interesting syndrome
Radiologic Manifestations of Proteus Syndrome
Proteus syndrome is a sporadic disorder named for its highly variable manifestations. The disease causes tissue overgrowth in a mosaic pattern and may affect tissues derived from any germinal layer. The disease process is not usually apparent at birth but develops rapidly in childhood. Common manifestations include macrodactyly, vertebral abnormalities, asymmetric limb overgrowth and length discrepancy, hyperostosis, abnormal and asymmetric fat distribution, asymmetric muscle development, connective-tissue nevi, and vascular malformations. The features of Proteus syndrome indicate that the condition may be caused by a somatic alteration in a gene, but no specific genetic mutation has yet been identified. Therefore, the diagnosis and management of the disease depend heavily on clinical evaluation and imaging. Although the manifestations of Proteus syndrome are highly variable, accurate diagnosis is possible if standard diagnostic criteria are followed and if disease features are assessed in comparison with those found in similar syndromes.
RadioGraphics 2004;24:1051-1068
Proteus syndrome is a sporadic disorder named for its highly variable manifestations. The disease causes tissue overgrowth in a mosaic pattern and may affect tissues derived from any germinal layer. The disease process is not usually apparent at birth but develops rapidly in childhood. Common manifestations include macrodactyly, vertebral abnormalities, asymmetric limb overgrowth and length discrepancy, hyperostosis, abnormal and asymmetric fat distribution, asymmetric muscle development, connective-tissue nevi, and vascular malformations. The features of Proteus syndrome indicate that the condition may be caused by a somatic alteration in a gene, but no specific genetic mutation has yet been identified. Therefore, the diagnosis and management of the disease depend heavily on clinical evaluation and imaging. Although the manifestations of Proteus syndrome are highly variable, accurate diagnosis is possible if standard diagnostic criteria are followed and if disease features are assessed in comparison with those found in similar syndromes.
RadioGraphics 2004;24:1051-1068
interesting syndrome
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Reviewed by Sumer Sethi
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Sunday, October 10, 2004
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Sumer Sethi
Unique blend of academic excellence and entrepreneurship, heading leading firms in India- Teleradiology Providers, pioneering company providing teleradiology services and DAMS (Delhi Academy of Medical Sciences) Premier test preparation institute in India for MD/MS/MCI preparation. He has also been an invited faculty member at various conferences, including Teleradiology in IRIA 2008 and 2011, Hospital Build Middle East, Congress of the Brain Tumor Radiology in Neuro-oncology Society. Dr. Sethi is Editor-in-Chief of Internet Journal of Radiology. He has a keen interest in Web 2.0 technologies and in maintaining his famous radiology blog, which has been featured in multiple international journals.
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