Short rib polydactyly syndrome

Dr Henry Knipe and Dr Yuranga Weerakkody et al.

Short rib polydactyly syndrome(s) (SRPS) comprise 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:

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

Collectively, most cases carry an autosomal recessive inheritance.

Multiple anomalies of major organs are common 5. These include:

SRPSs are lethal anomalies and management is often supportive.

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Article information

rID: 13709
Section: Syndromes
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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