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Potentially life-threatening cardiac dysrhythmia in a child with selenium deficiency and phenylketonuria

L G Greeves1, D J Carson, B G Craig

  • 1Department of Child Health and Medicine, Royal Belfast Hospital for Sick Children, Queen's University, Belfast, N. Ireland.

Acta Paediatrica Scandinavica
|December 1, 1990
PubMed

Insights

Children with phenylketonuria (PKU) on dietary treatment often have selenium deficiency. This case highlights episodic ventricular tachycardia in an infant with PKU, linked to low selenium levels.

Area of Science:

  • Biochemistry
  • Pediatrics
  • Cardiology

Background:

  • Phenylketonuria (PKU) requires strict dietary management, often leading to nutrient deficiencies.
  • Selenium (Se) is an essential trace element crucial for antioxidant defense and overall health.
  • Glutathione peroxidase (GSHPX) is a key selenoenzyme involved in protecting cells from oxidative damage.

Observation:

  • A 9-month-old infant with PKU presented with episodic ventricular tachycardia.
  • The infant exhibited significantly low serum selenium (Se) levels (0.13 mumol/l) and low whole blood glutathione peroxidase (GSHPX) activity (16 U/g Hb).
  • These biochemical markers indicated a state of selenium deficiency.

Findings:

  • A direct correlation was observed between PKU dietary management, selenium deficiency, and cardiac dysrhythmia.
  • The infant's low Se and GSHPX levels suggest impaired antioxidant capacity, potentially contributing to cardiac issues.
  • Ventricular tachycardia in this context points to a possible novel complication of PKU treatment.

Implications:

  • Routine cardiac auscultation is advised for young children with PKU during follow-up.
  • Selenium deficiency should be considered as a potential etiological factor in cardiac dysrhythmias in PKU patients.
  • Prompt Se supplementation may be necessary to correct deficiency and manage associated cardiac complications.

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