title: Proteus syndrome: Clinical profile of six patients and review of literature.
abstract: OBJECTIVE
  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.
  
  
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Pediatrics, Genetic and Metabolic Unit, Advanced Pediatric Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
authors.forename: Suresh Kumar
authors.initials: SK
authors.lastname: Angurana
authors.forename: Renu Suthar
authors.initials: RS
authors.lastname: Angurana
authors.forename: Inusha
authors.initials: I
authors.lastname: Panigrahi
authors.forename: Ram Kumar
authors.initials: RK
authors.lastname: Marwaha
keywords: Hamartoma; hemihypertrophy; lipoma; localized overgrowth; macrodactyly
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