Pyruvate kinase (PK) deficiency in newborns: the pitfalls of diagnosis

J Pediatr. 2007 Apr;150(4):443-5. doi: 10.1016/j.jpeds.2007.01.039.

Abstract

Pyruvate kinase (PK) deficiency is asymptomatic in heterozygotes, but it can lead in homozygous neonates to a severe neonatal hemolysis, sometimes life-threatening. We report five cases, with a 1- to 17-month delayed diagnosis, highlighting the need to measure PK activity in neonates and parents in case of an hemolysis at birth.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Anemia / etiology
  • Female
  • Genetic Testing / methods
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Metabolism, Inborn Errors / complications
  • Metabolism, Inborn Errors / diagnosis*
  • Metabolism, Inborn Errors / genetics
  • Metabolism, Inborn Errors / therapy
  • Mutation / genetics
  • Neonatal Screening / methods
  • Polymorphism, Restriction Fragment Length
  • Pyruvate Kinase / deficiency*
  • Treatment Outcome

Substances

  • Pyruvate Kinase