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Journal of Child Neurology
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A Patient With Propionic Acidemia Managed With Continuous Insulin Infusion and Total Parenteral Nutrition

Avedis Kalloghlian, MD

Department of Pediatrics, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia

Helmut Gleispach, PhD

Department of Biological and Medical Research, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia

P.T. Ozand, MD, PhD

Department of Pediatrics, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia, Department of Biological and Medical Research, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia

An infant newly diagnosed with propionic acidemic coma was managed successfully with total parenteral nutrition (TPN) and continuous infusion of insulin. The urinary excretion of 3-hydroxypropionic acid was reduced to 3% of the admission value in 4 days, gradually decreasing to 1.5% in 16 days. The treatment did not prevent a prolonged episode of thrombocytopenia. The infant tolerated TPN well, except for continued hyper-lactic acidemia (2 to 4 times normal). Metabolic acidosis and mild hyperammonemia recurred only when the patient had sepsis secondary to Candida albicans and Staphylococcus aureus infection. ( J Child Neurol 1992;7(Suppl):S88-S91.)

Journal of Child Neurology, Vol. 7, No. 1 Suppl, S88-S91 (1992)
DOI: 10.1177/08830738920070011411
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Home page
 J Child NeurolHome page
P. T. Ozand, E. B. Devol, and G. G. Gascon
Neurometabolic Diseases at a National Referral Center: Five Years Experience at the King Faisal Specialist Hospital and Research Centre
J Child Neurol, April 1, 1992; 7(1_suppl): S4 - S11.
[Abstract] [PDF]