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A pediatric case of relapsed pulmonary alveolar proteinosis despite successful whole lung lavage

Seung Young Jin1, Hye Ri Yun1, Yun Jung Choi1

  • 1Department of Pediatrics, Seoul National University Hospital, Seoul, Korea.

Korean Journal of Pediatrics
|September 2, 2017
PubMed

Insights

Pulmonary alveolar proteinosis (PAP) in children is rare. This case details a pediatric patient who relapsed after whole lung lavage (WLL), highlighting a challenge in treating this severe lung disease.

Area of Science:

  • Pediatric Pulmonology
  • Rare Diseases
  • Respiratory Medicine

Background:

  • Pulmonary alveolar proteinosis (PAP) is a rare pediatric lung disease.
  • Characterized by surfactant protein accumulation, it impairs gas exchange.
  • Whole lung lavage (WLL) is the primary treatment for severe pediatric PAP.

Purpose of the Study:

  • To report a pediatric case of PAP treated with WLL.
  • To document the first reported case of PAP relapse in a child despite successful WLL in Korea.

Main Methods:

  • Diagnosis of PAP confirmed via video-assisted thoracoscopic lung biopsy.
  • Therapeutic WLL performed on each lung on separate days as a first-line treatment.
  • Repeat WLL administered due to symptom relapse.

Main Results:

  • Initial WLL provided brief symptom relief in an 11-year-old boy.
  • The patient experienced symptom exacerbation and relapse post-initial treatment.
  • This represents the first documented pediatric PAP relapse in Korea following WLL.

Conclusions:

  • Whole lung lavage may not always provide a long-term solution for pediatric PAP.
  • Relapse of PAP after WLL presents a significant clinical challenge.
  • Further research into long-term management strategies for pediatric PAP is warranted.

Keywords:
ChildPulmonary alveolar proteinosisRecurrenceTherapeutic irrigationVideo-assisted surgery

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