A study of the response to protein-modified diets for propionic acidemia in twelve patients.
The charts of twelve patients with propionic acidemia (PA) were analyzed retrospectively for the outcome of dietary treatment. The diet was prescribed for each patient based on their individual needs. We established good diet compliance in four PA patients who were alive and well with healthy life-styles at the time of this report. The main difficulty causing non-compliance was the parents' inability to take care of the patient due to other family responsibilities. In two cases, the parents believed their children to be cured and they abandoned diet and medications, resulting in the sudden death of both children. In four cases, the parents were unable to cope with the demands of the diet and medications, while complications and severe classic PA provided no hope for two patients.
Thomas
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Department of Food and Nutrition Services, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.
D000596:Amino Acids / Q000008:administration & dosage; D001927:Brain Diseases / Q000178:diet therapy* / Q000209:etiology; D002675:Child, Preschool; D004032:Diet; D004734:Energy Metabolism / Q000502:physiology; D005260:Female; D005526:Food, Formulated; D006128:Growth; D006801:Humans; D007223:Infant; D008297:Male; D008661:Metabolism, Inborn Errors / Q000097:blood / Q000150:complications / Q000178:diet therapy*; D010349:Patient Compliance; D011422:Propionates / Q000097:blood*; D012189:Retrospective Studies
D000596:Amino Acids; D011422:Propionates