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

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

Backgrounds: Diabetes insipidus(DI) is a clinical syndrome characterized by excretion of copious volumes of dilute urine combined with persistent intake of abnormally large quantities of fluid. Central DI, caused by lack of antidiuretic hormone(ADH), most often results from lesions in the hypothalamic-neurohypophyseal axis. Magnetic resonance(MR) imaging is particularly useful in documenting the presence of a structural lesion, as opposed to assigning a diagnosis of idiopathic DI for which only symptomatic therapy is prescribed. Recently, several reports have described a specific MR finding in central DI, that is absence of normal posterior pituitary bright spot(PPBS). Methods: We retrospectivesly studied the clinical and MR findings in 25 patients with central DI, diagnosed by warter deprivation test. Results: 1) The subjects included 17 males and 8 females, between the ages of 2 and 58 years. 2) 24-hour urine volumes were 2,340~13,750 mL, and mean urine osmolarity was 147.7±65.8 mOsm/kg. The 23 subjects diagnosed complete central DI by warter deprivation test. 3) We found that the most common cause of cntral DI was infiltrative lesions of hypothalmic-neurohypophyseal axis(60%). Germ cell tumor was the single leading cause in present study, accounting for 36% of cases. The other causes were found, including pituitary apoplexy, meningitis, and trauma. Idiopathic central DI accounted for 20% of all cases. 4) Growth hormone deficiency was the most common accompanying anterior pituitary deficit, and panhypopituitarism was found in 7 cases, Hyperprolactinernia was seen in 6 cases. 5) In all patients, PPBS on Tl weighted MR images were not observed. A thickened pituitary stalk was seen in 15 cases(9 patients with germ cell tumor, 3 patients with histiocytosis X, 1 patient with tuberculosis, 2 patients with unknown origin). Conclusion: In our results, the most common causes of central DI was suprasellar infiltrative lesions. MR is currently the imaging methods of choice in the evaulation of dysfunction of the hypothalamic-neurohypophyseal system in patients with central DI. A specific MR finding, that is loss of normal PPBS allows a confirmative diagnosis of central DI.
Diagnosis ; Female ; Growth Hormone ; Histiocytosis, Langerhans-Cell ; Humans ; Male ; Meningitis ; Neoplasms, Germ Cell and Embryonal ; Osmolar Concentration ; Pituitary Apoplexy ; Pituitary Gland ; Tuberculosis

Objectives: Adolescent suicide is a serious social problem. Adolescent alcohol use is one of the most important risk factors for adolescent suicide. This study aimed to identify the relationship between drinking habits and suicide among Korean adolescents. Methods: Data from the 14th and 15th Korean Youth Risk Behavior Web-based Survey, conducted in 2018 and 2019, were used for analysis. Multiple logistic regression analysis was used to identify the relationship between drinking habits—including the age of drinking initiation, frequency of drinking, average drinking amount, frequency of drunkenness—and suicidal ideation, plans, and attempts. Results: Even after adjusting for age, sex, school grade, academic achievement, socioeconomic status, depression, stress, and drinking habits, the frequencies of drinking and drunkenness increased the risk of suicide attempts. Suicide attempts were associated with the frequency of drinking in girls and middle school students, and with the frequency of drunkenness in boys and high school students. Conclusion This study identified associations between drinking habits (the age of drinking initiation, frequency of drinking, average amount of drinking, frequency of drunkenness) and suicidal behavior in adolescents. Our findings suggest that to prevent adolescent suicide, it might be necessary to investigate drinking habits, including the frequencies of drinking and drunkenness. Moreover, considering the differences in sex and school grade, it is important to include the individual group characteristics when evaluating drinking habits.

Background: Puberty is a biologically and psychologically unstable period, and pubertal changes differ by sex. However, most previous studies on pubertal timing and suicide have focused on girls. This study investigated the association between early spermarche and suicide attempts in boys. Methods: We analyzed a nationally representative sample of Korean adolescents (The Korea Youth Risk Behavior Web-Based Survey, KYRBS) that included approximately 35,000 boys annually from 2011 to 2015. Pubertal timing in boys was defined by spermarche. Complex sampling logistic regression analyses were performed to evaluate the odds ratios (ORs) for suicide attempts between the early and average spermarche groups. Results: The ORs for suicide attempts in boys with early spermarche were significantly higher than those in boys with average spermarche after adjustment for age, perceived stress, depressive symptoms, and suicidal ideation. The ORs from 2011 to 2015 were as follows: 1.782 (P < 0.001), 1.490 (P = 0.002), 1.693 (P < 0.001), 1.541 (P = 0.001), and 1.393 (1.024– 1.895; P = 0.035), respectively. Conclusion These findings suggest that early pubertal timing is a risk factor for suicide attempts in Korean boys after adjustment for depressive symptoms, perceived stress, and suicidal ideation, which have been previously reported as risk factors for suicide attempts. Therefore, careful attention should be paid to the prevention of suicide in boys who experience early spermarche in Korea.

Objectives: Adolescent suicide is a serious social problem. Adolescent alcohol use is one of the most important risk factors for adolescent suicide. This study aimed to identify the relationship between drinking habits and suicide among Korean adolescents. Methods: Data from the 14th and 15th Korean Youth Risk Behavior Web-based Survey, conducted in 2018 and 2019, were used for analysis. Multiple logistic regression analysis was used to identify the relationship between drinking habits—including the age of drinking initiation, frequency of drinking, average drinking amount, frequency of drunkenness—and suicidal ideation, plans, and attempts. Results: Even after adjusting for age, sex, school grade, academic achievement, socioeconomic status, depression, stress, and drinking habits, the frequencies of drinking and drunkenness increased the risk of suicide attempts. Suicide attempts were associated with the frequency of drinking in girls and middle school students, and with the frequency of drunkenness in boys and high school students. Conclusion This study identified associations between drinking habits (the age of drinking initiation, frequency of drinking, average amount of drinking, frequency of drunkenness) and suicidal behavior in adolescents. Our findings suggest that to prevent adolescent suicide, it might be necessary to investigate drinking habits, including the frequencies of drinking and drunkenness. Moreover, considering the differences in sex and school grade, it is important to include the individual group characteristics when evaluating drinking habits.