Cartilage Hair Hypoplasia: Two Unrelated Cases with g.70 A > G Mutation in RMRP Gene

Indian J Pediatr. 2016 Sep;83(9):1003-5. doi: 10.1007/s12098-015-1947-4. Epub 2016 Feb 1.

Abstract

Cartilage-hair hypoplasia is an autosomal recessive disorder, characterized by short stature, metaphyseal dysplasia, hypotrichosis and immunodeficiency. More than 90 different biallelic mutations in RMRP gene have been identified to cause this condition. Three cases previously reported from India showed novel mutations in RMRP gene. The authors report two unrelated cases with the more common g.70A > G mutation, stressing the need to screen for this mutation in Indian population having features of cartilage-hair hypoplasia.

Keywords: Cartilage-hair hypoplasia; RMRP.

Publication types

  • Case Reports

MeSH terms

  • Cartilage
  • Child
  • Child, Preschool
  • Endoribonucleases
  • Hair / pathology*
  • Humans
  • India
  • Male
  • Mutation
  • Osteochondrodysplasias / diagnosis
  • Osteochondrodysplasias / genetics*
  • RNA, Long Noncoding / genetics*

Substances

  • RMRP non-coding RNA, human
  • RNA, Long Noncoding
  • Endoribonucleases