Klubdaeng A, Tovichien P. Clinical approach for pulmonary alveolar proteinosis in children. World J Clin Cases 2024; 12(30): 6339-6345 [PMID: 39464322 DOI: 10.12998/wjcc.v12.i30.6339]
Corresponding Author of This Article
Prakarn Tovichien, MD, Associate Professor, Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, 2 Wanglang Road, Bangkok Noi, Bangkok 10700, Thailand. prakarn.tov@mahidol.edu
Research Domain of This Article
Respiratory System
Article-Type of This Article
Editorial
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Clin Cases. Oct 26, 2024; 12(30): 6339-6345 Published online Oct 26, 2024. doi: 10.12998/wjcc.v12.i30.6339
Clinical approach for pulmonary alveolar proteinosis in children
Anuvat Klubdaeng, Prakarn Tovichien
Anuvat Klubdaeng, Prakarn Tovichien, Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok 10700, Thailand
Author contributions: Klubdaeng A and Tovichien P designed the overall concept, outlined the manuscript, reviewed the literature, and wrote and edited the manuscript; All authors have read and approved the final manuscript.
Conflict-of-interest statement: Anuvat Klubdaeng and Prakarn Tovichien have nothing to disclose.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: Https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Prakarn Tovichien, MD, Associate Professor, Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, 2 Wanglang Road, Bangkok Noi, Bangkok 10700, Thailand. prakarn.tov@mahidol.edu
Received: March 12, 2024 Revised: July 2, 2024 Accepted: July 10, 2024 Published online: October 26, 2024 Processing time: 176 Days and 1.7 Hours
Abstract
In this editorial, we discuss the clinical implications of the article by Zhang et al. Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by excessive surfactant accumulation in the alveoli. It is classified into four categories: Primary, secondary, congenital, and unclassified forms. Primary PAP is caused by the disruption of granulocyte-macrophage colony-stimulating factor (GM-CSF) receptor signaling, which is necessary for the clearance of surfactant by alveolar macrophages. It is further divided into autoimmune PAP, caused by anti-GM-CSF antibodies blocking alveolar macrophage activation, and hereditary PAP, resulting from mutations in genes encoding GM-CSF receptors. Secondary PAP develops due to conditions affecting the number or function of alveolar macrophages, such as infections, immunodeficiency, hematological disorders, or exposure to inhaled toxins. Congenital PAP is linked to mutations in genes involved in surfactant protein production. Notably, the causes of PAP differ between children and adults. Diagnostic features include a characteristic "crazy-paving" pattern on high-resolution computed tomography, accompanied by diffuse ground-glass opacities and interlobular septal thickening. The presence of PAP can be identified by the milky appearance of bronchoalveolar lavage fluid and histological evaluation. However, these methods cannot definitively determine the cause of PAP. Whole lung lavage remains the standard treatment, often combined with specific therapies based on the underlying cause.
Core Tip: Both pulmonary alveolar proteinosis and X-linked agammaglobulinemia are rare diseases in children, each presenting unique challenges in diagnosis and treatment. This article aims to shed light on the clinical approach, diagnosis, and treatment processes for managing these conditions effectively.
