Short rib polydactyly syndrome

Short rib polydactyly syndrome(s) (SRPS) comprise of a rare group of severe osteochondrodysplasias. There are four major recognised types present:

There may also be other very rare types described, such as 9:

Clinical features

While there is some variation in clinical spectrum, they can be collectively characteristed by 2-3:

Pathology

Genetics

Collectively, most cases carry an autosomal recessive inheritance.

Associations

Multiple anomalies of major organs are common 5. These include

Treatment and prognosis

SRPSs are lethal anomalies and management is often supportive.

See also

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Article Information

rID: 13709
Section: Syndromes
Tags: cases, syndrome
Synonyms or Alternate Spellings:
  • Short rib polydactyly syndromes
  • Short rib polydactyly syndromes (SRPS)
  • Short rib polydactyly (SRP) syndrome
  • SRP syndrome
  • SRPS

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