Premature pubarche in children with Pompe disease.

Tan, Queenie K-G; Stockton, David W; Pivnick, Eniko; Choudhri, Asim F; Hines-Dowell, Stacy; Pena, Loren D M; Deimling, Melissa A; Freemark, Michael S et al. · J Pediatr · 2015

case_series · Level IV

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Abstract

Pompe disease (PD), or glycogen storage disease type II, results from deficiency of acid α-glucosidase. Patients with infantile-onset PD die by early childhood if untreated. Patient survival has improved with enzyme replacement therapy. We report a case series of 8 patients with infantile-onset PD on enzyme replacement therapy with premature pubarche.

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