Open Access Case Study

Alagille Syndrome: About Two Cases and Literature Review

M. Akhrif, A. Radi, M. Kmari, A. Ourrai, A. Hassani, R. Abilkassem, A. Agadr

Asian Journal of Pediatric Research, Page 1-6,
DOI: 10.9734/ajpr/2020/v4i330148

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.

Open Access Original Research Article

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

Cristina Malzoni Ferreira Mangia, Alexandre Carneluti, Maria Cristina Andrade

Asian Journal of Pediatric Research, Page 7-17
DOI: 10.9734/ajpr/2020/v4i330149

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.

Open Access Original Research Article

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, Naima El Hafidi, Fadoua Benbrahim, Soumia Benchekroun, Chafiq Mahraoui

Asian Journal of Pediatric Research, Page 18-25
DOI: 10.9734/ajpr/2020/v4i330150

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.

Open Access Original Research Article

Mortality from Surgical Conditions in Children: An Insight from a Tertiary-Level Government Hospital in Bangladesh

Tanvir Kabir Chowdhury, S. M. Humayun Kabir, Md. Abdullah Al Farooq, Md. Khurshid Alam Sarwar, Rajib Khastagir, Md. Minhajuddin Sajid

Asian Journal of Pediatric Research, Page 26-34
DOI: 10.9734/ajpr/2020/v4i330151

Aims: While the developed world has significantly reduced mortality from surgical conditions in children, there is lack of adequate data on the outcome of children’s surgical conditions in the developing world. This study aims at assessing the spectrum of mortalities from children’s surgical conditions in a low-middle income country (LMIC).

Methods: Mortality data were collected for a period of 12 years (2008 to 2019) among 0 to12-year old children to analyse the diseases which had higher mortality trends, age -group specific mortality trends and categorize those according to body system.

Results: Among a total of 30,301 admitted children, 1228 (4.05%) patients died. Among them 53.01% were neonates, 20.28% were infants and 26.71% were children.  Male to female ratio was 1.95:1. Mortality from neonatal surgical conditions was 24.21%. Infant mortality rate was 5.65% and child mortality rate was 1.41%. Neonatal surgical admission and mortality had a higher yearly trend. Anorectal malformation (ARM) was the most common cause of death (12.05%), followed by intestinal obstruction (11.40%) and gastroschisis (8.63%). Tracheo-oesophageal fistula/ oesophageal atresia (TOF/OA) had the highest mortality rate (83.33%), followed by gastroschisis (80.92%) and intestinal atresia (42.535) among the respective admissions. Other important causes of mortality were jejuno-ileal atresia (5.37%), trauma (5.05%), Hirschsprung disease (4.64%), intestinal perforation (4.48%), omphalocele (4.15%), burn (4.07%) and posterior urethral valves (PUV), 3.58%.  

Conclusion: Overall mortality rate was much higher than developed countries and lower than some African countries. Neonatal mortality rate was very high and among them TOF/OA and gastroschisis had extremely high mortality rates.

Open Access Original Research Article

Prevalence of Biochemical Abnormalities in Neonatal Seizures in Term and Preterm Neonates

K. M. Vinay, Goutham Shankargoud Patil, S. S. Prakash

Asian Journal of Pediatric Research, Page 35-45
DOI: 10.9734/ajpr/2020/v4i330152

Background: The most vulnerable period of life to develop seizures is the neonatal period. These events very often signify serious damage or malfunction of the immature developing central nervous system. Neonatal seizures may arise as a result of diverse etiologies and can have varied presentations. Biochemical abnormalities are commonly observed in neonates who are admitted in neonatal intensive care unit with seizures. Early recognition and treatment of biochemical disturbances is essential for optimal management and satisfactory long-term outcome.

Objective: To assess clinical types and importance of biochemical abnormalities in neonatal seizures and to evaluate clinical type & time of onset of seizures in term and preterm neonates.

Methods: A prospective observational study, where 100 neonates presenting with seizures admitted to neonatal intensive care unit of Jagadguru Jayadeva Murugarajendra Medical College, Davangere, from September 2015 to August 2017 were enrolled in the study. The detailed history along with clinical examination, baseline characteristics of convulsing neonate were recorded at admission. Clinical details of each seizure episode reported by the mother and subsequently observed by the resident doctors on duty were recorded. The relevant biochemical investigations were done immediately after baby had seizures and before instituting any specific treatment. The descriptive statistics such as mean and standard deviation (SD) for continuous variables, frequencies and percentages were calculated for categorical variables. The association between gestational age and other categorical variables were analyzed using chi-square test of independence. The comparison of mean of various quantitative variables was analyzed using ANOVA test. Etiology of neonatal seizures and associated biochemical abnormalities were diagnosed.

Results: In the present study, out of 100 neonates studied, 64 were full term of which 49(76.5%) were appropriate for gestational age and 15(23.5%) were small gestational age, whereas 36 cases were preterm. Most neonatal seizures occur in first 3 days of life, i.e. 59%. Most of them occurred on first day of life (34%). Birth asphyxia was the cause of neonatal seizures in 82.35% neonates who developed seizures on day-1 of life. Birth asphyxia and septicemia are common cause of neonatal seizures in our study (38 cases each), followed by pure metabolic disturbances 19%. In pure metabolic seizures, hypoglycemia (47.8%) is most common more in preterm babies (55%) followed by hypocalcemia. In cases of non- metabolic seizures, which showed associated biochemical abnormalities, hypoglycemia was most common abnormality 23 of 52 cases (44.2%). 12 cases (52.1%) are associated with birth asphyxia and 11 cases (47.9%) are associated with septicemia. Subtle seizures were most common type of seizures in our study, followed by focal clonic, multifocal clonic, generalized tonic, subtle with GTC and subtle with clonic.

Conclusion: Biochemical abnormalities are common in neonatal seizures and often go unorganized. These abnormalities may significantly contribute to seizure activity correction of these abnormalities work up is necessary for all cases of neonatal seizures.