Proteus syndrome: Clinical profile of six patients and review of literature.

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dc.contributor.authorAngurana, Suresh Kumar
dc.contributor.authorAngurana, Renu Suthar
dc.contributor.authorPanigrahi, Inusha
dc.contributor.authorMarwaha, Ram Kumar
dc.date.accessioned2013-11-20T09:00:54Z
dc.date.available2013-11-20T09:00:54Z
dc.date.issued2013-04
dc.description.abstractOBJECTIVE: Proteus syndrome (PS) is characterized by patchy or segmental overgrowth and hyperplasia of multiple tissues and organs, along with susceptibility to development of tumors. Very few cases are reported in literature from developing countries. Due to certain overlapping features with other overgrowth syndromes, diagnosis is usually delayed. Our aim was to describe clinical profile of this rare condition in six patients. MATERIALS AND METHODS: Retrospective case sheet review of patients followed in a Pediatric Genetic and Metabolic clinic at a tertiary care institute of North India with a diagnosis of hemihypertrophy/overgrowth syndrome. RESULTS: Six cases presented with asymmetric overgrowth and peculiar features suggestive of PS were included in this study. Age at presentation was 2 months to 10 years; two were males and four were females. Hemihypertrophy was noticed in only one case at birth, and focal overgrowths in rest of other patients were seen later during childhood. CONCLUSION: Due to certain overlapping features with other overgrowth syndromes, diagnosis of PS is usually delayed. Pediatricians are the first persons who come across such patients and they should be aware about this rare condition.en_US
dc.identifier.citationAngurana Suresh Kumar, Angurana Renu Suthar, Panigrahi Inusha, Marwaha Ram Kumar. Proteus syndrome: Clinical profile of six patients and review of literature. Indian Journal of Human Genetics. 2013 Apr; 19(2): 202-206.en_US
dc.identifier.urihttps://imsear.searo.who.int/handle/123456789/149430
dc.language.isoenen_US
dc.source.urihttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC3758728/?report=classicen_US
dc.subjectHamartomaen_US
dc.subjecthemihypertrophyen_US
dc.subjectlipomaen_US
dc.subjectlocalized overgrowthen_US
dc.subjectmacrodactylyen_US
dc.subject.meshChild, Preschool
dc.subject.meshFemale
dc.subject.meshFingers --abnormalities
dc.subject.meshHamartoma --diagnosis
dc.subject.meshHamartoma --epidemiology
dc.subject.meshHypertrophy --congenital
dc.subject.meshHypertrophy --diagnosis
dc.subject.meshHypertrophy --epidemiology
dc.subject.meshHumans
dc.subject.meshInfant
dc.subject.meshLimb Deformities, Congenital --diagnosis
dc.subject.meshLimb Deformities, Congenital --epidemiology
dc.subject.meshLipoma --diagnosis
dc.subject.meshLipoma --epidemiology
dc.subject.meshMale
dc.subject.meshProteus Syndrome --diagnosis
dc.subject.meshProeteus Syndrome --epidemiology
dc.subject.otherChild
dc.titleProteus syndrome: Clinical profile of six patients and review of literature.en_US
dc.typeArticleen_US
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