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.
MeSH terms
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Adult
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Amino Acids / blood
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Amino Acids / urine
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Carbon-Carbon Ligases*
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Carnitine / blood
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Carnitine / therapeutic use
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Carnitine / urine
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Citrates / urine
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Citric Acid
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Female
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Fetal Growth Retardation / etiology
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Humans
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Ketones / urine
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Lactates / urine
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Lactic Acid* / analogs & derivatives*
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Ligases / blood
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Maple Syrup Urine Disease / complications
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Maple Syrup Urine Disease / diet therapy*
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Maple Syrup Urine Disease / drug therapy*
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Pregnancy
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Pregnancy Complications / blood*
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Pregnancy Complications / urine*
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Propionates / blood*
Substances
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Amino Acids
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Citrates
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Ketones
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Lactates
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Propionates
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Citric Acid
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Lactic Acid
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hydracrylic acid
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Ligases
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Carbon-Carbon Ligases
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propionyl CoA carboxylase (ATP-hydrolyzing)
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propionic acid
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Carnitine