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Background During perimenopause, the rise in serum follicle-stimulating hormone (FSH) is associated with increased adiposity, insulin resistance (IR), and metabolic syndrome (MetS). However, data for the pubertal period, which is characterized by increasing FSH levels and changing body composition, are limited. Objectives To investigate the relationships between FSH and anthropometric changes, IR markers, and development of MetS in the peripubertal period. Methods Uppsala Longitudinal Study of Childhood Obesity (ULSCO) is an ongoing study that aims to understand the factors contributing to childhood obesity and the development of obesity-related diseases. We analysed the subset of participants who were prepubertal at the first visit (n = 95, 77 with obesity). Mean follow-up time was 3.0 +/- 1.4 years. Results Higher serum FSH levels at the first visit were associated with an increased likelihood of elevation in body mass index (BMI SDS) (p = 0.025, OR = 16.10) and having MetS (p = 0.044, OR = 4.67) at the follow-up. We observed nonlinear relationships between varying serum FSH levels and markers of adiposity and IR, especially in girls. At the first visit, when girls were prepubertal, FSH was negatively associated with BMI (beta = -0.491, p = 0.005) and positively associated with sex hormone-binding globulin (SHBG) (beta = 0.625, p = 0.002). With the progression of puberty, negative associations between BMI and SHBG disappeared while FSH became positively associated with HOMA-IR (beta = 0.678, p = 0.025) and fasting insulin (beta = 0.668, p = 0.027). Conclusions Higher serum FSH levels in prepubertal children were associated with an increased risk of MetS development during pubertal transition. Along with nonlinear associations between varying serum FSH levels and IR markers, our results might imply a relationship between FSH and IR of puberty.

Additional file 1. Supplementary information about diagnostics and measured parameters in the study.

The study focuses on examining the eventual effects of corporate culture on customer satisfaction. The culture at Handelsbanken AB was examined due to its historical background of high customer satisfaction in contrast to the other large banks in Sweden. The study was made by finding previous theories discussing the relationship between organizational culture and customer satisfaction. Through these previous theories, three significant corporate culture traits were found that has shown positive correlation with customer satisfaction. These three variables will serve as guidelines when examining the culture of Handelsbanken through interviewing people associated to the organisation. The findings of the study show that due to Handelsbankens very decentralized culture and local presence, they have managed to attain high customer satisfaction through good customer service and having a culture that engages change.

Myxedema is extremely rare in children, and guidelines are lacking. We treated a 12-year-old girl with myxedema and cardiogenic shock with initial low dose (0.3-2.5 μg/kg body weight/day) of oral levothyroxine and intensive care. Oral administration may safely revert children's myxedema in a dosage resembling that for hypothyroidism.

Context: Recombinant human GH (rhGH) therapy in Prader-Willi syndrome (PWS) has been used by the medical community and advocated by parental support groups since its approval in the United States in 2000 and in Europe in 2001. Its use in PWS represents a unique therapeutic challenge that includes treating individuals with cognitive disability, varied therapeutic goals that are not focused exclusively on increased height, and concerns about potential life-threatening adverse events. Objective: The aim of the study was to formulate recommendations for the use of rhGH in children and adult patients with PWS. Evidence: We performed a systematic review of the clinical evidence in the pediatric population, including randomized controlled trials, comparative observational studies, and long-term studies (>3.5 y). Adult studies included randomized controlled trials of rhGH treatment for ≥6 months and uncontrolled trials. Safety data were obtained from case reports, clinical trials, and pharmaceutical registries. Methodology: Forty-three international experts and stakeholders followed clinical practice guideline development recommendations outlined by the AGREE Collaboration (www.agreetrust.org). Evidence was synthesized and graded using a comprehensive multicriteria methodology (EVIDEM) (http://bit.ly.PWGHIN). Conclusions: Following a multidisciplinary evaluation, preferably by experts, rhGH treatment should be considered for patients with genetically confirmed PWS in conjunction with dietary, environmental, and lifestyle interventions. Cognitive impairment should not be a barrier to treatment, and informed consent/assent should include benefit/risk information. Exclusion criteria should include severe obesity, uncontrolled diabetes mellitus, untreated severe obstructive sleep apnea, active cancer, or psychosis. Clinical outcome priorities should vary depending upon age and the presence of physical, mental, and social disability, and treatment should be continued for as long as demonstrated benefits outweigh the risks. Copyright

A method for experimental study in vitro of the outlet region (bladder base, bladder neck and proximal urethra) from the cat is described which allows simultaneous recording of resistance to flow through the region and of isometric tension in the longitudinal muscles. The results suggest that acetylcholine (ACh) may influence the smooth muscles in the outlet region by several mechanisms. Contractions in the longitudinal muscles are mediated by atropine sensitive cholinergic receptors. The resistance to flow could be either increased or decreased following ACh stimulation and in neither case could the response be blocked with atropine. If the response was an increase, however, it was blocked by phentolamine and if it was a decrease, it was blocked by propranolol. It is concluded that ACh stimulates the circular musculature in the outflow region through α- and β-adrenergic receptors. It is suggested that the effects of ACh on flow are brought about through short intramural adrenergic neurons. ACh stimulates their ganglion-cells which results in a release of nor-adrenaline at the nerve endings and in activation of adrenergic receptors in the circular muscle layer.

UMMARY A group of 27 infants with myelomeningoceles was investigated in the newborn period by neurologic examination and by micturating cystourethrography. An abnormal configuration of the bladder, trabeculation of the bladder wall, narrowing of the urethra at the external sphincter and alteration in the shape of the bladder neck were common findings. The incidences of trabeculation and a wide bladder neck were significantly related to neurologic lesions at a low thoracic and high lumbar level. Possible pathogenetic mechanisms presented in the literature are reviewed and the hypothesis is advanced that the alterations ate related to interruption of sympathetic innervation of the bladder. RESUME Les voies urinaires inferieures chez le nouveau-ne avec myelomeningocele 27 nourrissons porteurs de myelomeningocele ont ete examines durant la periode neonatale du point de vue neurologique et par cisto-urethrographie avec mixion. Une configuration anormale de la vessie, trabeculation de la paroi vesicale, un retrecissement de l'urethre an niveau du sphincter externe et une alteration de la forme du col vesical ont ete communement trouves. La survenue d'une trabeculation ou d'un col vesical elargi ont ete relies significativement a des lesions neurologiques au niveau thoracique bas ou lombaire haut. Les mecanismes pathogeniques possibles trouves dans la litterature sont revus et une hypothese est formulee, que les alterations observees sont reliees a l'interruption de l'innervation sympathique de la vessie. ZUSAMMENFASSUNG Der untere Harntrakt bei Neugeborenen mit Myelomeningocele Eine Gruppe von 27 Kindern mit Myelomeningocele wurde in der Neugeborenen-Phase neurologisch und cysto-urethrographisch untersucht. Haufige Befunde waren eine abnorme Form der Harnblase, Trabekel in der Blasenwand, eine Verengung der Urethra am auseren Sphinkter und eine veranderte Form im Bereich des Blasenhalses. Die Haufigkeit von Trabekeln und eines weiten Blasenhalses war signifikant bezogen auf neurologische Schaden im unteren Thorax- und oberen Lumbarbereich. Die in der Literatur beschriebenen moglichen pathogenetischen Faktoren werden diskutiert und die Hypothese gestellt, das die Veranderungen auf eine Unterbrechung der sympathischen Blaseninnervation zuruckgehen. RESUMEN Las vias urinarias bajas en recien nacidos con mielomeningocele Un grupo de 27 lactantes con mielomeningocele fue investigado en el periodo de recien nacido con examen neurologico y cisto-uretrografia miccional. Fueron hallazgos comunes una configuracion anormal de la vejiga, trabeculacion de la pared vesical, estrechamiento de la uretra a nivel del esfinter externo y alteration de la forma del cuello vesical. La incidencia de la trabeculacion y del cuello vesical ancho estaba significativamente relacionada con las lesiones neurologicas a nivel toracico bajo y lumbar alto. Se revisan los posibles mecanismos patogenicos citados en la literatura y se adelanta la hipotesis de que las alteraciones estan en relation con una interruption de la inervacion simpatica de la vejiga.

Inherited metabolic diseases usually present in the neonatal period or before school age. A growing portion of the disorders can be treated successfully, and an increasing number of patients are now treated in adult medicine. Several of the disorders also exist as attenuated variants without distinct symptoms in childhood. They can present as an acute onset event during metabolic stress in adulthood. We describe three patients with acute clinical decompensation in adulthood with severe sequelae and propose investigations to help diagnose such patients. One patient was unconscious from ammonia intoxication and developed severe neurological sequelae because of a defect in the urea cycle. A second patient had seizures caused by a defect in the conversion of homocysteine to methionine, resulting in lack of S-adenosylmethionine. A third patient had a stroke and grossly elevated homocysteine caused by undiagnosed homocystinuria. Recently there have been dramatic improvements in diagnostic methods, and new therapies are continuously being developed. Knowledge of these disorders is therefore of increasing importance also in adult medicine.

Nonlinear regression (± SEM) of acylated (AG) (A), unacylated (UAG) ghrelin (B) levels and AG/UAG ratio (C) according to age in both groups. Black line: control; Red line: PWS infants. For Supplementary Figure1, we used nonlinear regressions by B-splines to draw the curves. Because the curves are compatible with linear regressions, we did not use nonlinear regressions for the statistical analysis. (DOCX 86 kb)

The hypothesis that treatment with neuropepticle Y (NPY) can increase running activity and decrease food intake and body weight was tested. Female rats with a running wheel lost more weight than sedentary rats and ran progressively more as the availability of food was gradually reduced. When food was available for only 1 h/day, the rats lost control over body weight. Correlatively, the level of NPY mRNA was increased in the hypothalamic arcuate nucleus. This phenomenon, activity-based-anorexia, was enhanced by intracer-ebroventricular infusion of NPY in rats which had food available during 2h/day. By contrast, NPY stimulated food intake but not wheel running in rats which had food available continuously. These findings are inconsistent with the prevailing theory of the role of the hypothalamus in the regulation of body weight according to which food intake is a homeostatic process controlled by "orexigenic" and "anorexigenic" neural networks. However, the finding that treatment with NPY, generally considered an "orexigen", can increase physical activity and decrease food intake and cause a loss of body weight is in line with the clinical observation that patients with anorexia nervosa are physically hyperactive and eat only little food despite having depleted body fat and up-regutated hypothalamic "orexigenic" peptides. (C) 2007 Elsevier Ltd. All rights reserved.

Summary: Purpose: To evaluate the effect of low-dose clonazepam (CZP) on the amount of epileptiform activity in children with focal and generalized epilepsy. Methods: In a single-blind pilot study, followed by a double-blind, placebo-controlled, randomized, crossover study, 15 children with epilepsy were evaluated by using 24-h long-term EEG recordings during baseline days and days after injections of placebo and CZP. The drug was given as a single i.m. injection of 0.02 mg/kg BW. Blood samples were obtained regularly for analysis of plasma concentrations of CZP. The number of epileptiform discharges was determined during corresponding periods with the individual child in the same state of alertness, the same real time of day, and with concomitant antiepileptic drugs (AEDs) unchanged. Results: In the double-blind study, low-dose CZP produced a highly significant (p = 0.0015) decrease in the amount of epileptiform activity (mean, -69% vs. placebo, -2%) obtained during periods when median plasma concentrations ranged from 18 to

Objective : To evaluate the impact of the time factor on the amount of epileptiform activity in long-term EEG recordings in children with epilepsy. Methods : Ten children with epilepsy of different types underwent three 24h EEG examinations during two consecutive days and with a month's interval. The number of epileptiform discharges during selected corresponding periods of time was counted. Results : The number of epileptiform discharges on three repeated examination days showed no significant difference (ANOVA P=0.88) as intraindividual increases and decreases on different days counterbalanced each other within the group. However the standard deviations of the relative changes were larger between recordings with a month's interval compared to those for consecutive days (86% and 33%). The mean magnitude of change was 55% between days separated by a month compared to 24% on consecutive days. The difference was non-significant but showed a trend towards larger changes with a longer interval (P=0.07). Conclusions : The variability of epileptiform activity was larger when the interval between recordings was 1 month compared to consecutive days. The magnitude of the relative changes between intervals of 1 and 30 days showed a trend towards a difference although not statistically significant. When evaluating repeated long-term EEGs in relation to therapy in children, these variations should be considered.

SUMMARY In 39 infants and children with myelomeningocele, no lower incidence of urinary tract infection was obtained by a trial of prophylaxis treatment with nitrofurantoin. Instead, nitrofurantoin-resistant strains appeared in some children, with the accompanying higher risk of re-infection. RESUME Chez 39 enfants atteints de spina bifida, il n'a pas ete observe de baisse de frequence des infections du tractus urinaire par un essai de traitement prophylactique avec le nitrofurantoine. En revanche, des colonies resistantes a la nitrofurantoine sont apparues chez quelques enfants avec le haut risque de reinfection que cela presente. ZUSAMMENFASSUNG Bei 39 Kindern mit Spina bifida wurde die Zahl der Harnwegsinfekte durch eine Prophylaxe mit Nitrofurantoin nicht herabgesetzt. Im Gegenteil, bei einigen Kindern traten Nitrofurantoin-resistente Stamme auf, durch die ein viel hoheres Risiko einer Reinfektion gegeben war. RESUMEN En 39 ninos con espina bifida no se obtuvo una incidencia menor de infeccion de vias urinarias con el ensayo de un tratamiento profilactico con nitrofurantoina. En cambio, en algunos ninos aparecieron capas resistentes a la nitrofurantoina, con el mayor riesgo acompanante de una reinfection.

Summary: Purpose: We report a double-blind, placebo-controlled crossover study of lamotrigine (LTG) as add-on treatment in therapy-resistant, generalized epilepsy in children and adolescents (n = 30). Methods: Twenty patients had Lennox-Gastaut syndrome. Each patient acted as his or her own control. LTG and placebo were randomly added to existing antiepileptic medication (AEDs). The LTG dosage was individualized in an open phase preceding the placebo/treatment phase. Patients who responded to LTG in the open phase went on to the double-blind phase. “Responders” were defined as patients with a >50% seizure reduction or less severe seizures or both, or improved behavior or improved motor skills or both. “Nonresponders” were defined as children who showed no positive effects of LTG with plasma levels of 10 μg/ml or children who had adverse events during the open phase. Results: There was a clear statistically significant reduction of seizure frequency in LTG compared with placebo treatment. None of the children studied showed abnormal biochemical or hematologic findings, or changes in plasma levels of concomitantly administered AEDs. Conclusions: LTG is a well-tolerated and effective treatment in children with intractable generalized epilepsies, including those with Lennox-Gastaut syndrome. The study design allowed a double-blind placebo-controlled assessment of LTG although the participating children used 19 different AED combinations at entry.