No abstract available.
Aortic Aneurysm, Thoracic*

No abstract available.
Carcinoid Tumor*

BACKGROUND: The immunohistochemical detection of endothelial differentiation in skin tumors has been hindered by the relative paucity of reliable markers that are applicable to rou- tinely-processed specimens, which are both specific and sensitive as well. OBJECTIVE: This study was designed to evaluate the utility of CD31 and CD34, newly introduced vascular markers, in the immunohistochemical differentiation of vascular neoplasms from other mesenchymal tumors and to compare their sensitivity and specificity with factor VII-related antigen (FVIIIRA). METHODS: Paraffin-embedded specimens of 26 cases of benign and malignant vascular tumors and 25 cases of non-vascular mesenchymal neoplasms of the skin and subcutis were investigated for CD31, CD34, and FVIIIRA expression using immunohistochemical methods. RESULTS: CD31-immunoreactivity was observed in all of the 26 vascular lesions. CD34 and FVlllRA were labelled in 23 cases and 16 cases of vascular tumors, respectively. All of the 25 non-vascular mesenchymal tumors were negative for CD31 and FVIIIRA. In contrast, positive reactivity for CD34 was seen in 14 cases, among which dermatofibrosarcoma protuberans(2/2), neurofibroma(8/8), neuriilemmoma(2/2), and leiomyoma(2/3) were included. CONCLUSION: Our results indicate that CD31 is a more sensitive and specific vascular marker than CD34 and FVIIIRA. A small panel composed of these three antibodies will constitute a comprehensive and reliable method for identifying tumors of vascular origin.
Antibodies ; Dermatofibrosarcoma ; Methods ; Sensitivity and Specificity ; Skin* ; Vascular Neoplasms ; von Willebrand Factor*

Pulmonary agenesis is a very rare anomaly. It is defined as total absence of the pulmonary parenchyma, vascular structures, and bronchi beyond the carina. We experienced a case of right pulmonary agenesis in association with other congenital defects who died at 1 day of age. The other defects included: esophageal atresia, tracheoesophageal fistula, cardiac malformation, anal atresia and a malformed left thumb. The cardiac malformations were a type of Pentalogy of Fallot, composed of right ventricular hypertrophy, ventricular septal defect, an overiding of aorta, pulmonary atresia, and an atrial septal defect. Hand roentgenograms of the malformed left thumb showed an unarticulated metacarpopharyngeal joint. This unique combination of anomalies is extremely rare.

No abstract available.
Humans ; Irritable Bowel Syndrome*

Bromidrosis is a disorder characterized by rancid body odor which influences a patient's social life and mental health. The therapeutic modalities and the mechanism of bromidrosis have been carefully studied, however, there have been few reports about the genetic inheritance of bromidrosis. We investigated the family history of 42 patients who were operated on for bromidrosis and followed up to the third generation in 10 cases. The results were as follows: Results of investigation which were followed up the second generation. The fathers of five patients and the mothers of 11 patients had bromidrosis in 18 male patients. The fathers of six patients and the mothers of 12 patients had bromidrosis in 24 female patients. Thirty-four patient (81.0%) among a total of 42 have a single parent with bromidrosis. Result of investigation which were followed up to the third generation Bromidrosis was occurred in 17 of 42 patients (40.5%) in the second generation, and 18 of 27 patients (66.7%) in the third generation. In one case, a father transmitted bromidrosis to his three sons, and as a result, X-linked inheritance could be ruled out Bromidrosis was not skipped in every generation of all families. We on conclude that bromidrosis is an autosomal dominant inherited disorder.
Fathers ; Female ; Genes, X-Linked ; Humans ; Male ; Mental Health ; Mothers ; Odors ; Single Parent ; Wills*

No abstract available.
Child* ; Humans ; Scrub Typhus*

No abstract available.
Humans ; Inpatients*

PURPOSE:Autonomic neuropathy in adult diabetics is known to be common and poor in prognosis. Cardiac autonomic neuropathy increases sudden cardiac death and is present before apparant cardiac symptoms. Subclinical diabetic peripheral neuropathy is common with type 1 diabetes. In children, few studies for subclinical peripheral neuropathy and cardiac autonomic neuropathy were reported but the results were inconsistent. The authors investigated subclinical cardiac and peripheral neuropaties and evaluated correlation between nerve conduction velocity(NCV) and cardiac autonomic function(CAF) test in type 1 diabetic children and adolescents METHODS:Over 5 years from 1997 to 2002, 27 IDDM without clinical neuropathy were registered. Total 57 CAF and NCV were tested every 2 or 3 years. Duration of diabetes, average HbA1c during 1 year prior to the test, and urinary microalbumin excretion for 24 hours were assessed. Diabetic autonomic nervous function was evaluated by cardiovascular reflex test:falling systolic blood pressure in response to standing, heart rate changes in response to standing, beat-to-beat rate variation during deep breathing, RR interval change during and after Valsalva maneuver. Subclinical peripheral neuropathy was evaluated by NCV in both upper and lower extremities. Correlation of parameters according to NCV abnormality and microalbuminuria were analyzed. RESULTS:The age, duration of diabetes and microalbuminuria were not statistically different between children with normal and abnormal NCV. The level of HbA1c was associated with NCV abnormality. In a multivariate logistic model for NCV abnormality, level of HbA1c showed statistical significance after controlling the effects of age, duration of diabetes, and urinary microalbumin level, and the odd ratio was 1.532. The degree of CAF abnormality was marginally significant in the logistic model. Children with microalbuminuria showed older age and longer duration of diabetes than those without microalbuminuria. However, mean HbA1c level was not significantly different between the two groups. Degree of CAF abnormality was not significantly associated with microalbuminuria, either. In a general linear model including urinary albumin level as the dependent variable, none of independent variables was statistically significant. CONCLUSION: Therapy against neuropathy should be considered in patients with high HbA1c and abnormal CAF test even without clinically apparent neuropathy.
Adolescent ; Adult ; Blood Pressure ; Child* ; Death, Sudden, Cardiac ; Diabetes Mellitus, Type 1 ; Diabetic Neuropathies ; Heart Rate ; Humans ; Linear Models ; Logistic Models ; Lower Extremity ; Neural Conduction ; Peripheral Nervous System Diseases ; Prognosis ; Reflex ; Respiration ; Valsalva Maneuver

We treated a case of extramammary Paget's disease that affected the scalp of a 45-year-old female. It is extremely rare that the disease arises in areas other than the anogenital region and the axillae. The lesion was a round erythematous oozing crusted hairless patch. Histopathologically, many pagetoid cells were found within the epidermis and dermis. The cytoplasms of these cells stained with alcian blue at pH 2.5, CEA, EMA and low-molecular-weight-cytokeratin. The patient underwent a wide local excision. We used a mapping technique to reveal the distribution of microscopically involved lesions.
Alcian Blue ; Axilla ; Cytoplasm ; Dermis ; Epidermis ; Female ; Humans ; Hydrogen-Ion Concentration ; Middle Aged ; Paget Disease, Extramammary* ; Scalp*