Morphology and chemical identity of periarticular and vascular calcification in a patient with the rare genetic disease of arterial calcification due to deficiency of CD73 (ACDC)
| Autor: | Shirley F. Rollison, Manfred Boehm, Deepak R. Lakshmipathy, Han Wen, Elisa A. Ferrante, Wenqian Xu, Alessandra Brofferio, Leon J. Nesti, Marcus Y. Chen, Katherine M. Carney, David T. Nguyen, Frederick Dyda, Cornelia Cudrici |
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| Rok vydání: | 2020 |
| Předmět: |
lcsh:Medical physics. Medical radiology. Nuclear medicine
Diagnostic Imaging Pathology medicine.medical_specialty Morphology (linguistics) lcsh:R895-920 Disease 030218 nuclear medicine & medical imaging 03 medical and health sciences Ectopic calcification 0302 clinical medicine medicine Synovial fluid Radiology Nuclear Medicine and imaging ACDC Vascular calcification business.industry medicine.disease X-ray diffraction Radiography Arterial calcification Periarticular calcification Alkaline phosphatase medicine.symptom Claudication business 030217 neurology & neurosurgery CT |
| Zdroj: | Radiology Case Reports Radiology Case Reports, Vol 15, Iss 10, Pp 1883-1886 (2020) |
| ISSN: | 1930-0433 |
| Popis: | A 54-year old female patient with the genetic disease of arterial calcification due to deficiency of CD73 was studied under the Undiagnosed Disease Program of the National Institutes of Health. She presented with symptoms of claudication in her 40s and later developed arthritic symptoms, ectopic calcification in her left hand and severe arterial calcifications of the lower extremities. Since little was known about the composition of the calcifications in arterial calcification due to deficiency of CD73, we investigated their chemical identity and microscopic morphology in this patient with imaging and x-ray diffraction analysis. We found that, microscopically, the bulk calcifications consisted of fragments of either solid or porous internal structure. Both periarticular and arterial calcifications were primarily hydroxyapatite crystals of the same crystalline anisotropy, but different crystalline grain sizes. This was consistent with the presence of hydroxyapatite crystals along with birefringent calcium pyrophosphate dihydrate crystals in the synovial fluid of the patients by polarized light microscopy. The result suggests that tissue calcification in both locations follow a similar biochemical mechanism caused by an increase in extracellular tissue-nonspecific alkaline phosphatase activity. |
| Databáze: | OpenAIRE |
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