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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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Anteroposterior chest x-rayDense bilateral pulmonary consolidation with minimal sparing of the lung apices.
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FIG1: Anteroposterior chest x-rayDense bilateral pulmonary consolidation with minimal sparing of the lung apices.

Mentions: The patient had presented as a young 35-year-old female post a motor vehicle accident. She denied past medical and surgical history. She was born in the United States and denied a history of occupational inhalation exposure. She denied smoking tobacco. Upon admission, she was afebrile and hemodynamically stable. Her oxygen saturation was 96.3% on two liters of oxygen delivered via nasal cannula. On physical examination, she had coarse crackles diffusely in bilateral lung fields. Anterior-posterior chest radiograph showed no evidence of rib fractures, but she did have extensive diffuse dense alveolar infiltrates (Figure 1).


Pulmonary Alveolar Microlithiasis “ Stone Lungs ” : A Case of Clinico-Radiological Dissociation
Anteroposterior chest x-rayDense bilateral pulmonary consolidation with minimal sparing of the lung apices.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

FIG1: Anteroposterior chest x-rayDense bilateral pulmonary consolidation with minimal sparing of the lung apices.
Mentions: The patient had presented as a young 35-year-old female post a motor vehicle accident. She denied past medical and surgical history. She was born in the United States and denied a history of occupational inhalation exposure. She denied smoking tobacco. Upon admission, she was afebrile and hemodynamically stable. Her oxygen saturation was 96.3% on two liters of oxygen delivered via nasal cannula. On physical examination, she had coarse crackles diffusely in bilateral lung fields. Anterior-posterior chest radiograph showed no evidence of rib fractures, but she did have extensive diffuse dense alveolar infiltrates (Figure 1).

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