SFTPC gene mutation p.R167Q in a premature infant

Pediatr Pulmonol. 2014 Mar;49(3):E66-8. doi: 10.1002/ppul.22825. Epub 2013 Jun 17.

Abstract

We present an infant who was born premature at 23 weeks gestation with bronchopulmonary dysplasia and a SFTPC gene mutation, p.R167Q, who had a complicated neonatal course requiring 4 months of mechanical ventilation. Over time, his clinical course has improved, and he only requires oxygen by nasal cannula and low dose hydroxychloroquine, suggesting that p.R167Q mutation contributed to his clinical course and may manifest with a variable disease pattern making long-term prognostication difficult in the immediate newborn period.

Keywords: bronchopulmonary dysplasia; hydroxychloroquine; surfactant deficiency.

Publication types

  • Case Reports

MeSH terms

  • Anti-Inflammatory Agents / therapeutic use
  • Bronchopulmonary Dysplasia / complications
  • Bronchopulmonary Dysplasia / physiopathology*
  • Bronchopulmonary Dysplasia / therapy
  • Disease Progression
  • Humans
  • Infant, Extremely Premature
  • Infant, Newborn
  • Male
  • Mutation
  • Pulmonary Alveolar Proteinosis / complications
  • Pulmonary Alveolar Proteinosis / genetics*
  • Pulmonary Alveolar Proteinosis / physiopathology
  • Pulmonary Surfactant-Associated Protein C / deficiency
  • Pulmonary Surfactant-Associated Protein C / genetics*
  • Respiration, Artificial

Substances

  • Anti-Inflammatory Agents
  • Pulmonary Surfactant-Associated Protein C
  • SFTPC protein, human