What cardiac complications occur in propionic acidemia?

Dilated cardiomyopathy occurs in 23-26% of PA patients and can be the presenting or life-threatening complication; QTc prolongation also occurs independently.

How is metabolic control assessed in PA?

Plasma propionylcarnitine (C3), plasma ammonia, blood gas bicarbonate, and urinary organic acids (3-hydroxypropionate, methylcitrate) are key monitoring parameters.

What is the role of biotin in PA treatment?

Biotin supplementation (10-40 mg/day) is indicated when biotin-responsive propionyl-CoA carboxylase deficiency is confirmed; most cases are not biotin-responsive.

When should liver transplantation be considered in PA?

Liver transplant reduces metabolic decompensations in PA but does not prevent cardiac, neurological, or renal complications; indicated for recurrent life-threatening crises.

What are neurological complications of PA?

Basal ganglia injury (especially globus pallidus) during crises, cortical atrophy, and peripheral neuropathy occur; metabolic strokes can cause permanent deficits.

Propionic Acidemia Monitoring Calculator

Monitors propionic acidemia metabolic control using plasma propionylcarnitine, ammonia, bicarbonate, and ketones with recommendations for biotin and carnitine supplementation and cardiac surveillance.

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Frequently Asked Questions

What cardiac complications occur in propionic acidemia?

How is metabolic control assessed in PA?

What is the role of biotin in PA treatment?

When should liver transplantation be considered in PA?

What are neurological complications of PA?