Pearson marrow-pancreas syndrome with cardiac conduction abnormality necessitating prophylactic pacemaker implantation.

Pearson marrow-pancreas syndrome (PS), an exceedingly rare mitochondrial disorder, involves multiple systems including hematologic system and pancreas. Other mitochondrial disorders have been associated with progressive infrahisian block but this has not yet been described as a major feature of PS. We report a 7-year-old girl with classical features of PS and cardiac conduction defect. Her electrocardiogram revealed QRS prolongation with right bundle and left anterior fascicular blocks. Follow-up Holter revealed bifascicular block, alternating left and right bundle branch blocks, supraventricular tachycardia (with alternating bundles), and suspicion for nonsustained ventricular tachycardia. She underwent successful transvenous single-chamber ventricular pacemaker.

Unintentional injuries among children aged 1-5 years: understanding the burden, risk factors and severity in urban slums of southern India.

BACKGROUND: Globally, 5.82 million deaths occurred among children under the age of five years in 2015 and injury specific mortality rate was 73 per 100,000 population. In India, injury specific mortality rate is around 2.1 per 1000 live births contributing to 4% of the total under 5 mortality rate. This study aims to estimate the burden and understand factors associated with unintentional injuries among children aged 1-5 years residing in urban slums of Vellore, southern India. We also attempted to assess the hazards posed by the living environment of these children and study their association with unintentional injury patterns. METHODS: This cross-sectional study was conducted in eight urban slums of Vellore, southern India and primary caregivers of children aged 1-5 years were interviewed with a questionnaire to obtain the details of injuries sustained in the past three months. Environmental hazard risk assessment was conducted at places frequented by these children and their scores calculated. Baseline prevalence and incidence rates of unintentional injuries were estimated. Multivariate logistic regression and poisson regression analysis were performed to examine factors associated with unintentional injuries and repeated injuries respectively. Association between environmental hazard risk and unintentional injuries was estimated. RESULTS: Prevalence of unintentional injuries was 39.1% (95% CI 35.4-42.9%) and incidence rate was 16.5 (95% CI 14.7-18.3) per 100 child months (N = 662). Bivariate analysis revealed that children of working mothers (OR 1.48; 1.01-2.18) and children from overcrowded families (OR 1.78; 1.22-2.60) had increased odds of sustaining unintentional injuries. Multivariate regression analysis revealed that children from overcrowded families had increased odds of sustaining unintentional injuries (AOR 1.66, 95% CI 1.14-2.41). Boys (IRR 1.33, 95% CI 1.07-1.66) and children from overcrowded families (IRR 1.50; 1.14-1.98) were at increased risk of having repeated injuries. There is an increase in incidence rate of injuries with an increased environmental hazard risk, although not statistically significant. CONCLUSIONS: The burden of unintentional injuries was very high among study children when compared to studies in other urban slums in India. Environment plays an important role in the epidemiology of unintentional injuries; providing safe play environment and adequate supervision of children is important to reduce its burden.

A Comparison of Diarrheal Severity Scores in the MAL-ED Multisite Community-Based Cohort Study.

OBJECTIVES: There is a lack of consensus on how to measure diarrheal severity. Within the context of a multisite, prospective cohort study, we evaluated the performance of a modified Vesikari score (MAL-ED), 2 previously published scores (Clark and CODA [a diarrheal severity score (Community DiarrheA) published by Lee et al]), and a modified definition of moderate-to-severe diarrhea (MSD) based on dysentery and health care worker diagnosed dehydration. METHODS: Scores were built using maternally reported symptoms or fieldworker-reported clinical signs obtained during the first 7 days of a diarrheal episode. The association between these and the risk of hospitalization were tested using receiver operating characteristic analysis. Severity scores were also related to illness etiology, and the likelihood of the episode subsequently becoming prolonged or persistent. RESULTS: Of 10,159 episodes from 1681 children, 143 (4.0%) resulted in hospitalization. The area under the curve of each score as a predictor of hospitalization was 0.84 (95% confidence interval: 0.81, 0.87) (Clark), 0.85 (0.82, 0.88) (MAL-ED), and 0.87 (0.84, 0.89) (CODA). Severity was also associated with etiology and episode duration. Although families were more likely to seek care for severe diarrhea, approximately half of severe cases never reached the health system. CONCLUSIONS: Community-based diarrheal severity scores are predictive of relevant child health outcomes. Because they require no assumptions about health care access or utilization, they are useful in refining estimates of the burden of diarrheal disease, in estimating the effect of disease control interventions, and in triaging children for referral in low- and middle-income countries in which the rates of morbidity and mortality after diarrhea remain high.