scholarly journals Pulmonary Alveolar Microlithiasis: A Unique Case of Familial PAM Complicated by Transplant Rejection

2021 ◽  
Vol 2021 ◽  
pp. 1-4
Author(s):  
Austin Helmink ◽  
Samir Atiya ◽  
Ernesto Martinez Duarte
Keyword(s):  
Lung Disease ◽  
Lung Transplant ◽  
Unusual Case ◽  
Transplant Rejection ◽  
Alveolar Cells ◽  
Pulmonary Alveolar Microlithiasis ◽  
Hypoxic Respiratory Failure ◽  
Type Ii Alveolar Cells ◽  
Bilateral Lung ◽  
Alveolar Microlithiasis

Background. Pulmonary alveolar microlithiasis (PAM) is a rare lung disease characterized by the deposition of calcium phosphate microliths or calcospherites, within the alveolar airspace. Typical imaging findings demonstrate a “sandstorm” appearance due to bilateral, interstitial sand-like micronodularities with basal predominance. Methods and Results. We describe an unusual case of a 48-year-old male with severe, familial PAM ultimately treated with a bilateral lung transplant. Conclusions. PAM is a rare lung disease caused by a mutation in the SLC34A2 gene, which encodes for a sodium-phosphate cotransporter in type II alveolar cells, leading to accumulation of intra-alveolar phosphate causing microlith formation. PAM has an indolent course but can progress to chronic hypoxic respiratory failure, ultimately requiring lung transplant, the only known effective treatment.

Bilateral lung transplant: the procedure of choice for end-stage septic lung disease

2001 ◽  
Vol 33 (1-2) ◽  
pp. 1622-1623 ◽  
Author(s):  
J.N Rao ◽  
J Forty ◽  
A Hasan ◽  
C.J Hilton ◽  
S Ledingham ◽  
...  
Keyword(s):  
Lung Disease ◽  
Lung Transplant ◽  
Bilateral Lung ◽  
End Stage

scholarly journals PULMONARY ALVEOLAR MICROLITHIASIS. DISCREPANCIES BETWEEN RADIOLOGICAL FINDINGS AND CLINICAL PATTERN- CASE STUDY

2021 ◽  
Vol 74 (9) ◽  
pp. 2235-2240
Author(s):  
Aleksandra Oraczewska ◽  
Marek Ochman ◽  
Mariola Ograbek-Król ◽  
Wiktoria Kowalska ◽  
Klaudia Glinka ◽  
...  
Keyword(s):  
Lung Transplantation ◽  
Computer Tomography ◽  
Lung Transplant ◽  
Genetic Disorder ◽  
Clinical Signs ◽  
Pulmonary Alveolar Microlithiasis ◽  
Computer Tomography Scan ◽  
Alveolar Microlithiasis ◽  
Tomography Scan

Pulmonary alveolar microlithiasis is a rare genetic disorder, inherited autosomally recessively, which is characterized by intra-alveolar deposition of microliths built mostly of calcium salts and phosphorus. This case study describing management of patient with pulmonary alveolar microlithiasis. A 49-year-old woman, diagnosed with pulmonary microlithiasis in 1979 was admitted to Pneumology Department due to increased dyspnea. On admission there were no clinical signs of active infection. The chest computer tomography scan confirmed the presence of advanced microlithiasis. Pulmonary function test revealed mild restriction with moderate diffusion impairment, due to severe hypoxemia present on 6-minute walking test patient was sent for specific assessment to local lung transplant team in Zabrze for consideration for lung transplantation. According to International Society for Heart & Lung Transplantation guidelines the patient was observed in 6 months intervals to reveal whether further disease progression will be observed. Clinical condition of our patient does not correlate with radiological scans, severe respiratory symptoms and cardiological complications. Computer tomography scan should not be the only indication for lung transplant.

Sequential bilateral lung transplantation for pulmonary alveolar microlithiasis

1993 ◽  
Vol 56 (4) ◽  
pp. 972-975 ◽  
Author(s):  
George Stamatis ◽  
Hans-reinhard Zerkowski ◽  
Norbert Doetsch ◽  
Dieter Greschuchna ◽  
Nikolaus Konietzko ◽  
...  
Keyword(s):  
Lung Transplantation ◽  
Pulmonary Alveolar Microlithiasis ◽  
Bilateral Lung Transplantation ◽  
Bilateral Lung ◽  
Alveolar Microlithiasis

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
Keyword(s):  
Lung Transplantation ◽  
Lung Disease ◽  
Effective Treatment ◽  
Surgical Management ◽  
Poor Prognosis ◽  
State Of The Art ◽  
Pulmonary Alveolar Microlithiasis ◽  
Current State ◽  
Treatment Paradigm ◽  
Alveolar Microlithiasis

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.

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