Case reports of successful pregnancy in women with maple syrup urine disease and propionic acidemia.

Abstract

We report on 2 women with organic acidemias, one with classical maple syrup urine disease and another with mild propionic acidemia in which protein restricted diets and carnitine supplementation were successfully employed to manage pregnancies. Healthy infants were delivered without maternal metabolic decompensation.

• Authors: S C Van Calcar, C O Harding, S R Davidson, L A Barness, J A Wolff
• Journal: American journal of medical genetics (Am J Med Genet) Vol. 44 Issue 5 Pg. 641-6 (Nov 15 1992) ISSN: 0148-7299 [Print] United States
• PMID: 1481826 (Publication Type: Case Reports, Journal Article)

Chemical References

• Amino Acids
• Citrates
• Ketones
• Lactates
• Propionates
• Citric Acid
• Lactic Acid
• hydracrylic acid
• Ligases
• Carbon-Carbon Ligases
• propionyl CoA carboxylase (ATP-hydrolyzing)
• propionic acid
• Carnitine

Topics

• Adult
• Amino Acids (blood, urine)
• Carbon-Carbon Ligases
• Carnitine (blood, therapeutic use, urine)
• Citrates (urine)
• Citric Acid
• Female
• Fetal Growth Retardation (etiology)
• Humans
• Ketones (urine)
• Lactates (urine)
• Lactic Acid (analogs & derivatives)
• Ligases (blood)
• Maple Syrup Urine Disease (complications, diet therapy, drug therapy)
• Pregnancy
• Pregnancy Complications (blood, urine)
• Propionates (blood)