Search

BackgroundFibroblast growth factor 21 (FGF21) is a novel metabolic regulator that has beneficial effects on glucose and lipid metabolism. However, plasma FGF21 levels are paradoxically increased in type 2 diabetes mellitus (T2DM) and obesity, suggesting resistance to this ligand. FGF21 acts mainly on adipose tissue and ectopic fat accumulation is a typical feature in metabolic deterioration such as diabetes, metabolic syndrome, and cardiovascular disease.ObjectiveTo investigate the relationship between FGF21 resistance and ectopic fat accumulation.Research design and methodsSubjects who underwent 64-slice multidetector CT (MDCT) were enrolled (n=190). Plasma FGF21 levels and MDCT data of ectopic fats at various sites were analyzed. Human visceral and subcutaneous fat tissues from abdominal and coronary artery bypass surgery were obtained. FGF21 receptor expression and postreceptor signaling in different fat deposits of both control and T2DM subjects were analyzed.ResultsPlasma FGF21 levels were significantly associated with body mass index, triglyceride, homeostatic model assessment of insulin resistance, and Matsuda index. Plasma FGF21 levels were significantly higher in patients with T2DM than in the pre-diabetes and normal glucose tolerance groups. The ectopic fat phenotypes (visceral, epicardial, intrahepatic, and intramuscular fat) of T2DM were significantly higher than controls. Plasma FGF21 levels were elevated and exhibited a strong positive correlation with ectopic fat accumulation in T2DM. The expression of genes comprising the FGF21 signaling pathway was also lower in visceral fat than in subcutaneous fat in this disease.ConclusionsHuman FGF21 resistance in T2DM could result from increases in FGF21-resistant ectopic fat accumulation. Our study provides novel clinical evidence linking FGF21 resistance and T2DM pathogenesis.

We wanted to investigate the changes in beta cell function and insulin sensitivity during long-term CSII therapy in patients with type 2 diabetes (T2D) up to for 3 years. We discontinued oral antidiabetic drugs and applied CSII therapy to T2D patients (number, 110 with 64% of male; age, 59.9 ± 9.1 years; duration, 12.0 ± 9.2 years; HbA1c 8.9 ± 2.0%; BMI 23.5 ± 2.5 kg/m2). Blood samplings were performed yearly for 3 years at overnight fasting and 120 minutes after ingestion of a standard mixed meal (500 kcal). Serum C-peptide, glucose, and HbA1c were measured, and Matsuda Index (MI) were calculated. The patients were grouped into high MI (insulin sensitive group) and low MI (insulin resistant group) groups by the mean value of baseline MI. Mean HbA1c decreased significantly from 8.9% to 6.5% and there was no difference in glycemic control between the two groups. In insulin sensitive group, mean serum C-peptide increased significantly during CSII therapy. In contrast, mean serum C-peptide did not change but mean MI increased significantly in insulin resistant group (Fig. 1). In conclusion, by the physiological delivery of insulin using insulin pumps, the two main pathophysiologies of T2D, either insulin deficiency or insulin resistance, seemed to be corrected, respectively. Disclosure S. Choi: Stock/Shareholder; Self; SOOIL Developments Co., Ltd.. E. Hong: None. Y. Noh: None.

Background and Aims: Artificial pancreas combines continuous glucose monitoring (CGM) with insulin pump by using a control algorithm to direct insulin delivery. Although several control algorithms have been developed, the control algorithms are beyond reach of most of diabetic patients in need. The Open Artificial Pancreas System project (openAPS) is an open control algorithm for artificial pancreas, which is available worldwide in type 1 diabetes (T1D). Here, we present several interesting clinical experiences using openAPS. Materials and Methods: Twenty T1D patients using openAPS, CGM (Dexcom G4®), and insulin pump (Sooil, Dana R®) were studied. Normal glycemic range was set as 80∼180 mg/dl. Results: Mean age was 11.9 ± 6.9 years and 10 patients were male. Median openAPS duration was 180 (30-240) days. By the use of openAPS, CGM analysis showed significant decrease in A1C (6.8 ± 1.0% to 6.3 ± 0.7%, p Conclusion: Open artificial pancreas system reduced hypoglycemia and improved glycemic control in patients with T1D. Disclosure S. Choi: Stock/Shareholder; Self; SOOIL Developments Co., Ltd.. E. Hong: None. Y. Noh: None.

Background Dipeptidyl peptidase-4 (DPP-4) inhibitor add-on therapy is a new option for patients with inadequately controlled type 2 diabetes who are taking combined metformin and sulfonylurea (SU). We evaluated the efficacy and safety of this triple therapy and the characteristics of rapid responders and hypoglycemia-prone patients. Methods We included 807 patients with type 2 diabetes who were prescribed a newly added DPP-4 inhibitor to ongoing metformin and SU in 2009 to 2011. Glycemia and other metabolic parameters at baseline, 12, 24, and 52 weeks, as well as episodes of hypoglycemia were analyzed. Rapid responders were defined as patients with ≥25% reduction in glycosylated hemoglobin (HbA1c) within 12 weeks. Results At baseline, while on the submaximal metformin and SU combination, the mean HbA1c level was 8.4%. Twelve weeks after initiation of DPP-4 inhibitor add-on, 269 patients (34.4%) achieved an HbA1c level ≤7%. Sixty-six patients (8.2%, 47 men) were rapid responders. The duration of diabetes was shorter in rapid responders, and their baseline fasting plasma glucose (FPG), HbA1c, C-peptide, and homeostasis model assessment of insulin resistance were significantly higher. Patients who experienced hypoglycemia after taking DPP-4 inhibitor add-on were more likely to be female, to have a lower body weight and lower triglyceride and FPG levels, and to have higher homeostasis model assessment of β-cells. Conclusion An oral hypoglycemic triple agent combination including a DPP-4 inhibitor was effective in patients with uncontrolled diabetes. Proactive dose reduction of SU should be considered when a DPP-4 inhibitor is added for rapid responders and hypoglycemia-prone patients.

Background: Measurement of the plasma adrenocorticotropic hormone (ACTH) level has been recommended as the first diagnostic test for differentiating between ACTH-independent Cushing syndrome (CS) and ACTH-dependent CS. When plasma ACTH values are inconclusive, a differential diagnosis of CS can be made based upon measurement of the serum dehydroepiandrosterone sulfate (DHEA-S) level and results of the high-dose dexamethasone suppression test (HDST). The aim of this study was to assess the utility of plasma ACTH to differentiate adrenal CS from Cushing’ disease (CD) and compare it with that of the HDST results and serum DHEA-S level. Methods: We performed a retrospective, multicenter study from January 2000 to May 2012 involving 92 patients with endogenous CS. The levels of plasma ACTH, serum cortisol, 24-hour urine free cortisol (UFC) after the HDST, and serum DHEA-S were measured. Results: Fifty-seven patients had adrenal CS and 35 patients had CD. The area under the curve of plasma ACTH, serum DHEAS, percentage suppression of serum cortisol, and UFC after HDST were 0.954, 0.841, 0.950, and 0.997, respectively (all P

The clinical implications of prediabetes for development of type 2 diabetes may differ for Asian ethnicity. We investigated various indices derived from a 2-h oral glucose tolerance test (OGTT) in people with prediabetes to predict their future risk of diabetes.We recruited 406 consecutive subjects with prediabetes from 2005 to 2006 and followed them up every 3-6 months for up to 9 years. Prediabetes was defined as isolated impaired fasting glucose (IFG), isolated impaired glucose tolerance (IGT), combined glucose intolerance (CGI), or isolated elevated HbA1c (5.7-6.4%, 39-46 mmol/mol) without IFG or IGT. The rate of diabetes conversion was compared between prediabetes categories. The association of glycemic indices with development of diabetes was also investigated.Eighty-one patients were diagnosed with diabetes during the 9-year follow-up (median 46.0 months). The rate of diabetes conversion was higher in subjects with CGI (31.9%), or isolated IGT (18.5%) than in those with isolated IFG (15.2%) or isolated elevated HbA1c (10.9%). Surrogate markers reflecting β-cell dysfunction were more closely associated with diabetes conversion than insulin resistance indices. Subjects with a 30-min postload glucose ≥ 165 mg/dL and a 30-min C-peptide5 ng/mL had 8.83 times greater risk (95% confidence interval 2.98-26.16) of developing diabetes than other prediabetic subjects.In Asians, at least Koreans, β-cell dysfunction seems to be the major determinant for diabetes conversion. A combination of high glucose and low C-peptide levels at 30 min after OGTT may be a good predictor for diabetes conversion in this population.

Objective Abdominal obesity is considered to be a risk factor for mortality. However, recent studies indicate that overweight may be negatively associated with mortality (“obesity paradox”). The relationships between mortality and various obesity markers in an elderly Asian cohort were evaluated. Methods Subjects of the Korean Longitudinal Study on Health and Aging (KLoSHA) (n = 1000, age ≥65 years) were included. The visceral fat area (VFA) and subcutaneous fat area (SFA) were measured using computed tomography. Results A total of 222 deaths occurred during the 6-year follow-up (median = 5.2 [range 0.1–6.3] years). Body mass index (BMI), VFA, SFA, and total fat mass were negatively associated with all-cause mortality in the univariable analyses (hazard ratio [HR] 0.67 per 1 SD [95% CI 0.57-0.77], 0.66 [0.55-0.79], 0.73 [0.61-0.86], and 0.74 [0.63-0.87], respectively). BMI and VFA were significantly associated with all-cause mortality in the multivariable analyses (HR 0.85 per 1 SD [95% CI 0.73-0.99] and 0.64 [0.47-0.87], respectively). When stratified by quartiles, the HR associated with VFA was the lowest in the third quartile. Conclusions In this observational study with a short follow-up of elderly Asian people, higher amounts of visceral fat, a marker for central obesity, were associated with decreased all-cause mortality.

Background and Aim Effective medicines have not been introduced for insulin resistance-related fatty liver. The efficacy and safety of treatment between a combination of metformin and carnitine-orotate complex and metformin alone in a 12-week, double-blind, randomized, placebo-controlled study on drug-naive patients with impaired glucose metabolism and fatty liver were compared. Methods Fifty-two patients with fasting glucose 100–240 mg/dL or glycosylated hemoglobin (HbA1c) ≥ 6.0% and alanine aminotransferase (ALT) 40–250 IU/L were randomized to receive metformin (250 mg t.i.d.), or metformin (250 mg t.i.d.) and carnitine-orotate complex (300 mg t.i.d.) for 12 weeks (n = 26 per group). The primary end-point was a change from baseline ALT level. Secondary end-points were changes in fasting glucose, HbA1c, aspartate aminotransferase levels, mitochondrial DNA (mtDNA) copy number in the peripheral blood, and urinary output of 8-hydroxy-2′-deoxyguanosine, a marker of oxidative stress. Results The combined treatment reduced ALT level significantly more than metformin alone (–51.5 ± 33.2 IU/L vs −16.7 ± 31.3 IU/L, P = 0.001). The HbA1c levels also decreased significantly in both groups but there was no significant difference between them (–0.9% ± 1.0% vs −0.7% ± 0.9%). Treatment with the complex decreased the urinary 8-hydroxy-2′-deoxyguanosine level and increased mtDNA copy number significantly compared with metformin alone (both P

ObjectiveBody adiposity, especially ectopic fat accumulation, has a range of metabolic and cardiovascular effects. This study aimed to investigate whether thyroid function is associated with various regional fat quantities in euthyroid subjects.MethodsA total of 100 euthyroid men (free triiodothyronine (fT3), 4.77±1.21 pg/ml; free thyroxine (fT4), 1.38±0.21 ng/dl; and TSH, 2.09±0.91 μIU/ml) were enrolled in this cross-sectional study. We measured accumulated regional fat using 64-slice multi-detector computed tomography. Multiple linear regression analysis was used to determine whether accumulated fat in each region was associated with clinical parameters after adjusting for age.ResultsFT3 was inversely correlated with BMI (r=−0.232, P=0.029) and LDL cholesterol level (r=−0.277, P=0.019). FT4 was inversely correlated with waist circumference (r=−0.350, P=0.008) and BMI (r=−0.355, P3 and fT4 levels were significantly associated with pericardial fat volume (fT3: B=−0.079, 95% CI −0.142 to −0.017, P=0.013; fT4: B=−0.411, 95% CI −0.780 to −0.042, P=0.030) in euthyroid men, independent of age. FT3 level was inversely associated with intramuscular fat area (B=−0.059, 95% CI −0.106 to −0.011, P=0.016) and hepatic fat quantity (B=−0.237, 95% CI −0.441 to −0.033, P=0.024) in euthyroid men, independent of age.ConclusionsIn euthyroid men, low levels of fT3 and fT4 were significantly associated with increased pericardial fat volume and BMI.

Mediastinal ectopic thyroid is a very rare condition, with few reported cases in the literature and no reported cases in Korea. This report describes an asymptomatic 65-year-old man with a right paratracheal mass compressing the superior vena. Additionally, the epidemiology, clinical manifestation, diagnosis, and management of mediastinal ectopic thyroids are discussed. A mediastinal ectopic thyroid should be considered in the differential diagnosis of all mediastinal masses. Surgical excision is recommended for both the diagnosis and treatment of this condition, because of its potential for malignancy and compression of mediastinal structures. This case demonstrates the clinical importance of mediastinal etopic thyroid.

We evaluated the malignancy and nondiagnostic rates using fine needle aspiration cytology (FNAC) results in thyroid nodules smaller than 1 cm according to the subdivided size. We retrospectively reviewed the medical records of all subjects underwent FNAC from 2003 to 2009 in our hospital, and 2,756 patients of subcentimeter thyroid nodules with one or more suspicious sonographic features and 7,105 with nodule sized 1 cm or more were included. The malignancy rate was higher in those subcentimeter nodules with suspicious sonographic findings than the nodule sized 1cm or more (19.7% vs 7.8%, P < 0.001). We grouped the nodules based on size with mm interval and observed that the malignancy rate did not decrease but the nondiagnostic results increased its size decrement. When we divided the subjects arbitrarily into a 5 mm or smaller and a 6-9 mm sized group, nondiagnostic cytology findings were reported more frequently in the smaller group (24.3% vs 18.1%, P = 0.001), while the rate of "malignant" was similar (18.3% vs 15.5%, P = 0.123) and the rate of "suspicious for malignancy" was higher (6.8% vs 2.9%, P < 0.001). Therefore when we decide to perform FNAC or not in subcentimeter-sized nodules, we should consider sonographic findings and other clinical risk factors but not the nodular size itself.

Background: The aim of this study was to investigate sarcopenia and its association with cardiometabolic risk factors in a community-based elderly cohort in Korea. Methods: We recruited 287 men and 278 women aged 65 years or older and without physical disability. Appendicular skeletal muscle mass (ASM) was measured with dual energy X-ray absorptiometry. We used two definitions for sarcopenia-ASM divided by height (kg/m) or by weight (%)

Background The complement component C1q triggers activation of the classical immune pathway and can bind to adiponectin (APN). Recently, some studies have been reported that serum C1q-APN/total APN ratio correlates with atherosclerosis and coronary artery disease (CAD). We assessed the relationships between C1q related variables and the severity of CAD, and investigated the localization of the C1q–APN complex. Methods The sample included 153 subjects comprising healthy controls and patients with subclinical or overt CAD. We measured the serum concentrations of C1q, total APN, and high-molecular weight (HMW)-APN, and the amount of C1q–APN complex. We identified the sites of C1q–APN complex deposition in various adipose tissues and blood vessels. Results Serum concentrations of C1q and HMW-APN and the C1q/HMW-APN ratio were independently associated with the severity of coronary stenosis. The amount of C1q–APN complex was significantly higher in patients with CAD compared with controls. C1q and APN co-localized in perivascular areas of subcutaneous, visceral, and pericardial fat tissues, and the internal mammary artery of patients with severe CAD. Conclusions Serum C1q concentration and the C1q/HMW-APN ratio were independent markers of coronary artery stenosis. The amount of C1q–APN complex was significantly greater in serum from CAD patients. C1q and APN co-localized to perivascular areas in adipose tissue and blood vessels. The association between the increased amount of the C1q–APN complex and CAD should be investigated further.

Background The relationship between adiponectin concentration and mortality is unclear. We examined whether serum adiponectin concentration is associated with all-cause and cardiovascular mortality in elderly Asians. Methods We analyzed the data for community-dwelling adults ≥65years of age (439 men and 561 women) who were enrolled in the Korean Longitudinal Study on Health and Aging (KLoSHA) cohort in prospective manner. The baseline serum total and high molecular weight adiponectin were measured using an enzyme-linked immunosorbent assay. Using Cox regression, we determined the associations between serum adiponectin concentration and all-cause and cardiovascular mortality after adjusting for well-known cardiovascular risk factors. Results Over a mean follow-up time of 6.2years, 222 individuals died, and 52 deaths (23.4%) were by cardiovascular disease. After adjusting confounding factors, elevated baseline serum adiponectin concentration was independently associated with all-cause mortality (adjusted hazard ratio [HR] 1.38; 95% confidence interval [CI] 1.17–1.64) and cardiovascular mortality (HR 1.50; 1.06–2.14). We evaluated the effect modification by baseline body mass index (BMI). High serum adiponectin and low BMI were synergistically associated with increased all-cause mortality (HR 6.25; 3.08–12.71) and cardiovascular mortality (HR 13.94; 1.82–106.58). Conclusions Higher serum adiponectin concentration was associated with increased all-cause and cardiovascular mortality in community-dwelling elderly Asian population. Our data supported the recent theory so called "adiponectin paradox". This relationship was strengthened when combined with low BMI. We suggest that measurement of adiponectin concentration and BMI together could be an additional predictive marker of survival among elderly adults.

SummaryObjective Dyslipidaemia and central obesity are the major factors underlying the dramatic increase in metabolic syndrome (MS). We compared the effects of early combined therapy with pitavastatin and intensive lifestyle modification (LSM) on the amelioration of each component of MS with those of LSM only. Design/Participants/Measurements PROPIT (a PROspective comparative clinical study to evaluate the efficacy and safety of PITavastatin in patients with metabolic syndrome) was a prospective, randomized, multicenter open-label 48-week trial. We enrolled 187 patients with MS (central obesity and prediabetes) and randomized them into two treatment groups: 2 mg pitavastatin daily + intensive LSM or intensive LSM only. The primary outcome was the improvements in the components of MS and in the percentage of non-MS converters. Results After 1 year treatment, the improvement of MS score was significantly higher in the pitavastatin + LSM group (P = 0·039). However, non-MS converters (MS score ≤2) did not differ between the groups. The secondary outcomes, namely lipid profiles, the Apo B/A1 ratio, visceral fat/subcutaneous fat ratio and the Framingham risk score, were significantly improved in the pitavastatin group. There was no deterioration in glucose metabolism after treatment with pitavastatin for 1 year. Conclusions Early statin treatment can be an effective option in obese patients with MS, prediabetes and mild dyslipidaemia with further improvement of cardiovascular risk factors. We could not observe the increase rate of glucose intolerance in statin group. Future longitudinal studies are needed to test the benefits of early statin treatment compared with LSM.

Abdominal obesity is considered to be a risk factor for mortality. However, recent studies indicate that overweight may be negatively associated with mortality ("obesity paradox"). The relationships between mortality and various obesity markers in an elderly Asian cohort were evaluated.Subjects of the Korean Longitudinal Study on Health and Aging (KLoSHA) (n = 1000, age ≥65 years) were included. The visceral fat area (VFA) and subcutaneous fat area (SFA) were measured using computed tomography.A total of 222 deaths occurred during the 6-year follow-up (median = 5.2 [range 0.1-6.3] years). Body mass index (BMI), VFA, SFA, and total fat mass were negatively associated with all-cause mortality in the univariable analyses (hazard ratio [HR] 0.67 per 1 SD [95% CI 0.57-0.77], 0.66 [0.55-0.79], 0.73 [0.61-0.86], and 0.74 [0.63-0.87], respectively). BMI and VFA were significantly associated with all-cause mortality in the multivariable analyses (HR 0.85 per 1 SD [95% CI 0.73-0.99] and 0.64 [0.47-0.87], respectively). When stratified by quartiles, the HR associated with VFA was the lowest in the third quartile.In this observational study with a short follow-up of elderly Asian people, higher amounts of visceral fat, a marker for central obesity, were associated with decreased all-cause mortality.

Effective medicines have not been introduced for insulin resistance-related fatty liver. The efficacy and safety of treatment between a combination of metformin and carnitine-orotate complex and metformin alone in a 12-week, double-blind, randomized, placebo-controlled study on drug-naïve patients with impaired glucose metabolism and fatty liver were compared.Fifty-two patients with fasting glucose 100-240 mg/dL or glycosylated hemoglobin (HbA1c) ≥ 6.0% and alanine aminotransferase (ALT) 40-250 IU/L were randomized to receive metformin (250 mg t.i.d.), or metformin (250 mg t.i.d.) and carnitine-orotate complex (300 mg t.i.d.) for 12 weeks (n = 26 per group). The primary end-point was a change from baseline ALT level. Secondary end-points were changes in fasting glucose, HbA1c, aspartate aminotransferase levels, mitochondrial DNA (mtDNA) copy number in the peripheral blood, and urinary output of 8-hydroxy-2'-deoxyguanosine, a marker of oxidative stress.The combined treatment reduced ALT level significantly more than metformin alone (-51.5 ± 33.2 IU/L vs -16.7 ± 31.3 IU/L, P = 0.001). The HbA1c levels also decreased significantly in both groups but there was no significant difference between them (-0.9% ± 1.0% vs -0.7% ± 0.9%). Treatment with the complex decreased the urinary 8-hydroxy-2'-deoxyguanosine level and increased mtDNA copy number significantly compared with metformin alone (both P 0.05). No severe adverse events were observed.A 12-week treatment with metformin and carnitine-orotate complex significantly improved liver function enzyme levels. This was associated with changes in oxidative stress and mtDNA copy number compared with metformin alone in patients with impaired glucose metabolism and fatty liver (clinical trial number: KCT0000193).

Adipose tissue is known to secrete hormones actively and produces many biologically active proteins called adipocytokines. Typically, obesity is followed by low-grade inflammation, which is characterized by increased circulating levels of pro-inflammatory cytokines. Macrophages play a role in the inflammatory process by secreting many cytokines such as tumor necrosis factor alpha, interleukin-6, resistin, and retinol binding protein-4. These cytokines and chemokines participate in low-grade pro-inflammatory processes leading to insulin resistance, metabolic impairment, and cardiovascular diseases. More metabolic regulators, such as fibroblast growth factor (FGF)21, FGF19, FGF1, vaspin, and visfatin have now been discovered but their exact roles in human diseases are still unclear. This review focuses on recent research regarding the role of adipokines and new metabolic factors in metabolic derangement or cardiovascular disease.

SummaryObjective Fibroblast growth factor 21 (FGF21) is an emerging metabolic regulator associated with glucose and lipid metabolism. However, previous studies of FGF21 have been largely confounded by obesity, and data are limited for advanced outcomes such as coronary artery disease (CAD) and ectopic fat accumulation. We investigated the associations between serum FGF21 concentrations and glucose/lipid metabolism, CAD, and pericardial fat deposition in subjects strictly matched for obesity parameters. Design, Patients and Measurements We enrolled 189 patients who had undergone cardiac multidetector coronary computed tomography. We measured cardiometabolic parameters and serum FGF21 levels within body mass index (BMI)-matched groups. Correlations and linear regressions were analysed among serum FGF21 levels, pericardial fat volumes and cardiometabolic parameters. Serum FGF21 concentrations were compared in patients with and without diabetes, metabolic syndrome (MS) or CAD. Results Serum FGF21 concentrations were significantly higher in BMI-matched patients with MS (107·2 ± 83·6 vs 82·1 ± 67·4 ng/l without MS, P

99m Tc-sestamibi) parathyroid scan is a sensitive diagnostic tool for the localization, although it sometimes shows a false-negative result. Here we report a case of presumed cystic parathyroid adenoma based on clinical findings and analysis of cystic fluid with negative findings in a parathyroid scan. A 44-year-old male patient visited the hospital due to leg pain and compressive symptoms (dysphagia, hoarseness) that had started 4-5 months before. His serum calcium level was 14.4 mg/dL and his intact parathyroid hormone (iPTH) had increased to 478.1 pg/mL. On neck computed tomography, a cystic nodule measuring 6.2 cm was detected in the inferior part of the right thyroid gland. Sestamibi uptake for this nodule was not detected on 2-h delayed imaging, and fluorodeoxyglucose positron emission tomography showed only subtle uptake. Fine-needle as piration was performed and intracystic iPTH had increased to 61,600 pg/mL. Focused parathyroidectomy guided by intraoperative iPTH monitoring led to successful enucleation of the right inferior parathyroid gland. A parathyroid adenoma was confirmed, and his laboratory results had normalized. This study shows that cystic parathyroid adenoma can sometimes be difficult to detect on a 99m Tc-sestamibi parathyroid scan. (Endocrinol Metab 27:83-88, 2012)

Background: The objective of this study is to demonstrate the relationship between the volume of a parathyroid adenoma and the preoperative biochemical parameters in patients undergoing surgery for primary hyperparathyroidism. Methods: The medical records of 68 patients who underwent a parathyroidectomy for a single parathyroid adenoma were retro spectively reviewed. The volume of the adenoma was estimated using its measured size and a mathematical formula. The correla tion between the volume of the parathyroid adenoma and the preoperative laboratory data was assessed. Results: There were no correlations between the estimated volume of the adenoma and the serum calcium, alkaline phosphatase and parathyroid hormone levels. However, the volume of the adenoma was associated with the preoperative level of serum phosphorus. After excluding 5 adenomas with cystic degeneration, a positive correlation was noted between the adenoma volume and the preoperative levels of alkaline phosphatase and parathyroid hormone. Conclusion: The preoperative serum levels of calcium, alkaline phosphatase and parathyroid hormone are of limited use to predict the volume of the parathyroid adenoma in patients with a single parathyroid adenoma. We suggest that the absence of a correlation between the volume of the adenoma and the biochemical parameters can be attributed to the cystic degeneration of the adenomas. (Endocrinol Metab 26:62-66, 2011)

Received: 27 July 2010, Accepted: 12 September 2010 Corresponding author: Seong Yeon Kim Division of Endocrinology, Department of Internal Medicine, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul 110-744, Korea Tel: +82-2-2072-3216, Fax: +82-2-762-9662, E-mail: seongyk@plaza.snu.ac.kr Clinical Characteristics of Langerhans Cell Histiocytosis with Hypothalamo-Pituitary Involvement

Von Hippel-Lindau (VHL) disease is an autosomal dominant disorder that results from a germline mutation of the VHL gene. The affected individuals might develop several benign or malignant tumors such as central nervous system or retinal haemangioblastomas, endolymphatic sac tumors, renal cell carcinomas, pheochromocytomas or pancreatic cysts and neuroendocrine tumors. We report here on a case of a 21 year old female with von Hippel-Lindau disease and she presented with only pancreatic neuroendocrine tumor and no evidence of haemangioblastomas or other visceral complications. Further, direct sequencing of the VHL gene reveals a novel germline frameshift mutation of codon 198 from the deletion of nucleotide 592 (cytosine), leading to truncation of the VHL protein. (Endocrinol Metab 26:89-91, 2011)