Asian Journal of Pediatric Research

Alagille Syndrome: About Two Cases and Literature Review

M. Akhrif — 2020-08-22

Alagille syndrome is a multi-systemc genetic disorder with variable phenotypic penetrance that was first described in 1969 by Daniel Alagille.It is characterized by anomalies of the intrahepatic bile ducts, heart, eye and skeleton, which are associated with facial features . The prognosis depends on the severity of the liver and heart diseases. The authors reported two cases characterized by the variability of clinical expression and evolution. The study concerned two girls aged of 2 and 4 months with no family history, who developed cholestatic jaundice evolving from the first month of life. The aim of this work is to remind the different clinical expressivity and the differentmodalities to manage the patients in order to ensure a best quality of life.

Reference Bioelectrical Impedance Values for Children of Normal Body Mass Index in Southeastern Brazil

Cristina Malzoni Ferreira Mangia — 2020-09-02

Background: Bioelectrical analysis measures two bioelectrical vectors: Resistance (R) and reactance (Xc). Resistance is the pure opposition of a biological conductor to the flow of an alternating current through the intra and extra-cellular ionic solution and it is inversely related to the dynamics of body fluids and body composition.

Objective: The purpose of this study was to determine the reference values of the indexes bioelectrical impedance (BI) for children of normal body mass index in southeastern Brazil of middle-income country.

Methods: Two hundred eighty-one children with normal body mass index were included in the study (135 female and 146 male), aged 4 to 129 months, selected from federal public urban school in São Paulo, São Paulo, Brazil, where bioelectrical impedance values resistance (R) and reactance (Xc) values were measured in order to establish reference values of these parameters.

Results: The anthropometric variables, body mass index, z-scores and bioelectrical impedance parameters were evaluated. For both genders, the mean and standard deviation of anthropometric variables were: age (months): 73.42 ± 34.65; weight (kg): 23.5 ± 9.46; height (m): 1.16±0.22; BMI (kg/m²): 16.65±1,75; Xc (ohms): 63.92±9.6; R (ohms): 749±75.26. For analysis, the children were stratified into three groups for each gender, being divided by ages: 4 to 23 months; 24 to 71 months and 72 to 129 months. Linear regression analysis showed R had a significant progressive decrease with age (p=0.0003) while Xc had a progressive increase (p=0.0065) with age increase. We analyzed by multiple regression the associations between R and Xc with anthropometric variables by age group to establish the reference values, confidence intervals and the tolerance limits for a new individual observation.

Conclusion: The BI reference values were established, in a field where there is a relative lack of publications, and we collected relevant information about resistance and reactance in a population of middle income setting that could be used in epidemiologic studies and could be used reference value in children with altered body composition.

Comparative Study of Nebulization with Salbutamol vs Saline Solution at the Acute Phase of Bronchiolitis of 100 Children Aged 1 to 23 Months

Nadia Mebrouk — 2020-09-03

Introduction: Infant bronchiolitis is the most common acute viral infection infection of the lower respiratory tract in children. Many pharmacological interventions have been suggested, including bronchodilators, yet the efficacy of bronchodilators in the treatment of this infection is still controversial.

Objectives: To evaluate the effect of nebulization of salbutamol vs. saline solution in the acute phase of bronchiolitis.

Materials and Methods: Using a randomized selection process, 52 infant patients received salbutamol, and 48 received a saline solution. The patients included in the study were aged 1 to 23 months and presented with their first episode of wheezing. During patient selection, we excluded children who were asthmatic or had other pulmonary issues prior to their bronchiolitis episode. Three nebulizations were performed for each patient, at one-hour intervals, after admission to the hospital. Wang's score and oxygen saturation were recorded for each patient on arrival, then at 30 minutes after each nebulization.

Results and Conclusion: During the treatment period (which lasts three hours), the mean Wang score decreased from 7 to 3.5, and the mean oxygen saturation increased from 92.5% to 95.6%. Statistical analysis of the data, based on a parametric statistical test of the Student type (T-test), shows that there is no significant difference between nebulization with salbutamol and those given saline solution. The evolution of the clinical scores leads to the general conclusion that we cannot recommend using salbutamol nebulization over saline solution for the treatment of bronchiolitis.