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| Year:
2024 |
Month:
October
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Volume:
13 |
Issue:
4 |
Page:
PC05 - PC08 |
Pulmonary Alveolar Proteinosis in a Three-month-old Infant: An Unusual Case Report
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Correspondence
A Saranya, K Chandramouleeswari, M Dougul Regis,
Dr. M Dougul Regis,
Assistant Professor, Department of Pathology, Institute of Child Health and Hospital for Children, Madras Medial College, Chennai-600003, Tamil Nadu, India.
E-mail: dougulregis@gmail.com :
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Pulmonary Alveolar Proteinosis (PAP) is an unusual diffuse lung disease characterised by the alveolar accumulation of phospholipoprotein material, with a peak incidence in the 3rd to 4th decades of life and a male predominance. However, it has also been described in children. The recorded prevalence is 0.1 per 100,000 individuals. The major clinicopathogenetic subtypes include the autoimmune (idiopathic) form, which is associated with Granulocyte-macrophage Colony-stimulating Factor (GM-CSF) autoantibodies, the secondary form and the congenital form (associated with surfactant gene mutations). Common presenting features include dyspnoea, cough, low-grade fever, inspiratory crackles and digital clubbing. Pulmonary function tests typically show restrictive ventilatory defects. Herein, the authors present a case of a three-month-old male infant who presented with cough, dyspnoea and failure to thrive over the past 10 days. X-rays revealed a bilateral reticular pattern with non homogeneous opacities. Computed Tomography (CT) of the chest showed features consistent with interstitial lung disease. High-resolution Computed Tomography (HRCT) demonstrated a crazy-paving appearance, suggestive of interstitial lung disease or a surfactant-related genetic disorder. Bronchial Alveolar Lavage (BAL) yielded milky fluid. Characteristic microscopic findings on lung biopsy included the filling of terminal bronchioles and alveolar spaces with deep pink granular Periodic Acid Schiff (PAS)-positive eosinophilic material, while the alveolar architecture remained preserved. Patients with minimal symptoms are managed conservatively, whereas those with hypoxaemia require a more aggressive approach. Whole Lung Lavage (WLL) is the safest and most effective form of treatment. To date, there have been 240 case reports and 410 case series documented in the literature. This is the first case of PAP reported in the study institution. The authors present this case report here due to its rarity and diagnostic significance.
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