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PAM is a rare chronic disease with poorly defined etiology which shows clinico-radiological dissociation. It is characterized by deposition of calcospherites/microliths within alveoli in bilateral lung parenchyma with predominance in lower and mid zones causing dense micro- nodular opacities with classical sandstorm appearance with black pleura sign (due to subpleural cystic changes) on chest radiograph and micro- calcification, subpleural cystic changes and calcified pleura on HRCT.






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Pulmonary Alveolar Microlithiasis - Clinico-Radiological dissociation - A case report with Radiological review

Free full text article: Pulmonary Alveolar Microlithiasis -  Clinico-Radiological dissociation -  A case report with Radiological review

Abstract
Pulmonary alveolar microlithiasis (PAM) is a rare chronic lung disease characterized by deposition of intra alveolar calcium and phosphate in bilateral lung parenchyma with predominance in lower and mid zones. Etiology and pathogenesis is not fully understood. However, mutation in SLC34A2 gene that encodes a sodium phosphate co-transporter in alveolar type-II cells resulting in formation and accumulation of microliths rich in calcium phosphate due to impaired clearance is considered the cause of disease. Patients with PAM are asymptomatic till development of hypoxemia and cor pulmonale. It remains static, while in some it progresses to pulmonary fibrosis, respiratory failure and cor pulmonale. We report a case of 44 year old male patient presenting with progressive shortness of breath on exertion for one year in duration with dry cough, more since last six months. Chest radiograph showed dense micronodular opacities giving classical sandstorm appearance. High resolution computed tomography (HRCT) showed microcalcification, subpleural cystic changes and calcified pleura. Lung biopsy showed calcospherites within alveolar spaces.






References



1. Harbitz E. Extensive calcification of the lungs as a distinct disease. Arch Intern Med 1918; 21:139-46.
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2. J?nsson ?L, Simonsen U, Hilberg O, Bendstrup E. Pulmonary alveolar microlithiasis: two case reports and review of the literature. EurRespir Rev 2012 Sep 1; 21(125):249-56
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


3. Kashyap S1, Mohapatra PR. Pulmonary alveolar microlithiasis Lung India. 2013 Apr; 30(2):143-7
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


4. Castellana G, Lamorgese V. Pulmonary alveolar microlithiasis. World cases and review of the literature Respiration 2003; 70:549-55
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


5. Castellana G1, Gentile M, Castellana R, Fiorente P, Lamorgese V. Pulmonary alveolar microlithiasis: clinical features, evolution of the phenotype, and review of the literature. Am J Med Genet 2002 Aug 1; 111(2):220-4
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


6. Abdalla G1, Marchiori E, Zanetti G, Mucillo A, Pereira ML, Ventura N, Martins P, Constantino CP, Canellas R, Brandão V, Varella de Oliveira R. Pulmonary alveolar microlithiasis: a case report with emphasis on imaging findings. Case Rep Med 2010; 2010:819242
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


7. Pracyk JB, Simonson SG, Young SL, Ghio AJ, Roggli VL, Piantadosi CA. Composition of lung lavage in pulmonary alveolar microlithiasis. Respiration 1996; 63:254-60
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


8. Siddiqui NA, Fuhrman CR. Best cases from the AFIP: Pulmonary alveolar microlithiasis. Radiographics 2011 Mar-Apr; 31(2):585-90
Get full text
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


9. Webb WR. Diffuse lung diseases associated with calcification. In: Webb WR, Higgins CB (eds.) Thoracic imaging: Pulmonary and cardiovascular imaging
Find similar topics on Read this article on PubMed :: Find similar articles on Google scholarScholar :: Search for similar topics with the Radiology specific search engine Radiology search engine


10. Mariotta S, Ricci A, Papale M, De Clementi F, Sposato B, Guidi L. Pulmonary alveolar microlithiasis: Report on 576 cases published in the literature. Sarcoidosis Vasc Diffuse Lung Dis 2004; 21:173-81
Get full text
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1. Pulmonary Alveolar microlithiasis

2. HRCT

3. Type-II sodium dependent phosphate co-transporter

4. calcospherites

5. microliths and micronodular opacities


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