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Unlabelled: Constipation, faecal incontinence, soiling and difficult toilet training remain significant problems in children with Hirschsprung's disease after corrective surgery. Chronic defecation problems can have various negative implications. At the University Medical Centre Nijmegen, a multidisciplinary behavioural treatment was developed to treat defecation problems. In this paper, a prospective controlled study is presented concerning the effect of this treatment upon children suffering from chronic defecation problems following corrective surgery for Hirschsprung's disease. The effect of treatment was studied in 27 children (21M, 6F, mean age 5.2y, range 2-11 y). Fourteen children were allocated to the experimental treatment group. The 13 children allocated to the waiting-list control group were also treated following a waiting period of 6 mo. On all outcome variables, children in the experimental treatment group had significantly better results after treatment than children in the waiting-list control group after the waiting period. No effect of age upon treatment was found. The effect of treatment remained significant on all outcome variables at a mean follow-up of 7 mo after the end of treatment.Conclusion: Multidisciplinary behavioural treatment is successful in decreasing chronic defecation problems in children with Hirschsprung's disease. [ABSTRACT FROM AUTHOR]

Aim: Fatty acid beta-oxidation defects comprise a heterogeneous group of disorders that may precipitate acute life threatening metabolic crises particularly during catabolic episodes. Several studies have demonstrated a possible association between fatty acid beta-oxidation defects, including long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency and severe pregnancy complications. However, the precise percentage of women with haemolysis, elevated liver enzymes, low platelets (HELLP) syndrome associated with foetal fatty acid beta-oxidation defects is not known.Methods: We carried out a multicentre retrospective study on 88 infants, born to women with HELLP syndrome. Acylcarnitine profiles from blood dried on filter paper cards were analysed by tandem mass spectrometry for the diagnosis of fatty acid beta-oxidation defects. In addition, we screened for the common long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency mutation using a standard restriction fragment length polymorphism polymerase chain reaction method.Results: None of the infants studied carried the common long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency mutation. There was no evidence of fatty acid beta-oxidation defects, including long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency, as expected by unremarkable acylcarnitine profiles, while three infants with fatty acid beta-oxidation defects were diagnosed in the control group.Conclusions: Neither foetal long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency, including heterozygosity for the common long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency mutation, nor fatty acid beta-oxidation defects in general are a major risk factor for HELLP syndrome in Austria. [ABSTRACT FROM AUTHOR]

Aim: To assess the prevalence of neurodevelopmental problems in adolescents with severe obesity and their associations with binge eating and depression.Methods: Data were collected at inclusion in a randomised study of bariatric surgery in 48 adolescents (73% girls; mean age 15.7 ± 1.0 years; mean body mass index 42.6 ± 5.2 kg/m2 ). Parents completed questionnaires assessing their adolescents' symptoms of attention-deficit/hyperactivity disorder and autism spectrum disorder and reported earlier diagnoses. Patients answered self-report questionnaires on binge eating and depressive symptoms.Results: The parents of 26/48 adolescents (54%) reported scores above cut-off for symptoms of the targeted disorders in their adolescents, but only 15% reported a diagnosis, 32% of adolescents reported binge eating, and 20% reported symptoms of clinical depression. No significant associations were found between neurodevelopmental problems and binge eating or depressive symptoms. Only a third of the adolescents reported no problems in either area.Conclusion: Two thirds of adolescents seeking surgical weight loss presented with substantial mental health problems (reported by themselves or their parents). This illustrates the importance of a multi-professional approach and the need to screen for and treat mental health disorders in adolescents with obesity. [ABSTRACT FROM AUTHOR]

Aim: Troponin is a sensitive marker of asphyxia in term infants mirroring the myocardial injury sustained in global hypoxia-ischaemia. In addition, troponin is a sensitive marker of severity of stroke in adults and neonatal encephalopathy (NE). We aimed to examine the relationship between troponin T in infants with perinatal asphyxia and brain injury on MRI and correlate with neurodevelopmental outcome.Methods: Serum troponin was sampled in infants requiring resuscitation at birth and/or neonatal encephalopathy in a tertiary referral neonatal centre. Birth history, clinical parameters, neuroimaging and developmental outcome (Bayley Scores of Infant Development [BSID] III) were evaluated.Results: Infants with perinatal asphyxia (n = 54) had serum troponin T measured and 27 required therapeutic hypothermia. Troponin T levels on days 1 and 2 were predictive of need for TH, development of seizures and grade II/III NE (AUC = 0.7; P-values < .001), troponin T levels on days 1, 2 and 3 were highly significant predictors of mortality (AUC = 0.99, P-values .005). The cut-off values of troponin T for best prediction of mortality were 0.84, 0.63 and 0.58 ng/mL on days 1, 2 and 3, respectively. Troponin T on day 3 of life was predictive of injury in the combined area of basal ganglia/watershed on MRI (AUC 0.70; P-value = .045).Conclusion: Infants with brain injury on neuroimaging following perinatal asphyxia had significantly elevated serum troponin, and troponin also correlated with developmental scores at 2 years. Further studies combining troponin and MRI may assist in the classification of neonatal brain injury to define aetiology, prognosis and response to treatment. [ABSTRACT FROM AUTHOR]

Aim: We investigated the association between active hypothermia and hypocapnia in neonates with moderate-to-severe hypoxic-ischaemic encephalopathy (HIE) transported after birth.Methods: This was a retrospective cohort study of neonates with HIE born between 2007 and 2011 and transported to Semmelweis University, Hungary, for hypothermia treatment before and after we introduced active cooling during transport in 2009. Of these, 71 received intensive care plus controlled active hypothermia during transport, while the 46 controls just received standard intensive care. Incident hypocapnia was defined as a partial pressure of carbon-dioxide (pCO2 ) that decreased below 35 mm Hg during transport. Multivariable logistic regression investigated the relationship between hypothermia and incident hypocapnia.Results: Incident hypocapnia was more frequent in the actively cooled transport group (36.6%) than control group (17.4%; p = 0.025). pCO2 decreased from a median of 45 to 35 mm Hg (p < 0.0001) in the intervention group, but remained unchanged in the controls. After adjusting for confounders, hypothermia remained an independent risk factor for hypocapnia with an odds ratio (OR) of 4.23 and 95% confidence interval (95% CI) of 1.30-13.79. Sedation was associated with a reduction in OR of hypocapnia, at 0.35 (95% CI 0.12-0.98).Conclusions: Hypothermia increased the risk of hypocapnia in neonates with HIE during transport. [ABSTRACT FROM AUTHOR]

Follow-up study found that vitamin D deficiency and weight gain increased the risk of impaired fasting glycaemia Vitamin D deficiency, as measured by 25-hydroxyvitamin D, has been related to obesity and impaired fasting glycaemia (IFG), but the causality is unclear. No data on the duration of vitamin D deficiency were available, but the IFG risk did not increase for children who developed vitamin D deficiency during the follow-up period. [Extracted from the article]

Unlabelled: The objective of this study was to investigate the association hypothesis that outcome following respiratory syncytial virus (RSV) induced bronchiolitis (RSVB) and RSV induced wheeze (RSVW) are different. At 3 years respiratory symptoms were more common in those with RSV infection than the control group but there was no increase in allergic sensitisation (11% vs 10%). Those with RSVW were more likely to have evidence of allergic sensitisation when compared with RSVB subjects (22% vs 7%), and have increased symptoms and increased use of inhaled steroids.Conclusion: The data argue that RSV infection during infancy does not induce allergic asthma and that host factors rather than the virus determine long-term outcomes. [ABSTRACT FROM AUTHOR]

Investigates the prevalence of urinary tract symptoms in 30 children with cerebral palsy (CP). Urological problems associated with spina bifida; Observation of symptoms of bladder dysfunction, urgency, and forced voiding in ten of the children studied; High prevalence of kidney affection, bladder symptoms and urodynamical abnormalities.