Literature DB >> 22433957

Hip pathology in Hutchinson-Gilford progeria syndrome: a report of two children.

Pouya Akhbari1, Shilpa Jha, Kyle D James, Barry L Hinves, Jamie A F Buchanan.   

Abstract

Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic disorder. The estimated incidence is one in 4 million births. Orthopaedic manifestations include abnormality of the hips occurring early in the disease process. Severe coxa valga can be apparent by the age of 2 years. We report two cases of HGPS, one in a 7-year-old girl with avascular necrosis of the left hip and the second in a 13-year-old girl with recurrent traumatic hip dislocations. We demonstrate the pathoanatomical changes in the hip with HGPS using a combination of imaging modalities including radiographic, computed tomographic and MRI scans. These include coxa magna, coxa valga and acetabular dysplasia. We also comment on how these would affect the surgical management of this high-risk group of patients.
© 2012 Wolters Kluwer Health | Lippincott Williams & Wilkins.

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Year:  2012        PMID: 22433957     DOI: 10.1097/BPB.0b013e3283528ddd

Source DB:  PubMed          Journal:  J Pediatr Orthop B        ISSN: 1060-152X            Impact factor:   1.041


  1 in total

1.  Radiological Diagnosis of a Rare Premature Aging Genetic Disorder: Progeria (Hutchinson-Gilford Syndrome).

Authors:  Haji Mohammed Nazir; Akshiitha Ramesh Baabhu; Yuvaraj Muralidharan; Seena Cheppala Rajan
Journal:  Case Rep Radiol       Date:  2017-09-12
  1 in total

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