scholarly journals Comprehensive Imaging-Guided Optimization of the Surgical Management of Patients with Pulmonary Alveolar Microlithiasis

2020 ◽  
pp. 1-3
Author(s):  
Laurent Dercle ◽  
Aiping Chen ◽  
Fatima-Zohra Mokrane ◽  
Laurent Dercle

Pulmonary alveolar microlithiasis (PAM) is a rare lung disease with poor prognosis. The only effective treatment to date, is lung transplantation in the severest cases. However, the etiology of PAM has been recently deciphered, and the treatment paradigm is shifting. We report a case of PAM and propose an optimized imaging-guided management based on the current state of the art.

2010 ◽  
pp. 3454-3455
Author(s):  
D.J. Hendrick

Pulmonary alveolar microlithiasis is caused by mutations of the type IIb sodium phosphate cotransporter gene, which by an unknown mechanism leads to the accretion of calcified microliths in the lungs. Almost invariably the patient is symptom free when the diagnosis is made after a chest radiograph is taken incidentally (or during family screening) and reveals profuse small calcified nodules. Patients usually survive 10–20 years from diagnosis, lung transplantation being the only effective treatment in severe cases....


2007 ◽  
Vol 84 (1) ◽  
pp. 290-292 ◽  
Author(s):  
Timothy J.P. Batchelor ◽  
Dumbor L. Ngaage ◽  
Damian V. McGivern ◽  
Michael E. Cowen

2020 ◽  
pp. 4265-4267
Author(s):  
S. J. Bourke

Pulmonary alveolar microlithiasis is characterized by the deposition of calcium phosphate in the alveolar air spaces as a result of mutations of the SLC34A2 gene. The diagnosis is often made before symptoms have developed when a chest radiograph is performed for other reasons, and shows a dramatic typical ‘sandstorm’ pattern of diffuse bilateral calcified micronodules. The patient is often symptom-free when the diagnosis is made after a chest radiograph is taken incidentally and reveals calcified micronodules, but typically the disease progresses to respiratory failure over about 10–20 years. Etidronate has led to improvement in some cases that have been detected early. Lung transplantation is the main option in advanced disease. The severity of the disease and prognosis are variable, and this may be influenced by the specific type of gene mutation. Survival of 10–20 years from the onset of symptoms is typical.


2013 ◽  
Vol 107 (1) ◽  
pp. 1-9 ◽  
Author(s):  
Flávia Angélica Ferreira Francisco ◽  
Jorge Luiz Pereira e Silva ◽  
Bruno Hochhegger ◽  
Gláucia Zanetti ◽  
Edson Marchiori

2010 ◽  
Vol 139 (3) ◽  
pp. e50-e52 ◽  
Author(s):  
Norihisa Shigemura ◽  
Christian Bermudez ◽  
Brack G. Hattler ◽  
Bruce Johnson ◽  
Maria Crespo ◽  
...  

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