We experienced two cases of congenital adrenal hyperplasia(CAH), complicated by true precocious puberty with early maturation of the hypothalamic-pituitary-gonadal axis in a boy and a girl. The boy was diagnosed as CAH at 8 years of age, when he showed pseudoprecocity with adrenal rest tumor. The girl was diagnosed at neonate, when she showed ambiguous genitalia and salt-losing symptom. They developed precocious puberty during steroid treatment. Clinically they showed precocity, advanced bone age, and decreased predicted adult height, even though they showed partial gonadotrophin response to luteinizing hormone releasing hormone (LHRH) stimulation test. They were treated with LHRH analogue(leuprolide depot) resulting no further progression of precocity.
Adrenal Hyperplasia, Congenital* ; Adrenal Rest Tumor ; Adult ; Axis, Cervical Vertebra ; Disorders of Sex Development ; Female ; Gonadotropin-Releasing Hormone ; Humans ; Infant, Newborn ; Male ; Puberty, Precocious*

No abstract available.
Atherosclerosis* ; Hyperuricemia* ; Risk Factors*

The technique of plssma exchange has been applied to a number of immune disorders in which c4eulating antibodies are present. We observed a patient with bullous pemphigoid and serious side effects of longterm use of steroid therspy. We applied ten times of plasmapheresis to him over six months period. After the plasmapheresis, clinical symptoms have greatly improved and steroid maintenance doses have been decreased. We have not observed any side effect of plasmapheresis such as thrambotopenia or hypogarnrnaglobulinemia during the whole therapeutic period.
Antibodies ; Humans ; Immune System Diseases ; Pemphigoid, Bullous* ; Plasmapheresis*

Rasch was the first to use the term carcinoma erysipelatoides in the dermatologic literature, and it is most frequently associated with breast carcinoma. This inflammatory type of rnetastatic carcinoma of the breast is an uncommon but well known entity. It occurrs almost exclusively in women, tends to appear in the anterior chest wall, and is usually found after recognition of the primary tumor which is usually adenocarcinoma or undifferentiated carcinoma. It has an acute inflammatory process with rapid progress, with many fatalities resulting in a short time. We report a case of carcinoma erysipela,toides originating from a breast carcinoma with characteristic clinical and histopathologic findings.
Adenocarcinoma ; Breast ; Breast Neoplasms ; Carcinoma ; Female ; Humans ; Skin* ; Thoracic Wall

Serum IgE values were determined in 100 patients vith atogic dermatitis in the Department of Dermatology at Severance Hospital, Yonsei University College of Medicine, from Oct. 1, 1978 to Mar. 31, 1980. Mean IgE value of patients is 293. 4 unit/ml which is significantly higher than reference value, and the following results were obtained. 1. The mean IgE value in adults with atopic dermatitis, 339.8 unit/ml, 30 cases: more tian 16 years old) was significantly higher than in children(251. 4 unit /ml, 70 cases). 2. Male patierts showed higher IgE va,lue (mean 324. 5 unit/ml, 62 cases,' than female patients (242.7 unit/ml, 38 cases). 3. No close correlation was demonstrated between IgE values and eosiniphils in blood. 4. Patients with parasitic infesta,tion (5 cases) showed relatively increased IgE values (mean; 116. 2 unit/ml;. 5. Patients wifh bronchial asthma (2 cases) showed high IgE values exceeding 1000 unit/ml, 6. IgE. values roughly correlated with severity of the dermatitis. Four cases with high IgE values exceeding 1000 unit/ml were included in the clinically severe group.
Adult ; Asthma ; Dermatitis ; Dermatitis, Atopic* ; Dermatology ; Female ; Humans ; Immunoglobulin E* ; Male ; Reference Values

No abstract available.
Nipples* ; Poroma*

BACKGROUND: Follicular thyroid carcinoma (FTC) is the second most common thyroid malignancy and its differential diagnosis includes follicular adenoma (FA) and adenomatous goiter (AG). Several ancillary markers have been suggested to aid in the diagnosis of FTC, but the successful use of these methods still needs to be validated. METHODS: In the present study, we verified the immunoexpression of HMGA2, CEACAM6, survivin, and SFN/14-3-3 delta in lesions including 41 AGs, 72 FAs, and 79 FTCs. We evaluated their diagnostic usefulness, combined with galectin 3, Hector Battifora mesothelial 1 (HBME1), cytokeratin 19, and cyclin D1, in diagnosing FTC. RESULTS: The expressions of HBME1 (65.8%) and HMGA2 (55.7%) were significantly higher in FTCs than in FAs and AGs (p<.001 and p=.005, respectively). HBME1 was the only marker that was more frequently expressed in FTCs than in FAs (p=.021) and it was more frequently expressed in follicular neoplasms than in AGs (p<.001). Among the novel markers, the combination of HMGA2 and HBME1 showed the highest sensitivity (72.2%) and specificity (76.1%) for diagnosing FTC. CEACAM6, survivin, and SFN/14-3-3 delta were barely expressed in most cases. CONCLUSIONS: Our present results show that only HMGA2 can be beneficial in differentiating FTC using the novel markers.
Adenocarcinoma, Follicular* ; Adenoma ; Cyclin D1 ; Diagnosis ; Diagnosis, Differential ; Galectin 3 ; Goiter ; Keratin-19 ; Sensitivity and Specificity ; Thyroid Gland

Tracheal tumors are uncommon comprising less than 0.1% of all malignancies. Metastatic tracheal tumors, especially form the extrathoracic sites, are exceedingly rare. Ovarian cancer tends to metastasize to the serous cavities and the lymph nodes. One large autopsy study reported tracheal involvement in 1% of patients who had died from ovarian cancer. Other studies have not mentioned tracheal involvement at all. Since the main symptoms of cough, hemoptysis, or wheezing are nonspecific, patients may be initially treated for other conditions including asthma or bronchitis. Here we describe a metastatic tracheal tumor from an overain carcinoma that was initally treated for bronchial asthma.
Asthma ; Autopsy ; Bronchitis ; Cough ; Hemoptysis ; Humans ; Lymph Nodes ; Ovarian Neoplasms ; Respiratory Sounds

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

BACKGROUND: Recent studies have suggested that loss of p27Kip1 expression correlates with poor prognosis in gastric carcinomas, although the published data is still controversial. However, there are very few reports on the immunohistochemical expression of p27Kip1 in gastric adenomas and its significance in the progression of gastric adenomas to early gastric carcinomas (EGCs) is unclear. We therefore performed an immunohistochemical study for p27Kip1 expression in gastric adenomas with low- and high-grade dysplasia and EGCs to investigate the role of p27Kip1 expression in gastric carcinogenesis. METHODS: We collected 45 cases of endoscopic mucosal resection specimens which were diagnosed as gastric adenomas and 20 cases of gastrectomy specimens showing EGCs. Using a monoclonal antibody against p27Kip1, the im- munohistochemistry was performed on paraffin-embedded specimens. RESULTS: The loss of p27Kip1 immunoreactivity (<5% of the tumor cells) tended to be observed more frequently in high-grade adenomas than in the low-grade. The loss was found significantly higher in the EGCs than in both high-grade and low-grade adenomas (p=0.000). Gastric adenomas with villous component showed significant loss of p27Kip1 expression (p=0.057). CONCLUSION: These results suggest that loss of p27Kip1 expression can play a role in the progression of gastric adenomas into adenocarcinomas and the villous component allows reliable estimation of the possibility for malignant transformation.
Adenocarcinoma ; Adenoma* ; Carcinogenesis ; Gastrectomy ; Immunohistochemistry ; Prognosis ; Stomach