Adrenocorticotropin (ACTH) independent bilateral macronodular adrenal hyperplasia (AIMAH) is a rare form of Cushing's syndrome, in which unique endocrinological, clinical and histopathological features have been described. In AIMAH, cortisol secretion is autonomous and independent of ACTH, thus plasma ACTH levels are persistently suppressed. Various etiological mechanisms have been proposed to explain the development of AIMAH, the development of aberrant adrenal sensitivity to gastric inhibitory polypeptide (GIP), vasopressin, beta-adrenergic receptor agonists or the presence of circulating adrenal stimulating immunoglobulins have been suggested. We report on a 46-year-old female who had Cushing's syndrome, due to AIMAH, with a positive response to vasopressin.
Adrenergic beta-Agonists ; Adrenocorticotropic Hormone ; Cushing Syndrome ; Female ; Gastric Inhibitory Polypeptide ; Humans ; Hydrocortisone ; Hyperplasia* ; Immunoglobulins ; Middle Aged ; Plasma ; Vasopressins

BACKGROUND: Leptin has been suggested as a possible cause of atherosclerotic disease. The small dense low-density lipoprotein cholesterol (LDL-C) has also been regarded as a new surrogate marker in atherosclerotic disease. The aim of this study was to evaluate the relationship between the leptin concentration and the small dense LDL-C concentration in Korean type 2 diabetic patients. METHODS: One hundred-ninety one type 2 diabetic patients, who did not use any medication that could affect the concentration of lipid such as statin, fibrate, thiazolidinediones and corticosteroid, were enrolled in this study. We analyzed the relationship between leptin, the small dense LDL-C and the other metabolic parameters. RESULTS: The small dense LDL-C concentrations were higher in the group with the highest tertile of the leptin value, both in males and females than those patients in the group with the lowest tertile of the leptin value. The small dense LDL-C concentrations were also higher in the group with the highest tertile of leptin divided by the BMI value both in males and females than those patients in the group with the lowest tertile of the leptin value. The leptin concentration was positively correlated with the small dense LDL-C, total cholesterol, triglyceride, LDL-C, insulin and HOMAIR values after adjusting for age, gender and BMI. CONCLUSION: The association between leptin and small dense LDL-C could be a factor that explains the association between leptin and cardiovascular disease.
Biomarkers ; Cardiovascular Diseases ; Cholesterol ; Cholesterol, LDL* ; Diabetes Mellitus, Type 2 ; Female ; Humans ; Hydroxymethylglutaryl-CoA Reductase Inhibitors ; Insulin ; Leptin* ; Lipoproteins ; Male ; Thiazolidinediones ; Triglycerides

BACKGROUND: Leptin has been suggested as a possible cause of atherosclerotic disease. The small dense low-density lipoprotein cholesterol (LDL-C) has also been regarded as a new surrogate marker in atherosclerotic disease. The aim of this study was to evaluate the relationship between the leptin concentration and the small dense LDL-C concentration in Korean type 2 diabetic patients. METHODS: One hundred-ninety one type 2 diabetic patients, who did not use any medication that could affect the concentration of lipid such as statin, fibrate, thiazolidinediones and corticosteroid, were enrolled in this study. We analyzed the relationship between leptin, the small dense LDL-C and the other metabolic parameters. RESULTS: The small dense LDL-C concentrations were higher in the group with the highest tertile of the leptin value, both in males and females than those patients in the group with the lowest tertile of the leptin value. The small dense LDL-C concentrations were also higher in the group with the highest tertile of leptin divided by the BMI value both in males and females than those patients in the group with the lowest tertile of the leptin value. The leptin concentration was positively correlated with the small dense LDL-C, total cholesterol, triglyceride, LDL-C, insulin and HOMAIR values after adjusting for age, gender and BMI. CONCLUSION: The association between leptin and small dense LDL-C could be a factor that explains the association between leptin and cardiovascular disease.
Biomarkers ; Cardiovascular Diseases ; Cholesterol ; Cholesterol, LDL* ; Diabetes Mellitus, Type 2 ; Female ; Humans ; Hydroxymethylglutaryl-CoA Reductase Inhibitors ; Insulin ; Leptin* ; Lipoproteins ; Male ; Thiazolidinediones ; Triglycerides

BACKGROUND: Tibolone is a novel synthetic compound with tissue-specific effects in bone, breast tissue and the endometrium. Tibolone, and active vitamin D, effectively prevent bone loss, and the maintain skeletal integrity of postmenopausal women. The aim of the present study was to examine the effect of tibolone, and active vitamin D (1-hydroxyvitamin D3), therapies given alone, or in combination, against bone loss in postmenopausal women. METHODS: One hundred and three postmenopausal women were treated with tibolone (n=40), alphacalcidol (n=27) or both drugs (n=36) for 12 months. All subjects took supplemental calcium carbonate (500 mg daily). The bone mineral densities (BMD) of the lumbar spine and proximal femur were measured by dual-energy x-ray absorptiometry (DXA) at the baseline and after 12 months. RESULTS: Tibolone therapy produced significant increase of 4.1 and 1.8% in the BMD at the lumbar spine (p<0.001) and femoral neck (p=0.009), respectively. The combination of tibolone and active vitamin D increased the BMD by 8.0 and 4.4% (p<0.001) at the spine and femoral neck, respectively. The differences in the change of BMD from the baseline at the lumbar spine was significant (p=0.038) in the combination treatment group compared that in the tibolone alone group. CONCLUSION: Tibolone alone, and in combination with active vitamin D, effectively increased the BMD at all skeletal sites in postmenopausal women. Combination treatment for osteoporosis is emerging as a promising modality in Korean postmenopausal women.
Absorptiometry, Photon ; Bone Density* ; Breast ; Calcium Carbonate ; Endometrium ; Female ; Femur ; Femur Neck ; Humans ; Osteoporosis ; Spine ; Vitamin D* ; Vitamins*

Klinefelter's syndrome is one of the most common forms of primary hypogonadism presenting with gynecomastia, azospermia and increased follicle-stimulating hormone. It is well known that this syndrome has an increased incidence of neoplasia, especially breast cancer and extragonadal germ cell tumors. However, it is rarely associated with an intracranial tumor of maldevelopmental origin, especially in the suprasellar area. We report, for the first time, a case of Klinefelter's syndrome, with a Rathke's cleft cyst is the patient was a 32-year-old male who was known to have an incidentaloma form brain computed tomography, which was clinically diagnosed as a suprasellar tumor. After operating, the suprasellar mass was confirmed as a Rathke's cleft cyst, and his hormonal abnormality, an elevated level of follicle-stimulating hormone, was not normalized. Therefore, we performed chromosomal analysis, and diagnosed Klinefelter's syndrome with the XXY karyotype.
Adult ; Brain ; Breast Neoplasms ; Follicle Stimulating Hormone ; Gynecomastia ; Humans ; Hypogonadism ; Incidence ; Karyotype ; Klinefelter Syndrome* ; Male ; Neoplasms, Germ Cell and Embryonal

BACKGROUND: High sensitivity C-reactive protein (hsCRP) is more sensitive than standard CRP assay for evaluation of risk of coronary heart diseases and other atherosclerotic events. But, there were no data of association of serum hsCRP with risk factors of cardiovascular diseases and nonalcoholic fatty liver in Korean type 2 diabetic and nondiabetic subjects. METHODS : A hundred type 2 diabetic subjects (51 men and 49 women) from Severance Hospital and 200 nondiabetic subjects participating medical checkup in Health Promotion Center (105 men and 95 women) were recruited and subjects with acute illnesses and chronic inflammatory diseases such as upper respiratory infection, rheumatoid arthritis, osteoarthritis, or viral hepatitis were excluded. A standardized interview was conducted by trained personnel; detailed information was collected on medical history, dietary habits and lifestyle characteristics, including smoking, alcohol and physical activity. Body mass index (BMI) was computed and biochemical study were undergone using fasting blood. All subjects were done abdominal ultrasonography for evaluation of fatty liver. Serum hsCRP concentration was measured by Nephelometer AnalyzerII (Behring Co.) and a lower detection limit of test was 0.18 mg/L. RESULTS : There was no difference in sex, BMI, presence of fatty liver, concentration of total cholesterol, triglyceride, high density lipoprotein-cholesterol (HDL-C), low density lipoprotein cholesterol (LDL-C) and uric acid between diabetic and nondiabetic subjects. Age, total colesterol/HDL-C ratio, fasting blood glucose and incidence of hypertension were higher in diabetic than nondiabetic subjects, but a rate of smoking was higher in nondiabetic than diabetic subjects. The mean concentration of serum hsCRP was remarkably increased in type 2 diabetic subjects than nondiabetic subjects (1.34 +/- 1.87 vs 0.71 +/- 0.80 mg/L, p<0.05). After adjustment of different variables between both groups, there was significantly difference of the concentration of serum hsCRP (p<0.05). In nondiabetic subjects, by univariate analysis, there was a positive correlation between hsCRP and age (r=0.26, p<0.05), BMI (r=0.34, p<0.05), systolic blood pressure (r=0.21, p<0.05), diastolic blood pressure (r=0.16, p<0.05), triglyceride (r=0.27, p<0.05), total cholesterol/HDL-C ratio (r=0.22, p<0.05), uric acid (r=0.15, p<0.05) and a negative correlation between serum hsCRP and HDL-C (r=-0.16, p<0.05). Interestingly, subjects with fatty liver had shown increased serum hsCRP concentration than subjects without fatty liver (0.99 +/- 0.96 vs 0.58 +/- 0.69 mg/L, p<0.05). But there were no correlation of serum hsCRP with the history of smoking, sex, physical activity, fasting plasma glucose and presence of hypertension. After multiple regression analysis, only BMI and age were associated with serum hsCRP. In diabetic subjects, there were significant correlation of serum hsCRP with HDL-C and fasting plasma glucose, but other risk factors of cardiovascular diseases and fatty liver were not. When we compared serum hsCRP according to numbers of risk factors of cardiovascular diseases in nondiabetic subjects, group without risk factors had 0.41 +/- 0.55 mg/L, group with one risk factor had 0.48 +/- 0.40 mg/L, group with two risk factors had 0.75 +/- 0.88 mg/L, group with three risk factors had 1.08 +/- 0.87 mg/L and group with four risk factors had 1.55 +/- 1.21 mg/L. There was significant difference of serum hsCRP according to numbers of risk factors of cardiovascular diseases (p<0.05). CONCLUSION : Serum hsCRP is correlated with risk factors of cardiovascular diseases and may be useful tool for prediction of accelerated, atherosclerotic process in nondiabetic subjects. Although there is association of serum hsCRP with few risk factors of cardiovascular diseases, serum hsCRP is elevated in diabetic subjects. Therefore it is necessary to evaluate usefulness of serum hsCRP using carefully selected diabetic subjects. In addition, our study had shown that subjects with nonalcoholic fatty liver have increased risk of cardiovascular events.
Arthritis, Rheumatoid ; Blood Glucose ; Blood Pressure ; Body Mass Index ; C-Reactive Protein* ; Cardiovascular Diseases* ; Cholesterol ; Cholesterol, LDL ; Coronary Disease ; Fasting ; Fatty Liver ; Food Habits ; Health Promotion ; Hepatitis ; Humans ; Hypertension ; Incidence ; Life Style ; Limit of Detection ; Male ; Motor Activity ; Osteoarthritis ; Risk Factors* ; Smoke ; Smoking ; Triglycerides ; Ultrasonography ; Uric Acid

Pendred syndrome is an autosomal recessive genetic disorder, which is characterized by sensorineural hearing loss, goiter and a positive perchlorate discharge test. It is caused by mutations of the PDS gene, and its clinical characteristics vary widely. The thyroid function in most cases is normal, or shows only mild hypothyroidism. In Pendred syndrome, there is an organification defect that leads to defective thyroid hormone synthesis, followed by chronic TSH stimulation. Herein is reported a case of a follicular thyroid carcinoma associated with Pendred syndrome. To our knowledge, this is the first case reported in Korea. The patient presented with a huge anterior neck mass, sensorineural hearing loss and a positive perchlorate discharge test. Fine needle aspiration cytology suggested malignancy of the thyroid, and a total thyroidectomy, with central compartment node dissection, was performed. The pathology from the thyroid mass showed a poorly differentiated follicular thyroid carcinoma
Adenocarcinoma, Follicular* ; Biopsy, Fine-Needle ; Goiter ; Hearing Loss, Sensorineural ; Humans ; Hypothyroidism ; Korea ; Neck ; Pathology ; Thyroid Gland ; Thyroidectomy

Pendred syndrome is an autosomal recessive genetic disorder, which is characterized by sensorineural hearing loss, goiter and a positive perchlorate discharge test. It is caused by mutations of the PDS gene, and its clinical characteristics vary widely. The thyroid function in most cases is normal, or shows only mild hypothyroidism. In Pendred syndrome, there is an organification defect that leads to defective thyroid hormone synthesis, followed by chronic TSH stimulation. Herein is reported a case of a follicular thyroid carcinoma associated with Pendred syndrome. To our knowledge, this is the first case reported in Korea. The patient presented with a huge anterior neck mass, sensorineural hearing loss and a positive perchlorate discharge test. Fine needle aspiration cytology suggested malignancy of the thyroid, and a total thyroidectomy, with central compartment node dissection, was performed. The pathology from the thyroid mass showed a poorly differentiated follicular thyroid carcinoma
Adenocarcinoma, Follicular* ; Biopsy, Fine-Needle ; Goiter ; Hearing Loss, Sensorineural ; Humans ; Hypothyroidism ; Korea ; Neck ; Pathology ; Thyroid Gland ; Thyroidectomy

An adrenal cortical carcinoma is a rare malignancy associated with poor prognosis. On diagnosis, most patients present with large tumor masses, which are often detected at an advanced stage. The most effective treatment is a complete resection, which is the only curative treatment for adrenal cortical carcinomas. The most important prognostic factor is a successful resection of the primary tumor, as long as it is low-grade and has not spread to distant sites. However, with advanced adrenal cortical carcinomas, with distant metastasis, there is no strict effective treatment program, and the prognosis is poor. The case of a 50-year-old female patient, presenting with an adrenal cortical carcinoma and Cushing's syndrome, who had a long-term survival of 78 months, is reported. The mass was completely resected on diagnosis, but 16 months later liver metastasis was discovered. She had received chemotherapy, with cisplatin, etoposide and bleomycin, for the liver metastasis for a period of 15 months, but with no response, furthermore, the size had increased after 10 months. Afterward, she received her 10th session of intrahepatic artery cisplatin chemotherapy and her 3rd hepatic artery embolization. Although the patient had a large degree of liver metastasis, this was tolerated. The tumor mass is presently not aggravated, and she still survives after 78 months.
Adrenocortical Carcinoma* ; Arteries ; Bleomycin ; Cisplatin ; Cushing Syndrome ; Diagnosis ; Drug Therapy ; Etoposide ; Female ; Fibrinogen ; Hepatic Artery ; Humans ; Liver* ; Middle Aged ; Neoplasm Metastasis* ; Prognosis

No abstract available.
Candida* ; Cerebrospinal Fluid Shunts ; Meningitis*