A Case of Papillary Thyroid Carcinoma Combined with Multiple Endocrine Neoplasia Type 2A / 대한내분비학회지

Multiple endocrine neoplasia (MEN) type 2A is a syndrome of medullary thyroid carcinomas, pheochromocytomas and parathyroid hyperplasia. The simultaneous occurrence of medullary, and papillary, thyroid carcinomas is rare because they are derived from, apparently, different germ layers, the former from the neuroectoderm and the latter from the endoderm. We report a case of a papillary thyroid carcinoma, combined with a medullary thyroid carcinoma, in a patient with MEN type 2A. Molecular genetic studies for screening a RET proto-oncogene mutation revealed a point mutation in codon 631 on chromosome 10, which is reported as highly uncommon in MEN type 2A.

A case of primary amenorrhea due to 17alpha- hydroxylase deficiency / 대한내과학회지

The enzyme, 17 -hydroxylase, is necessary for both cortisol and estrogen synthesis. Deficiency of the hormone results in increased adrenocorticotrophic hormone (ACTH), follicle-stimulating hormone (FSH). Synthesis of progesterone, 11-deoxycorticosterone (DOC), corticosterone and aldosterone don't require the enzyme. The lack of estrogen results in primary amenorrhea and absent sexual maturation. The replacement of dexamethasone and estrogens has lowered the blood pressure and produced feminization. A 19-year-old female had 46,XX genotype and presented amonorrhea, absence of sexual characteristics, hypertension and hypokalemia. Endocrinologic studies demonstrated increased plasma progesterone, ACTH levels and low production of 17 -hydroxyprogesterone and testosterone. We report a rare case of 17 -hydroxylase deficency with a brief history and review of the literature.

The Effect of alpha MSH Analogues on Rat Bones

Melanocortin is the downstream mediator of leptin signaling and absence of leptin signaling in ob/ob and db/db mice revealed the enhancement of bone formation through the central regulation. While alpha-melanocyte-stimulating hormone (alpha MSH) inhibits the secretion of interleukin-1 alpha and tumor necrosis factor-alpha from the inflammatory cells, alpha MSH can also enhance clonal expansion of pro B cells linked to stimulation of osteoclastogenesis. Therefore, we tested the effect of melanocortin on bones. alpha MSH analogues [6His] alpha MSH-ND and [6Asn] alpha MSH-ND were synthesized and the radio-ligand receptor binding- and cyclic AMP generating activity were analyzed in China Hamster Ovary cell line over- expressing melanocortin receptors. The EC50 of [6His] alpha MSH-ND measured from melanocortin-1, 3, 4 and 5 receptors were 0.008 0.0045, 1.523 0.707, 0.780 0.405, and 250.320 42.234 nM, respectively, and the EC50 of [6Asn] alpha MSH-ND were 16.8 6.94, 271.8 21.95, 8.0 1.21, and 1132.5 635.46 nM, respectively. Four weeks after the subcutaneous injection of the analogues, the body weights in the [6His] alpha MSH-ND and the [6Asn] alpha MSH-ND treated groups (346.0 20.63 g vs. 350.0 13.57 g) were lower than that of the vehicle treated group (375.8 17.31 g, p 0.05). There was no difference in the total femoral BMD measured by dual x-ray absorptiometry among the three groups. Among the three groups, there were no differences in the total numbers of crystal violet positive- or alkaline phosphatase positive colonies, in the expression of Receptor Activator of Nuclear Factor Kappa-B ligand on the tibia and the total number of multinucleated osteoclast-like cells differentiated from primary cultured bone marrow cells. From the above results, no evidence of bone gain or loss was found after treatment of the alpha MSH analogues peripherally.

Association of serum high sensitivity C-reactive protein with risk factors of cardiovascular diseases in type 2 diabetic and nondiabetic subjects without cardiovascular diseases / 대한내과학회지

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.

A Case of Bilateral Macronodular Adrenal Hyperplasia with Cushing's Syndrome Treated by Unilateral Adrenalectomy / 대한내분비학회지

In order to differentiate the causes of Cushing's syndrome; whether it is pituitary or adrenal-dependent, a high dose dexamethasone suppression test is usually performed but this does not always correlated with the imaging diagnosis. We report a case of bilateral macronodular adrenal hyperplasia, which was thought to be independent to adrenocorticotrophin (ACTH) stimulation. At first we thought it was pituitary-dependent Cushing's syndrome, due to its suppression by high dose dexamethasone. However, we found no abnormal findings on the brain sella magnetic resonance image (MRI). A significant finding, however, we found bilateral adrenal masses on the abdominal computed tomography (CT). We performed percutaneous selective adrenal venous sampling (PSAVS), and confirmed hypercortisolism of the left adrenal mass only. Therefore, we decided to remove the left adrenal gland to preserve the residual function of the right adrenal gland. After the left adrenalectomy, the patient became normotensive, and their buffalo hump disappeared, and her 24 hour urinary free cortisol level returned to normal.

Effects of cyclosporin A on sex hormone and estrogen receptor in male rat with special reference to cyclosporin A-induced osteoporosis

The mechanisms of high turnover bone loss induced by Cyclosporin A (CsA) are not clearly understood. Deficiencies in sex hormones result in high turnover osteoporosis, and not only androgen but also estrogen plays an important role in maintaining bone mass in men. To study whether or not there are any changes in the levels of sex hormones, aromatization, and the expression of estrogen receptors in CsA-induced osteoporosis, we treated 39 rats with vehicle, low-dose CsA (5 mg/kg) and high dose CsA (15 mg/kg) for 28 days, and measured sex hormone levels by radioimmunoassay. Aromatase activities in ROS cells and 3T3-L1 cells were determined by measuring the conversion rate of 3H-androstenedione into 3H-estrone. ER and ER mRNA were measured by competitive RT-PCR in collected marrow cells and ROS cells. The levels of free testosterone in the serum in low-dose CsA-treated rats were unchanged, but the levels were significantly decreased in those treated with high-dose CsA as previously reported. The levels of total estradiol in the serum were significantly increased in the low-dose CsA-treated group (5 mg/kg) and were comparable to levels of the control group in the high-dose CsA-treated group (15 mg/kg). CsA increased the conversion of 3H-androstenedione to 3H-estrone in ROS cells, but not in 3T3-L1 cells. Meanwhile, CsA treatment did not change the rates of ER or ER mRNA expression in ROS cells or in collected bone marrow cells. In conclusion, CsA treatment decreased the level of free testosterone in the serum, but did not decrease the level of serum estradiol by enhancing aromatization. High-turnover osteoporosis induced by clinical dosage CsA treatment may not be caused by lowering the levels of circulating estrogen or by decreasing the expression of estrogen receptors.

A Case of Propylthiouracil Induced Anti: Neutrophil Cytoplasmic Antibody (ANCA) Positive Vasculitis / 대한내분비학회지

A 40-year-old woman just had started to take propylthiouracil for Graves disease, However, the treatment had to be interrupted because she developed skin rash, arthritis, chest pain, fever and proteinuria after 2 months. The serologic study revealed antineutrophil cytoplasmic antibody(ANCA) positivity, especially showing perinuclear pattern. The anti-myeloperoxidase titer was high. The hematoxylin & eosin stain of the specimen obtained from kidney was compatible with pauci-immune glomerulonephritis with crescent formation. There were no immune complex deposits under electron microscope. Such findings suggested propylthiouracil-induced vasculitis. Vasculitis is a rare side effect of propylthiouracil. Recently more cases of vasculitis associated with anti-thyroid drug with ANCA positivity are being reported up to about 36 cases worldwide. There are possibilities of underdiagnosis of this side effect, meaning more cautions on the patients under anti-thyroid drug treatment. We present a case with review of related literature.

A Case of Endobronchial Lipoma causing left lung collapse / 대한내과학회지

Endobronchial lipoma is a rare, benign neoplasm of the large bronchi, which makes up about 0.1 percent of all lung tumors. It can produce irreversible parenchymal lung damage or bronchiectasis if diagnosis and treatment are delayed. The treatment of endobronchial lipoma is early removal and surgical procedures depend on the status of distal parenchymal lung damage. We experienced a case of endobronchial lipoma in 53 year-old female patient, which caused total collapse of left lung and save left lung by enucleation of tumor via bronchotomy.

A Case of Pulmonary Eosinophilic Granuloma in a Passive-Smoking Reproductive Female / 결핵

Pulmonary eosinophilic granuloma, a rare form of Langerhans cell histiocytosis confined to lung, has no known etiology and variable natural history. It is characterized by discrete proliferation of Langerhans cells. It is known that over 90% of the patients are cigarette smokers, and it is rarely reported in non- or passive-smoking patients, especially female. Here we describe a case of pulmonary eosinophilic granuloma in a passive-smoking, reproductive female patient presented with spontaneous pneumothorax. We identified S-100 and CDla positive histiocytes on immunohistochemical stain of the lung tissue obtained by open lung biopsy.