Primary Pigmented Nodular Adrenal Dysplasia (PPNAD) is a rare cause of Cushing's syndrome in infants and young adults. The familial occurrence, it may be variably associated with a complex of other pathologic characteristics that manifests extraadrenal disorders (includes cardiac myxomas, lentigines, mammary myxoid lesions, testicular tumors, pituitary adenomas, and neuroectodermal tumors) was considered indicative of Carneys complex. This was based on the failure of cortisol suppression by high-dose dexamethasone, either normal or suppressed basal adrenocorticotropic hormone (ACTH) levels, and normal radiographic studies of the sellar turcica, and adrenals glands is almost normal or slightlg eulaged.. Bilateral adrenalectomy has thus the only effective means of cure. The disease may be a component of a rare, but potentially dangerous complex of abnormalities that follow an autosomal-dominant mode of inheritance. Recently we experienced a case of Carney's complex composed by Cushings syndrome due to PPNAD with familial purple colored lentigines on their lips and report it with reviews of the literatures.
Adrenalectomy ; Adrenocorticotropic Hormone ; Cushing Syndrome* ; Dexamethasone ; Humans ; Hydrocortisone ; Infant ; Lentigo ; Lip ; Myxoma ; Neural Plate ; Pituitary Neoplasms ; Testicular Neoplasms ; Wills ; Young Adult

No abstract available.
Animals ; Calcium ; Glucose ; Insulin ; Magnesium ; Pancreas ; Rats

BACKGROUND: Positive correlations between bone mass and androgen levels have been observed in premenopausal and postmenopausal women as well as in men. Androgen production was decreased in women with osteoporosis compared to that in age-matched controls. We hypothesized that androgen metabolism might be also deranged in osteoporosis. To clarify our hypothesis, we investigated the relationship between urinary metabolites of androgen and bone mineral density (BMD) in Korean postmenopausal osteoporotics. METHODS: We examined the anthropometry and bone turnover marker in 67 postmenopausal women. BMD was measured by dual energy X-ray absorptiometry (DEXA). Serurn levels of estrone, estradiol, free testosterone were measured by radioirnmunoassay and serum level of sex hormone binding globulin (SHBG) was measured by two site immunoradiometric assay. The urinary metabolites of androgen were determined by gas chromatography-mass spectrometry (GC-MS) at Korean Institute of Science and Technology Doping Control Center. RESULTS: 1. Spinal BMD had a positive correlation with height (r 0.3049, p<0.05), weight (r=0.4114, p<0.001) and body mass index (BMI, r=0.2638, p<0,05). 2. Spinal and femoral neck BMD had no correlation with serum levels of estrone, estradiol and ten major urinary metabolites of androgen, but serum free testosterone had positive correlation with spinal BMD (r=0.3622, p<0.01) and SHBG had negative correlation with femoral neck BMD (r=-0.2625, p< (0.05). 3. Serum free testosterone in osteoporotics was lower than non-osteoporotics with spinal BMD (p<0.05) and SHBG in patients with osteopenia was higher than non-osteopenic subjects with femoral neck BMD (p <0.05). 4. In multiple stepwise regression analysis, weight and serum free testosterone were statistically significant for spinal BMD (R =0.3072). As for femoral neck BMD, weight was the independent determinant (R 0.1307). 5. Serum level of osteo#ealcin and urinary deoxypyridinoline/creatinine had a positive correlation with urinary 11-ketoandrosterone (p<0.05). SHBG was positive correlation with osteocalcin (r=0.3190, p<0.05). 6. Serum free testosterone (r=-0.2740, p<0.05) decreased with aging. CONCLUSION: Our data suggest that androgen metabolism is not deranged in osteoporotics, but serum free testosterone is important than estrogen on postmenopausal osteoporosis after 5-10 years menopause.
Absorptiometry, Photon ; Aging ; Anthropometry ; Body Mass Index ; Bone Density* ; Bone Diseases, Metabolic ; Estradiol ; Estrogens ; Estrone ; Female ; Femur Neck ; Gas Chromatography-Mass Spectrometry ; Humans ; Immunoradiometric Assay ; Male ; Menopause ; Metabolism ; Osteocalcin ; Osteoporosis ; Osteoporosis, Postmenopausal ; Sex Hormone-Binding Globulin ; Testosterone

Background: Prolactin(PRL) secretion is tonically inhibited by doparnine that originates from the hypothalamic tuberoinfundibular tract and reaches the lactotroph via the hypophyseal portal vessel. Hyperprolactinemia associated with oligomenorrhea-amenorrhea, galactorrhea and/or infertility is mainly due to PRL-secreting pituitary adenoma(PA). The diagnosis of idiopathic hyperprolac- tinemia(IHP) is made, when hyperprolactinemia is sustained and all causes of hyperprolactinemia are excluded without radiological abnormality. It is not known, whether IHP and PA are two distinct entities or two subsequent phases of the same disease. The etiology of both disorders remains unresolved. We investigated that PRL hypersecretion in patients with IHP and PA may be the result of a defect in the central nervous system(CNS)-dopamine release, and that there may be some differences in pathogenesis of both diseases. Methods: We measured 24 hour-urinary dopamine, norepinephrine, epinephrine, and serum and 24 hour-urinary VMA(vanillyl rnandelic acid), HVA(homovanilic acid), DOPAC(3,4-dihydroxy phenylaceticacid), MHPG(3-methoxy 4-hydroxy phenylglycol) in 10 normal controls, 9 patients with IHP, and 17 patients with PA in the early follicular phase. Results: Urinary HVA and DOPAC concentrations, the major metabolites of CNS dopaminergic activity, were signficantly lower in both patients with IHP and PA compared with those in normal controls(p 0.05), whereas they were not different in both disease groups. Dopamine, norepine-phrine, epinephrine, MHPG concentrations were similar to those of the normal controls. Although VMA concentrations of both disease groups were significantly higher than those of normal controls, all of them were within normal range. Conelusion: Although our data are unable to establish the precise biochemical defect responsible for central dopamine deficiency in pathogensis of IHP and PA, we can support the presence of a pathological reduction of brain dopamine activity in IHP and PA.
3,4-Dihydroxyphenylacetic Acid ; Brain ; Diagnosis ; Dopamine ; Epinephrine ; Female ; Follicular Phase ; Galactorrhea ; Humans ; Hyperprolactinemia ; Infertility ; Lactotrophs ; Methoxyhydroxyphenylglycol ; Norepinephrine ; Pregnancy ; Prolactinoma ; Reference Values

Objective: Atrial fibrillation (AF) increases the risk of thromboembolic events, making oral anticoagulants (OACs) essential for high-risk patients. This fact sheet provides nationwide statistics on AF management for stroke prevention in Korea. We aimed to evaluate current anticoagulation treatment trends and strategies in Korea.MethodThe Korean national health claims database from the National Health Insurance Service was used. AF patients aged ≥ 18 years from 2013 to 2022 were included. OAC use, including warfarin and non-vitamin K antagonist OACs (NOACs), was tracked through prescription data. The rates of OAC use were calculated based on continued use, considering prescription dates and amounts. For patients with multiple encounters, the last encounter was used for analysis. Results: During the study, 20.4% of strokes were accompanied by AF, with AF diagnosed within 6 months before or after the stroke. The number of patients diagnosed with AF after a stroke increased from 4893 in 2013 to 6978 in 2022. Among newly diagnosed AF patients requiring OACs, 51% were not prescribed OACs within 6 months. OAC treatment rates for high-risk AF patients increased from 44.6% in 2013 to 77.5% in 2022, with NOAC prescriptions rising significantly after 2015. Regional variations in OAC prescription rates were observed, with lower rates in suburban/rural areas than in urban regions (76.0% vs. 79.6%, p < 0.001). Conclusions Considerable strokes could have been prevented with earlier AF detection and OAC treatment through more intensive electrocardiogram screening.

Objective: Atrial fibrillation (AF) increases the risk of thromboembolic events, making oral anticoagulants (OACs) essential for high-risk patients. This fact sheet provides nationwide statistics on AF management for stroke prevention in Korea. We aimed to evaluate current anticoagulation treatment trends and strategies in Korea.MethodThe Korean national health claims database from the National Health Insurance Service was used. AF patients aged ≥ 18 years from 2013 to 2022 were included. OAC use, including warfarin and non-vitamin K antagonist OACs (NOACs), was tracked through prescription data. The rates of OAC use were calculated based on continued use, considering prescription dates and amounts. For patients with multiple encounters, the last encounter was used for analysis. Results: During the study, 20.4% of strokes were accompanied by AF, with AF diagnosed within 6 months before or after the stroke. The number of patients diagnosed with AF after a stroke increased from 4893 in 2013 to 6978 in 2022. Among newly diagnosed AF patients requiring OACs, 51% were not prescribed OACs within 6 months. OAC treatment rates for high-risk AF patients increased from 44.6% in 2013 to 77.5% in 2022, with NOAC prescriptions rising significantly after 2015. Regional variations in OAC prescription rates were observed, with lower rates in suburban/rural areas than in urban regions (76.0% vs. 79.6%, p < 0.001). Conclusions Considerable strokes could have been prevented with earlier AF detection and OAC treatment through more intensive electrocardiogram screening.

Objective: Atrial fibrillation (AF) increases the risk of thromboembolic events, making oral anticoagulants (OACs) essential for high-risk patients. This fact sheet provides nationwide statistics on AF management for stroke prevention in Korea. We aimed to evaluate current anticoagulation treatment trends and strategies in Korea.MethodThe Korean national health claims database from the National Health Insurance Service was used. AF patients aged ≥ 18 years from 2013 to 2022 were included. OAC use, including warfarin and non-vitamin K antagonist OACs (NOACs), was tracked through prescription data. The rates of OAC use were calculated based on continued use, considering prescription dates and amounts. For patients with multiple encounters, the last encounter was used for analysis. Results: During the study, 20.4% of strokes were accompanied by AF, with AF diagnosed within 6 months before or after the stroke. The number of patients diagnosed with AF after a stroke increased from 4893 in 2013 to 6978 in 2022. Among newly diagnosed AF patients requiring OACs, 51% were not prescribed OACs within 6 months. OAC treatment rates for high-risk AF patients increased from 44.6% in 2013 to 77.5% in 2022, with NOAC prescriptions rising significantly after 2015. Regional variations in OAC prescription rates were observed, with lower rates in suburban/rural areas than in urban regions (76.0% vs. 79.6%, p < 0.001). Conclusions Considerable strokes could have been prevented with earlier AF detection and OAC treatment through more intensive electrocardiogram screening.

Objective: Atrial fibrillation (AF) increases the risk of thromboembolic events, making oral anticoagulants (OACs) essential for high-risk patients. This fact sheet provides nationwide statistics on AF management for stroke prevention in Korea. We aimed to evaluate current anticoagulation treatment trends and strategies in Korea.MethodThe Korean national health claims database from the National Health Insurance Service was used. AF patients aged ≥ 18 years from 2013 to 2022 were included. OAC use, including warfarin and non-vitamin K antagonist OACs (NOACs), was tracked through prescription data. The rates of OAC use were calculated based on continued use, considering prescription dates and amounts. For patients with multiple encounters, the last encounter was used for analysis. Results: During the study, 20.4% of strokes were accompanied by AF, with AF diagnosed within 6 months before or after the stroke. The number of patients diagnosed with AF after a stroke increased from 4893 in 2013 to 6978 in 2022. Among newly diagnosed AF patients requiring OACs, 51% were not prescribed OACs within 6 months. OAC treatment rates for high-risk AF patients increased from 44.6% in 2013 to 77.5% in 2022, with NOAC prescriptions rising significantly after 2015. Regional variations in OAC prescription rates were observed, with lower rates in suburban/rural areas than in urban regions (76.0% vs. 79.6%, p < 0.001). Conclusions Considerable strokes could have been prevented with earlier AF detection and OAC treatment through more intensive electrocardiogram screening.

Multiple endocrine neoplasia (MEN) is a combination of endocrine tumor associations and consists of 3 types: 1, 2A and 2B. Herein, we report a case of insulinoma which is combined with pheochromocytoma discovered at the left adrenal gland which was treated by surgical resection. We think there is a possibility of a new type of multiple endocrine neoplasia (MEN).
Adrenal Glands ; Insulinoma* ; Multiple Endocrine Neoplasia ; Pheochromocytoma*

Hyperprolactinemia is the most common hypothalamo-pituitary disorder encountered in clinical endocrinology. Excluding the drug-induced hyperprolactinemia, the most common cause of this disorder is a pituitary tumor. Prolactinoma is mainly made up of prolactin-secreting cells but pseudoprolactinoma is tumor that does not secrete prolactin itself. The pseudoprolactinoma interrupts the flow of prolactin inhibiting factor, dopamine, from the hypothalamus through the pituitary stalk to the normal pituitary. The differentiation prolactinoma from pseudoprolactinoma is vitally important since true prolactinomas are most commonly responded well in terms of tumor shrinkage to medical treatment using dopamine agonist therapy, whereas pseudoprolactinomas do not. Thus surgical treatment is clearly indicated as first-line treatment if we know that a lesion is a pseudoprolactinoma. We compared prolactinoma with pseudoprolactinoma in clinical and endocrinologic characteristics of 48 cases after immunohistochemical diagnosis. We could not find any differential point of both tumors in clinical and radiological characteristics although some differences were exist. But we had found the relationship between the mean level of pretreatment serum prolactin and the presence of positive immunohistochemical stain for prolactin. The pretreatment serum prolactin level was significantly higher in patients with tumors showing many prolactin immunohistochemical staining cells than in those with none(p<0.05). When the pretreatment serum prolactin exceeded 100ng/ml, the tumors contain 94% of prolactin positive cells in stain. So, if the pretreatment serum prolactin exceeds 100ng/ml, we primarily suspect prolactinoma and medical treatment should be considered. If the pretreatment level below 100ng/ml, we suspect pseudoprolactinoma and surgical treatment should be considered.
Diagnosis ; Dopamine ; Dopamine Agonists ; Endocrinology ; Humans ; Hyperprolactinemia ; Hypothalamus ; Pituitary Gland ; Pituitary Neoplasms ; Prolactin ; Prolactinoma