Clinical spectrum of inborn errors of metabolism in children in a tertiary care hospital

Background: Inborn Errors of Metabolism (IEM) are a group of disorders occurring due to disruption of normal biochemical process. Prompt diagnosis is often only the beginning of a long medical journey for the affected children and their family. Pediatricians play a vital role in establishing the continuity of care, providing treatment when needed and referrals to specialists. Reported prevalence of IEM is 1 in 2497 newborn though, true pan India prevalence is still unknown. This study was carried to determine the clinical spectrum of inborn errors of metabolism in a tertiary care hospital in South India.Methods: Records of all patients suspected and diagnosed to have inborn errors of metabolism in Institute of Child Health and Hospital for Children, Madras Medical College from April 2018 to October 2019 were sequentially included in the study. Details of clinical presentation, investigations and treatment were noted and analysed.Results: In this study 65 children diagnosed to have inborn errors of metabolism were included in the study and of them 27(41.5%) had derangement in carbohydrate metabolism, 16(24.6%) in protein metabolism and 22(33.9%) in lipid metabolism. Mean age at presentation was 37 months with range of 2 months to 10 years of age. Most common clinical manifestation was poor feeding (67.7%) followed by fever (64.6%) and dyspnea (63.1%). Of these 65 children, mortality was observed in 10 cases (15%).Conclusions: IEM contribute to a significant cause of global child morbidity and mortality. A high index of suspicion is most important in making the diagnosis. IEM should be considered in children with features mimicking sepsis and unexplained course. Screening programmes and prenatal diagnosis of IEM will go a long way in preventing these disorders and early diagnosis helps initiate prompt therapy which is very much essential to prevent lethal complications.

Effect of tween 80 added to the soil on the degradation of endosulfan by pseudomonas aeruginosa

Endosulfan, a chlorinated cyclodiene insecticide is of environmental concern because of its apparent persistence and toxicity to many non target organisms. Endosulfan is hydrophobic and persists in soil for more than a year. To overcome the problem of hydrophobic and limited availability, surfactants play a major role in soil remediation. In the present study, the effect of Tween 80 added to the soil on the degradation of endosulfan by Pseudomonas aeruginosa at different pH [7.0 and 8.5] was studied. The addition of synthetic surfactant Tween 80 enhanced the solubility and degradation of endosulfan. A significant degradation [94%] was observed in pH 8.5 and Tween 80 added soil; the bacterial population in the treatment unit T8 was 75 x 109 CFU/g of soil. The unit T4 inoculated at pH 8.5 showed 86% alpha and 60% beta endosulfan degradation, the bacterial population was 73 x 108 CFU / g of soil. The degradation of both the isomers were observed and accompanied with formation of endodiol and endosulfan sulfate

Surfactants enhanced recovery of endosulfan from contaminated soils

Soil pollution due to hydrophobic organic compounds is a wide environmental problem. Extensive use of endosulfan in cotton and paddy to meet the needs of the growing population has led to contamination of soil and other ecosystems. Endosulfan is hydrophobic, highly toxic to aquatic and human population and persists in soil for more than a year. To overcome the problem of hydrophobicity and limited availability, surfactants play a major role in soil remediation. In the present study, the potential of non-ionic synthetic surfactants [Tween 80, Triton X-100] and biosurfactant [Surfactin produced by Bacillus subtilis] for enhancing the release of endosulfan from contaminated agricultural soils was evaluated using the batch method. Incorporation of the surfactant concentrations at above Criticle Miceller Concentration [0.5, 1 and 2 g/L] into soil enhances the release of endosulfan. Surfactin produced from Bacillus subtilis recorded maximum [91.5%] recovery. The observed order of recovery being surfactin > Tween 80 > Triton X-100. The result suggest that surfactants could help in the remediation of soils polluted by pesticides

Prevalence of overweight and obesity in affluent adolescent girls in Chennai in 1981 and 1998.

To assess the prevalence of obesity and overweight in adolescent girls between 10-15 years of age, among the affluent families of Chennai--two studies are compared using body mass index (BMI) as a parameter. The first study done in the year 1981 (Group I) was compared with the second study in 1998 (Group II). Group I had 707 and group II had 610 girls. Overweight and obesity were denoted by BMI above 85th and 95th percentile respectively. Results showed a 9.6% prevalence of overweight and 6% prevalence of obesity in both studies. It was also observed that the BMI for the same age in the two study periods showed an increase from 1981 to 1998. BMI approximated the international reference values for BMI at age 13 years in the year 1998.