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ORIGINAL ARTICLE
Year : 2013  |  Volume : 17  |  Issue : 6  |  Page : 1053-1056

Proteus syndrome: Clinical diagnosis of a series of cases


1 Pediatric Endocrinology Unit, Hospital Universitario Prof. Edgard Santos, Faculty of Medicine, Federal University of Bahia, Salvador, Bahia, Brazil
2 Genetic Service, Hospital Universitario Prof. Edgard Santos, Faculty of Medicine, Federal University of Bahia, Salvador, Bahia, Brazil

Correspondence Address:
Cresio Alves
Pediatric Endocrinology Unit, Hospital Universitario Prof. Edgard Santos, Faculty of Medicine, Federal University of Bahia, Salvador, Bahia
Brazil
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2230-8210.122621

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Objectives: This paper describes the clinical diagnosis of Proteus syndrome (PS) in children referred for evaluation of asymmetric disproportionate overgrowth. Materials and Methods: Retrospective, descriptive, cross-sectional study conducted from January 1998 to December 2010. Results: During the study period, 2011 new patients were evaluated. Thirteen (0.65%) patients presented features suggestive of PS. These patients were formally evaluated based on the revised diagnostic criteria proposed by Biesecker. The mean age was 6.92 ± 5.1 years. Ten patients (76.9%) were females. All subjects had asymmetric disproportionate overgrowth. Other dysmorphic features were as follows: macrodactily (84.6%); linear epidermal nevus (41.6%); hemangioma (30.7%); and lipoma (23%). Six patients fulfilled the diagnostic criteria for PS. Conclusions: The diagnostic rate of only 46.1% of patients with PS confirms the diagnostic difficulties and the need for continuous monitoring and periodic review of these patients since the clinical manifestations of this syndrome become more evident with aging. Molecular tests may help the differential diagnosis of Proteus syndrome when they became commercially available.


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