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Pulmonary Alveolar Proteinosis in Children: Diagnosis and Treatment Outcomes

Maryam Hassanzad1, Sabereh Tashayoie-Nejad1, Mohammadreza Boloursaz1

  • 1Pediatric Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Tanaffos
|October 21, 2022
PubMed

Insights

Pulmonary Alveolar Proteinosis (PAP) treatment using lung lavage was effective in improving patient prognosis. Regular follow-up care is recommended to enhance therapeutic efficacy and patient longevity in PAP cases.

Area of Science:

  • Pulmonology
  • Pediatric Medicine
  • Critical Care

Background:

  • Pulmonary Alveolar Proteinosis (PAP) is a rare lung disease caused by impaired surfactant clearance.
  • Accumulation of surfactant in alveoli leads to respiratory compromise.
  • Current standard treatment involves lung lavage, with specific indications for its performance.

Purpose of the Study:

  • To evaluate the efficacy of lung lavage in treating pediatric patients with Pulmonary Alveolar Proteinosis.
  • To analyze patient demographics, diagnostic methods, and treatment outcomes.

Main Methods:

  • Retrospective study of 17 pediatric PAP patients over 15 years.
  • Data collection included demographics, clinical presentation, diagnosis (Transbronchial Lung Biopsy/open lung biopsy), and treatment (lung lavage).

Main Results:

  • 17 pediatric patients (7 males, 10 females) diagnosed with PAP.
  • Lung lavage was the primary treatment for 15 patients.
  • Five patients (29.4%) died due to severe disease complications.

Conclusions:

  • Whole Lung Lavage (WLL) is effective for Pulmonary Alveolar Proteinosis patients meeting therapeutic indications.
  • WLL plays a significant role in improving prognosis for PAP patients.
  • Regular follow-up is crucial for maximizing therapeutic benefits and patient survival.
Abstract

Keywords:
DiagnosisOutcomePulmonary alveolar Proteinosis

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