5 alpha-reductase deficiency resulting in male pseudohermaphroditism is a rare disease characterized by clitoral-like phallus, bifid scrotum, urogenital sinus, testis cited in labioscrotal folds. Evaluation of plasma T/DHT ratios in infancy, particularly after hCG stimulation of the testes and elevated urinary tetrahydrocortisol (THF) to 5 alpha-tetrahydrocortisol(5 alpha-THF) ratios provide a valuable dianostic test for 5 alpha-reductase deficiency. We report one case of 5 alpha-reductase deficiency who were presented with ambiguous genitalia and elevated T/DHT ratio before and after hCG stimulation.
46, XY Disorders of Sex Development ; Cholestenone 5 alpha-Reductase* ; Disorders of Sex Development ; Plasma ; Rare Diseases ; Scrotum ; Testis ; Tetrahydrocortisol

Nesidioblastosis, also known as persistent hyperinsulinemic hypoglycemia of infancy(PHHI) or familial hyperinsulinsm, is the most common cause of recurrent severe hypoglycemia in infancy. It is an autosomal recessive disorder characterized by irregular insulin secretion leading to inappropriately raised plasma insulin concentration compared to blood glucose levels. Recently, mutations in the sulfonylurea receptor(SUR) have been described in association with PHHI. The mainstay of medical treatment is glucose infusion and diazoxide or long acting somatostatin. If medical treatment fails in preventing hypoglycemia, near total pancreatectomy is recommended. We report one case of nesidioblastosis cured by near total pancreatectomy with brief review of literatures.
Blood Glucose ; Congenital Hyperinsulinism* ; Diazoxide ; Glucose ; Humans ; Hypoglycemia ; Infant, Newborn* ; Insulin ; Nesidioblastosis* ; Pancreatectomy ; Plasma ; Somatostatin

The allergic skin test and RAST are used commonly to demonstrate the presence of skin sensitizing IgE antibody in the diagnosis of allergic disease. To evaluate the agreement rate and correlation between results of skin test and RAST (radioallergosorbent test) in allergic subject, 83 allergic childhood with positive skin test (bronchial asthma: 26, allergic rhinitis: 27, atopic dermatitis and urticaria: 6 bronchial asthma and allergic rhinitis: 24) and 16 nonallergic childhood were studied. The results were as follows. 1) Peripheral eosinophil counts were significantly elevated in groups with bronchial asthma, allergic rhinitis, bronchial asthma & allergic rhinitis except atopic dermatitis & urticaria group in comparison with normal control group. 2) Serum IgE levels were significantly higher in groups with bronchial asthma, allergic rhinitis, bronchial asthma & allergic rhinitis than in normal control group except atopic dermatitis & urticaria group but there was no significant differences among study groups. 3) RAST units of D.p and D.f were significantly higher in study group except atopic dermatitis & urticaria group. RAST units of cockroach, however, were not significantly increased but in D.f, combined group of bronchial asthma & allergic rhinitis was significantly higher than bronchial asthma or allergic rhinits (p<0.05). 4) Each agreement rate between 3+ skin test and positive rate of RAST in D.p, D.f, cockroach was 37.5%, 63.6% and 12.5% respectively and 4+ skin test and positive rate of RAST was 84.0%, 89.1% and 38.4%. 5) Correlation coefficient between skin test and RAST was 0.71 in D.P, 0.59 in D.f and 0.23 in cockroach. In conclusion, there was difference in agreement rate between positive skin test and RAST to each allergen. Agreement rate between positive skin test and RAST was higher in D.p and D.f. These results suggest that RAST can be used simultaneously with skin test for detecting allergens.
Allergens ; Asthma ; Cockroaches ; Dermatitis, Atopic ; Diagnosis ; Eosinophils ; Humans ; Hypersensitivity* ; Immunoglobulin E ; Rhinitis ; Skin Tests* ; Skin* ; Urticaria

No abstract available.
Anemia, Dyserythropoietic, Congenital*

PURPOSE: Obesity is a significant health problem with medical and psychological consequences for children and adolescents. The purpose of this study was to assess the correlation between body fat percent using bioelectrical impedance(BI) and other variable methods. METHODS: We measured height, weight, body mass index(BMI) and body fat percent by skinfold thickness(ST) and BI in 1,035(496 male; 539 female) children from seven to 18 years of age. The correlation coefficients between BI and each of the other different methods were obtained. The sensitivity and specificity to predict obesity by these several methods were studied. RESULTS: Fat percent estimated by BI analysis and BMI showed a strong correlation(r=0.749). Fat percent estimated by BI analysis and ST showed a very strong correlation(r=0.835). At the 95th percentile cut-off point for BI, ST showed a sensitivity of 57.7%, and a specificity of 97.6% for estimating body fat. At the 95th percentile cut-off point for BI, BMI showed a sensitivity of 84.9%, and a specificity of 99.3% for estimating body fat. CONCLUSION: The fat percent estimated by BI analysis correlated strongly with ST or BMI. BI analysis is an objective and accurate method for estimating body fat in childhood obesity.
Adipose Tissue* ; Adolescent ; Body Mass Index ; Body Weight ; Child ; Electric Impedance* ; Humans ; Male ; Obesity ; Pediatric Obesity ; Sensitivity and Specificity ; Skinfold Thickness

Pulmonary artery sling is a rare vascular anomaly in which the left pulmonary artery arises from the right pulmonary artery and then traverses between the esophagus and the trachea toward the hilum of the left lung. Despite the availability of a corrective operation, the mortality rate remains very high due to the high incidence of associated obstructive anomalies of tracheobronchial trees. We experienced a 8 month old female infant who was admitted because of recurrent pneumonia and expiratory wheezing. She was diagnosed as pulmonary sling by esophagography, echocardiography, computed tomography. We report this with a brief review and related literature.
Echocardiography ; Esophagus ; Female ; Humans ; Incidence ; Infant ; Lung ; Mortality ; Pneumonia ; Pulmonary Artery* ; Respiratory Sounds ; Trachea

No abstract available.
Collagen* ; Interferons* ; Ureter* ; Ureteral Obstruction*

Formaldehyde is a colorless irritating gas of pungent odor, which is used as a disinfectant, antiseptic, deodorant, tissue fixative and embalming fluid, It is used in the formation of resins, alcohols and acids, and in permanent press processes in the textile industry. If formaldehyde is swallowed, severe abdominal pain and vomiting is immediately noted. Corrosive gastritis can follow with diffuse ulceration, fibrosis and contracture of the stomach, resembling linitis plastica, which may be severe enough to require gastrectomy. We report a case of cicatricial contracted stomach due to the ingestion of formalin.
Abdominal Pain ; Alcohols ; Contracture ; Deodorants ; Eating* ; Embalming ; Fibrosis ; Formaldehyde* ; Gastrectomy ; Gastritis ; Linitis Plastica ; Odors ; Stomach* ; Textile Industry ; Ulcer ; Vomiting

Carcinosarcoma is a rare malignant neoplasm consisting of both epithelial and nonepithelial components, that is, carcinoma and sarcoma in the same tumor. Since Queckenstedt reported the case of carcinosarcoma of the stomach in 1904, only 30 cases have been reported. We present a case of carcinosarcoma and adenocarcinoma of the stomach in 63-year-old man with the review of the literature.
Adenocarcinoma* ; Carcinosarcoma* ; Humans ; Middle Aged ; Sarcoma ; Stomach*

Preoperative evaluation of metastasis to internal iliac lymph nodes are very important in the designing of the patient's therapeutic plan and the predicting the prognosis of carcinoma of the uterine cervix. Until now no study has reported on the subject of selective visualization of the internal iliac lymph nodes. Indirect lymphangiography is a simple method for evaluating local lymphatic system. The internal iliac lymph nodes can be visualized by combining indirect lymphangiography of the upper one-third area of the vagina and thin section CT scan. We applied this method to 25 cases of FIGO B and A stage of the carcinoma of the uterine cervix, and correlated our findings with results of radical abdominal hysterectomy, We were able to visualize the internal iliac lymph nodes in 24 cases out of 25, and their size range was 3-15 mm. No nodal metastasis were found in all the patients and these were varified by subsequent pathologic examination from radically dissected lymph nodes.
Cervix Uteri* ; Female ; Humans ; Hysterectomy ; Lymph Nodes* ; Lymphatic System ; Lymphography ; Methods* ; Neoplasm Metastasis ; Prognosis ; Tomography, X-Ray Computed* ; Vagina