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Hereditary Osteodystrophy with Multiple Hormone Resistance— A Case Report

  • Ines Velez1,*,
  • Melanie Bond2
  • Steven Ellen3
  • Diane Ede-Nichols2
  • Jose Larumbe2
  • Victor Oramas2
  • Daniel Arnold2

1Oral and Maxillofacial Pathology, Nova Southeastern University, College of Dental Medicine

2Pediatric Dentistry Resident, Nova Southeastern University, College of Dental Medicine

3Especial Needs Program, Nova Southeastern University, College of Dental Medicine

4,

DOI: 10.17796/jcpd.34.1.33017umx4n144285 Vol.34,Issue 1,January 2010 pp.67-70

Published: 01 January 2010

*Corresponding Author(s): Ines Velez E-mail: ivelez@nova.edu

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Abstract

Hereditary Osteodystrophy, also called pseudohypoparathyroidism, Type 1A (PHP), is a very rare condition composed of a heterogeneous group of autosomal dominant disorders with the common feature of organ resistance to multiple hormones. These patients produce the right amount of hormones but there is resistance to its effect. PHP is difficult to diagnose and the lack of diagnosis may have serious implications for the patient. We report a case of PHP, diagnosed by the dentist, due to the dental and jaw manifestations.

Keywords

Pseudohypoparathyroidism, Osteodystrophy, Albright syndrome

Cite and Share

Ines Velez,Melanie Bond,Steven Ellen,Diane Ede-Nichols,Jose Larumbe,Victor Oramas,Daniel Arnold. Hereditary Osteodystrophy with Multiple Hormone Resistance— A Case Report. Journal of Clinical Pediatric Dentistry. 2010. 34(1);67-70.

URL:

https://www.jocpd.com/articles/10.17796/jcpd.34.1.33017umx4n144285

References

1. De Wijn E M, Steendijk. Growth and development in a girl with pseudohypoparathyroidism. Acta Paediat. Scand, 71: 657–660, 1982

2. Farfel Z, Friedman E. Mental deficiency in psuedohypoparathyroidism type I is associated with Ns protein deficiency. Ann Intern Med, 105: 197–199, 1986.

3. Gorlin R, Cohen R, Hennekam RCN. Syndromes of the Head and Neck. Oxford 4th Ed, 164–168, 2001.

4. Hall J.G. Genomic imprinting review and relevance to human diseases. Am J. Hum. Genet, 46: 857–872, 1990.

5. Hedeland H, Berntorp K, Arheden K, Kristofferson U. Pseudohy-poparathyroidism type 1 and Albright’s hereditary Osteodystrophy with a proximal 15q chromosomal deletion in mother and daughter. Clin Genet, 42: 129–134. 1992.

6. Burgert T, Markowitz M. Understanding and recognizing pseudohy-perparathyroidism. Pediatrics in review. American Academy of Pedi-atrics, 26(8) 308–309, 2005.

7. Albright F, Burnett CH, Smith PH, Parson W: Pseudo-hypoparathy-roidism an example of “Seabright-Bantam syndrome” report of 3 cases. Endocrinology, 30: 922–932, 1942.

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