کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
8625028 | 1568110 | 2018 | 29 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Clinical-pathological correlations in three patients with fibrodysplasia ossificans progressiva
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کلمات کلیدی
موضوعات مرتبط
علوم زیستی و بیوفناوری
بیوشیمی، ژنتیک و زیست شناسی مولکولی
زیست شناسی تکاملی
پیش نمایش صفحه اول مقاله

چکیده انگلیسی
This autopsy case series provides valuable information about the underlying complications of FOP and contributes significantly to our knowledge of this rare yet debilitating disorder. Thoracic insufficiency syndrome, right heart dysfunction, widespread heterotopic ossification, spinal ligament ossification, and CNS malformations were clearly evident; however, most other non-bone tissues appeared to be spared from gross malformations. Finally, the ability to isolate live cells from cadaveric skin is an important technique that will facilitate future studies, particularly as induced pluripotent stem cells and other cell-based technologies evolve. This case series highlights the importance of post-mortem examinations and their contribution to our current knowledge of disease pathophysiology and comorbidities.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Bone - Volume 109, April 2018, Pages 104-110
Journal: Bone - Volume 109, April 2018, Pages 104-110
نویسندگان
Kelly L. Wentworth, Katherine Bigay, Tea V. Chan, Jennifer P. Ho, Blanca M. Morales, Joseph Connor, Erin Brooks, M. Shahriar Salamat, Henry Charles Sanchez, Geoffrey Wool, Robert J. Pignolo, Frederick S. Kaplan, Edward C. Hsiao,