative cells in heterogeneous tumors showed positive methlyation. In the cell line from salivary adenoid cystic carcinoma with methylated E-cadherin, 5-azacytidine restored the E-cadherin expression. Our results indicate that: (1) E-cadherin gene promoter is frequently methylated in adenoid cystic carcinoma, leading to reduced E-cadherin expression, (2) variable E-cadherin expression might result from the intratumoral heterogeneity, and (3) increased extent of methylated areas may be associated with progression and advancement of the disease.     

Angiotensin I-converting enzyme inhibitor improves reactive hyperemia in elderly hypertensives with arteriosclerosis obliterans.

Endothelial function in elderly hypertensive patients with arteriosclerosis obliterans has not been evaluated. We examined whether antihypertensive drugs improve vasodilatory response to reactive hyperemia of the limbs in elderly hypertensive patients (83 +/- 8 [SD] years) without (n=46, 0.9 < or = ankle-brachial pressure index < or = 1.4) and with (n=24) arteriosclerosis obliterans (ankle-brachial pressure index < 0.2). Patients were randomized for treatment with monotherapy of either temocapril (14 with and 26 without arteriosclerosis obliterans) or amlodipine (10 with and 20 without arteriosclerosis obliterans) for 6 months. Blood flows of the forearms and legs were measured by strain-gauge plethysmography. The vasodilatory response to the release of compression of the forearms and thighs at 200 mmHg or 20 mmHg more than systolic blood pressure for 5 min and to sublingual administration of nitroglycerin (0.3 mg) was assessed. The maximum reactive hyperemic flow in 35 legs with arteriosclerosis obliterans was significantly (p < 0.001) decreased compared to the value in legs in the control hypertensive subjects. Moreover, maximum reactive hyperemic flow in the forearms of patients with arteriosclerosis obliterans was significantly (p = 0.002) decreased compared to that in the control subjects. Blood pressure was similarly decreased by treatment with temocapril or amlodipine. Response to nitroglycerin (0.3 mg) was not changed by either drug. Treatment with temocapril significantly improved maximum reactive hyperemic flow of not only the legs and forearms in control hypertensives but also the legs and forearms in patients with arteriosclerosis obliterans, and attenuated the worsening of activity of daily living in these patients, although treatment with amlodipine did not. These results suggest that the angiotensin-converting enzyme inhibitor temocapril has a beneficial effect on endothelial function in elderly patients with arteriosclerosis obliterans.     

NEW METHOD OF STENTING TREATMENT FOR MALIGNANT DUODENAL STENOSIS: USING DOUBLE‐BALLOON ENTEROSCOPY

Recently, a self‐expandable metallic stent has been recognized for treatment of malignant duodenal stenosis. But the complications by stenting are important problems even now. In the present study, we report our new method of duodenal stenting by using of double‐balloon enteroscopy considered safe and effective.     

Urinary dysfunction and autonomic control in amyloid neuropathy

Abstract Uro-neurological assessment was performed in four patients with small-fiber neuropathy due to amyloidosis (2 transthyretin-type/2 immunoglobulin light-chain-type). Voiding difficulties were due to detrusor weakness and impaired bladder sensation. In two patients cholinesterase inhibition treatment caused urge incontinence, indicating detrusor denervation supersensitivity. The underlying mechanisms of urinary dysfunction seem to involve postganglionic cholinergic and afferent somatic nerves.     

Long-Term Outcome of Adult-to-Adult Living Donor Liver Transplantation for Post-Kasai Biliary Atresia

Our objective was to analyze problems in the perioperative management and long-term outcome of living donor liver transplantation (LDLT) for biliary atresia (BA). Many reports have described the effectiveness of liver transplantation (LT) for BA, particularly in pediatric cases, but little information is available regarding LT in adults (> or =16 years old). Between June 1990 and December 2004, 464 patients with BA underwent LDLT at Kyoto University Hospital, of whom 47 (10.1%) were older than 16 years. In this study, we compared the outcomes between adult (> or =16 years old) and pediatric (<16 years old) patients. The incidence of post-transplant intestinal perforation, intra-abdominal bleeding necessitating repeat laparotomy and biliary leakage was significantly higher (p < 0.0001, <0.001 and <0.001, respectively) in adults. Overall cumulative 1-, 5- and 10-year survival rates in pediatric patients were significantly higher (p < 0.005) than in adults. Two independent prognostic determinants of survival were identified: a MELD score over 20 and post-transplant complications requiring repeat laparotomy. Outcome of LDLT in adult BA patients was poorer than in pediatric patients. It seems likely that LT will be the radical treatment of choice for BA and that LDLT should be considered proactively at the earliest possible stage.     

A case of Sweet's syndrome (acute febrile neutrophilic dermatosis) associated with mixed connective tissue disease]

Sweet's syndrome (acute febrile neutrophilic dermatosis) is an unusual condition characterized by fever, polymorphonuclear neutrophil leukocytosis of the blood, thick painful plaques on the face, neck and limbs, and a dense dermal infiltrate of mature neutrophils seen histologically. Recently, this disease has also been reported in association with various malignant neoplasms and chronic inflammatory disorders. In the literature, seven cases of Sweet's syndrome associated with collagen diseases have been reported, but no cases with mixed connective tissue disease (MCTD). The first case of Sweet's syndrome associated with MCTD was herein described and discussed. A 49-year-old man was admitted to our hospital with the complaints of high fever and painful erythema on his face, neck and limbs. Six months ago, MCTD was suspected, with the presence of limited cutaneous sclerosis of the hands, Raynaud's phenomenon, polyarthralgia, an elevation of CPK value and a positive anti-RNP antibody. Just before hospitalization, he suffered a prodromal infection of the upper respiratory tract for two weeks. He was diagnosed as Sweet's syndrome by the clinical and histological features. He began receiving corticosteroid therapy (prednisolone 60 mg/day), and within a week he showed dramatic improvement in the above symptoms.     

Malignant Rhabdoid Tumor of the Breast: A Case Report

A 66-year-old woman time of 10 days. One month after radicalmastectomy, there was local recurrence, followed by multiplepulmonary metastases, and the patient died of respiratory failure5 months after surgery. The gray-white-colored tumor measured13x12x;10 cm, and its border was well defined. The tumor wascomposed of diffusely growing round or polygonal cells withvesicular nuclei, prominent nucleoli, and ample cytoplasm containingeosinophilic inclusions. Lymph node involvement was widespread.Both vimentin and keratin were clearly demonstrated by immunohistochemicalstaining. Ultrastructural studies revealed that the MRT cellscontained cytoplasmic whorls of intermediate filaments.     

Iron in the enameloid of perciform fish.

It is known that a high concentration of iron is deposited in the enameloid of some teleostean fish. Previously, Suga et al. (1989) pointed out that the iron concentration in the enameloid is related to the phylogeny of fish rather than to the feeding habits, according to the results of quantitative iron analyses on the teeth of marine teleost fish of the Tetraodontiformes. In the present study, in order for the previous idea to be verified, quantitative iron analysis was made with an electron microprobe on the enameloid of fish belonging to the Perciformes, which is the largest group of teleostean fish in the world and consists of both marine and freshwater species. The enameloid of all the fish examined (57 species) contained high iron concentrations ranging from 0.2% to 10.2% at the surface or middle layer, whereas that of an advanced suborder, Tetraodontoidei, of the Tetraodontiformes was very low in iron, at a level which could not be discriminated from the background value of the emission intensity. The distribution pattern of iron in the enameloid was classified into at least two types, namely, type A, in which a high iron concentration was observed mainly in the surface layer, and type B, in which iron was deposited throughout the entire layer, although there were differences in concentration. There were some differences in the concentration and distribution of iron in the enameloid for the families; for example, those of the Scaridae had a type A distribution, with about 0.2% iron only at the surface layer, whereas those of the Cichlidae, Centrarchidae, and Acanthuridae, which showed a type B distribution, contained iron ranging from 2.9% to 10.5% at the surface or middle layer of enameloid. Such differences seemed to be associated with the difference in timing of the commencement of the iron deposition into the developing enameloid, which is probably related to the phylogeny of fish. There was no evidence to support the idea that the iron concentration in the enameloid is associated with the feeding habits of fish, as proposed by previous investigators.     

Relationship between left ventricular systolic function and the myocardial gray level distribution in patients with idiopathic dilated cardiomyopathy]

Dilated cardiomyopathy (DCM) is characterized by progressive left ventricular (LV) systolic dysfunction of nonspecific etiology. Fifty-nine DCM patients were serially observed by echocardiography for 4.5 +/- 2.6 years, and 7.3 +/- 3.4 times M-mode and two-dimensional echocardiography was performed during the observation period using SSH-11A (Toshiba). To assess LV systolic function, ejection fraction was calculated by Pombo's method. Myocardial gray level distribution shown by echocardiography was calculated to assess the myocardial tissue character. Two-dimensional echocardiographic images were obtained in the parasternal short-axis view, recorded on U-matic videotape, and transferred to an image processing computer system (MIPRON, Kontron). The images were digitized and stored on the computer. The regions of interest (ROI) were placed in the LV septum, posterior papillary muscle, posterior wall, anterior papillary muscle and entire LV wall. The gray level distributions in each ROI and its quantitative parameters (mean, SD, skewness, excess) were calculated. The corrected myocardial gray level of every ROI (CMD) was also calculated and expressed as the ratio to the mean gray level of the LV cavity. Seven patients exhibited significant decreases in ejection fraction (more than 10%) during the observation period (group A); the remainders showed less change (group B).(ABSTRACT TRUNCATED AT 250 WORDS)