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Pulmonary Alveolar Microlithiasis “ Stone Lungs ” : A Case of Clinico-Radiological Dissociation

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ABSTRACT

Pulmonary alveolar microlithiasis (PAM) is a rare infiltrative lung disease characterized by deposition of spherical calcium phosphate microliths called calcospherites within the alveoli. PAM was first described by Friedrich in 1856 and then by Harbitz in 1918. The disease pathogenesis is based on mutations in the SLC34A2 gene that encodes for the Type IIb sodium-phosphate cotransporter. The majority of the patients are diagnosed at an early age, usually between the ages of 20 and 40 years. The hallmark of this disease is a striking dissociation between the radiological findings and the mild clinical symptoms. 

We report a case of 35-year-old woman who presented post-motor vehicle accident with back pain and with minimal dyspnea on exertion. The final diagnosis was made after computed tomography and lung biopsy. The present case exhibits the remarkable clinico-radiological dissociation with complete calcification of the lungs on radiographic images with a relatively mild clinical presentation.

No MeSH data available.


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Computed tomography of the chestA: Axial view, intense calcification of the interstitium and pleural serosa. B: Axial view lung window, intense calcification giving a “white lung” appearance. C: Coronal view, intense calcification of the interstitium and pleural serosa sparing the lung apices. D: Coronal view lung window, dense calcification of the lower lung fields with moderate calcification of the apices.
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FIG2: Computed tomography of the chestA: Axial view, intense calcification of the interstitium and pleural serosa. B: Axial view lung window, intense calcification giving a “white lung” appearance. C: Coronal view, intense calcification of the interstitium and pleural serosa sparing the lung apices. D: Coronal view lung window, dense calcification of the lower lung fields with moderate calcification of the apices.

Mentions: Arterial blood gas showed pH: 7.40, PaCO2: 35.2 mmHg, PaO2: 60.8 mmHg, and HCO3: 22.5 mmol/L on 2 liters of oxygen via nasal cannula. Computed tomography was performed which demonstrated diffuse pulmonary interstitial and alveolar calcification with minimal sparing of the apices (Figure 2).


Pulmonary Alveolar Microlithiasis “ Stone Lungs ” : A Case of Clinico-Radiological Dissociation
Computed tomography of the chestA: Axial view, intense calcification of the interstitium and pleural serosa. B: Axial view lung window, intense calcification giving a “white lung” appearance. C: Coronal view, intense calcification of the interstitium and pleural serosa sparing the lung apices. D: Coronal view lung window, dense calcification of the lower lung fields with moderate calcification of the apices.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC5037061&req=5

FIG2: Computed tomography of the chestA: Axial view, intense calcification of the interstitium and pleural serosa. B: Axial view lung window, intense calcification giving a “white lung” appearance. C: Coronal view, intense calcification of the interstitium and pleural serosa sparing the lung apices. D: Coronal view lung window, dense calcification of the lower lung fields with moderate calcification of the apices.
Mentions: Arterial blood gas showed pH: 7.40, PaCO2: 35.2 mmHg, PaO2: 60.8 mmHg, and HCO3: 22.5 mmol/L on 2 liters of oxygen via nasal cannula. Computed tomography was performed which demonstrated diffuse pulmonary interstitial and alveolar calcification with minimal sparing of the apices (Figure 2).

View Article: PubMed Central - HTML - PubMed

ABSTRACT

Pulmonary alveolar microlithiasis (PAM) is a rare infiltrative lung disease characterized by deposition of spherical calcium phosphate microliths called calcospherites within the alveoli. PAM was first described by Friedrich in 1856 and then by Harbitz in 1918. The disease pathogenesis is based on mutations in the SLC34A2 gene that encodes for the Type IIb sodium-phosphate cotransporter. The majority of the patients are diagnosed at an early age, usually between the ages of 20 and 40 years. The hallmark of this disease is a striking dissociation between the radiological findings and the mild clinical symptoms. 

We report a case of 35-year-old woman who presented post-motor vehicle accident with back pain and with minimal dyspnea on exertion. The final diagnosis was made after computed tomography and lung biopsy. The present case exhibits the remarkable clinico-radiological dissociation with complete calcification of the lungs on radiographic images with a relatively mild clinical presentation.

No MeSH data available.


Related in: MedlinePlus