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Background Familial chylomicronemia syndrome (FCS) is caused by a genetic defect in triglyceride (TG) metabolism that leads to severe hypertriglyceridemia, which in turn is associated with multiple morbidities and may cause severe pancreatitis that is both recurrent and progressive. Management of hypertriglyceridemia in FCS is challenging, as both dietary and medical interventions are often ineffective. Therapeutic plasma exchange (TPE) has been shown to rapidly decrease circulating levels of chylomicrons and TGs in patients presenting with acute hypertriglyceridemic-associated pancreatitis. Conversely, limited evidence exists to suggest that prophylactic use of TPE is effective at preventing recurrence of acute pancreatitis. Case report Herein, we report our experience with the use of chronic, prophylactic TPE to reduce the incidence of recurrent acute pancreatitis in a patient with FCS.

Background Crepidiastrum denticulatum (CD) is a well-known, traditionally consumed vegetable in Korea, which was recently reported to contain bioactive compounds with detoxification and antioxidant properties. Ischemia-reperfusion injury (IRI) is a major problem after renal transplantation. Furthermore, inflammatory responses to IRI exacerbate the resultant renal injury. In the present study, we investigated whether CD extract exhibits renoprotective effects against IR-induced acute kidney injury in mice. Materials and methods Renal IRI was induced in male C57BL/6 mice by bilateral renal pedicle occlusion for 30 minutes followed by reperfusion for 48 hours. CD extract (75 mg/kg) was administered orally 5 days before IRI. Results Treatment with CD extract significantly decreased blood urea nitrogen and serum creatinine levels as well as kidney tubular injury. CD also prevented IRI-induced renal glutathione depletion and increased malondialdehyde levels. Western blotting and reverse transcriptase polymerase chain reaction indicated that CD extract significantly attenuates inducible nitric oxide synthase and toll-like receptor 2/4 protein levels 48 h after IRI. The expression of tumor necrosis factor-α and interleukin-1β was significantly decreased in the CD extract treatment group. Conclusion CD extract improved acute renal IRI through its antioxidant and anti-inflammatory effects. These findings suggest that CD extract is a potential therapeutic agent for acute ischemia-induced renal damage.

Objectives This study was designed to evaluate the usefulness of intra-operative frozen section for the evaluation of microscopic extrathyroidal extension (ETE) in papillary thyroid carcinoma (PTC). Design Retrospective cohort study. Setting Dong-A University Medical Center, Busan, Korea. Participants Three hundred and sixty-four patients who underwent thyroid surgery from January 2000 to December 2010 with PTC confined to one unilateral lobe as diagnosed using preoperative ultrasonography were enrolled. Main outcome measures The patients who had microscopic ETE on frozen section were classified into “group A,” and those who did not have microscopic ETE on frozen section were classified into “group B.” Clinicopathologic factors including age, gender, size of the tumour, extent of operation, ETE, multifocality, bilaterality, lymph node metastasis and recurrence were compared between the two groups. Results Of the 364 patients enrolled, ETE was confirmed in 100 patients (group A, 27.5%) on frozen biopsy. The nodule size in group A (0.94±0.87 cm) was larger than that in group B (0.86±0.79 cm) (P=.042). In group A, 15 patients (15%) showed multifocality and 11 patients (14.47%) showed bilaterality. In group B, 37 patients (14.02%) showed multifocality and seven patients (43.35%) showed bilaterality. They did not differ significantly between the two groups (P=.811, P=.182). There was a higher frequency of lymph node metastases in group A (52/86, 60.47%) than in group B (7/16, 43.75%, P=.214). Recurrence was observed in only two patients who had received thyroid lobectomy as the initial surgery in group A. Conclusions Intra-operative frozen biopsy can be a useful method for identifying the microscopic ETE. During the surgery, it can also help the surgeon to decide the optimal extent of surgery and the need for central compartment neck dissection in PTC patients.

Persistent mullerian duct syndrome (PMD) with antimullerian hormone (AMH) deficiency is usually associated with mutations or deletions of the AMH gene, although many cases have no identified gene association. We report on a genetic male with PMD and AMH deficiency associated with distal monosomy 10q. A term 3,230 g infant was born to a healthy 27-year-old. Fetal ultrasound had shown possible genital ambiguity. Postnatal exam showed a 0.5 cm phallus with basal meatus, normal scrotum with no palpable gonads, no vaginal orifice, and a rectal fistula with an imperforate anus. Voiding cystourethrogram with ultrasound, cystoscopy, and laparoscopy showed normal bladder, urethral orifice, distal vagina, cervix, and bilateral abdominal testis. At 24 hours of life, testosterone was within normal range with low AMH level. Chromosome microarray analysis showed 46, XY, del10(10q25.3q26.13) involving an 8.2 MB interstitial deletion. Whole exome sequencing identified a NOTCH2 variant (1p11.2). AMH sequencing revealed no abnormalities. Following multidisciplinary team and parent discussion, male gender was assigned. Testosterone treatment resulted in penile length of 1.5 cm. Bilateral orchiopexy and posterior sagittal anorectoplasty were performed at 11 months of age; rudimentary mullerian structures were identified. This observation suggests an association of 10qter elements with male differentiation including AMH expression and is similar to a patient with 46, XY, del(10q26.1) in which AMH levels were not reported. Regional candidate genes include FGFR2 (10q26.13). The possible contribution of a NOTCH2 variant cannot be excluded. © 2015 Wiley Periodicals, Inc.

Osteocalcin (OCN), a marker of osteoblast activity, has been implicated in the regulation of energy metabolism by the skeleton and thus may affect body fat measures. We examined the relationships of OCN to body fat measures and whether they vary according to markers of energy and vitamin D metabolism. Data was obtained from 58 obese adolescents aged 13–17.9 years (38 females, 8 black or African-American). We calculated total fat mass (FM) [dual X-ray absorptiometry (DXA)] and visceral adipose tissue (VAT) [computerized axial tomography (CT)]. Blood tests included leptin, OCN, 25-hydroxyvitamin D [25(OH)D], parathyroid hormone (PTH), thyroid function tests, and triglycerides. Markers of glucose metabolism were obtained from fasting and OGTT samples. Adolescents with 25(OH)D

In-vessel cryo-pump (IVCP) of the Korea Superconducting Tokamak Advanced Research (KSTAR) has been designed, fabricated, and installed in the vacuum vessel for effective particle control by pumping through a divertor gap. For the final engineering design of the IVCP supports to withstand all external forces, a structure analyses were performed for two cases. The first is the thermal stress due to cool-down from room temperature to operating temperature (cryo-panel: 4.4 K, thermal shield: 77 K), and the other is the electro-magnetic stress due to the induced eddy currents during plasma disruptions. When the plasma disrupts, the maximum stress and displacement on the supports were estimated to be 849 MPa and 5.36 mm, respectively. These results were taken into account in the support design. The IVCP system was fabricated in two half-sectors and a pre-assembling test was successfully completed in the factory. Final installation of the IVCP in the vacuum vessel was fulfilled in parallel with a pressurization test (thermal shield: 30 bar, cryo-panel: 10 bar), a helium leak test, and a thermal shock test using liquid nitrogen. As a result, the IVCP system was successfully installed in the vacuum vessel.

It is possible to detect the presence of small field errors in a tokamak with an electron beam. This was demonstrated earlier on T-15 and TEXTOR. This paper discusses the concept, past experience on these tokamaks, calculations for the Korea Superconducting Tokamak Advanced Research (KSTAR) device, an electron beam source, measurement devices for these measurements, and some results. It is shown that small toroidally averaged field errors can be detected by this method. A low voltage electron beam (e-beam) gun and fluorescent screen were mounted in a vertical port and inserted into the vacuum vessel at the end of the KSTAR 2nd campaign plasma experiments. A camera with a narrow field of view was mounted in midplane port in a tube tangent to the field lines at R ∼ 1.3 m and photographed the beam striking the screen. The poloidal field (PF) currents were held constant during the camera exposure period. Many shots with various PF coils energized were made and the deflections of the e-beam were measured. The measurements were made with a camera integration time of 300 ms because of the low light intensity. The results show that there are large field errors that diminish as the PF currents are raised. There appears to be no significant up–down asymmetry for static fields. Measurements with a 7 PF coil scenario with a calculated field null located at e-beam radial position show much larger fields than calculated. KSTAR was constructed with Incoloy 908 conduit using cable-in-conduit conductors (CICC) in 10 of the 14 PF coils and all 16 of the toroidal field (TF) coils. Incoloy 908 has a relative magnetic permeability, μ, of about 10. The field errors appear to be largely due to Incoloy 908.

This paper studies the effect of mesostructured materials in the stereo-selective hydrogenation of 3-hexyne at 298 K and 40 psig pressure of H 2 over Pd-supported catalysts at different substrate:palladium (S:Pd) molar ratios. The catalysts were prepared by impregnation using a toluene solution of Pd(acac) 2 to obtain a metal content close to 1 wt.% over SBA-15 with one-dimensional hexagonal structure, MCM-48 silica with cubic structure and three-dimensional pore system and MSU-γ alumina with a lathlike particle morphology. All the supports were characterised by nitrogen adsorption–desorption isotherms at 77 K, TEM, XRD and H 2 chemisorption and TEM measurements. The reactions were found to be zero order with respect to 3-hexyne concentration. The starting 3-hexyne produces primarily cis -3-hexene, which subsequently is either hydrogenated to hexane or isomerized to trans -3-hexene and 2-hexenes that are found in very small amounts depending on the nature of the support used. Palladium catalysts supported on SBA-15 was the most active and selective catalyst.

Background: Data on the relation between vitamin D status and body fat indexes in adolescence are lacking. Objective: The objective was to identify factors associated with vitamin D status and deficiency in obese adolescents to further evaluate the relation of body fat indexes to vitamin D status and deficiency. Design: Data from 58 obese adolescents were obtained. Visceral adipose tissue (VAT) was measured by computed tomography. Dual-energy X-ray absorptiometry was used to measure total bone mineral content, bone mineral density, body fat mass (FM), and lean mass. Relative measures of body fat were calculated. Blood tests included measurements of 25-hydroxyvitamin D [25(OH)D], parathyroid hormone (PTH), osteocalcin, type I collagen C-telopeptide, hormones, and metabolic factors. Vitamin D deficiency was defined as 25(OH)D 65 ng/mL. Results: The mean (±SD) age of the adolescents was 14.9 ± 1.4 y; 38 (66%) were female, and 8 (14%) were black. The mean (±SD) body mass index (in kg/m2) was 36 ± 5, FM was 40.0 ± 5.5%, and VAT was 12.4 ± 4.3%. Seventeen of the adolescents were vitamin D deficient, but none had elevated PTH concentrations. Bone mineral content and bone mineral density were within 2 SDs of national standards. In a multivariate analysis, 25(OH)D decreased by 0.46 ± 0.22 ng/mL per 1% increment in FM (β ± SE, P = 0.05), whereas PTH decreased by 0.78 ± 0.29 pg/mL per 1% increment in VAT (P = 0.01). Conclusions: To the best of our knowledge, our results show for the first time that obese adolescents with 25(OH)D deficiency, but without elevated PTH concentrations, have a bone mass within the range of national standards (±2 SD). The findings provide initial evidence that the distribution of fat may be associated with vitamin D status, but this relation may be dependent on metabolic factors. This study was registered at www.clinicaltrials.gov as {"type":"clinical-trial","attrs":{"text":"NCT00209482","term_id":"NCT00209482"}}NCT00209482, NCT00120146.

During the ICP (inductively coupled plasma) process, a very high self-bias voltage is induced along the coil, which can cause sputtering of dielectric materials covering the coil. This problem can be solved by installing a capacitor between the coil end and ground. The effect of the capacitance termination was investigated by measuring the voltages at both of coil ends, plasma parameters and the impurity contents on the Si substrate after Ar plasma treatment at different ICP powers. The voltages at the coil ends decreased by half and the induced self-bias voltage approached zero under resonance conditions. The capacitance was approximately 200 pF, with both coil inlet and outlet voltages the same. In the range of this critical capacitance of 200 pF, the harmonics between coil and plasma decreased and the plasma density increased to 120–250% by controlling pressure and power compared to that with the grounded coil end. When the pressure was 1 and 5 mTorr, the plasma density doubly increased. Impurity concentrations were also suppressed drastically by the capacitance termination.

Iron-overload associated endocrinopathy is the most frequently reported complication of chronic transfusion therapy in patients with thalassaemia (Thal). This study compared iron-overloaded subjects with Thal (n = 142; 54%M; age 25.8 +/- 8.1 years) and transfused sickle-cell disease (Tx-SCD; n = 199; 43%M, 24.9 +/- 13.2 years) to non-transfused SCD subjects (non-Tx-SCD; n = 64, 50%M, 25.3 +/- 11.3 years), to explore whether the underlying haemoglobinopathy influences the development of endocrinopathy. Subjects were recruited from 31 centres in the USA, Canada and the UK. Subjects with Thal had more evidence of diabetes (13% vs. 2%, P < 0.001), hypogonadism (40% vs. 4%, P < 0.001), hypothyroidism (10% vs. 2%, P =

The ability of Loligo opalescens paralarvae to resist and recover from starvation was examined by measuring their survival, growth rate and RNA/DNA ratios during starvation and refeeding. Paralarvae were fed Artemia sp. nauplii, zooplankton and mysid shrimp. Fourteen days after hatching they were separated into five feeding treatments: a control treatment (food was always available) and treatments starved for 2, 3, 4 and 5 days, and then refed. Each day, 5–7 paralarvae from each treatment were anesthetized to measure mantle length and wet weight (WW), and then RNA and DNA were extracted using an ethidium bromide fluorometric technique. Paralarvae did not survive 4 and 5 days of starvation, showing that at 15 days of age and at 16 °C the limit to recovery was 3 days of starvation. Paralarvae starved for 2 and 3 days showed compensatory growth that mitigated the effects of starvation, in that at the end of the experiment (10 days), they attained mean final body weights similar to the control treatment. Differences in the RNA/DNA ratios between control and starved paralarvae were detected within 2 days of food deprivation. For paralarvae starved 2 and 3 days, it took 1 day after refeeding to attain RNA/DNA ratios not significantly different from the control treatment. Additionally, RNA/DNA ratios were highest during the day (0800, 1200, 1600 h) and lowest at night (0000, 0400 h), suggesting daytime feeding activity. Growth rates ranged from − 14% to 21% WW day− 1 and the resulting equation between RNA/DNA ratio and growth rate (GR) of paralarvae was GR = 1.74 RNA/DNA − 11.79 (R2 = 0.70). After starvation, there was a reduction in growth variability in all starved treatments, while growth variability remained high in the control treatment. Findings from the present study indicate that nucleic acids are a valid indicator of nutritional condition and growth in squid paralarvae.

The KSTAR (Korea Superconducting Tokamak Advanced Research) superconducting magnet system adopts a superconducting CICC(Cable-In-Conduit Conductor) type conductor. It consists of 16 TF (Toroidal Field) coils and 14 PF (Poroidal Field) coils and it also uses two different types of CICCs-Nb3Sn cable with Incoloy 908 conduit and NbTi cable with 316LN stainless-steel conduit. A special CICC jacketing system is developed for the KSTAR CICC fabrication: the tube-mill process, which consists of forming, welding, sizing and squaring procedure. The cabling process for TF and PF superconducting cable and the fabrication process of each CICCs (TF CICC and PF CICC) is described. The welding of conduit materials are also discussed. The fabrication results such as the geometrical specification, micro structure and the void fraction will be discussed

Carbon nitride films were deposited on a Si (100) wafer by an inductively coupled plasma assisted chemical vapor deposition (ICP-CVD) at room temperature. A mixture of N 2 and C 2 H 2 were used as the precursor. Additional r.f. power (13.56 MHz) was applied to the substrate with various negative self bias voltages ( V self =0 to −60 V), and the effect of the substrate bias on the structure and properties of the films was investigated. The composition and chemical bonding of the films were analyzed by Raman spectroscopy, Auger electron spectroscopy (AES) and X-ray photoelectron spectroscopy (XPS). The surface roughness of films was investigated by atomic force microscopy (AFM). It was found that nitrogen content of films were in the range of 13.5–21.4 at.%, decreased with increasing bias voltage. As the bias voltage was increased, the deposition rate decreased due to resputtering and the substrate temperature increased as a result of the energetic ions. The film hardness increased with increasing bias voltage up to 30 GPa at −60 V. The results from Raman and XPS analyses showed that the amount of sp 3 C C or sp 3 C N bonds increased with increasing bias voltage, while the number of the C H, N H and sp 2 C N bonds decreased.

Although it is life saving, transfusion therapy has resulted in the majority of sickle cell anemia and thalassemia patients being at risk for hemosiderosis-induced organ damage. It is unknown whether the complications of iron overload are affected by the underlying disease. In order to address this problem, we compared the prevalence of organ dysfunction in both groups of patients receiving chronic transfusion therapy (beta thalassemia, N = 30; sickle cell anemia, N = 43). Both groups had similar quantitative liver iron. Thalassemia patients had greater cardiac disease (20% vs. 0%), growth failure (27% vs. 9%), and endocrine failure (37% vs. 0%). The strongest predictors of combined endocrine and cardiac disease in multivariate analysis were duration of chronic transfusion (P = 0.03) and diagnosis (P = 0.03). Quantitative liver iron concentration on a single liver biopsy was not predictive of cardiac or endocrine injury. Viral hepatitis is the strongest predictor of hepatocellular damage (P = 0.009), while the development of liver fibrosis is more closely related to liver iron concentration (P = 0.04). In conclusion, sickle cell anemia and thalassemia differ in the prevalence of organ injury. This difference is related to the duration of iron exposure and the specific hemoglobinopathy. A prospective study with a larger number of subjects is needed to confirm the relationships between specific diagnosis, liver iron concentration over time, and organ dysfunction.

In high temperature superconducting (HTS) transformer with double pancake windings, the perpendicular component of leakage magnetic flux density (B/sub r/) applied to HTS tapes of double pancake windings is larger than that of solenoid windings, which acts as a cause to decrease the critical current in HTS tape and increase AC loss. In this paper, in order to reduce the B/sub r/ applied to HTS windings, the use of ferrite flux diverters is presented. Flux diverters are located in between the primary windings and the secondary windings, and must be made of low-loss materials to minimize the heat. And electromagnetic analysis of a single-phase 1 MVA 22.9 kV/6.6 kV HTS transformer with flux diverters in HTS windings was accomplished. The variation of B/sub r/ applied to the secondary windings and magnetic flux density in flux diverters were analyzed by changing their relative permeability of flux diverters respectively. Suitable relative permeability of flux diverters to reduce B/sub r/ applied to HTS windings was selected, considering flux diverter losses by increment of B in flux diverters.

Strong red photoluminescence (PL) spectra appeared at porous silicon (PS) samples prepared by a chemical anodization of Fe-contaminated Si substrates. The Fe1000 sample with Fe contamination of 1000 ppb showed a ten times stronger red PL than that of the reference PS sample without any Fe contamination, and this sample also showed the higher thermal stability for PL spectra as compared with the reference PS sample. Furthermore, the PL intensity from the PS with Fe contamination is linearly proportional to the Fe-related trap concentrations of Si substrates obtained from DLTS. Especially, all the PS samples exhibit the same PL peak position regardless of Fe contamination concentrations, as compared with that of the reference PS. This means that there is no significant effect such as the variation of size distribution of nanocrystalline Si in PS layer formed on Fe-contaminated Si substrate. Based on the results of PL and DLTS, we found that the PL efficiency depends strongly on the Fe-related trap concentration in Si substrates.

The central solenoid (CS) magnet system of the Korea Superconducting Tokamak Advanced Research (KSTAR) device consists of four pairs of segmented CS coils and a CS coil structure. The maximum repulsive force between CS coils is about 12 MN. The functions of the CS structure are to apply preload on the CS coils and to support the repulsive force between CS coils during operation. The designed axial compression of 15 MN at 4.5 K will be applied partly by assembling the preload structure at room temperature with preload of about 13.4 MN and partly by the thermal contraction difference between the CS coils and the structure during cool down. Additional preload will be given by minute adjustment of wedges. The structural analysis of the CS magnet system has been performed to verify the CS structure design reliability.

Several types of Lorenz forces and Joule heating generated in the magnet system of the Korea Superconducting Tokamak Advanced Research (KSTAR) device have been studied numerically and analytically. We have examined 225 plasma equilibrium states to calculate the maximum magnetic forces and to determine which equilibrium state(s) generate such forces. The obtained results are used as input for structural analysis. A precompression should be applied to the eight-segmented central solenoid (CS) coil assembly to prevent free motion of each coil due to attractive and repulsive forces during operation. We have also evaluated the maximum values of the vertical and lateral forces for each of the CS coils and poloidal field coils and also for the entire CS coil stack. The in-plane force due to toroidal field (TF) coil charging and the out-of-plane force due to interaction of the TF coil current with the poloidal field have been computed. The Joule heating on the TF structure due to plasma disruption has also been calculated.

The toroidal field (TF) magnet system of Korea Superconducting Tokamak Advanced Research (KSTAR) device consists of 16 superconducting coils enclosed in steel cases. The TF cases are wedged along the inboard straight legs to sustain in-plane centering forces. The inter-coil structures contain adjustable shear keys and conical bolts to provide pre-loading in toroidal direction and to resist in-plane and out-of-plane forces, which are the most critical loads on the TF magnet system. For effective cooling of the TF case, a pad-type cooling channel has been designed to ensure structural and thermal stability. In order to investigate the structural integrity and to increase the structural reliability of the KSTAR magnet system, structural analyses have been conducted. From the analysis results, it has been found that the TF magnet structure can safely withstand the reference scenario operations.

The objective of this study was to examine the impact of crowding (stocking density) on food consumption and growth of juvenile Sepia officinalis reared at 17 and 25 °C. Two groups of 75 cuttlefish each were reared in closed seawater systems with water temperatures of 17 and 25 °C. Each group was subdivided into two treatments (three replicates per treatment): low-density (equivalent to 100 Sepia m−2) and high-density (equivalent to 400 Sepia m−2). Food consumption was measured daily and live wet body weight (g) was measured weekly over a 5-week study. The 25 °C treatment resulted in significantly higher growth rates and food consumption compared to the 17 °C treatment. Stocking density had no statistically significant effect on food consumption, gross growth efficiency (GGE), or weight at either temperature. However, the high-density treatments had slightly lower GGE values overall and growth in weight at 25 °C was slightly but consistently lower in the high-density treatment suggesting that stocking densities of 400 Sepia m−2 may be approaching levels that impact feeding and growth.

The extensive design effort for KSTAR has been focused on two major aspects of the KSTAR project mission - steady-state-operation capability and advanced tokamak physics. The steady state aspect of the mission is reflected in the choice of superconducting magnets, provision of actively cooled in-vessel components, and long pulse current drive and heating systems. The advanced tokamak aspect of the mission is incorporated in the design features associated with flexible plasma shaping, double null divertor and passive stabilizers, internal control coils and a comprehensive set of diagnostics. Substantial progress in engineering has been made on superconducting magnets, the vacuum vessel, plasma facing components and power supplies. The new KSTAR experimental facility with cryogenic system and deionized water cooling and main power systems has been designed, and the construction work is under way for completion in 2004.

In order to provide better estimates of the thermal‐induced stress encountered by salmon migrating to their spawning grounds, a model is used to hindcast temperatures throughout the mainstem Fraser and Thompson Rivers back to 1953. Tributary and headwater temperatures that are not available prior to 1993 are estimated with both linear regression and neural network techniques. The average root mean square difference between model temperatures and those observed at Hell's Gate, on the lower Fraser River, is computed to be 1.12°C. Historical flow and temperature observations are also used to establish patterns and trends for the Fraser River watershed. The Julian day numbers by which one‐third and one‐half of the integrated yearly discharge had occurred were computed and found to be progressing earlier at the rates of 0.11 and 0.09 days per year, respectively. Both values are significant at the 95% level. Average summer Hell's Gate temperatures from 1941 to 1998 are warming at the rate of 0.012°C pe...

The Korea Superconducting Tokamak Advanced Research (KSTAR) device is a steady-state-capable experimental fusion device with a fully superconducting magnet system, including toroidal field (TF) coils, central solenoid (CS) coils, and poloidal field (PF) coils. The major design consideration of the magnet system is to meet the KSTAR mission with plasma current of 2 MA and toroidal field of 3.5 T at the major radius 1.8 m and z=0. The preliminary analyses show that the magnet structure design has mechanical, electrical, and thermal stability during operation. The TF magnets have a wedged structure, including coil cases, inter-coil structures, and inter-octant joints. The CS and PF structures are designed to support the electromagnetic forces. To support the coil system against gravity and lateral loads, gravity support and lateral load structures are designed.

Standard weight-for-age and height-for-age charts have been used for many years to assess growth and body compositions of children, including curves specific to the Prader-Willi syndrome (PWS). However, a number of direct measures of body compositions have been developed in recent years. Dural-energy x-ray absorptiometry (DXA) provides a measure of bone mineral content and bone mineral density for the whole body and at specific bone regions (spine, hip, forearm). This technology now has been extended to include assessment of the body's lean tissue mass and body fat mass. An alternate technique, called bioelectrical impedance analysis (BIA), has gained wide use in recent years as an indirect measure of the fat-free mass. The older, underwater weighing method is giving way to the air-displacement plethysmographic technique for assessing body fatness. Total body potassium still remains the method of choice for a direct assessment of body cell mass, and body nitrogen is recognized as the best assay for body protein mass. Imaging techniques, such as a computer tomography and magnetic resonance imaging, continue to receive increasing attention for body composition assays, especially for the subcutaneous vs. visceral distribution of body fat. Although total body water and extracellular water can be measured by dilution techniques, these methods are slowly being replaced by the BIA assay. When these various techniques are used in combination, the patient's nutritional status can be obtained based on a comprehensive 5-level multicompartment model of the body composition. Each of these methods will be reviewed, and their specific strengths and limitations presented, relative to their application for assessing changes in body composition of the PWS patient.▪ The Endocrinologist 2000; 10: 57S-62S

Prader-Willi syndrome (PWS) is a disabling condition characterized by hypotonia, hyperphagia, obesity, short stature, delayed or absent puberty, and mental retardation. The syndrome complex was first described in 1956 by Dr. Andrea Prader and colleagues [1]. In the 1980s, a characteristic genetic defect was identified involving deletion of paternal alleles at chromosome 15q11-13 [2-6]. This occurs by deletion of alleles on the paternal copy of chromosome 15q, an absent paternal chromosome 15q with maternal disomy, or, rarely, by mutations of the imprinting center of chromosome 15q. The estimated population prevalence of PWS is 1 in 15,000 live births.

Modulation of growth factors by growth hormone in children with chronic renal failure. Anthropometric measurements and circulating growth factors were studied serially in 44 prepubertal children with growth failure and chronic renal failure (GFR = 10 to 40 ml/min/1.73m2) who were randomized to receive either recombinant human growth hormone (rhGH; N = 30) or no treatment (N = 14). RhGH was given as Nutropin, 0.05 mg/kg/day, and the studies were carried out at baseline and after 3 and 12 months. At baseline, serum insulin-like growth factor binding protein (IGFBP)-1 and -2 levels were, while IGFBP-3 levels were not, higher than those of children with normal renal function. In addition, height SDS at baseline correlated inversely with serum IGFBP-2 levels (r = -0.461, P = 0.0016), but did not correlate significantly with any other factor. After 12 months of study, the 30 children receiving rhGH showed: (i) greater increase in height (9.1 ± 2.8 vs. 5.5 ± 1.9 cm, P < 0.0001); (ii) increases in serum levels of IGF-I, IGF-II, free IGF-I, IGFBP-3 and acid labile subunit (ALS); (iii) a greater decrease in serum IGFBP-1 levels; and (iv) no significant difference in serum IGFBP-2 levels, when compared to the 14 control patients. The change in height SDS after 12 months of rhGH (+ 0.8) in the 30 treated children correlated significantly and positively with serum ALS, IGFBP-3, total IGF, IGF-I, IGF-II and free IGF-I levels measured during treatment. These observations suggest that, in children with growth failure associated with chronic renal failure: (i) IGFBP-2, and not IGFBP-3, is likely to be a growth inhibitor; (ii) rhGH stimulates catch-up growth in part by increasing serum levels of IGF peptides; and (iii) linear growth is influenced by the balance between growth stimulating IGFs and growth inhibitory IGFBPs.

Weight loss is a common, persistent characteristic of long term human immunodeficiency virus (HIV-1) infection; its full etiology remains unknown. Because treatment with GH has induced nitrogen retention in various catabolic conditions, we designed this study to determine whether a moderate dose of insulin-like growth factor I (IGF-I) combined with a low GH dose could impede the catabolic response seen in HIV-1 infection. A double blind, placebo-controlled study design was used. Subjects in the GH/IGF-I treatment group (n = 44) and control group (n = 22) continued to receive their routine stable antiretroviral therapy. No patient had a recent history of opportunistic infection, malignancy, or Kaposi's sarcoma and had dietary intakes of at least 25 Cal/kg weight.day at study entry. During the 12-week study period, dietary instruction was given, and subjects were encouraged to maintain an intake of 35 Cal/kg and 1 g protein/kg. All subjects had a body mass index of 19.8 kg/m2 or less at the time of study entry or a weight loss of 10% or more of their premorbid weight and a body mass index below 26.1 kg/m2. The treatment group received 0.34 mg (0.68 mg/day) GH, twice daily, and 5.0 mg (10 mg/day) IGF-I, twice daily. Changes in body composition of total body potassium (TBK), total body nitrogen (TBN), fat-free mass (FFM), and body fat (Fat) were examined at 6 and 12 weeks during the treatment period. TBK, TBN, FFM, and Fat for the treatment and placebo groups were, on the average, below normal at study entry. At 6 weeks, the GH/IGF-I group showed a significant increase in FFM (P0.0001), a minimal increase in TBK (P0.05), and a substantial decrease in Fat (P0.01) compared with baseline values. The loss of body fat continued to be significant (P0.01) in the GH/IGF-I group treatment at 12 weeks, whereas the increase in FFM was minimal (P0.05). No significant changes in the mean body composition occurred at 6 or 12 weeks in the placebo group. By 12 weeks, neither TBK (body cell mass) nor TBN (total protein mass) had significantly increased relative to the values at baseline, although the FFM remained elevated. Thus, the combined GH and IGF-I doses used in this study in adult males with HIV-associated weight loss were ineffective in producing a sustained anabolic response and, in fact, resulted primarily in a significant loss of body fat.

Children with chronic renal failure (CRF) often fail to attain an adult height consistent with their genetic potential. The growth hormone (GH)/insulin-like growth factor (IGF)/growth plate chondrocyte axis has been intensively studied in these children to determine the basis for this growth failure. Evidence suggests that hepatic GH resistance results in deficient expression of IGF-I. However, serum IGF-I levels are usually normal and it is IGF-I action on target tissues which is inhibited, possibly by the presence of excess high-affinity IGF binding proteins (IGFBPs) in CRF serum. In this paper we evaluate the roles of IGFBP-1, -2, and -3 as growth inhibitors in CRF children. The data support a role for each of these IGFBPs as growth inhibitors. Currently, IGFBP-1 meets most criteria expected of a growth inhibitor, but IGFBP-2 and -3 will likely also meet these criteria and may well be important contributors to the growth failure of CRF. Ultimately, many or all of the six IGFBPs may be found to contribute to the excess high-affinity IGF binding sites which are a hallmark of CRF serum and are possible contributors to the growth failure of CRF children.

The motional Stark effect (MSE) diagnostic has been used to obtain accurate measurements of the internal magnetic pitch angle tan−1(BZ/BT) in finite‐pressure tokamaks. The MSE data, together with external magnetic probe data, are used to reconstruct self‐consistently the equilibrium safety factor (q) profile and, hence, the plasma current density, in the Tokamak Fusion Test Reactor (TFTR) [Plasma Phys. Controlled Nucl. Fusion Res. 1, 51 (1986)] and the Princeton Beta Experiment‐Modified (PBX‐M) tokamak [Phys. Fluids B 2, 1271 (1990)]. An efficient computational scheme, based on an inverse coordinate representation of the magnetic field, has been developed to solve the coupled nonlinear equations describing both the magnetohydrodynamic (MHD) equilibrium and the q profile, which best match all the experimental data.

Insulin-like growth factor-binding protein (IGFBP)-1 is one of six homologous proteins that specifically bind and modulate the mitogenic and metabolic actions of insulin-like growth factor (IGF)-I and IGF-II. Of the six IGFBP, IGFBP-1 is the only one that displays rapid dynamic regulation in vivo, with serum levels varying 10-fold or more in relation to meals. The complementary cDNA for IGFBP-1 was first reported in 1988. The predicted 234-amino acid sequence has a molecular mass of 25.3 kDa. The N-terminal and C-terminal regions are highly homologous among rat, human, and bovine sequences, and contain 18 conserved cysteines which are postulated to provide a framework for ligand binding. The 65-residue midregion is less homologous and does not contain cysteines, but does include a Pro-Glu-Ser-Thr (PEST) domain that is typical of rapidly metabolized proteins. The gene for IGFBP-1 has been localized to human chromosome region 7p12-p14, where it is contiguous with the gene for IGFBP-3. IGFBP-1 mRNA and protein expression have been identified in human liver and uterine decidua, and in nonhuman kidney. In vitro and in vivo studies indicate that insulin is the primary regulator of IGFBP-1 expression in these tissues, and that the primary effect of insulin is rapid inhibition of transcription. On the other hand, cortisol, glucagon, and cAMP stimulate IGFBP-1 production. Limited data also show a potent stimulatory effect of phorbol esters. A detailed review of IGFBP-1 levels and physiology in vivo and in vitro is presented. The function of IGFBP-1 is not completely defined. However, several studies demonstrate that IGFBP-1 inhibits IGF binding to cell surface receptors and thereby inhibits IGF-mediated mitogenic and cell metabolic actions. Furthermore, IGFBP-1 regulation by insulin and glucoregulatory hormones in vitro and limited in vivo data are consistent with a role for IGFBP-1 in glucose counterregulation.

An explicit formulation is presented to determine widths of magnetic island separatrices generated by magnetic field perturbations in a general toroidal stellarator geometry. The method, which is based on a representation of three-dimensional flux surfaces by double Fourier series, allows rapid and accurate calculation of the island widths in real vacuum field configurations, without the need to follow field lines through numerical integration of the field line equations. The procedure does not involve any simplifying approximations and can be used for fields with multiple small perturbations. Numerical results of the island width obtained in the flux co-ordinate representation for the Advanced Toroidal Facility agree closely with those determined from Poincare puncture points obtained by following field lines. The effects of resonance coupling are analysed.

Pediatric pituitary diagnosis is complicated by the unique developmental characteristics, secretory patterns and regulation of each pituitary hormone system. Although dynamic test procedures have been described, validation of each procedure is limited and universally agreed diagnostic criteria are not available. Clinical acumen, experience and judgment continue to be primary elements of pituitary diagnosis.

Pituitary gigantism, a condition of endogenous growth hormone (GH) hypersecretion prior to epiphyseal closure, is a rare condition. In the adult condition of GH excess, acromegaly, the occurrence of type 2 diabetes mellitus (T2DM) and diabetic ketoacidosis (DKA) have been reported, with resolution following normalization of GH levels. We report the case of a 16-year-old male with pituitary gigantism due to a large invasive suprasellar adenoma who presented with T2DM and DKA. Despite surgical de-bulking, radiotherapy and medical treatment with cabergoline and pegvisomant, GH and insulin-like growth factor-I (IGF-I) levels remained elevated. However, the T2DM and recurrent DKA were successfully managed with metformin and low-dose glargine insulin, respectively. We review the pathophysiology of T2DM and DKA in growth hormone excess and available treatment options.

Cells are known to undergo apoptosis when cultured in high serum concentrations. However, the serum factors responsible for this induction of apoptosis have not been identified. The IGF-binding protein-3 (IGFBP-3), a negative growth regulator, is found at concentrations of 5 microgram/ml in serum. We have recently demonstrated that IGFBP-3 induces apoptosis in PC-3 cells, a prostate cancer cell line, at a concentration of 500 ng/ml. In this communication, we demonstrate the role of IGFBP-3 as one of the apoptosis-inducing agents in high serum concentrations. Treatment of PC-3 cells with increasing concentrations (40% to 90%) of intact human serum (HS) resulted in a dose-dependent decrease in cell growth. Valinomycin, an ionophore, was used as a positive control to measure the induction of apoptosis by serum treatment in PC-3 cells. Treatment with 90% serum showed significant suppression of growth (P

The possible occurrence of increased non-enzymatic glycosylation of serum insulin-like growth factor binding protein-3 (IGFBP-3) in vivo and the changes that would simultaneously occur in serum levels of IGFBP-3 and insulin-like growth factor-1 (IGF-I) were investigated. We measured levels of IGF-I and IGFBP-3 and the degree of glycation of total serum protein and IGFBP-3, in serum samples obtained from patients with poorly controlled non-insulin-dependent diabetes (type 2) and from age-matched non-diabetic controls. Type 2 diabetic patients had significantly higher glycated serum protein (GlyP) levels. GlyP significantly correlated with age in the control (r = 0.315, P, Facultad de Ciencias Médicas

In 1988, insulin-like growth factor-binding protein-1 (IGFBP-1) became the first characterized member of a group of structurally related soluble proteins which specifically bind and modulate the actions of the IGFs. Since then, a wealth of information has accumulated regarding the physiology of this dynamic serum protein. In this review, we update our 1993 summary (Lee PDK et al. Proc Soc Exp Biol Med 204:4-29) of the status of IGFBP-1 research. The IGFBP-1 protein sequence contains 12 N-terminal and 6 C-terminal cysteine residues which are conserved in other mammalian IGFBP-1 sequences and amongst other IGFBPs; both of the cysteine-rich regions are required for optimal IGF binding. The nonconserved IGFBP-1 midregion may act as both a hinge which defines ligand binding characteristics and as a specific target for protease activity. Integrin-binding and phosphorylation sites within the IGFBP-1 sequence have functional significance in vitro, but their physiologic relevance in vivo have not been defined. The human IGFBP-1 and IGFBP-3 genes are contiguous and located in close proximity to the homeobox A (HOXA) gene cluster on chromosome 7. The other IGFBP genes, located on chromosomes 2, 12, and 17, are also associated with HOX clusters, suggesting evolutionary linkage of the IGFBP and HOX gene families. Similarities between the hIGFBP-1 and phosphoenolpyruvate kinase (PEPCK) promoters, including regions conferring insulin, glucocorticoid, and cyclic adenosine-monophosphate responses, are consistent with our previous hypothesis that IGFBP-1 is involved in regulation of glucose metabolism. The tissue-specific patterns of IGFBP-1 gene expression in liver, kidney, decidua, and ovary may be due to stimulation of IGFBP-1 transcription by hepatic nuclear factor 1 (HNF1) proteins. Clinical and basic studies of IGFBP-1 physiology have been aided by several recently developed assay methods. Numerous investigations have confirmed that insulin, via inhibition of IGFBP-1 transcription, is the primary determinant of IGFBP-1 expression both in vitro and in vivo. IGF-I and IGF-II also have specific inhibitory effects on IGFBP-1 expression. Glucocorticoids and cAMP stimulate IGFBP-1 transcription, but these effects are observed only in conditions of low or absent insulin effect. Other stimulants of IGFBP-1 expression include thyroid hormones and epidermal growth factor. Phorbol ester stimulation of IGFBP-1 expression can supersede the effects of insulin in vitro;however, the mechanism and in vivo correlates of this effect have not been determined. Cytokines and, perhaps, growth hormones may affect IGFBP-1 expression, perhaps by altering the regulatory actions of insulin; this effect may have important clinical relevance. IGFBP-1 expression is upregulated in liver and (nonhuman) kidney during postinjury regeneration. The IGF-inhibitory actions of IGFBP-1 has been confirmed by numerous in vitro studies and several in vivo animal investigations, including administration of recombinant IGFBP-1 and IGFBP-1 transgenic models. IGFBP-1 has been shown to inhibit somatic linear growth, weight gain, tissue growth, and glucose metabolism. Moreover, IGFBP-1 appears to be a primary determinant of free IGF-I levels in serum. Excess levels of IGFBP-1 may contribute to growth failure in intrauterine growth restriction and in pediatric chronic renal failure, while low IGFBP-1 levels are associated with obesity and with cardiovascular risk factors in insulin resistance syndromes. Serum IGFBP-1 measurements may be useful biochemical marker in these pathologic conditions. IGFBP-1 is expressed in decidualized stromal cells of the uterine endometrium and in ovarian granulosa cells. IGFBP-1, together with IGFs, insulin, ovarian steroids, cytokines, and other factors, is involved in a complex system which regulates menstrual cycles, ovulation, decidualization, blastocyst implantation, and fetal growth. (ABSTRACT TRUNCATED)

Since the successful first plasma generation in the middle of 2008, three experimental campaigns were successfully made for the KSTAR device, accompanied with a necessary upgrade in the power supply, heating, wall-conditioning and diagnostic systems. KSTAR was operated with the toroidal magnetic field up to 3.6 T and the circular and shaped plasmas with current up to 700 kA and pulse length of 7 s, have been achieved with limited capacity of PF magnet power supplies. The mission of the KSTAR experimental program is to achieve steady-state operations with high performance plasmas relevant to ITER and future reactors. The first phase (2008–2012) of operation of KSTAR is dedicated to the development of operational capabilities for a super-conducting device with relatively short pulse. Development of start-up scenario for a super-conducting tokamak and the understanding of magnetic field errors on start-up are one of the important issues to be resolved. Some specific operation techniques for a super-conducting device are also developed and tested. The second harmonic pre-ionization with 84 and 110 GHz gyrotrons is an example. Various parameters have been scanned to optimize the pre-ionization. Another example is the ICRF wall conditioning (ICWC), which was routinely applied during the shot to shot interval. The plasma operation window has been extended in terms of plasma beta and stability boundary. The achievement of high confinement mode was made in the last campaign with the first neutral beam injector and good wall conditioning. Plasma control has been applied in shape and position control and now a preliminary kinetic control scheme is being applied including plasma current and density. Advanced control schemes will be developed and tested in future operations including active profiles, heating and current drives and control coil-driven magnetic perturbation.