Growth hormone (GH) and thyroid stimulating hormone (TSH)-secreting pituitary adenomas are very rare and they account for only 0.5% for all pituitary adenomas. These adenomas are usually treated with surgery, but this surgery is not easy because the tumor is usually huge and invasive. We reported here on a case of a GH-TSH-secreting adenoma in a 23-year-old male patient who was initially treated with octreotide LAR. He presented with symptoms of headache, palpitation and a visual defect that he had for the 3 months. He had hypertrophy of the frontal bone and enlargement of both the hands and feet. The visual field test showed bitemporal hemianopsia. The laboratory examinations showed high serum levels of free T4, TSH and free alpha-subunit. Additionally, the serum levels of GH and insulin-like growth factor-I (IGF-I) were increased. GH was not suppressed below 1microg/L by an oral 75g glucose loading test, and TSH was not stimulated by thyrotropin-releasing hormone (TRH). Because sellar MRI showed invasive macroadenoma encasing the vessels, we initially tried octreotide LAR for treatment. A year later, the IGF-I and thyroid function tests were normalized and the size of the tumor was reduced with cystic change. The symptoms of palpitation and headache were improved without a change of the visual field defect.
Acromegaly ; Adenoma ; Foot ; Frontal Bone ; Glucose ; Growth Hormone ; Hand ; Headache ; Hemianopsia ; Humans ; Hypertrophy ; Insulin-Like Growth Factor I ; Male ; Octreotide ; Pituitary Neoplasms ; Thyroid Function Tests ; Thyrotropin ; Thyrotropin-Releasing Hormone ; Visual Field Tests ; Visual Fields ; Young Adult

BACKGROUND: Type 2 diabetes develops because of defects in both insulin secretion and action. The half-life of insulin in uremia is prolonged because the metabolic clearance rate of insulin in diabetic end stage renal disease (ESRD) patients is reduced with consequence that the dose of insulin and/or oral hypoglycemic agent (OHA) administered in normal renal function make them increase the risk of hypoglycemia. Therefore, we should usually reduce the dose of insulin and/or OHA, or stop administration of insulin and/or OHA if type 2 diabetic patients are progressed to ESRD. But in some patients, that is not true. The aim of this study was to test the hypothesis that insulin resistance plays an important role in (re)evaluation of optimal insulin and/or OHA dose for glycemic control after type 2 diabetic patients are progressed to ESRD. METHODS: Insulin resistance was examined in 23 type 2 diabetic ESRD patients with tight control of glycemia using the K index of the insulin tolerance test (Kitt). We divided 23 patients into three groups. Group 1 (n=10) was defined as patients who were administered neither insulin nor OHA after ESRD. Group 2 (n=9) was defined as patients who were changed from insulin to OHA as drug for glycemic control after ESRD. Group 3 (n=4) was defined as patients in whom insulin or OHA was continuously administered after ESRD without a change of them for glycemic control. We compared the degree of insulin resistance among these three groups. RESULTS: Insulin resistance determined by Kitt was significantly different between group 1 (Kitt, 2.1422/0.94-4.01%/min), group 2 (Kitt, 1.3811/0.79- 3.90%/min) and group 3 (Kitt, 0.8550/0.44-1.81%/min) by using Kruskal-Wallis test (p=0.048). Kitt in group 3 was significantly lower than in group 1 by using Mann-Whitney test (p=0.016). CONCLUSION: Although metabolic clearance of insulin is reduced by renal failure, demand of insulin/ OHA for optimal glycemic control is not reduced in higher insulin-resistant type 2 diabetic ESRD patients on hemodialysis. Insulin resistance plays an important role in determination of optimal insulin/ OHA dose for glycemic control after type 2 diabetic patients are progressed to ESRD.
Half-Life ; Humans ; Hypoglycemia ; Insulin Resistance* ; Insulin* ; Kidney Failure, Chronic* ; Metabolic Clearance Rate ; Renal Dialysis* ; Renal Insufficiency ; Uremia

BACKGROUND: Chemerin is a novel adipokine that is associated with inflammation and adipogenesis. However, it remains unclear whether chemerin is involved in patients with cardiovascular disease. We investigated whether the serum chemerin levels of Korean patients with coronary artery disease correlated with specific cardiometabolic parameters. METHODS: In total, 131 patients, all of whom had coronary artery stenosis exceeding 50%, participated in this study. Their serum chemerin levels and cardiometabolic parameters were measured. The serum chemerin levels of two groups of patients were compared; those with one stenotic vessel (n=68) and those with multiple stenotic vessels, including left main coronary artery disease (n=63). RESULTS: Serum chemerin levels correlated positively with the degree of coronary artery stenosis and fasting glucose, triglyceride, total cholesterol, low density lipoprotein cholesterol, and high sensitive C-reactive protein levels. The group with multiple stenotic vessels, including left main disease, had higher chemerin levels than the group with one stenotic vessel (t=-2.129, P=0.035). Multiple binary logistic regression showed chemerin was not an independent risk factor of multiple vessel disease (odds ratio, 1.018; confidence interval, 0.997 to 1.040; P=0.091). CONCLUSION: Serum chemerin levels have a significant correlation with several cardiometabolic risk factors and the degree of coronary artery stenosis in Korean patients with coronary artery disease. However, multiple binary logistic regression showed chemerin was not an independent risk factor of multiple vessel disease. Additional investigations are necessary to fully elucidate the role of chemerin in cardiovascular disease.
Adipogenesis ; Adipokines ; C-Reactive Protein ; Cardiovascular Diseases ; Cholesterol ; Cholesterol, LDL ; Coronary Artery Disease ; Coronary Stenosis ; Coronary Vessels ; Fasting ; Glucose ; Glycosaminoglycans ; Humans ; Inflammation ; Lipoproteins ; Logistic Models ; Risk Factors

Primary sternal osteomyelitis without predisposing factors is a rare condition, and it is hardly differentiated from metastatic bone tumor especially in patient with the history of primary malignancy because osteomyelities shares frequently common findings with metastatic bone lesion on 18F-FDG PET and bone scan. Although there have been several publications of primary osteomyelitis mimicking bone metastasis in the spine or extremities, we report a case of primary sternal osteomyelitis in the patient with lung cancer, which has, to our knowledge, not been reported before.
Extremities ; Fluorodeoxyglucose F18 ; Humans ; Lung ; Lung Neoplasms ; Neoplasm Metastasis ; Osteomyelitis ; Positron-Emission Tomography ; Spine ; Sternum

The fibromatosis is a broad group of benign fibrous tissue proliferations of similar microscopic appearance that are intermediate in their biological behavior between benign fibrous lesions and fibrosarcoma. Although various series have been reported of abdominal wall and extra-abdominal desmoid tumors, intra-abdominal desoids are extremely rare. We experienced a case with mesenteric fibroma-tosis occuring in a 30 year-old male. He was admitted to the Kangbuk Samsung hospital complaining of right lower quadrant abdominal mass and abdominal bloating sense. Utrasonography and computed tomography of the abdomen showed a solid mass in the left abdomen surrounded by loops of small bowel. At explorative laparotomy, there was a hard, well circumscribed round mass (25 X 15 X 12 cm) in the mesentery of the terminal ilem. After the tumor was dissected from the retro-peritoneum and surrounding tissues, segmental re- section of ileum with end-to-end anastomosis was performed. On the histopathologic examination, it was confirmed as mesenteric fibromatosis. A brief review of the literature on mesentery fibromatosis was done.
Abdomen ; Abdominal Wall ; Adult ; Fibroma* ; Fibromatosis, Aggressive ; Fibrosarcoma ; Humans ; Ileum ; Laparotomy ; Male ; Mesentery

BACKGROUND/AIMS: Fabry disease is an X-linked recessive and progressive disease caused by alpha-galactosidase A (alpha-GaL A) deficiency. We sought to assess the prevalence of unrecognized Fabry disease in dialysis-dependent patients and the efficacy of serum globotriaosylceramide (GL3) screening. METHODS: A total of 480 patients of 1,230 patients among 17 clinics were enrolled. Serum GL3 levels were measured by tandem mass spectrometry. Additionally, we studied the association between increased GL3 levels and cardiovascular disease, cerebrovascular disease, or left ventricular hypertrophy. RESULTS: Twenty-nine patients had elevated serum GL3 levels. The alpha-GaL A activity was determined for the 26 patients with high GL3 levels. The mean alpha-GaL A activity was 64.6 nmol/hr/mg (reference range, 45 to 85), and no patient was identified with decreased alpha-GaL A activity. Among the group with high GL3 levels, 15 women had a alpha-GaL A genetics analysis. No point mutations were discovered among the women with high GL3 levels. No correlation was observed between serum GL3 levels and alpha-GaL A activity; the Pearson correlation coefficient was 0.01352 (p = 0.9478). No significant correlation was observed between increased GL3 levels and the frequency of cardiovascular disease or cerebrovascular disease. CONCLUSIONS: Fabry disease is very rare disease in patients with end-stage renal disease. Serum GL3 measurements as a screening method for Fabry disease showed a high false-positive rate. Thus, serum GL3 levels determined by tandem mass spectrometry may not be useful as a screening method for Fabry disease in patients with end stage renal disease.
Adult ; Aged ; Fabry Disease/blood/*diagnosis ; Female ; Humans ; Kidney Failure, Chronic/blood/*therapy ; Male ; Middle Aged ; *Renal Dialysis ; Trihexosylceramides/*blood ; alpha-Galactosidase/genetics/metabolism

Background: The PLASMIC score is a convenient tool for predicting ADAMTS13 activity of ＜10%.Lactate dehydrogenase (LDH) is widely used as a marker of haemolysis in thrombotic thrombocytopenic purpura (TTP) monitoring, and could be used as a replacement marker for lysis. We aimed to validate the PLASMIC score in a multi-centre Asia Pacific region, and to explore whether LDH could be used as a replacement marker for lysis. Methods: Records of patients with thrombotic microangiopathy (TMA) were reviewed. Patients’ ADAMTS13 activity levels were obtained, along with clinical/laboratory findings relevant to the PLASMIC score. Both PLASMIC scores and PLASMIC-LDH scores, in which LDH replaced traditional lysis markers, were calculated. We generated a receiver operator characteristics (ROC) curve and compared the area under the curve values (AUC) to determine the predictive ability of each score. Results: 46 patients fulfilled the inclusion criteria, of which 34 had ADAMTS13 activity levels of ＜10%. When the patients were divided into intermediate-to-high risk (scores 5‒7) and low risk (scores 0‒4), the PLASMIC score showed a sensitivity of 97.1% and specificity of 58.3%, with a positive predictive value (PPV) of 86.8% and negative predictive value (NPV) of 87.5%. The PLASMIC-LDH score had a sensitivity of 97.1% and specificity of 33.3%, with a PPV of 80.5% and NPV of 80.0%. Conclusion Our study validated the utility of the PLASMIC score, and demonstrated PLASMIC-LDH as a reasonable alternative in the absence of traditional lysis markers, to help identify high-risk patients for treatment via plasma exchange.

BACKGROUND: AND PURPOSE: The aim of this study was to determine the diagnostic performance and safety of a new ¹⁸F-labeled amyloid tracer, ¹⁸F-FC119S. METHODS: This study prospectively recruited 105 participants, comprising 53 with Alzheimer's disease (AD) patients, 16 patients with dementia other than AD (non-AD), and 36 healthy controls (HCs). In the first screening visit, the Seoul Neuropsychological Screening Battery cognitive function test was given to the dementia group, while HC subjects completed the Korean version of the Mini Mental State Examination. Individuals underwent ¹⁸F-FC119S PET, ¹⁸F-fluorodeoxyglucose (FDG) PET, and brain MRI. The diagnostic performance of ¹⁸F-FC119S PET for AD was compared to a historical control (comprising previously reported and currently used amyloid-beta PET agents), ¹⁸F-FDG PET, and MRI. The standardized uptake value (SUV) ratio (ratio of the cerebral cortical SUV to the cerebellar SUV) was measured for each PET data set to provide semiquantitative analysis. All adverse effects during the clinical trial periods were monitored. RESULTS: Visual assessments of the ¹⁸F-FC119S PET data revealed a sensitivity of 92% and a specificity of 84% in detecting AD. ¹⁸F-FC119S PET demonstrated equivalent or better diagnostic performance for AD detection than the historical control, ¹⁸F-FDG PET (sensitivity of 80.0% and specificity of 76.0%), and MRI (sensitivity of 98.0% and specificity of 50.0%). The SUV ratios differed significantly between AD patients and the other groups, at 1.44±0.17 (mean±SD) for AD, 1.24±0.09 for non-AD, and 1.21±0.08 for HC. No clinically significant adverse effects occurred during the trial periods. CONCLUSIONS ¹⁸F-FC119S PET provides high sensitivity and specificity in detecting AD and therefore may be considered a useful diagnostic tool for AD.