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

Patients with NIDDM are at increased risk for the development of coronary atherosclerosis and experience more silent myocardial infarction than non-diabetic subjects. The screening tools for early detection of coronary artery disease without significant narrowing has been requested in diabetic patients. Coronary artery calcification (CAC) score, quantified by electron beam computed tomography (EBT), have been reported to correlate with the amount of atherosclerotic plaque and vascular luminal narrowing. We investigated the distribution of CAC score and associated risk factors in asymptomatic NIDDM patients and patients with ischemic heart disease to estimate the usefulness of CAC as a screenig tool for ischemic heart disease in asymptomatic diabetes. METHOD: 136 NIDDM patients without any symptom of coronary artery disease and 37 patients with significant coronary artery stenosis were included. CAC were measured by electron bean tomography (ultrafast CT). Forty contiguous 3-mm thickness transverse two-dimensional sections were obtained through root of aorta and heart. Coronary calcification were defined as the presense of at least two adjacent pixel within the border of visualized coronary artery with CT number of at least 130 HU. Body mass index, waist-hip ratio were measured and body fat components were counted by impedence method. Visceral fat versus subcutaneous fat ratio were calculated by abdominal computed tomography. Plasma lipid profile, fasting insulin, C-peptide level, HbA1c concentration were measured. Correlations between natural log of CAC score and clinical parameters were evaluated and multiple regression analysis with natural log of CAC score as a independent variable was performed. Coronary angiography were performed in 17 asymptomatic NIDDM patients.. RESULT: CAC score was significantly higher in male than female subjects and increased significantly with aging (p<0.01). In patients with hypertension, previous history of cerebrovascular or peripheral vascular disease (p<0.05), CAC score was significantly increased. The CAC score showed significant positive correlations with smoking amount, duration of diabetes and a negative correlation with HDL-cholesterol (p<0.05). There were no association between CAC score and total cholesterol, LDL-cholesterol, waist to hip circumference ratio, or fasting insulin levels. After adjustment of compounding variables (age, sex), duration of diabetes, amount of smoking and previous history of atherosclerotic vascular disease were shown to be associated with CAC score. In multiple logistic regression analysis with natural log of CAC score as dependent variable, age, HDL-cholesterol, duration of diabetes, male gender were found to be significant independent variables. Seventeen diabetic patients with high CAC score, were taken coronary angiography and significant luminal narrowings (more than 50%) of coronary artery were documented in 16 patients. But, In 7 out of 17 patients with coronary one-vessel disease, coronary calcification were not detected by EBT. CONCLUSION: coronary artery calcium score quantified by electron beam computed tomography may be useful for screening of preclinical or asymptomatic coronary artery disease in asymptomatic NIDDM patients.
Adipose Tissue ; Aging ; Aorta ; Body Mass Index ; C-Peptide ; Calcium ; Cholesterol ; Coronary Angiography ; Coronary Artery Disease* ; Coronary Disease ; Coronary Stenosis ; Coronary Vessels* ; Diabetes Mellitus ; Diabetes Mellitus, Type 2 ; Fasting ; Female ; Heart ; Hip ; Humans ; Hypertension ; Insulin ; Intra-Abdominal Fat ; Logistic Models ; Male ; Mass Screening* ; Myocardial Infarction ; Myocardial Ischemia ; Peripheral Vascular Diseases ; Phenobarbital ; Plaque, Atherosclerotic ; Plasma ; Risk Factors ; Smoke ; Smoking ; Subcutaneous Fat ; Tomography, X-Ray Computed* ; Vascular Diseases ; Waist-Hip Ratio

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

BACKGROUND: Visceral fat accumulation plays a major role in metabolic syndrome and increased cardiovascular risks. Clinical evaluation of visceral fat by CT is not widely used because of high cost, low availability, and ionizing radiation risk, and alternative means are required. To assess the usefulness of ultrasound measurement of I-A fat distance for the evaluation of visceral obesity, we compared several length-associated parameters (I-A fat distance, I-A fat distance to thigh muscle length ratio, and I-A fat distance to abdominal subcutaneous fat length ratio) with area-associated parameters (VAT, visceral fat to subcutaneous fat area ratio [VSR] and VMR) measured by CT. METHODS: We evaluated 65 patients with type 2 diabetes (34 men and 31 postmenopausal women). CT scans were obtained at the L4-5 level and the mid-portion between greater trochanter and upper margin patella. I-A fat distance was defined as the distance between the internal face of rectus muscle and the anterior wall of the aorta. Also ultrasonography for measurement of I-A fat distance was performed in 20 men of subjects. RESULTS: Of the various parameters examined, I-A fat distance was most closely correlated with the VAT (r=0.76, p<0.001) and VMR (r=0.71, p<0.001) but not VSR (r=0.18, p=0.22). This distance positively correlated with serum triglyceride and free fatty acids in men (r=0.56 and r=0.59, p<0.05, respectively), and with uric acid (r=0.70, p<0.05), fasting insulin levels (r=0.70, p<0.001), insulin resistance index by HOMA (r=0.69, p<0.001), systolic blood pressure (SBP) (r=0.73, p<0.05), and diastolic blood pressure (DBP) (r=0.85, p<0.001) in women. When the clinical and laboratory data was considered by tertiles of I-A distance, serum uric acid, fasting insulin levels, insulin resistance index (HOMA), BMI, waist or hip circumference, SBP, and DBP were significantly higher in the top tertile, but not WHR. I-A fat distance measured by ultrasonography significantly correlated with VAT, VSR, and VMR (r=0.79, r=0.71, and r=0.79, p<0.05, respectively). CONCLUSION: These results suggest that 1) I-A fat distance may substitute for the VAT in assessment of visceral fat content and may be relatively good parameter indicative of visceral obesity and/or insulin resistance, and 2) several parameters (e.g., VAT, VSR, and VMR) related with visceral fat by CT may be replaced with ultrasonographic I-A fat distance.
Aorta ; Blood Pressure ; Fasting ; Fatty Acids, Nonesterified ; Female ; Femur ; Hip ; Humans ; Insulin ; Insulin Resistance ; Intra-Abdominal Fat* ; Male ; Muscle, Skeletal* ; Obesity, Abdominal ; Patella ; Radiation, Ionizing ; Subcutaneous Fat ; Subcutaneous Fat, Abdominal ; Thigh ; Tomography, X-Ray Computed ; Triglycerides ; Ultrasonography ; Uric Acid

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

Adrenal cysts are rare lesions usually discovered incidentally during surgery, or at the time of autopsy. Most adrenal cysts are generally asymptomatic and small, less than 10 cm in diameter. When adrenal cysts enlarge sufficiently, they cause pain and gastrointestinal disturbances or become palpable. When cystic lesions in upper abdomen are found, many cystic lesions such as hepatic, splenic, renal and pancreatic cysts should be considered. It's difficult to differentiate between benign and malignant lesions. Usually these cystic lesions have been excised to rule out malignancy. Herein we report a case of adrenal endothelial cyst which was detected in a 63-year-old female patient during a routine health examination by ultrasonography.
Abdomen ; Adrenal Glands* ; Autopsy ; Female ; Humans ; Middle Aged ; Pancreatic Cyst ; Ultrasonography

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

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