Abstract :
Pulmonary alveolar microlithiasis (PAM) is considered as one of the rarest diseases of respiratory system with chronicity in order. Its development is usually attributed to a genetic defect in phosphate transport mechanism, however comprehensible pathogenesis is not known, leading to accretion of calcium and phosphate units in the alveoli. These calcium-phosphate units resemble calculi, hence, called microliths. Concurrently, the name of the disease. The most common mutation associated with PAM found till date is the mutation of SLC34A2. In alveolar type II cells, the SLC34A2 gene, which codes for a sodium-phosphate co-transporter, causing microliths to accumulate and develop (owing to defective clearance). The clinical presentation of the disease has a variable spectrum. Radiology imaging remains the cornerstone of diagnosis for this disease. . A big lacuna is present in the field of research when it comes to this disease, ergo no standardized or definitive treatment at hand even today except lung transplantation.
Keyword :
PAM, Microlithiasis, Phosphate transport, Alendronate