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Aim: To assess parental understanding of idiopathic nephrotic syndrome (INS) and its management, to enable targeted education in areas of deficit.Methods: Families of children with at least one relapse of INS were interviewed, following a template covering key domains of (a) disease understanding, (b) management of INS and (c) access to information. Common trends and responses were identified and notable observations recorded.Results: Twenty-one parents were interviewed. The mean duration of INS was 4.4 years (range 0.5-14.5 years), with a mean of two steroid-sparing agents used. Although 90% parents self-reported that they understood INS, only 29% could appropriately define relapse and 24% name potential complications. The management of INS was generally good, with most parents appropriately testing urine (81%) and managing relapses (90%). Unnecessary dietary restrictions were imposed on 57% during remission. The Internet was searched by 90% of parents for disease and drug information. Further information was desired in paper form (71%), hospital website (81%) and face-to-face workshop (90%), plus educational materials for schools.Conclusion: Parents overestimated their understanding of INS; however, their management was generally well done. Parents desired more information and support in various forms. [ABSTRACT FROM AUTHOR]

Aim: This paper aimed to analyse the association between small for size at birth, stunting, recovery from stunting and pubertal development in a rural Bangladeshi cohort.Methods: The participants were 994 girls and 987 boys whose mothers participated in the Maternal and Infant Nutrition Interventions in Matlab trial. The birth cohort was followed from birth to puberty 2001-2017. Pubertal development according to Tanner was self-assessed. Age at menarche was determined and in boys, consecutive height measurements were used to ascertain whether pubertal growth spurt had started. The exposures and outcomes were modelled by Cox's proportional hazards analyses and logistic regression.Results: There was no difference in age at menarche between girls that were small or appropriate for gestational age at birth. Boys born small for gestational age entered their pubertal growth spurt later than those with appropriate weight. Children who were stunted had later pubertal development, age at menarche and onset of growth spurt than non-stunted children. Children who recovered from infant or early childhood stunting had similar pubertal development as non-stunted children.Conclusion: Infant and childhood stunting was associated with a later pubertal development. Recovery from stunting was not associated with earlier puberty in comparison with non-stunted children. [ABSTRACT FROM AUTHOR]

Severe primary paediatric immunodeficiency syndromes are rare and potentially fatal unless suspected, diagnosed and treated early. We provide clinical guidance and support for on-call consultant paediatricians working in secondary level hospitals on how to recognise and manage children with these conditions. Our paper addresses four conditions that risk the most severe outcomes if they are not adequately cared for during on-call periods, such as weekends: severe combined immunodeficiency, haemophagocytic lymphohistiocytosis, severe congenital neutropaenia and chronic granulomatous disease. CONCLUSION: On-call paediatricians are provided with advice on handling the most severe primary immunodeficiencies. [ABSTRACT FROM AUTHOR]

Unlabelled: Adolescent health surveys, like those for other segments of the population, tend to remain in the hands of researchers, where they can have no real impact on the way critical health issues are dealt with by policy makers or other professionals directly connected to young people in their everyday work. This paper reviews important issues concerning the dissemination of survey results among professionals from various fields. The content, length and wording of the messages should be tailored to the audience one wants to reach as well as the type of channels used for their diffusion. Survey data sets can be used to select priorities for interventions: ad hoc presentations, attractive summaries and brochures, or even films expressing young peoples' opinions have been used by European public health professionals to make data sets usable in various local, regional and national contexts.Conclusion: The impact of these diffusion strategies is, however, difficult to assess and needs to be refined. The adequate delivery of survey findings as well as advocacy and lobbying activities require specific skills which can be endorsed by specialized professionals. Ultimately, it is the researchers' responsibility to ensure that such tasks are effectively performed. [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 measure the relationship of resting energy expenditure (REE) and body composition, and to compare REE data calculated from anthropometric parameters using published equations with measurements obtained by indirect calorimetry (IC) in a population of obese paediatric patients.Methods: The study included 82 healthy obese paediatric subjects (49 boys, 33 girls; body mass index 29.6 +/- 5.0 kg/m , age 1 1.4 +/- 2.6 y, weight 72.4 +/- 20.9 kg, height 155 +/- 14 cm). REE was measured by IC, body composition was determined by dual energy X-ray absorptiometry (DXA). Bootstrap analysis was performed to validate the step-down linear regression analysis results.Results: Lean body mass (LBM) and weight were identified as the most significant determinants of REE. LBM was the best single predictor (r = 0.78; p < 0.001) for REE. Regression equations are given in the text. Prediction of REE on the basis of published anthropometric formulas was strongly dependent from the equation used. Some equations tend to underestimate REE in the population studied with a considerable systematic error.Conclusion: In the present paper we show that (1) the published equations to predict REE in obese subjects yield scattered data and some are even biased by a systematic error, and that (2) the inclusion of DXA-derived LBM improves accuracy and precision of predicted REE in boys and girls aged from 4 to 10 y and in boys from 11 to 15 y. [ABSTRACT FROM AUTHOR]

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]