No abstract available.
Humans ; Irritable Bowel Syndrome*

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*

No abstract available.
Carcinoid Tumor*

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.
Aortic Aneurysm, Thoracic*

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*

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*

BACKGROUND: The skin changes of X-linked recessive ichthyosis are cnused by the deficiency of the enzyme steroid sulfatase, which usually results from deletions of this gene in Caucasian populations. OBJECTIVE AND MEHTODS: To disgnose X-linked recessive ichthyosis and detect its carrier, we have investigated distinctive gene deletion and measured gene dosage of steroid sulfatase gene by southern blot hybridization in Korean patients with X-linked recessive ichthyosis. RESULTS: Patients from 8 of 9 unrelated families exhibited deletions, if the steroid sulfatase gene. Of 6 families showing a family history compatible with X-linked recessive inheritance, One family exhibited a normal pattern of hybridization. All but one family showed deletion of steroid sulfatase gene. All three patients lacking a fami1y history of the disease exhibited gene deletions. The ratio of the steroid sulfatsse specific band density to the Factor VIII specific band density was measured in 8 obligate carriers using a laser densitometer. The average ratio exhibited by the car riers was less than half that of normal women. Conclusian: These results suggest that the X-linked recessive ichth osis patient and its carrier can also be diagnosed and detected by Southern blot hybridization of steroid sulfatase gene in Korea.
Blotting, Southern* ; Diagnosis* ; Factor VIII ; Female ; Gene Deletion ; Gene Dosage ; Humans ; Ichthyosis* ; Korea ; Skin ; Steryl-Sulfatase ; Wills