Physical activity and aerobic fitness in children after liver transplantation

To determine physical activity (PA), aerobic fitness, muscle strength, health-related quality of life (HRQOL), fatigue, and participation in children after liver transplantation. Children, 6-12 years, at least one year after liver transplantation, participated in this cross-sectional study. Measurements: Time spent in moderate to vigorous PA (MVPA) was measured using an accelerometer, and aerobic fitness (VO2 peak) was measured by cardiopulmonary exercise testing. Muscle strength was measured by hand-held dynamometry. Fatigue was measured using the multidimensional fatigue scale, and HRQOL with the Pediatric Quality of life Core scales and leisure activities was measured using the Children's Assessment of Participation and Enjoyment. Outcomes (medians and interquartile range (IQR)) were compared to norm values. Twenty-six children participated in this study (14 boys, age 9.7 years, IQR 7.7;11.4). Children spent 0.8 hours/d (IQR 0.6;1.1) on MVPA. One child met the recommendation of at least 1 hour of MVPA every day of the week. Aerobic fitness was similar to norms (VO2 peak 1.4 (L)(/min), IQR 1.1;1.7, Z-score -0.3). Z-scores of muscle strength ranged between -1.4 and -0.4 and HRQOL and fatigue between -2.3 and -0.4. Participation was similar to published norms (Z-scores between -0.6 and 0.6). Young children after liver transplantation have similar MVPA patterns and aerobic fitness compared to published norms. Despite lower HRQOL, more fatigue, and less muscle strength, these children have similar participation in daily activities. Although children do well, it remains important to stimulate PA in children after liver transplantation in the context of long-term management

Atypical clinical presentation of mucopolysaccharidosis type II (Hunter syndrome): a case report

<p>Abstract</p> <p>Introduction</p> <p>We present a very rare case of mucopolysaccharidosis with atypical presentation such as mild mental retardation, an acrocephalic head and no corneal clouding. The purpose of presenting this case is to highlight the distinctive manifestation of mucopolysaccharidosis type II (Hunter syndrome).</p> <p>Case presentation</p> <p>A 10-year-old East Asian boy presented with abdominal distension of five years' duration and complained of shortness of breath on and off for the same period. On examination his head was large and his head circumference was 54.5 cm. His neck was short, he had coarse facial features, a depressed nasal bridge and small stubby fingers with flexion of distal interphalangeal joints, and a low arched palate was observed. There was mild mental retardation.</p> <p>Conclusion</p> <p>Based on clinical findings and radiological features it is possible to diagnose a case of mucopolysaccharidosis.</p> <p>Careful and systemic approach is needed to accurately diagnose the exact type as enzymatic studies are not available in most centers.</p

Lethal pneumatosis coli in a 12-month-old child caused by acute intestinal gas gangrene after prolonged artificial nutrition: a case report

<p>Abstract</p> <p>Introduction</p> <p>Pneumatosis coli is a rare disease with heterogeneous symptoms which can be detected in the course of various acute and chronic intestinal diseases in children, such as necrotizing enterocolitis, intestinal obstruction and intestinal bacteriological infections.</p> <p>Case presentation</p> <p>We report the case of a 12-month-old boy who died of pneumatosis coli caused by an acute intestinal gas gangrene after prolonged artificial alimentation.</p> <p>Conclusion</p> <p>While intestinal gas gangrene is a highly uncommon cause of pneumatosis coli, it is important to consider it as a differential diagnosis, especially in patients receiving a prolonged artificial food supply. These patients may develop intestinal gas gangrene due to a dysfunctional intestinal barrier.</p

Gram Negative Bacteria Are Associated with the Early Stages of Necrotizing Enterocolitis

Introduction: Necrotizing enterocolitis (NEC) affects 5–10 % of infants born weighing less than 1500 g. Most models of NEC recapitulate late-stage disease with gut necrosis and elevated inflammatory mediators. Evaluation of NEC at earlier, less lethal stages of disease will allow investigation of initial disease triggers and may advance our understanding of temporal relationships between factors implicated in NEC pathogenesis. In this manuscript, we describe our investigation of early NEC and test the hypothesis that bacteria and inflammatory mediators differ between animals with early NEC and disease fre

Association between Proximity to a Health Center and Early Childhood Mortality in Madagascar

Objective: To evaluate the association between proximity to a health center and early childhood mortality in Madagascar, and to assess the influence of household wealth, maternal educational attainment, and maternal health on the effects of distance. Methods: From birth records of subjects in the Demographic and Health Survey, we identified 12565 singleton births from January 2004 to August 2009. After excluding 220 births that lacked global positioning system information for exposure assessment, odds ratios (ORs) and their 95% confidence intervals (CIs) for neonatal mortality and infant mortality were estimated using multilevel logistic regression models, with 12345 subjects (level 1), nested within 584 village locations (level 2), and in turn nested within 22 regions (level 3). We additionally stratified the subjects by the birth order. We estimated predicted probabilities of each outcome by a three-level model including cross-level interactions between proximity to a health center and household wealth, maternal educational attainment, and maternal anemia. Results: Compared with those who lived >1.5–3.0 km from a health center, the risks for neonatal mortality and infant mortality tended to increase among those who lived further than 5.0 km from a health center; the adjusted ORs for neonatal mortality and infant mortality for those who lived >5.0–10.0 km away from a health center were 1.36 (95% CI: 0.92–2.01) and 1.42 (95% CI: 1.06–1.90), respectively. The positive associations were more pronounced among the second or later child. The distance effects were not modified by household wealth status, maternal educational attainment, or maternal health status. Conclusions: Our study suggests that distance from a health center is a risk factor for early childhood mortality (primarily, infant mortality) in Madagascar by using a large-scale nationally representative dataset. The accessibility to health care in remote areas would be a key factor to achieve better infant health

Platelet-Activating Factor Induces TLR4 Expression in Intestinal Epithelial Cells: Implication for the Pathogenesis of Necrotizing Enterocolitis

Necrotizing enterocolitis (NEC) is a leading cause of morbidity and mortality in neonatal intensive care units, however its pathogenesis is not completely understood. We have previously shown that platelet activating factor (PAF), bacteria and TLR4 are all important factors in the development of NEC. Given that Toll-like receptors (TLRs) are expressed at low levels in enterocytes of the mature gastrointestinal tract, but were shown to be aberrantly over-expressed in enterocytes in experimental NEC, we examined the regulation of TLR4 expression and signaling by PAF in intestinal epithelial cells using human and mouse in vitro cell lines, and the ex vivo rat intestinal loop model. In intestinal epithelial cell (IEC) lines, PAF stimulation yielded upregulation of both TLR4 mRNA and protein expression and led to increased IL-8 secretion following stimulation with LPS (in an otherwise LPS minimally responsive cell line). PAF stimulation resulted in increased human TLR4 promoter activation in a dose dependent manner. Western blotting and immunohistochemical analysis showed PAF induced STAT3 phosphorylation and nuclear translocation in IEC, and PAF-induced TLR4 expression was inhibited by STAT3 and NFκB Inhibitors. Our findings provide evidence for a mechanism by which PAF augments inflammation in the intestinal epithelium through abnormal TLR4 upregulation, thereby contributing to the intestinal injury of NEC