A recurrent ACAA2 variant causes a dominant syndrome of lipodystrophy, lipomatosis, infantile steatohepatitis, and hypoglycemia.

Simha, Vinaya; LoPiccolo, Mary Kate; Platt, Anna; Brown, Rebecca J; Johnson, Xandria; Carere, Deanna Alexis; Donnelly, Colleen; Snyder, Matthew T et al. · J Clin Invest · 2026

case_report · Level V

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Abstract

We report a novel variant in ACAA2 that causes hepatitis and hypoglycemia during infancy and lipodystrophy during adulthood accompanied by elevated plasma long chain acylcarnitines.

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