1. ABSTRACT
Nutritional Status of Children with Branched-Chain Amino Acids Disorders
in Riyadh City
Prepared By: Suzanne Mansour Alodaib
King Saud University – Collage of Food Sciences and Agriculture – Food Sciences and
Nutrition.
This study aims to evaluate the nutritional status of children with branched-
chain amino acids disorders (Methylmalonic Acidemea MMA, Propionic Acidemea PPA, Maple
Syrup Urine Disease MSUD). Methods: A two-year observational multicenter outpatient study
was conducted with children suffering from MMA, PA and MSUD. Main outcome measures
were anthropometrics ( height, weight, head circumference), protein status indices, diet records,
amino acids levels, ammonia and creatinine. Results: Fifteen patients completed the study. By
study end, there were significant differences between the affected children and the control group
in growth (p≤0.05). Required daily protein intake shows no significant values between the two
groups.
The amino acids concentrations were taken monthly during the study period and
then divided into three stages. The results showed that the concentration of Isoleucine in MMA
and PPA patients were significantly reduced (p≤0.05). Also, Valine was reduced in all patients
compared with control group (p≤0.05). Though, Glycine levels in PPA patients were
significantly higher (p≤0.05) than they are in control group, the same observation was for MMA
patients but at beginning of the study.
Ammonia were high in all patients (p≤0.05) while albumin were low (p≤0.05)
compared with the control group. Moreover, there were no significant differences in prealbumine
concentration between patients and control group..
Through this study, some children with branched-chain amino acids disorders
have a poor growth. All affected children had low Valine concentrations, while all PPA patients
had shown high levels of Glycine in plasma.