Pulmonary Alveolar Microlithiasis: A Unique Case of Familial PAM Complicated by Transplant Rejection
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 micronodu...
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| Format: | Article |
| Language: | English |
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Wiley
2021-01-01
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| Series: | Case Reports in Pathology |
| Online Access: | http://dx.doi.org/10.1155/2021/6674173 |
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| _version_ | 1832564918713319424 |
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| author | Austin Helmink Samir Atiya Ernesto Martinez Duarte |
| author_facet | Austin Helmink Samir Atiya Ernesto Martinez Duarte |
| author_sort | Austin Helmink |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-dcd7060b6239414584e9d9e0d7e73072 |
| institution | Kabale University |
| issn | 2090-6781 2090-679X |
| language | English |
| publishDate | 2021-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Pathology |
| spelling | doaj-art-dcd7060b6239414584e9d9e0d7e730722025-02-03T01:09:57ZengWileyCase Reports in Pathology2090-67812090-679X2021-01-01202110.1155/2021/66741736674173Pulmonary Alveolar Microlithiasis: A Unique Case of Familial PAM Complicated by Transplant RejectionAustin Helmink0Samir Atiya1Ernesto Martinez Duarte2Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha, NE 68198, USADepartment of Pathology and Microbiology, University of Nebraska Medical Center, Omaha, NE 68198, USADepartment of Pathology and Microbiology, University of Nebraska Medical Center, Omaha, NE 68198, USABackground. 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.http://dx.doi.org/10.1155/2021/6674173 |
| spellingShingle | Austin Helmink Samir Atiya Ernesto Martinez Duarte Pulmonary Alveolar Microlithiasis: A Unique Case of Familial PAM Complicated by Transplant Rejection Case Reports in Pathology |
| title | Pulmonary Alveolar Microlithiasis: A Unique Case of Familial PAM Complicated by Transplant Rejection |
| title_full | Pulmonary Alveolar Microlithiasis: A Unique Case of Familial PAM Complicated by Transplant Rejection |
| title_fullStr | Pulmonary Alveolar Microlithiasis: A Unique Case of Familial PAM Complicated by Transplant Rejection |
| title_full_unstemmed | Pulmonary Alveolar Microlithiasis: A Unique Case of Familial PAM Complicated by Transplant Rejection |
| title_short | Pulmonary Alveolar Microlithiasis: A Unique Case of Familial PAM Complicated by Transplant Rejection |
| title_sort | pulmonary alveolar microlithiasis a unique case of familial pam complicated by transplant rejection |
| url | http://dx.doi.org/10.1155/2021/6674173 |
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