Fowler syndrome

Fowler syndrome, also known as proliferative vasculopathy and hydranencephaly-hydrocephaly syndrome (PVHH), is a rare inherited condition.

Not to be confused with Fowler syndrome of urinary retention, a condition caused by primary failure of urethral sphincter relaxation resulting in urinary retention in young women.

It is characterized by:

Fowler syndrome is inherited in an autosomal recessive manner, caused by mutations in the cell-surface protein FLCVR2 4.

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Article information

rID: 13890
Section: Syndromes
Tag: cases
Synonyms or Alternate Spellings:
  • PVHH
  • Proliferative vasculopathy and hydranencephaly-hydrocephaly syndrome
  • Proliferative vasculopathy and hydranencephaly-hydrocephaly syndrome (PVHH)
  • Fowler's syndrome

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