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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]

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]

Aim: The aim of this study was to evaluate the HemoCue WBC DIFF system for point of care testing of fingerstick samples from paediatric patients.Methods: We analysed 158 white blood cell counts on both the point of care HemoCue WBC DIFF instrument and the Cell Dyn Sapphire cell counter used by our central laboratory and compared the results. The measurements were performed using fingerstick samples drawn by nurses working in paediatric emergency and paediatric oncology units.Results: There was good agreement between the two instruments for white blood cell and neutrophil counts. The correlation was weaker for lymphocytes, and the correlations were poor for monocytes and eosinophils. The HemoCue WBC DIFF flagged 56 of the 148 capillary drawn samples as abnormal, but none of the 10 venously collected samples. Only two of the flagged samples differed significantly between the instruments, with regard to the cell counts.Conclusion: The correlations between the white blood cell counts and neutrophil counts in this real-life study were good enough to diagnose children in emergency department and oncology unit settings. However, the high number of pathological flags from fingerstick samples, which made reruns necessary, limited the usefulness of the instrument. [ABSTRACT FROM AUTHOR]

A medical report is presented on the Survey of healthcare professional and parental experience in accessing support for breastfeeding during an acute hospital admission. The article mentions about the advantages Breastmilk to infants and mothers as well as promoting the formation of the parental bond; and breastmilk should be the preferred choice of feed for infants, and with current guidelines from the World Health Organization (WHO).

Aim: To evaluate common methods of investigation and treatment in children younger than 2 y of age with eczema, with or without sensitization to food allergens.Methods: One hundred and twenty-three children younger than 2 y of age with eczema and suspected food allergy were included in this prospective study. The children underwent skin-prick test with cow's milk, fresh hen's egg white and wheat. Specific IgE to milk and egg white was analysed. The eczema extent and severity was estimated with SCORAD before and after treatment. Children with a positive skin-prick test were instructed to exclude that food item from their diet. All children were treated with emollients and topical steroids when needed.Results: Sixty-two of the children were skin-prick positive to at least one of the allergens; 62% had mild, 30% moderate and 8% severe eczema at their first visit. After treatment, 90% had mild, 10% moderate and 0% severe eczema. Forty-six per cent of the children had circulating IgE antibodies to milk or egg white. Ten per cent had specific IgE but negative skin-prick test to the same allergen. This subgroup improved their eczema significantly without elimination diet.Conclusion: The conventional treatments for children with eczema, i.e. skin care and food elimination, are effective. The beneficial effect of skin care as the first step should not be neglected, and it may not be necessary to eliminate food allergens to relieve skin symptoms in all food-sensitized children with eczema. [ABSTRACT FROM AUTHOR]

Aim: To assess whether children with Noonan syndrome on long-term growth hormone (GH) therapy improve their final height to near mid-parental height.Methods: Twenty-five prepubertal children (13 girls) with Noonan syndrome (NS) were studied. A single clinician made the diagnosis based on clinical criteria. GH treatment started at an age ranging from 3.1 to 13.8 y and was continued for at least 2 y. Improvement or "gain" in final height (FH) was defined as either the difference between adult height SD scores (SDS) and pre-treatment height SDS (the childhood component of the Swedish reference) or height SDS compared to the Noonan reference.Results: Ten children received a GH dose of 33 microg/kg/d (mean age at start 7.7+/-2.1 y, mean age at stop 17.6+/-1.7 y) and 15 received a dose of 66 microg/kg/d (mean age at start 8.6+/-3.3 y, mean age at stop 18.4+/-2.1 y). Eighteen out of 25 patients reached FH. A substantial improvement in FH of 1.7 SDS, equivalent to 10.4 cm compared to pre-treatment height, was observed. No significant difference was seen between the two GH doses. Females gained a mean height of 9.8 cm and males 1-13 cm (FH 174.5+/-7.8 cm vs mean adult height of 162.5+/-5.4 cm for males with NS) at final height. Moreover, 60% reached a mid-parental height of+/-1 SD.Conclusion: GH treatment improves final height in patients with Noonan syndrome, with a mean gain of 1.7 SDS. The prepubertal height gain is maintained to final height and the children achieve a height close to their mid-parental height. [ABSTRACT FROM AUTHOR]

Background: Most of the world's children live in regions where laboratory facilities are not available. In these regions, clinical prediction rules can be useful to guide clinicians' decisions on antibiotic therapy for streptococcal pharyngitis, and to reduce routine presumptive antibiotic therapy for all pharyngitis.Methods: Prospective cohort study to assess diagnostic signs and develop a prediction rule. Bivariate and multivariate analyses were used to develop clinical rules. Participants were 410 children in Cairo, Egypt, aged from 2 to 12 y, presenting with complaint of sore throat and whose parents provided consent. Main outcome measures included presence of signs and symptoms, and positive group A beta hemolytic streptococcal (GABHS) culture.Results: 101 (24.6%) children had positive GABHS culture. Pharyngeal exudate, tender or enlarged anterior cervical lymph nodes, season, absence of rash, or cough or rhinitis were associated with positive culture in bivariate and multivariate analyses. Three variables (enlarged nodes, no rash, no rhinitis), when used in a cumulative score, showed 92% sensitivity and 38% specificity in these children.Conclusions: The proposed three-variable clinical prediction rule for GABHS may be useful when diagnostic laboratories are not available. In this setting, the rule identified more than 90% of true cases. Compared to universal treatment of all pharyngitis, the rule will reduce antibiotic use in GABHS-negative cases by about 40%. [ABSTRACT FROM AUTHOR]

Aims: To analyse mothers' self-assessed quality of interaction with their children and their opinions about child difficulty with respect to socio-economic status and subjective factors: postnatal depression, social isolation, sense of coherence and locus of control.Methods and Material: A comprehensive questionnaire was completed by 1039 mothers of 18-mo-old children participating in the baseline measurements of a Swedish multicentre study developing and testing a new psychosocial model for the child health services.Results: All subjective factors, including the number of factors, showed significant associations with perceived interaction and difficultness. Effect sizes of subjective factors ranged from about 0.3 to 1 SD for interaction, and from about 0.2 to 0.8 SD for difficultness. As for difficultness, effect sizes were larger for boys. There were no associations between high socio-economic status and high-quality interaction or low child difficultness: the few significant differences in fact favoured low-status children.Conclusion: The results provided some contradictory findings to the well-known association between high socio-economic status and favourable outcome. This result is of practical relevance for interventions: supportive programmes cannot be limited to areas and families of low socio-economic status. Positive effects may ensue if subjective factors like those studied here can be promoted among parents and children through the child health services. [ABSTRACT FROM AUTHOR]

Aim: To describe an evidence-based model for preventive child health care and present some findings from baseline measurements.Methods: The model includes: parent education; methods for interaction and language training; follow-up of low birthweight children; identification and treatment of postnatal depression, interaction difficulties, motor problems, parenthood stress, and psychosocial problems. After baseline measurements at 18 mo (cohort I), the intervention was tested on children from 0 to 18 mo at 18 child health centres in Uppsala County (cohort II). Eighteen centres in other counties served as controls. Two centres from a privileged area were included in the baseline measurements as a "contrasting" sample. Data are derived from health records and questionnaires to nurses and mothers.Results: Baseline experiment (n = 457) and control mothers (n = 510) were largely comparable in a number of respects. Experiment parents were of higher educational and occupational status, and were more frequently of non-Nordic ethnicity. Mothers in the privileged area (n = 72) differed from other mothers in several respects. Experiment nurses devoted considerably fewer hours per week to child health services and to child patients than did control nurses.Conclusions: Despite certain differences, experiment and control samples appeared comparable enough to permit, in a second step, conclusions about the effectiveness of the intervention. [ABSTRACT FROM AUTHOR]

Aim: To study the relationship between socio-economic factors and coeliac disease.Methods: This study was part of a prospective cohort study of 16 286 children born from 1 October 1997-1 October 1999 (the ABIS study; All Babies in Southeast Sweden). Eight paediatric departments recorded all children with coeliac disease in southeast Sweden. Coeliac disease was confirmed through biopsy. Socio-economic characteristics (maternal employment, civil status, whether parents were born in Sweden, parental education, place of living before pregnancy and during pregnancy, crowded living), infant sex, previous siblings, parental age and maternal alcohol consumption during pregnancy were analysed using logistic regression. All data, except for those related to diagnosis of coeliac disease, were obtained through a questionnaire distributed at birth.Results: Coeliac disease in the offspring was less common among mothers who had worked < 3 mo during pregnancy (odds ratio, OR = 0.29; 95% CI: 0.09-0.94; p = 0.039). This risk decrease remained after adjustment for confounders (adjusted OR = 0.28; 95% CI: 0.09-0.92; p = 0.035). No other socio-economic factor was related to coeliac disease.Conclusion: This study indicates that most socio-economic factors are probably of little importance to the development of coeliac disease. [ABSTRACT FROM AUTHOR]

Unlabelled: Owing to a lack of longitudinal studies, the effect of centralization of care on pulmonary function and survival remains unclear. Three different levels of involvement of centralized care in the treatment of paediatric cystic fibrosis patients were compared with regard to longitudinal pulmonary function and nutritional and microbiological status in a 3-y period, and the literature was reviewed on the possible advantages and disadvantages of centralized care. The study included 105 paediatric patients attending the Cystic Fibrosis Centre between January 1997 and January 2001. Twenty-three patients were treated by local paediatricians according to the protocol of the Centre and were seen only once a year at the Centre, for an annual check-up (local care). Forty-one patients were treated at the Centre only (centralized care). The remaining 41 patients were treated in close cooperation between the Centre and local hospitals, with patients visiting the doctors alternately (shared care). The mean annual changes in pulmonary function and body mass index from all patients, as well as a microbiological survey, were reviewed. No significant differences were found between the three groups for annual changes in FEV1, FVC and body mass index, nor did the review of microbial colonization show any significant differences between the groups. Because the groups in this study were relatively small, the results might have been influenced by lack of power.Conclusion: In this relatively small group, no differences in pulmonary function, nutritional status or microbiological colonization between the three levels of involvement of centralized care could be found. This could signify that local paediatricians have a special role in the care for patients with cystic fibrosis, in close cooperation with the specialists at the Centre. [ABSTRACT FROM AUTHOR]