THE SEVERE PULMONARY ALVEOLAR PROTEINOSIS IN A CHILD: CASE REPORT AND TREATMENT CHALLENGES

Abstract

Introduction: Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by the accumulation of surfactant in the alveoli, leading to worsened ventilatory function, primarily affecting young children and newborns. Imaging studies and biopsy are the main diagnostic tools. Lung lavage is the most effective treatment.

Objective: To describe a case report of severe pulmonary alveolar proteinosis in a child and demonstrate the challenges of total lung lavage in cases of severe lung impairment.
Methodology: This case report was developed through research articles from the PUBMED and BVS (BBO and MEDLINE) databases, as well as literature from reference authors and other reports concerning the disease.

Case Report: A 6-year-old patient was admitted with severe dyspnea and bilateral pulmonary infiltrates, showing no response to initial treatment. After 18 days, she was intubated due to ventilatory failure. A CT scan revealed a pattern of diffuse lung disease with proteinaceous deposits. Since surgery was contraindicated, a bronchoscopy with biopsy was performed, confirming the diagnosis of alveolar proteinosis. A total lung lavage was planned and conducted 12 days post-intubation, resulting in immediate improvement in the patient's ventilation. Due to the duration of intubation, a tracheostomy was performed, leading to good recovery and discharge after 58 days. Six months later, she presented with new respiratory complaints and underwent another procedure, with positive outcomes.

Conclusion: Alveolar proteinosis highlights the complexity in managing interstitial respiratory diseases. The initial outcome of the case emphasizes the importance of accurate differential diagnosis and rapid therapeutic adaptation. The success of treatment, culminating in significant recovery and discharge after a prolonged period, underscores the relevance of precise diagnosis and treatment. This not only contributes to the understanding of rare pulmonary diseases but also emphasizes the importance of flexible treatment protocols that can be adapted to each clinical situation.

Keywords:Pulmonary Alveolar Proteinosis, Respiratory Failure, Respiratory Distress Syndrome, Children.