Assessment of Mothers Care towards their Children Suffering from Branched Chain Keto-Aciduria (BCKA)

Abstract

Branched-chain keto-aciduria (BCKA) also called maple syrup urine disease (MSUD) is an autosomal recessive disease caused by defective function of Branched-Chain α-Keto-acid Dehydrogenase complex (BCKD). It was now considered one of the commonest metabolic disordered. The screening test should be done within one week of birth. In Egypt, this screening is not routine consequently, it was discovered late and numbers of pediatric patients with BCKA increasing. Aim of study The present study was conducted with the aim of study was to assess mothers care for children suffering from Branched Chain Keto Aciduria through: 1. Assess the mother`s knowledge about BCKA. 2. Assess the mother`s self-reported practice toward care their children suffering from BCKA. Research questions 1. What is the mother`s knowledge about the care of their children suffering from BCKA? 2. What the mother`s self-reported practices toward caring for their children suffering from BCKA? 3. Is there a relation between the mother`s characteristics and their care for children suffering from BCKA?