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We carried out a retrospective investigation of the 89 patients with extrahepatic biliary atresia born in The Netherlands during a 10 year period. Of these 89 patients 10 had a diagnostic laparotomy only. Eight patients had an anastomosis between the proximal bile duct and the intestine, and the remaining 71 had hepatic portoenterostomies. Bile drainage was re-established in 46 (65%). After successful hepatic portoenterostomy the development of cholangitis was the most important determinant of long term survival; five year survival was 54% in the 19 patients who had cholangitis and 91% in the 27 who did not. In the whole group of 71 patients the five year survival was 47%. Seventeen patients were at least 5 years of age at the time of writing, three of whom had had liver transplantation. Three patients have cirrhosis and hyperbilirubinaemia, and the other 11 have normal bilirubin concentrations and normal or slightly raised transaminase activities. To improve these results early surgical intervention in all children with extrahepatic biliary atresia is necessary, as are better methods of prophylaxis and treatment of cholangitis. [ABSTRACT FROM AUTHOR]

Summary Background In a murine model of allergic inflammation, B ifidobacterium breve M-16 V has been shown to reduce IL-4 and IgE by inducing IL-10 and IFN-γ. However, it remains unknown whether this strain has the same effect in humans with allergic disease. Objective To determine the effects of B ifidobacterium breve M-16 V combined with a prebiotic oligosaccharide mixture (synbiotic) on atopic markers, ex vivo cytokine production by peripheral blood mononuclear cells ( PBMCs) and circulating regulatory T cell percentage in infants with atopic dermatitis. Methods In a double-blind, placebo-controlled multi-centre trial, 90 infants with atopic dermatitis, age <7 months, were randomized to receive an infant formula with B ifidobacterium breve M-16 V and a mixture of short chain galactooligosaccharides and long chain fructooligosaccharides ( Immunofortis®), or the same formula without synbiotics during 12 weeks. At week 0 and 12, plasma levels of IL-5, IgG1, IgG4, CTACK and TARC, ex vivo cytokine responses by PBMCs and percentage of regulatory T cells, were determined. Results There were no significant differences between the synbiotic and the placebo group in IL-5, IgG1, IgG4, CTACK and TARC levels and ex vivo cytokine production by anti- CD3/anti- CD28-stimulated PBMCs. With allergen-specific stimuli, we found a decreased IL-12p40/70 and IL-12p70 production in response to egg allergen ( P = 0.04 and P = 0.01, respectively) and decreased IL-12p70 production in response to peanut allergen ( P = 0.003) in the synbiotic compared with the placebo group. Circulating regulatory T cell percentage did not significantly differ between the groups. Conclusions and Clinical Relevance This synbiotic mixture has no detectable effect on plasma levels of the analysed atopic disease markers, ex vivo cytokine production and circulating regulatory T cell percentage in infants with atopic dermatitis, besides down-regulation of IL-12 production in egg- and peanut-stimulated PBMCs. These results do not support the use of this synbiotic in clinical practice. [ABSTRACT FROM AUTHOR]

Unlabelled: We describe the clinical symptoms and biochemical findings of a patient with succinyl-CoA:acetoacetate transferase deficiency who presented in the neonatal period and review the current literature on this subject. Our patient was initially suspected to have distal renal tubular acidosis, and subsequently, a fasting test revealed severe metabolic ketoacidosis with normal blood glucose after 13 h which suggest a defect in ketolysis. In his cultured skin fibroblasts succinyl-CoA:acetoacetate transferase was deficient (residual activity 15%). Treatment in the acute phase consisted of sodium bicarbonate. At the present age of 9 years, psychomotor and physical development are within normal limits.Conclusion: Defects of ketolysis probably are underdiagnosed disorders and should be considered in infants and young children with persistent ketosis. [ABSTRACT FROM AUTHOR]

Regurgitation is a common manifestation in infants below the age of 1 year and a frequent reason of counselling of general practitioners and paediatricians. Current management starts with postural and dietary measures, followed by antacids and prokinetics. Recent issues such as an increased risk of sudden infant death in the prone sleeping position and persistent occult gastro-oesophageal reflux in a subset of infants receiving milk thickeners or thickened "anti-regurgitation formula" challenge the established approach. Therefore, the clinical practices for management of infant regurgitation have been critically evaluated with respect to their efficacy, safety and practical implications. The updated recommendations reached by the working party on the management of infant regurgitation contain five phases: (1 A) parental reassurance; (1 B) milk-thickening agents; (2) prokinetics; (3) positional therapy as an adjuvant therapy; (4 A) H2-blockers; (4 B) proton pump inhibitors; (5) surgery. [ABSTRACT FROM AUTHOR]

Children permanently exposed to hypoxia at altitudes of >3000 m above sea level show a phenotypical form of adaptation. Under these environmental conditions, oxygen uptake in the lungs is enhanced by increases in ventilation, lung compliance, and pulmonary diffusion. Lung and thorax volumes in children growing up at high altitude are increased. The haemoglobin concentration in highlanders is evevated. With respect to the decreased arterial oxygen tension at high altitude, this seems a useful adaptation. Blood viscosity also increases as a result of the increase in red blood cell concentrations however, and this has potentially negative effects on the microcirculation in the tissues. The decreased partial pressure of oxygen in the lungs of highland children is associated with a higher pulmonary artery pressure. Pulmonary hypertension, high altitude pulmonary oedema, and chronic mountain sickness form part of the pathophysiology afflicting highland dwellers. Birth weight at high altitude is decreased. Decreased postnatal growth has been widely reported in populations at high altitude, particularly in early studies from the Andes. Recent studies taking into account the effects of socio-economic deprivation, suggest that long-term exposure to altitudes of 2500-3900 m is associated with a moderate reduction in linear growth in children. [ABSTRACT FROM AUTHOR]

Children permanently exposed to hypoxia at altitudes of > 3000 m above sea level show a phenotypical form of adaptation. Under these environmental conditions, oxygen uptake in the lungs is enhanced by increases in ventilation, lung compliance, and pulmonary diffusion. Lung and thorax volumes in children growing up at high altitude are increased. The haemoglobin concentration in highlanders is elevated. With respect to the decreased arterial oxygen tension at high altitude, this seems a useful adaptation. Blood viscosity also increases as a result of the increase in red blood cell concentrations however, and this has potentially negative effects on the microcirculation in the tissues. The decreased partial pressure of oxygen in the lungs of highland children is associated with a higher pulmonary artery pressure. Pulmonary hypertension, high altitude pulmonary oedema, and chronic mountain sickness form part of the pathophysiology afflicting highland dwellers. Birth weight at high altitude is decreased. Decreased postnatal growth has been widely reported in populations at high altitude, particularly in early studies from the Andes. Recent studies taking into account the effects of socio-economic deprivation, suggest that long-term exposure to altitudes of 2500-3900 m is associated with a moderate reduction in linear growth in children. [ABSTRACT FROM AUTHOR]

In studies on atopic dermatitis (AD), different scoring systems are used to evaluate the severity of the disease. The objective of this study was to investigate agreement between observers in the assessment of the overall severity of AD, and interobserver variation in the assessment of severity of AD for each scoring item separately, using the Simple Scoring System (SSS), the Scoring Atopic Dermatitis (SCORAD) index, and the Basic Clinical Scoring System (BCSS), and, furthermore, to investigate agreement between these three scoring systems in the assessment of the overall severity of AD. Eighty-two patients (42 male) with AD, mean age 13.4 years (range 0.2- 67.0), were included. Agreement between observers in assessing the overall AD severity scores, and interobserver variation in assessing AD severity of each scoring item separately were determined in 34 of these 82 patients by two physicians scoring the severity of AD by the three scoring systems. To determine agreement between the scoring systems, one physician scored the severity of AD in all patients with the three scoring systems. Agreement between observers and agreement between the three scoring systems was calculated by Cohen's kappa (K) and by the measure of agreement according to Bland & Altman. K>0.4 represents fair agreement; K>0.75 excellent agreement. In addition, interobserver variation for each scoring item separately was calculated by the Wileoxon signed rank test. The mean differences (d) and the limits of agreement (d±2 SD of the differences) between observers by the SSS and the SCORAD were -0.82±5.58 and -0.28±7.49, respectively. K between observers for the BCSS was 0.90 (95% CI 0.79-1.03). By the SSS, significant interobserver variation was found in assessing the severity of excoriations (p=0.02) and scales (P=0.02). By the SCORAD, significant interobserver variation was found in assessing the severity of edema/papulation (p=0.04), erythema (p=0.04), and excoriations (p=0.01). No significant interobserver variation was found in assessing the extent of AD. The mean difference and the limits of agreement between the SSS and the SCORAD were -4.17±9.52. K between the SSS and the BCSS was 0.21 (95% CI 0.09-0.33), and K between the SCORAD and the BCSS was 0.38 (95% CI 0.26-0.51). We found good agreement between observers assessing the overall severity of AD in the lower and higher scoring rates by the SSS and the SCORAD, and excellent agreement by the BCSS. Significant interobserver variation was found on the isolated intensity items seales, excoriations, edema/population, and erythema. We found poor agreement between the three scoring systems in assessing the overall severity of AD, indicating that the SSS, the SCORAD, and the BCSS cannot be used interchangeably to assess the overall severity of AD. [ABSTRACT FROM AUTHOR]