bluuebunny/crossref_metadata_2025 huggingface dataset:
Bayesian Hierarchical Methods to Interpret the 13C-Octanoic Acid Breath Test for Gastric Emptying
Authors: Bluck Leslie J.C. , Jackson Sarah J. , Vlasakakis Georgios , Mander Adrian
Published: November 2010

Background:The 13C-octanoic acid breath test is a convenient method for assessing gastric emptying (GE). Success depends on obtaining a well-characterized time profile of the excretion of label in breath, which may not be the case if GE is delayed. Aims: To use Bayesian techniques in conjunction with hierarchical modelling as a method to increase the success of the modelling process. Methods: Retrospective analysis of 164 individual breath tests using the WinBUGS program. The approach was tested by analysing the complete dataset simultaneously, and also as individual studies. Results: The time required for Bayesian modelling was comparable with that needed for the usual methods. The results obtained were almost identical to those obtained from conventional modelling for well-behaved breath tests, but much more realistic in cases where the experimental data was poor, or when GE was delayed. Conclusions: The use of Bayesian estimation of the parameters of the 13C-octanoic acid breath test is demonstrated. By adopting a hierarchical model, realistic values for the lag phase and half-emptying time were obtained in situations when conventional parameter estimation failed. This is particularly relevant when GE is unexpectedly delayed. We recommend that WinBUGS become the method of choice for analysing breath test data.
Growth Patterns in the First Three Years of Life in Children with Classical Congenital Adrenal Hyperplasia Diagnosed by Newborn Screening and Treated with Low Doses of Hydrocortisone
Authors: Bonfig W. , Schmidt H. , Schwarz H.P.
Published: November 2010

Background: Linear growth is the best clinical parameter for monitoring metabolic control in classical congenital adrenal hyperplasia (CAH). Objective: To analyze growth patterns in children with CAH diagnosed by newborn screening and treated with relatively low doses of hydrocortisone during the first year of life. Patients and Methods: 51 patients (27 females) were diagnosed with classical CAH by newborn screening. All patients were treated with relatively low doses of hydrocortisone (9–15 mg/m2 body surface area). 47 patients were additionally treated with fludrocortisone. Results: At birth, height SDS (H-SDS) was 1.1 ± 1 in girls and 0.9 ± 1.5 in boys. After 3 months, H-SDS decreased to 0.4 ± 0.9 in girls and to 0.1 ± 1.3 in boys. Over the 3-year period, H-SDS further decreased to –0.4 ± 1.8 in girls and to –0.8 ± 1 in boys and approached the genetic height potential (target H-SDS of girls –0.5 ± 0.3 and target H-SDS of boys –0.9 ± 0.7). During the first 9 months of age, growth velocity was slightly decreased in girls (18.2 ± 1.9 cm) and boys (17.3 ± 1.6 cm) when compared to a healthy reference population (girls 19.0 ± 3.9 cm and boys 18.7 ± 4.7 cm). At the age of 3 years, bone age was appropriate for chronological age in both girls (2.7 ± 0.5 years) and boys (2.9 ± 0.5 years). Conclusion: Birth length is above average in children with classical CAH, which might be the result of untreated hyperandrogenism in utero. With relatively low doses of hydrocortisone treatment, growth velocity decreases slightly during the first 9 months and H-SDS then approaches the genetic height potential.
Risk Factors for Chronic Lung Disease and Mortality in Newborns with Congenital Diaphragmatic Hernia
Authors: van den Hout L. , Reiss I. , Felix J.F. , Hop W.C.J. , Lally Pamela A. , Lally Kevin P. , Tibboel D. ,   for the Congenital Diaphragmatic Hernia Study Group
Published: November 2010

Background: Congenital diaphragmatic hernia (CDH) is associated with a mortality rate of 10–35% in live-born infants. Moreover, CDH survivors have a substantial risk of developing long-term pulmonary sequelae, such as bronchopulmonary dysplasia (BPD). Objectives: This study aims to evaluate risk factors associated with BPD and mortality in neonates with CDH, with particular focus on the initial ventilation mode. Methods: Eligible for inclusion were live-born infants with CDH born from 2001 through 2006 at the centers participating in the CDH Study Group. BPD (defined as oxygen dependency at day 30) and/or mortality by day 30 served as the primary endpoint. Results: A total of 2,078 neonates were included in the analysis. At day 30, 56% of the patients had either died or met the criteria for BPD. In infants who survived until day 30, the prevalence of BPD was 41%. The overall mortality rate was 31%. High-frequency oscillatory ventilation as initial ventilation mode, a right-sided defect, a prenatal diagnosis, a lower Apgar score at 5 min, a cardiac anomaly, a chromosomal anomaly and a lower gestational age were all associated with BPD and/or mortality by day 30. Conclusions: Despite improvements in neonatal care, the rates of BPD and early mortality in newborns with CDH are still considerable. Several important risk factors for a worse outcome are reported in this nonrandomized prospective observational study.
Ghrelin Plasma Levels in Patients with Idiopathic Short Stature
Authors: Iñiguez Germán , Román Rossana , Youlton Ronald , Cassorla Fernando , Mericq Verónica
Published: November 2010

Background: Novel molecular insights have suggested that ghrelin may be involved in the pathogenesis of some forms of short stature. Recently, growth hormone secretagogue receptor (GHSR) mutations that segregate with short stature have been reported. Aim: To study plasma ghrelin levels in prepubertal patients with idiopathic short stature (ISS). Methods: Fasting total plasma ghrelin levels (radioimmunoassay) in 41 prepubertal patients with ISS (18 females, age 7.9 ± 0.5 years) compared with 42 age- and sex-matched controls (27 females, age 8.0 ± 0.3 years) with normal height. In a subset of 28 patients, the ghrelin receptor was sequenced. Results: ISS patients exhibited a higher level of ghrelin (1,458 ± 137 vs. 935 ± 55 pg/ml, p < 0.01) and similar IGF-I levels (–0.66 ± 1.29 vs. –0.32 ± 0.78 SDS) compared to controls. Ten patients with ISS had ghrelin levels greater than +2 SDS compared to controls. These patients did not differ in height, BMI or IGF-I SDS compared to ISS patients with ghrelin levels within the normal range. Molecular analysis of GHSR did not show any mutations, but showed some polymorphisms. Conclusion: These results suggest that in ISS patients, short stature does not appear to be frequently caused by abnormalities in ghrelin signaling.
Prospective Study Confirms Oxandrolone-Associated Improvement in Height in Growth Hormone-Treated Adolescent Girls with Turner Syndrome
Authors: Zeger Martha P.D. , Shah Kavita , Kowal Karen , Cutler, Jr. Gordon B. , Kushner Harvey , Ross Judith L.
Published: November 2010

Background/Aims: Untreated girls with Turner syndrome (TS) have growth failure, and adult height is, on average, 20 cm less than predicted height. Treatment with growth hormone (GH) is now standard of care. The objective of this study was to investigate the benefit of adding oxandrolone (Ox) to GH in a long-term, randomized, placebo (Pl)-controlled prospective trial to near adult height in TS. Methods: Prospective, randomized, Pl-controlled study: 76 girls with TS (ages 10–14.9 years) were randomized to receive Ox (0.06 mg/kg/day) or Pl in combination with GH (0.35 mg/kg/week, daily) over 2 years. Auxologic data, breast and pubic hair Tanner stages, and hormone and lipid levels were measured. Subjects who chose to continue were followed in a 2-year double-blind extension, also received estrogen therapy (years 3, 4), and had dual-energy X-ray absorptiometry evaluation of bone density (years 3, 4). Results: At year 4, the change in absolute height and height SDS was greater in the GH/Ox versus GH/Pl group [26.2 ± 6.7 vs. 22.2 ± 5.1 cm, analysis of covariance (ANCOVA) p < 0.001; 1.8 ± 0.9 vs. 1.2 ± 0.7 standard deviation scores, ANCOVA p < 0.001]. Bone mineral density (BMD) of the wrist (0.51 ± 0.17 vs. 0.54 ± 0.05 g/cm2) and spine (0.91 ± 0.34 vs. 0.96 ± 0.13 g/cm2) in the GH/Ox versus GH/Pl groups was similar after 4 years. Breast development was slower in the GH/Ox versus GH/Pl group [year 4: Tanner stage 2.9 ±1.3 (Ox) vs. 4.1 ± 1.3 (Pl), p = 0.003], and menarche was approximately 1 year later. Conclusions: The addition of Ox to GH at mean age 12.0 ± 1.7 year augmented height gain after 4 years of treatment, slowed breast development and did not affect BMD in girls with TS. Whether initiation of Ox prior to initiation of pubertal development would optimize height gain without impeding breast development will require further study.
Efficacy and Safety of the Selective Cyclooxygenase-2 Inhibitor Celecoxib in the Treatment of Rheumatoid Arthritis and Osteoarthritis in Japan
Authors: Sakamoto Choitsu , Soen Satoshi
Published: November 2010

Background/Aims: Gastrointestinal (GI) disorders are common adverse reactions of nonsteroidal anti-inflammatory drugs (NSAIDs). Loxoprofen is a representative NSAID widely used in East Asia. A selective cyclooxygenase-2 inhibitor, celecoxib, was introduced in Japan in 2007. In this study, we aimed to compare the efficacy and safety of celecoxib with those of loxoprofen in Japanese patients. Methods: We analyzed the data from 12 clinical studies conducted in Japan. These data of Japanese patients were compared with those of the patients in the West that had been published after 2000. Results: The efficacy of celecoxib as an analgesic was comparable to that of loxoprofen, whereas serious GI events, including symptomatic ulcers, were significantly less frequent with celecoxib than with loxoprofen in Japanese patients with rheumatoid arthritis (RA) and osteoarthritis (OA) (p = 0.039). These results were consistent with the findings of the studies conducted in the West. The incidence of serious cardiovascular events was 0.1% in 2,398 subjects on celecoxib, which was not statistically different from the incidence in subjects on loxoprofen (0.3%; p = 0.3404) and those on placebo (0.2%); this result was also consistent with the data of the studies conducted in the West. Conclusion: The analgesic activity of celecoxib, which was used for the treatment of RA, OA, and low back pain, was comparable to that of loxoprofen, and celecoxib was safer in terms of GI injury often caused by other nonselective NSAIDs.
Growth Hormone Secretory Status Evaluated by Growth Hormone Peak after Two Pharmacological Growth Hormone Release Stimuli Did Not Significantly Influence the Two-Year Catch-Up Growth Induced by Growth Hormone Therapy in 318 Prepubertal Short Children with Idiopathic Growth Retardation
Authors: Carrascosa A. , Audí L. , Fernández-Cancio M. , Yeste D. , Gussinye M. , Albisu M.A. , Clemente M. , Fábregas A. , Bel J. , Nosás R. , Rabanal M. , del Pozo C. , Gómez J.M. , Mesa J. ,   Catalan Group for Short Stature Study
Published: November 2010

Background/Aims: In prepubertal short children with idiopathic growth retardation, growth hormone (GH) peak after GH release stimuli classifies patients as growth hormone- deficient (GHD) or non-GHD. This study compared a 2-year growth response to GH therapy in 318 prepubertal short children. Methods: Patients were classified as: severe GHD (GH peaks <5 ng/ml after 2 stimuli; n = 54), mild GHD (GH peaks <10 ng/ml, but one or two between 5 and 10 ng/ml; n = 140), dissociated GH release (GH peak ≧10 ng/ml after 1 stimulus and <10 ng/ml after the other; n = 89), and normal GH release (GH peaks ≧10 ng/ml after 2 stimuli; n = 35). Results: Two-year height gain did not differ statistically among the 4 groups: 1.39 ± 0.51 SD, 16.4 ± 2.3 cm; 1.23 ± 0.56 SD, 15.8 ± 2.1 cm; 1.18 ± 0.53 SD, 15.3 ± 2.0 cm, and 1.14 ± 0.53 SD, 15.4 ± 2.0 cm, respectively, as was also the case for bone age gain: 2.5 ± 0.6, 2.4 ± 0.7, 2.6 ± 0.7 and 2.3 ± 0.5 years, respectively. Conclusions: Our results suggest that GH release stimuli are of little help for deciding on GH therapy in the clinical management of prepubertal short children with idiopathic growth retardation, while well-defined anthropometric and biochemical criteria may be useful.
National Audit of Patient Choice in Pediatric GH Therapy
Authors: Langham Shirley , Kirk Jeremy ,   on behalf of the British Society for Paediatric Endocrinology and Diabetes
Published: November 2010

Background: To assess free patient choice for pediatric patients commencing growth hormone (GH) therapy within the UK and Republic of Ireland. Methods: A semistructured questionnaire was sent to all members of the British Society for Paediatric Endocrinology and Diabetes. Results: Of 55 units responding, three do not commence patients on GH. The remaining 52 units included 21 from (all) historic growth centres, 13 from other tertiary centres, and 18 from district general hospitals. 46/52 units (89%) offer free patient choice, involving: demonstration of devices (n = 15), instructional DVDs (6), a combination of both (20), or other (3). Median (range) time spent choosing the GH device was 60 (25–150) min. Device demonstration involved: dialling up doses (39 units), assembling/dissembling (38), GH reconstitution (33), considering facilities provided by manufacturer (25), injecting patient (19), injecting parent (17), showing additional material (11), and cost consideration (4). Median (range) number of steps shown per unit was 5 (1–8), with correlation between numbers of manufacturers/devices and new patients commenced on GH per unit (R = 0.62 and 0.61, both p < 0.01), but not with time spent showing devices (R = 0.12 and 0.22, p > 0.05). Conclusion: Most units now offer some form of patient choice for new patients commencing GH therapy, although this involves several different methods, with larger units offering more manufacturers and more devices.
Effect of Oxandrolone on Glucose Metabolism in Growth Hormone-Treated Girls with Turner Syndrome
Authors: Menke Leonie A. , Sas Theo C.J. , Stijnen Theo , Zandwijken Gladys R.J. , de Muinck Keizer-Schrama Sabine M.P.F. , Otten Barto J. , Wit Jan M.
Published: November 2010

Background: The weak androgen oxandrolone (Ox) may increase height but may also affect glucose metabolism in girls with Turner syndrome (TS). Methods: In a randomized, placebo-controlled, double-blind study, we assessed the effect of Ox at a dosage of either 0.06 or 0.03 mg/kg/day on glucose metabolism in 133 growth hormone (GH)-treated girls with TS. Patients were treated with GH (1.33 mg/m2/day) from baseline, combined with placebo (Pl) or Ox from the age of 8, and estrogens from the age of 12. Oral glucose tolerance tests (OGTT) were performed, and HbA1c levels were measured before, during, and after discontinuing Ox/Pl therapy. Results: Insulin sensitivity, assessed by the whole-body insulin sensitivity index (WBISI) decreased during GH+Ox/Pl (p = 0.003) without significant differences between the dosage groups. Values returned to pre-treatment levels after discontinuing GH+Ox/Pl. On GH+Ox, fasting glucose was less frequently impaired (Ox 0.03, p = 0.001; Ox 0.06, p = 0.02) and HbA1c levels decreased more (p = 0.03 and p = 0.001, respectively) than on GH+Pl. Conclusions: We conclude that in GH-treated girls with TS, Ox at a dosage of 0.03 or 0.06 mg/kg/day does not significantly affect insulin sensitivity. Insulin sensitivity decreases during GH therapy, to return to a pre-treatment level after discontinuing therapy.
Effects of Growth Hormone Therapy on Cardiac Dimensions in Children and Adolescents with Prader-Willi Syndrome
Authors: Hauffa Berthold P. , Knaup Kathrin , Lehmann Nils , Neudorf Ulrich , Nagel Bert
Published: November 2010

Objectives: Growth hormone (GH) secretory status and GH therapy influence cardiac muscle mass and function. Most patients with Prader-Willi syndrome (PWS) are GH-deficient (GHD), and are at risk to develop obesity-related cardiac problems. Few data exist on the development of cardiac dimensions under GH treatment in PWS. Study Design: In a retrospective study, cardiac dimensions measured echocardiographically in 26 children with PWS before and after 22.9 ± 11.0 months of GH therapy (0.035 mg/kg/day) were compared to those of 13 GHD children treated with GH (0.025–0.035 mg/kg/day) over 13.5 ± 4.2 months. Results: Low-normal left ventricular end-systolic [diastolic] diameter SDS increased in PWS patients (1.41, p < 0.0001 [1.23, p < 0.0001]) and in controls (0.61, p = 0.04 [0.78, p = 0.013]), becoming elevated above normal in 2/26 PWS patients. There was a trend for a higher GH effect on left ventricular end-systolic diameter in PWS (p = 0.071). Mean values of all echocardiographic dimensions remained in the normal range in both PWS patients and controls, before and under GH therapy. Conclusion: The response of cardiac dimensions to GH treatment is similar in PWS patients and GHD controls, with a trend towards more elevated left ventricular parameters in PWS. Therefore, regular echocardiographic assessment may be considered in PWS children with longer treatment duration.
The Role of Tuba1a in Adult Hippocampal Neurogenesis and the Formation of the Dentate Gyrus
Authors: Keays David A. , Cleak James , Huang Guo-Jen , Edwards Andrew , Braun Andreas , Treiber Christoph D. , Pidsley Ruth , Flint Jonathan
Published: November 2010

The multitubulin hypothesis holds that each tubulin isotype serves a unique role with respect to microtubule function. Here we investigate the role of the α-tubulin subunit Tuba1a in adult hippocampal neurogenesis and the formation of the dentate gyrus. Employing birth date labelling and immunohistological markers, we show that mice harbouring an S140G mutation in Tuba1a present with normal neurogenic potential, but that this neurogenesis is often ectopic. Morphological analysis of the dentate gyrus in adulthood revealed a disorganised subgranular zone and a dispersed granule cell layer. We have shown that these anatomical abnormalities are due to defective migration of prospero-homeobox-1-positive neurons and T-box-brain-2-positive progenitors during development. Such migratory defects may also be responsible for the cytoarchitectural defects observed in the dentate gyrus of patients with mutations in TUBA1A.
Effects on Growth and Metabolism of Growth Hormone Treatment for 3 Years in 36 Children with Prader-Willi Syndrome
Authors: Colmenares A. , Pinto G. , Taupin P. , Giuseppe A. , Odent T. , Trivin C. , Laborde K. , Souberbielle J.C. , Polak M.
Published: November 2010

Background/Aims: Prader-Willi syndrome (PWS) is a complex genetic disorder whose many manifestations include obesity and short stature. Diabetes, osteoporosis, and scoliosis are common. We evaluated the effects of human growth hormone (hGH). Methods: A prospective cohort study of 36 children (1–15 years of age) with genetically confir med PWS who were given hGH (mean dose 0.033 ± 0.006 mg/kg/day) for 36 months. At baseline and once yearly, we evaluated growth, insulin-like growth factor-1 (IGF-1), body composition, bone mineral density (BMD), glucose tolerance, serum lipids, and spinal radiographs. Results: Height gain over the 3-year period was 1.2 SD score. Lean body mass increased significantly during each treatment year. Total body fat decreased by 5.42 and 1.17% in the 1st and 2nd years, respectively. BMD remained unchanged during therapy. IGF-1 and homeostasis model assessment index of insulin resistance increased, and glucose intolerance was found in 22.7% of patients at baseline and 0% at 3 years. None of the patients had diabetes. Their lipid profile improved. Scoliosis was present in 27.8% of the patients at baseline and 47.2% at 3 years. Conclusion:  GH treatment in children with PWS has multiple beneficial effects on growth and body composition. Tolerance is good, with an improvement in glucose metabolism, although IGF-1 levels and insulin resistance parameters should be monitored closely. The high rate of scoliosis warrants monitoring by a pediatric orthopedic surgeon.
Three New SF-1 (NR5A1) Gene Mutations in Two Unrelated Families with Multiple Affected Members: Within-Family Variability in 46,XY Subjects and Low Ovarian Reserve in Fertile 46,XX Subjects
Authors: Warman Diana Monica , Costanzo Mariana , Marino Roxana , Berensztein Esperanza , Galeano Jesica , Ramirez Pablo C. , Saraco Nora , Baquedano Maria Sonia , Ciaccio Marta , Guercio Gabriela , Chaler Eduardo , Maceiras Mercedes , Lazzatti Juan Manuel , Bailez Marcela , Rivarola Marco A. , Belgorosky Alicia
Published: November 2010

Background: Three novel heterozygous SF-1 gene mutations affecting multiple members of two unrelated families with a history of 46,XY disorders of sex development (DSD) and 46,XX ovarian insufficiency are described. Methods: Clinical and mutational analysis of the SF-1 gene in 9 subjects of two families. Results: Family 1 had 2 affected 46,XY DSD subjects. One, born with severe perineal hypospadias, was raised as a male, and presented normal adolescence. The other, born with ambiguous genitalia, uterus, and mild testicular dysgenesis, was raised as a female. A W279X heterozygous mutation and an intronic deletion (g3314-3317delTCTC (IVS 4 + 8) was found in the SF-1 gene. In family 2, 4/6 affected siblings had 46,XY DSD or hypospadias. An affected 46,XX sister had normal sexual development but increased FSH levels. The 37-year-old affected mother had entered menopause. An Y183X heterozygous mutation was detected. Conclusion: An extreme within-family phenotypic variability, ranging from severe prenatal undervirilization to normal pubertal development, was observed in 46,XY-affected siblings, indicating that other unknown factors might be involved in the phenotype. Low ovarian reserve and preserved fertility in 46,XX subjects can be observed in heterozygous SF-1 gene mutations.
Changes in Bone Mineral Density and Body Composition in Children with Central Precocious Puberty and Early Puberty before and after One Year of Treatment with GnRH Agonist
Authors: Ko Jung Hee , Lee Hyo Sung , Lim Jung Sub , Kim Shin Mi , Hwang Jin Soon
Published: November 2010

Background/Aims: Much concern has been raised and debated on the effects of obesity and bone mineral density (BMD) after treatment with GnRH agonist. The aim of this study was to assess BMD and body composition, especially percent body fat (%FM) based on dual-energy X-ray absorptiometry (DEXA), before and after 1 year of treatment with GnRH agonist in Korean girls. Methods: We assessed BMD and body composition in 121 Korean girls with precocious puberty before and after 1 year of treatment with GnRH agonist. BMD and body composition values were measured by using DEXA. Results: The BMD standard deviation score of each region of interest for chronological age (CA) were higher than zero, but lower than zero for bone age (BA) at baseline and the gap was decreased after 1 year of GnRH agonist treatment. The fat mass (FM) and %FM for both CA and BA were significantly higher than zero at baseline. After GnRH agonist treatment, FM and %FM decreased for CA, but increased for BA. Conclusion: Pituitary-gonadal axis suppression by GnRH agonist does not reverse the progression of bone mass acquisition and does not increase the prevalence of obesity in Korean children with precocious puberty.
Molecular Defects of the CYP21A2 Gene in Greek-Cypriot Patients with Congenital Adrenal Hyperplasia
Authors: Skordis Nicos , Kyriakou Andreas , Tardy Véronique , Ioannou Yiannis S. , Varvaresou Athanasia , Dracopoulou-Vabouli Maria , Patsalis Philippos C. , Shammas Christos , Neocleous Vassos , Phylactou Leonidas A.
Published: November 2010

Background/Aim: To determine the mutations in the CYP21A2 gene in Greek-Cypriots with congenital adrenal hyperplasia (CAH) and attempt a genotype-phenotype correlation. Subjects and Methods: Molecular analysis was performed by multiplex ligation-dependent probe amplification and direct sequencing of PCR products of the CYP21A2 gene in 32 CAH patients. Results: The most frequent genetic defect in the classic salt-wasting and simple virilizing forms was the IVS2-13A/C>G (55%) mutation, followed by Large lesion (20%) and in the non-classical form, the p.V281L (79.5%). Genotypes were categorized in 4 mutation groups (null, A, B and C). All 3 patients in the null group manifested the salt-wasting form and all 6 patients in mutation group A presented with the classical form. One patient in group B had the simple virilizing form and 22 patients in group C exhibited the non-classical form. Conclusion: The spectrum of mutations of the CYP21A2 gene in our population is comparable to the most common reported in similar ethnic groups. The knowledge of the ethnic specificity of the CYP21A2 mutations represents a valuable diagnostic tool for all forms of CAH.
Prevalence of Hepatitis B among Pregnant Women and Its Impact on Pregnancy and Newborn Complications at a Tertiary Hospital in the Eastern Part of Germany
Authors: Lobstein Simon , Faber Renaldo , Tillmann Hans L.
Published: November 2010

Background and Aims: Prevalence data of hepatitis B virus (HBV) in pregnant women in the Eastern part of Germany are missing. More importantly, no data on HBV’s impact on pregnancy is known in patients living in the Western hemisphere. Our aim was to assess the prevalence of hepatitis B surface antigen (HBsAg) and its impact on pregnancy in women admitted to the department of gynecology at a German university hospital. Patients and Methods: 8,193 women who delivered at the university hospital between 2001 and 2006 were retrospectively screened for HBsAg and if positive investigated for pregnancy and newborn complications in comparison to women without hepatitis B infection. Results:39 (0.48%) women were found to be HBsAg carriers, which is in line with other data showing lower rates in Eastern parts of Germany in relation to the Western part. Furthermore, prevalence was much lower in German-born women (0.2%) in comparison to Asian-born women (9.1%). Secondly, women with HBsAg had no significantly increased prevalence of adverse pregnancy outcomes. Conclusion: There is a low prevalence of HBV infection, especially among German-born women. There is no significant evidence to support increased complications in relation to HBV infection in our study.
A Novel Y332C Missense Mutation in the Intracellular Domain of The Human Growth Hormone Receptor Does Not Alter STAT5b Signaling: Redundancy of GHR Intracellular Tyrosines Involved in STAT5b Signaling
Authors: Derr Michael A. , Fang Peng , Sinha Sunil K. , Ten Svetlana , Hwa Vivian , Rosenfeld Ron G.
Published: November 2010

Background: The growth hormone receptor (GHR), upon binding with GH, induces JAK2-mediated phosphorylation of GHR intracellular tyrosines, which then recruit STAT5b. Aberrancies in STAT5b signaling, due to mutations in GHR or STAT5b genes, result in poor responses to GH and severe short stature. Objective: To evaluate and compare the role of a novel Y332C GHR variant identified in a patient with short stature to the other GHR intracellular tyrosines in the GHR-STAT5b signaling process. Results: Recombinant human GHR constructs carrying Y332C or single Y to F changes for each of the 7 intracellular tyrosines did not alter GH-induced GHR-STAT5b signaling in reconstitution studies. However, GH-induced STAT5b activation was specifically abrogated in an hGHR variant in which all 7 tyrosines were inactivated (MYF). When hGHR variants carrying single intracellular tyrosines were evaluated, STAT5b activation was comparable to that of wild-type hGHR only with variants carrying Y534, Y566 or Y627. Conclusion: We provide evidence that in human GHR, 3 intracellular tyrosines are critical and redundant in the GH-induced STAT5b signaling process. This redundancy may explain why an Y332C variant did not alter STAT5b signaling. Identification of missense variants in human GHR intracellular domain should be interpreted with caution and rigorously analyzed.
Placental IGF-I, Estrogen Receptor, and Progesterone Receptor Expression, and Maternal Anthropometry in Growth-Restricted Pregnancies in the Swedish Population
Authors: Akram S.K. , Sahlin L. , Östlund E. , Hagenäs L. , Fried G. , Söder O.
Published: November 2010

Background/Aims: Fetal growth restriction is a complex problem of pregnancy arising from multiple etiologies. Key regulatory elements of growth are the insulin-like growth factor (IGF) axis, and estrogen and progesterone receptors. The aims were to determine the relations of expression of IGF-I, estrogen receptors α and β (ERα and ERβ, respectively), and progesterone receptor (PR), with maternal anthropometry, focusing on birth weight outcomes. Methods: Placental samples were obtained from 33 patients following delivery. mRNA expression was determined by a solution hybridization technique. Samples were divided into normal control (NC) and growth-restricted (GR) groups. Results: IGF-I expression was lower in the GR as compared to the NC group. PR levels correlated positively with IGF-I expression, infant anthropometry, and gestational age (GR). ERα correlated positively with PR expression (NC), and maternal BMI at delivery (GR). ERβ correlated positively with maternal delivery weight and gestational age (NC). Conclusion: The differences in placental expression of IGF-I emphasize its key role in birth weight outcomes. We further suggest the importance of PR expression in the pathogenesis of intrauterine growth restriction, as there were direct correlations of PR expression with both IGF-I expression and infant anthropometric parameters, as well as gestational age.
Gender Dichotomy in Long Term Growth Trajectories of Children with 21-Hydroxylase Deficiency Congenital Adrenal Hyperplasia
Authors: Patel Leena , Chandrashekhar Sudha Rao , Gemmell Isla , O’Shea Elaine , Jones Julie , Banerjee Indraneel , Amin Rakesh , Clayton Peter
Published: November 2010

Aim: To evaluate longitudinal growth in 21-hydroxylase deficiency congenital adrenal hyperplasia (CAH), factors contributing to this and outcome for BMI, weight (Wt) and height (Ht) in adolescence. Methods: Multi-level longitudinal models were used to evaluate growth patterns of 45 salt wasters (SW) and 12 non-SW with CAH. Results: Ht, Wt and BMI growth curves differed between SW and non-SW, and by gender. In contrast to SW and males, non-SW females showed a markedly different pattern with a progressive increase in Wt and BMI SDS over childhood and adolescence and only a slight gain in Ht SDS. BMI SDS remained above 0 after early childhood. Over the 15 years, the growth variables were negatively associated with fludrocortisone (FC) (shorter children were receiving larger doses) but not hydrocortisone (HC) doses nor FC and HC doses in the first year. Conclusion: The growth patterns in these children with CAH were influenced by age, gender, phenotype and FC treatment. There was a trend to increasing BMI from an early age, greater adiposity during childhood and females had disproportionately greater adiposity but shorter stature during adolescence. These patients therefore have a predisposition to obesity in childhood and later life independent of early corticosteroid treatment.
Gonadotropin-Dependent Precocious Puberty in a Patient with X-Linked Adrenal Hypoplasia Congenita Caused by a Novel DAX-1 Mutation
Authors: Darcan Sukran , Goksen Damla , Ozen Samim , Ozkinay Ferda , Durmaz Burak , Lalli Enzo
Published: November 2010

Background/Aims: X-linked adrenal hypoplasia congenita (AHC) is typically characterized by a DAX-1 gene mutation and hypogonadotropic hypogonadism. However, rare cases with precocious puberty or normal puberty have been reported. Currently, the mechanism of action of the DAX-1 gene on puberty is not clearly known. Case Report: We report a male who was diagnosed as having AHC in the newborn period and detected as having stop codon Q155 X mutation in the DAX-1 gene. This subject developed central precocious puberty when he was 9 months old. Results: This paper is the first case report of AHC, central precocious puberty and a mutation in the DAX-1 gene. DAX-1 gene mutations can result in various phenotypes. Conclusion: In cases with AHC, central precocious puberty can develop rather than hypogonadotropic hypogonadism, which is the most frequently observed puberty disorder related to DAX-1 gene mutations.
Tumour Necrosis Factor-α Expression Is Associated with Increased Severity of Periprosthetic Breast Capsular Contracture
Authors: Tan K.T. , Wijeratne D. , Shih B. , Baildam A.D. , Bayat A.
Published: November 2010

Background: The pathogenesis of periprosthetic capsular contracture following breast implant surgery is unclear. The aim of this study was to identify the expression of tumour necrosis factor-α (TNF-α), collagen type III α1 (COL3A1), transforming growth factor-β1 (TGF-β1) and connective tissue growth factor (CTGF) in different Baker grades of breast capsular contracture. Methods: Seven periprosthetic breast capsule specimens were collected from 6 patients. TNF-α, COL3A1, TGF-β1 and CTGF gene expression were analysed using real-time quantitative polymerase chain reaction. Immunohistolocalisation of TNF-α was performed on paraffin-embedded sections. Significant correlations were analysed using the Pearson correlation coefficient. Results: TNF-α expression was associated with increased Baker grade of capsular contracture (Pearson correlation, r = 0.558; p = 0.02). COL3A1 gene expression was reduced with increasing severity of contracture (r = –0.490; p = 0.05). There were no significant correlations between TGF-β1 and CTGF expression with Baker grade. Positive TNF-α staining in breast capsules was localised to fibroblasts, macrophages, and extracellularly close to the prosthesis. Conclusion: The increased expression of TNF-α may play a key role in the inflammatory response associated with capsular contracture. The corresponding decrease in COL3A1 may contribute to the change in capsular physical properties seen in capsular contracture.
Against the Existence of Pied-Piping: Evidence from Tlingit
Authors: Cable Seth
Published: November 2010

I argue that pied-piping, as traditionally understood, might not exist. I reanalyze classic examples from English and other well-studied languages in light of new data from Tlingit, an understudied and endangered language of Alaska. I argue that the initial appearance of piedpiping in Tlingit is misleading and actually reflects structures where no true pied-piping occurs. I then show that a similar analysis is possible for putative cases of pied-piping in other, well-known languages. Consequently, both the phenomenon of pied-piping and the grammatical mechanisms introduced to derive it might be eliminable from the theory of grammar.
The Movement Derivation of Conditional Clauses
Authors: Haegeman Liliane
Published: November 2010

By analogy with the movement analysis of temporal clauses, some authors have proposed that conditional clauses be derived by leftward operator movement (Bhatt and Pancheva 2002, 2006, Arsenijević 2009, Tomaszewicz 2009). This movement analysis of conditional clauses is shown to account for the incompatibility of main clause phenomena and conditional clauses in terms of intervention effects. The cartographic implementation of this analysis predicts that conditional clauses will be incompatible with speaker-oriented modal expressions and that conditional clauses will lack the low-construal reading found in temporal clauses (Bhatt and Pancheva 2002, 2006). Thus, the absence of low construal in conditional clauses, which was initially taken to be an obstacle for the movement account of conditional clauses (see Citko 2000), becomes an argument in its favor.
Learning Long-Distance Phonotactics
Authors: Heinz Jeffrey
Published: November 2010

This article shows that specific properties of long-distance phonotactic patterns derived from consonantal harmony patterns (Hansson 2001, Rose and Walker 2004) follow from a learner that generalizes only on the basis of the order of sounds, not the distance between them. The proposed learner is simple, efficient, and provably correct, and does not require an a priori notion of tier or projection (contra the model in Hayes and Wilson 2008); nor does it rely on the additional structure provided by Optimality Theory grammars (Prince and Smolensky 1993, 2004) or grammars in the principles-and-parameters framework (Chomsky 1981, Dresher and Kaye 1990, Gibson and Wexler 1994). Not only does the noncounting nature of nonlocal dependencies automatically follow from the way the learner generalizes, it also explains the absence of blocking patterns from the typology. Finally, the learner lends support to the idea that long-distance phonotactic patterns are phenomenologically distinct from spreading patterns, contra the hypothesis of Strict Locality (Gafos 1999, et seq.).
Psycholinguistic Evidence for the VP-Internal Subject Position in Japanese
Authors: Koizumi Masatoshi , Tamaoka Katsuo
Published: November 2010

The question of whether the subject stays in its thematic position within the VP or moves to Spec, TP is difficult to answer with respect to free word order languages such as Japanese because the surface constituent orders in these languages do not necessarily provide sufficient information to determine syntactic positions. In this article, we present psycholinguistic evidence for the theoretical hypothesis that, in Japanese, the subject must move to Spec, TP in sentences with the subject-objectverb word order, but may stay within the VP in sentences with the object-subject-verb word order.
English-speaking Units of the International Brigades: War, Politics and Discipline
Authors: Stradling Rob
Published: November 2010

The moral and military aspects of the International Brigades’ record in Spain (1936—8) remain subjects of scholarly investigation and wider debate. Popular fascination was exemplified by the selection of Hemingway’s For Whom the Bell Tolls as a favourite novel by both main candidates during the US presidential elections of 2008. Postwar Francoist propaganda vilified Brigaders as criminals and cowards, but a counter-legend of the left venerated them as idealists and heroes. Whilst the one campaign has long since passed into oblivion, the other still marches on in the ceaseless commemorative work of veterans’ associations. Although few now question the ubiquitous role of the Comintern in the Brigades’ foundation, organization and command, the practical effects of this control on the environmental conditions and daily lives of the ‘volunteers for liberty’ — despite a plethora of published testimony by those involved — has tended to be overlooked. This article attempts to survey and assess the relevant experiences of the Brigades’ main English-speaking units, based on a wide range of archival and other sources.
Hungarian Aristocracy and the White Terror
Authors: Bodo Bela
Published: November 2010

This article looks at the response of the aristocracy to militia and mob violence in Hungary between 1919 and 1922. It argues that the experience with radical paramilitary groups in the era after the first world war colored the political behavior of the aristocracy and its political representatives, especially in their attitude to fascism, in the 1930s and 1940s.
Treacherous Allies: The Cold War in East Asia and American Postwar Anxiety
Authors: Kushner Barak
Published: November 2010

The postwar US federal government spent a decade, initiated two federal treason trials, and dispensed over one million dollars in legal pursuit of John Provoo for his crimes in the Philippines as a POW and as a propaganda agent for the Japanese. In a strange perversion of justice the US attorney even invited to the federal court Provoo’s former captors — Japanese propagandists, military officials and POW guards — to establish his guilt. Why was the US government so keen to pursue and charge a lone second world war POW with treason? What dread did Provoo strike in the hearts of US officials? This article examines how Provoo embodied wartime and postwar American panic concerning East Asia, just as the situation in the region dramatically spiraled out of American control.
‘Ish bin ein Bearleener’ - JFK’s 26 June 1963 Visit to Berlin: The Views from East Germany
Authors: Waite Robert G.
Published: November 2010

US President John Kennedy spent a single day in Berlin, where he gave one of his most famous speeches. The visit was intended to signalize America’s strong and undivided support of the divided city. Well before the formal announcement of the visit, East Germany agencies knew of the planned trip. East German records show the extent of the concern and the measures to counter the President’s visit.
Dunkirk and the Popular Memory of Britain at War, 1940—58
Authors: Summerfield Penny
Published: November 2010

The evacuation of the British Army from Dunkirk in 1940 has an iconic place in British culture. This article draws on a concept of popular memory that suggests that rival versions of the past compete for cultural centrality, to ask how Dunkirk acquired this position. During the war, accounts stressed the importance of the sea in the ‘deliverance’, but while some focused on the Navy, others concentrated on the civilian small boats, and criticism was rare. Immediately postwar there was a lull in representations. In the 1950s, attention switched to the land and to the problematic place of the defeated Army in the story, culminating in Ealing Studios’ film Dunkirk (1958). Ealing attempted to synthesize previous emphases and struggled to achieve agreement about the representation of the evacuation. The film ensured the public prominence of the memory of Dunkirk, yet its reception was fractured along class and gender lines, indicating the instabilities of Ealing’s negotiated consensus. The history of the contested inscription of Dunkirk in British culture emphasizes that at no point since the events occurred has the representation of the second world war been secure; the popular memory of the war is continually subject to construction, contestation and revision.
Introduction to the themed issue. Corporate power: Agency, communication, influence and social policy
Authors: Miller David , Mooney Gerry
Published: November 2010

This paper introduces this themed issue of Critical Social Policy on the question of corporate power. Corporate power is recognized as an important agent in social policy making and delivery. However, to date there has been comparatively little attention to the crucial role that lobbying and corporate ‘spin’ play in helping to shape policy making contexts. This special issue of Critical Social Policy is concerned to bring such issues to the mainstream of social policy analysis. It is argued here that the rise of spin and public relations is a key feature of neoliberalism in the past two decades. These have worked to reshape policy making, resulting in pronounced changes in the content and process of policy making and it is argued that these have tended to marginalize or undermine democratic processes.
Corporate and political strategy in relation to the Private Finance Initiative in the UK
Authors: Ruane Sally
Published: November 2010

The Private Finance Initiative (PFI) allows corporations to access guaranteed flows of public funds in exchange for the use of new facilities and the provision of asset-based services such as maintenance and estates management. The policy has been developed in the UK since 1992 and has been applied in fields of social policy analysis: notably, health and education. PFI has been a difficult policy to make work and since its introduction, governments and businesses have sought to develop strategies to shape both its evolution and its implementation. These strategies have aimed to overcome significant institutional, technical, ideological and practical barriers. Through business—government collaboration in relation to the restructuring of the institutions of the state, the production of a pro-PFI rationale and mechanisms for the spread of technical know-how, businesses have been brought to centre stage in policy making and policy delivery.
Forging a ‘neoliberal pedagogy’: The ‘enterprising education’ agenda in schools
Authors: Mccafferty Patricia
Published: November 2010

New Labour came to power with a stated commitment to ‘education, education, education’ and confirmed quickly that this commitment included a greater role for business in the modernization of state schools. One important, yet under-researched, element of direct business involvement is in school pupils’ personal and academic development evident in the increasingly pervasive embedding of rhetoric and practices of ‘enterprising education’. This paper argues that this is an aspect of a ‘neoliberal pedagogy’. It explores the uncritical promotion of values of enterprise and entrepreneurship through approaches that lead to greater ‘frontline’ business involvement in schools, helping to normalize free market values and ‘neoliberal commonplaces’. These promote a particular perspective on the relationships between education, the labour market, the economy and social justice characteristic of New Labour/Third Way approaches not incompatible with that of the Conservative—Liberal Democrat coalition, nor the current rhetoric and policies of the SNP administration in Scotland. Thus, despite elements of residual national distinctiveness, ‘enterprising education’ and the promotion of business values ‘on the ground’ is a problematic, yet increasingly core function of state education in developing the ideological apparatus of neoliberalism across the UK.
Business-managed democracy: The trade agenda
Authors: Beder Sharon
Published: November 2010

The architecture of global governance that has emerged in the past two decades has been strongly influenced by transnational policy actors. This article examines the role of transnational corporate agency in social policy by focusing in particular on the role of business coalitions, elite networking bodies and policy planning groups in fostering unity amongst corporate actors and enrolling political actors into managing democracies in the interests of business. The example of the General Agreement on Tariffs and Trade (GATT) and the World Trade Organization (WTO) is used to examine how corporate agency is wielded through elite networking organizations and how this is eroding national social policy.
Capitalist globalization, corporate social responsibility and social policy
Authors: Sklair Leslie , Miller David
Published: November 2010

This article outlines how the twin crises of capitalist globalization — of class polarization and ecological unsustainability — combine to produce the need for Corporate Social Responsibility (CSR) to attempt to bridge the gap between the rhetoric and reality of corporate conduct. The first section outlines how CSR relates to capitalist globalization and how it is integrated into the activities of the Transnational Capitalist Class (TCC). The role of CSR in relation to social policy is examined next leading on to an account of the uses to which CSR is put in policy discourse, particularly its strategic use in lobbying and the advance of corporate power.
Corporate strategy, corporate capture: Food and alcohol industry lobbying and public health
Authors: Miller David , Harkins Claire
Published: November 2010

This article examines two industry sectors — those making and selling fast food and alcoholic beverages or associated products. We examine their role in influencing policy and decision making on the regulation of their products for health reasons. We argue that the food and alcohol industries engage in a very wide range of tactics and strategies to defend and indeed to promote their ‘licence to operate’. We focus in on a specific component of these by examining public relations and lobbying strategies and their impacts on elite decision makers. We suggest that lobbying influence is a matter of both communication and action. We go on to outline the vertical and horizontal differentiation of lobbying strategies arguing that policy capture is the ultimate goal of lobbying, though influence is pursued by wide-ranging strategies to capture various arenas of decision making. We examine four key arenas; science, civil society, the media and policy, closing with an examination of two cases of the so-called ‘partnership’ model of governance.