Clinical approach for pulmonary alveolar proteinosis in children

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"crazypaving"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.

Diffuse panbronchiolitis in children misdiagnosed as asthma: A case report

BACKGROUND Diffuse panbronchiolitis(DPB)is a rare,chronic inflammatory lung disease mar-ked by chronic cough,breathlessness,and preceding sinusitis.Symptoms often persist for years and can be misdiagnosed as asthma,particularly in children.This report describes a DPB case resolved with long-term azithromycin therapy,em-phasizing the need for a timely and accurate diagnosis.CASE SUMMARY A 12-year-old girl,diagnosed with asthma at age five and managed with inhaled corticosteroids and long-acting beta-2 agonists,developed a history of chronic productive cough and chronic sinusitis for a year.On examination,she exhibited wheezing and coarse crackles.Despite receiving treatment for an asthma exacer-bation,her symptoms did not improve.A chest X-ray revealed reticulonodular infiltration in both lower lungs,prompting further evaluation with high-resolu-tion computed tomography(HRCT).The HRCT confirmed centrilobular nodule opacities,a'tree-in-bud'pattern,and non-tapering bronchi,suggesting DPB.Elevated cold hemagglutinin titers at 128 further supported the diagnosis.Her cough and sinusitis resolved within a month after starting azithromycin therapy,chosen for its anti-inflammatory and immunomodulatory effects.Follow-up HRCT scans after 1 year of continuous treatment showed complete normalization.CONCLUSION This case highlights the importance of early diagnosis and prompt treatment in achieving favorable outcomes for DPB.