Orphanet: Hydrocephalus obesity hypogonadism syndrome
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Hydrocephalus-obesity-hypogonadism syndrome

Disease definition

A rare form of syndromic obesity characterized by the association of congenital hydrocephalus, centripetal obesity, hypogonadism, intellectual deficit and short stature.

ORPHA:2183

Classification level: Disorder
  • Synonym(s):
    • Sengers-Hamel-Otten syndrome
  • Prevalence: <1 / 1 000 000
  • Inheritance: X-linked recessive 
  • Age of onset: Infancy, Neonatal
  • ICD-10: -
  • OMIM: -
  • UMLS: -
  • MeSH: -
  • GARD: 2775
  • MedDRA: -

Summary

Epidemiology

It has been described in two males from one family.

Genetic counseling

An X-linked recessive mode of inheritance was suggested.

- Last update: May 2009

  A summary on this disease is available in Deutsch (2009) Español (2009) Français (2009) Italiano (2009) Nederlands (2009) Português (2009)

Detailed information

Genetic Testing

ERN : produced/endorsed by ERN(s)
FSMR : produced/endorsed by FSMR(s)

Additional information

Further information on this disease

Patient-centred resources for this disease

Research activities on this disease

Specialised Social Services

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