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Hajdu-Cheney syndrome: case report

N T Prabhu1, A K Munshi

  • 1Department of Pedodontics and Preventive Dentistry, A.B. Shetty Memorial Institute of Dental Sciences, Mangalore, India.

The Journal of Clinical Pediatric Dentistry
|January 1, 1996
PubMed
Summary

Hajdu-Cheney syndrome, a rare genetic disorder, presents with distinctive bone abnormalities, skull malformations, and dental issues. This case highlights acro-osteolysis and early permanent tooth eruption, alongside a ventricular septal defect.

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Area of Science:

  • Genetics
  • Pediatrics
  • Radiology

Background:

  • Hajdu-Cheney syndrome is a rare autosomal dominant disorder characterized by skeletal abnormalities.
  • Key features include acro-osteolysis, delayed closure of fontanelles, and distinctive facial features.

Observation:

  • A case report detailing a child diagnosed with Hajdu-Cheney syndrome.
  • The patient exhibited acro-osteolysis of the left hand, a bizarrely shaped skull, and early eruption of permanent teeth.
  • A ventricular septal defect was also noted, indicating potential cardiovascular involvement.

Findings:

  • Biochemical investigations revealed elevated levels of acid phosphatase.
  • Dental management involved extraction of primary incisors due to premature lingual eruption of permanent incisors.

Implications:

  • This case underscores the complex presentation of Hajdu-Cheney syndrome, emphasizing the need for multidisciplinary care.
  • Early dental intervention may be necessary to manage complications arising from premature tooth eruption.
  • Further research into the biochemical markers and long-term management strategies for Hajdu-Cheney syndrome is warranted.

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