EVALUATION OF APPLIED DIETARY MANAGEMENT IN PATIENTS WITH UREA CYCLE DISORDERS

Document Type : Original Article

Authors

1 Genetic Research Unit, Ain Sham University Hospital Biochemistry

2 Department. Faculty of Agriculture, Ain Shams University

Abstract

Urea cycle disorders (UCDs) represent a group of rare inherited metabolic disorders resulting from a partial or complete deficiency of one of the urea cycle components, thereby resulting in accumulation of ammonia, as well as other nitrogenous products, including glutamine and alanine. However, hyperammonemia results in cerebral edema and neurologic injury. These disorders may present at birth, childhood, or adulthood and may range from a relatively mild encephalopathy to profound developmental disability. Early diagnosis and treatment may prevent some of the adverse outcomes, which are primarily neurological.
In the present work child growth parameters were used to check efficiency of dietary that introduce to children, through four growth parameters that including length for age, weight for age, head circumference for age and body mass index (BMI) for age. The study revealed the prevalence between the onset of diagnosis and follow up based on anthropometric measurements was 50% stunted, 25% underweight and 37% wasted for BMI, compared to 87.5%, 75% and 25% respectively for the same parameters, after applied urea cycle disorders diet. This finding shows that even with adequate management of cases with urea cycle disorders also, some fractions of cases were stunted, underweight, wasted and microcephaly.

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Journal of Environmental Science
Volume 49, Issue 4 - Serial Number 4
: Part 7 Agriculture
April 2020
Pages 59-80

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