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Pneumothorax in patients with severe combined immunodeficiency

Akihiro Hoshino1, Kohsuke Imai, Yusei Ohshima

  • 1Department of Pediatrics, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, Toyama, Japan.

Pediatrics International : Official Journal of the Japan Pediatric Society
|March 12, 2014
PubMed

Insights

Pneumothorax in infants with severe combined immunodeficiency (SCID) and Pneumocystis jirovecii pneumonia (PCP) is rare and often fatal. Prompt diagnosis and management are critical for improving outcomes in these critically ill infants.

Area of Science:

  • Pediatric Pulmonology
  • Immunology
  • Neonatology

Background:

  • Infants with pneumothorax often have underlying conditions.
  • Pneumocystis jirovecii pneumonia (PCP) is common in severe combined immunodeficiency (SCID).
  • This study investigates PCP-associated pneumothorax in SCID infants.

Observation:

  • Four SCID infants with pneumothorax and PCP were identified.
  • All patients required mechanical ventilation due to severe respiratory failure.
  • SCID diagnosis was concurrent with PCP diagnosis.

Findings:

  • PCP-associated pneumothorax in SCID infants carries a high mortality rate.
  • Only one of four patients survived after hematopoietic stem cell transplantation (HSCT).
  • Two patients died from respiratory failure; one died from HSCT complications.

Implications:

  • Pneumothorax in infants warrants investigation for underlying conditions like SCID and PCP.
  • Aggressive respiratory support and timely HSCT are crucial.
  • Early recognition and management can potentially improve outcomes in this vulnerable population.
Abstract

Keywords:
Pneumocystis jiroveciipneumothoraxsevere combined immunodeficiency

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