Acromegaly and hyperprolactinemia have been described in association with polyostotic fibrous dysplasia. The pathogenic mechanisms of this endocrinopathy are not clear. We experienced a 19-year-old male with hypersecretion of GH, hyperprolactinemia and fibrous dysplasia. He was referred for evaluation of suspected acromegaly. He had no skin pigmentation. Plasma GH, PRL, somatomedin-C, LH, FSH, testosterone, estradiol, progesterone, T3, T4, TSH and cortisol were measured. Among those, the levels of plasma GH, PRL and somatomedin-C were high. Serum alkaline phosphatase was increased. OGTT did not suppress plasma OH concentration and GH showed paradoxical response to TRH and LHRH. GH was suppressed after a test-dose of somatastatin and bromocriptine. Brain MRI demonstrated a mass lesion in sella turcica and another mass lesions in nasal cavity and posterior occipital bone. Whole body bone scan revealed increased uptake in skull, nasal bone, both 9th posterior rib, both femurs, both tibias, left scapular and pelvic bone. These fmdings were consistent with bone tumor such as fibrous dysplasia. We report a case with incomplete MeCune-Albright syndrome including acromegaly, hyperprolactinemia and polyostotic fibrous dysplasia.
Acromegaly ; Alkaline Phosphatase ; Brain ; Bromocriptine ; Estradiol ; Femur ; Fibrous Dysplasia, Polyostotic ; Glucose Tolerance Test ; Gonadotropin-Releasing Hormone ; Humans ; Hydrocortisone ; Hyperprolactinemia ; Magnetic Resonance Imaging ; Male ; Nasal Bone ; Nasal Cavity ; Occipital Bone ; Pelvic Bones ; Plasma ; Progesterone ; Ribs ; Sella Turcica ; Skin Pigmentation ; Skull ; Testosterone ; Tibia ; Young Adult

Nesidioblastosis is characterized by a diffuse proliferation of islet cells arising from pancreatic ducts and is the most common cause of hyperinsulinemic hypoglycemia in newborns and infantile. It is exceedingly rare in adults and no concensus regarding its diagnosis and management is available. We herein describe an elderly man with fasting hypoglycemia, inappropriate insulin hypersecretion. And pathologic examination of his pancreas revealed the characteristic finding of nesidioblastosis confirmed by immunohistochemical stain.
Adult ; Aged* ; Diagnosis ; Humans ; Hypoglycemia* ; Infant, Newborn ; Insulin ; Islets of Langerhans ; Nesidioblastosis* ; Pancreas ; Pancreatic Ducts

BACKGROUND: The relaxative response of blood vessels to acetylcholine (ACh) is known to be abnormal in diabetic rat due to changes in endothelium-derived relaxing factor (EDRF) and/or endothelium-derived hyperpolarizing factor (EDHF)-mediated action. Oxygen free radical (OFR) interferes with endothelium dependent relaxation to ACh in diabetic rats; this effect rnay be prevented by superoxide dismutase (SOD), OFR scavenger. Then, we determined the effect of SOD on modulation of OFR-induced damage to EDRF and EDHF-mediated relaxations to ACh in diabetic rat aortas. METHODS: After aortas were incubated with free radical generating system for 15 min with or without SOD pretreatment (150 U/mL) and contracted submaximally by norepinephrine (10 (-5) M), relaxative responses to cumulative concentrations (10 (-9) M to 10 (-5) M) of ACh were measured in aortas isolated from the control and 6-8 week streptozotocin-induced diabetic rat. We measured relaxative responses to ACh in these aortas treated with calmidazolium (100uM) or N-nitro-L-arginine methyl ester (luM) after exposure to OFR with/without SOD pretreatment, RESULTS: The ACh-induced relaxation (10 (-9)M to 10 (-5) M) was significantly decreased in diabetic than in control rat aortas (p<0.05). ACh-induced relaxation in diabetic rat aortas was significantly impaired from 79.3% to 71.2% after exposure to OFR (p<0.05), and the degree of ACh-induced relaxation was recovered from 71.2% to 84.0% after pretreatment with SOD (p<0.05). EDRF-mediated relaxation to ACh in diabetic rat aortas was significantly impaired from 71.2% to 61.6% after exposure to OFR (p<0.05), and the degree of impairment of ACh-induced EDRF-mediated relaxation was recovered from 61.6% to 76.0% after pretreatment with SOD. After exposure to OFR, EDHF-mediated relaxation to ACh in diabetic rat aortas was not significanlty impaired. However, the degree of impairment of EDHF-mediated relaxation to ACh was recovered from 46.0% to 59.5% after pretreatment with SOD. CONCLUSION: This study suggests that OFR may impair mainly EDRF-mediated relaxation to ACh and SOD may protect rnainly OFR-induced damage to EDRF-mediated relaxation to ACh in diabetic rat aortas.
Acetylcholine ; Animals ; Aorta* ; Blood Vessels ; Endothelium ; Endothelium-Dependent Relaxing Factors ; Free Radicals* ; Norepinephrine ; Oxygen* ; Rats* ; Relaxation* ; Superoxide Dismutase* ; Superoxides*

Patients with acromegaly have a reduced life expectancy rnainly due to cardiovascular, respiratory or cerebrovascular diseas-. Malignancy also seems to occur with greater than the expected incidence. In particular, the published retrospective or prospective studies have suggested a strong association of colonic neoplasia with acromegaly. But, there were a few reports of thyroid cancer in acrornegaly. We report a case of thyroid papillary cancer derived from diffuse goiter in acromegaly, sugge- sting the possible carcinogenic role of growth hormone.
Acromegaly ; Bites and Stings ; Colon ; Goiter ; Growth Hormone ; Humans ; Incidence ; Life Expectancy ; Prospective Studies ; Retrospective Studies ; Thyroid Gland ; Thyroid Neoplasms

Gastric adenocarcinoma of fundic gland type is known to originate from the gastric mucosa of the fundic gland region without intestinal metaplasia. It is difficult to detect during endoscopy and diagnose histopathologically. The development of immunohistochemistry has enabled the diagnosis of gastric adenocarcinoma of fundic gland type by gastric phenotype. A 34-year-old man visited us for treatment of a 5 cm-sized low-grade dysplasia in the gastric fundus which was incidentally found during a health check-up endoscopy. Endoscopic submucosal dissection was performed successfully, and the final histopathology showed gastric adenocarcinoma of fundic gland type. Herein, we report a rare case of gastric adenocarcinoma of fundic gland type and its endoscopic and histopathologic findings.

Gastric adenocarcinoma of fundic gland type is known to originate from the gastric mucosa of the fundic gland region without intestinal metaplasia. It is difficult to detect during endoscopy and diagnose histopathologically. The development of immunohistochemistry has enabled the diagnosis of gastric adenocarcinoma of fundic gland type by gastric phenotype. A 34-year-old man visited us for treatment of a 5 cm-sized low-grade dysplasia in the gastric fundus which was incidentally found during a health check-up endoscopy. Endoscopic submucosal dissection was performed successfully, and the final histopathology showed gastric adenocarcinoma of fundic gland type. Herein, we report a rare case of gastric adenocarcinoma of fundic gland type and its endoscopic and histopathologic findings.

BACKGROUND: Serum thyroglobulin(Tg) is a valuable and sensitive tool needed in the follow-up of patients with differentiated thyroid cancer(DTC), but antithyroglobulin antibody(Anti-Tg), common in patients with DTC, can interfere with the assay for Tg. In this study, we evaluated the influence of Anti-Tg on the measurement of Tg using the immunoradiometric assay(IRMA). METHODS: In using ELSA-hTg in vivo test(CIS international, Schering, France), a solid phase two-site IRMA was used to measure Tg(23.5ng/mL, 62.5ng/mL) under the absence or presence of three concentrations of Anti-Tg(25U/mL, 50U/mL, 100U/mL). We also performed Tg measurement using patients serum that was mixed with patients serum containing high Anti-Tg. ANOVA and Scheffe tests were performed to evaluate the effect of Anti-Tg on Tg IRMA, and an inverse regression was made to calculate the level of Tg from measured Tg and used Anti-Tg levels and also to assess the degree of effect of anti-Tg on Tg IRMA. RESULTS: In measuring Tg using the standard solution, the presence of Anti-Tg resulted in a falsely suppressed Tg value. The IRMAs for 23.5ng/mL of the standard Tg solution resulted in 24.5+/-.1 ng/mL under no Anti-Tg, 11.8+/-.4ng/mL under 25U/mL of Anti-Tg, 7.7+/-.1ng/mL under 50U/mL of Anti-Tg, and 4.5+/-.4ng/mL under 100U/mL of Anti-Tg. IRMAs 62.5ng/mL of the standard Tg solution resulted in 65.9+/-.7ng/mL under no Anti-Tg, 36.3+/-.2ng/mL under 25U/mL of Anti-Tg, 23.7+/-.7ng/mL under 50U/mL of Anti-Tg, and 14.0+/-.0ng/mL under 100U/mL of Anti-Tg. (ANOVA test, p=0.000). The degree of suppression of the measured Tg value was positively correlated with the Anti-Tg level (Quadratic model regression, Sig T=0.000). The presence of Anti-Tg also resulted in a falsely suppressed Tg value for the Tg measurement using patient's serum. CONCLUSION: The presence of Anti-Tg could consist of the use of Tg as a tumor, therefore Anti-Tg should be measured in all patients diagnosed with DTC. The interpretation of the Tg level must be performed with extreme caution in patients with Anti-Tg.
Humans ; Immunoradiometric Assay* ; Thyroglobulin* ; Thyroid Gland

Hutchinson-Gilford progeria syndrome (HGPS) and Werner's syndrome are representative types of progeroid syndrome. LMNA (Lamin A/C) gene mutation with atypical Werner's syndrome have recently been reported. Atypical Werner's syndrome with the severe metabolic complications, the extent of the lipodystrophy is associated with A133L mutation in the LMNA gene and these patients present with phenotypically heterogeneous disorders. We experienced a 15-yr-old Korean female with progeroid features, generalized lipodystrophy, hypertriglyceridemia, fatty liver, steatohepatitis, and type 2 diabetes mellitus. Skin fibroblasts from the patient showed marked abnormal nuclear morphology, compared with that from normal persons. Gene analysis revealed that this patient had T506del of exon 2 in the LMNA gene. We report here the first case of atypical Werner's syndrome with frameshift mutation that was caused by T506del.
Adolescent ; DNA/*genetics ; Exons ; Female ; Genetic Predisposition to Disease ; Humans ; Lamin Type A/*genetics/metabolism ; Lipodystrophy ; *Mutation ; Sequence Analysis, DNA ; Skin/metabolism/pathology ; Werner Syndrome/diagnosis/*genetics/metabolism

Approximately 10% of pheochromocytomas are malignant and its major criteria are tumor invasion of capsular blood vessel as well as metastatic invasion of other tissues. It is general rule that all resectable masses have to be removed surgically. However, there is no definite treatment modality about unresectable masses or microinvasive lesions. We experienced a case of 45 year-old male patient who was referred to our hospital for treatment of hypertension and headache. The plasma and urine catecholamine were increased above normal values and its metabolites also were increased. 131I-metaiodobenzylguanidine (MIBG) scan showed right adrenal mass and metastatic lesion of left iliac bone. This lesion was consistent with findings of the abdomen computed tomography (CT) and electron beam tomography (EBT) scan. We diagnosed this case as malignant pheochromocytoma. We removed primary tumor mass by wide excision and treated this patient with high dose 131I-MIBG. We report this case who shows good response to the high dose 131I-MIBG after surgery.
3-Iodobenzylguanidine ; Abdomen ; Blood Vessels ; Headache ; Humans ; Hypertension ; Male ; Middle Aged ; Neoplasm Metastasis ; Pheochromocytoma* ; Plasma ; Reference Values ; Tomography, X-Ray Computed