Osteogenesis Imperfecta Medication

  • Author: Manoj Ramachandran, MBBS, MRCS, FRCS; Chief Editor: Harris Gellman, MD   more...
 
Updated: Mar 29, 2012
 

Medication Summary

The goals of pharmacotherapy are to reduce morbidity and prevent complications.[21]

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Bisphosphonates

Class Summary

Bisphosphonates are the only drugs specifically licensed for the treatment of osteogenesis imperfecta (OI). The most commonly used drug in this class is pamidronate.[24, 25, 22]

Bisphosphonates are analogues of inorganic pyrophosphate and act by binding to hydroxyapatite in bone matrix, thereby inhibiting the dissolution of crystals. They prevent osteoclast attachment to the bone matrix and osteoclast recruitment and viability.

For maximum gut absorption, all oral bisphosphonates should be taken at least 2 hours before or after meals. The newer bisphosphonates are not completely free of the risk of causing a mineralization defect, but their safe therapeutic window is much wider. They clearly are more potent than etidronate in reducing disease activity and normalizing alkaline phosphatase levels

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Pamidronate (Aredia, APD)

 

Pamidronate is a potent second-generation bisphosphonate that acts principally by inhibiting osteoclastic bone resorption. Cyclic intravenous (IV) pamidronate is given in a dose of 7.5 mg/kg/y at 4- to 6-month intervals.

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Alendronate (Fosamax)

 

Alendronate is a potent third-generation bisphosphonate that principally acts by inhibiting osteoclastic bone resorption.

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Risedronate (Actonel, Atelvia)

 

Risedronate is a potent aminobisphosphonate that principally acts by inhibiting osteoclastic bone resorption.

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Tiludronate (Skelid)

 

Tiludronate is a sulfur-containing bisphosphonate of intermediate potency between etidronate and newer nitrogen-containing bisphosphonates. No food, indomethacin, or calcium should be ingested within 2 hours before and 2 hours after administration. A 3-month posttreatment evaluation follows.

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Zoledronate (Reclast, Zometa)

 

Zoledronate inhibits bone resorption. It inhibits osteoclastic activity and induces osteoclastic apoptosis.

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Contributor Information and Disclosures
Author

Manoj Ramachandran, MBBS, MRCS, FRCS  Consultant Trauma and Orthopaedic Surgeon, Barts and the London NHS Trust; Honorary Senior Lecturer, William Harvey Research Institute, Barts and The London School of Medicine and Dentistry, Queen Mary's, University of London, UK

Manoj Ramachandran, MBBS, MRCS, FRCS is a member of the following medical societies: British Orthopaedic Association

Disclosure: Nothing to disclose.

Coauthor(s)

David H A Jones, MB, ChB, FRCS, FRCS Ed(Orth)  Consultant Orthopedic Surgeon, Great Ormond Street Hospital for Children; Senior Clinical Lecturer, University College London Hospitals, UK

David H A Jones, MB, ChB, FRCS, FRCS Ed(Orth) is a member of the following medical societies: British Orthopaedic Association

Disclosure: Nothing to disclose.

Pramod Achan, MBBS, FRCS(Orth)  Senior Registrar, Royal National Orthopaedic Hospital, UK

Disclosure: Nothing to disclose.

Chief Editor

Harris Gellman, MD  Consulting Surgeon, Broward Hand Center; Voluntary Clinical Professor of Orthopedic Surgery and Plastic Surgery, Departments of Orthopedic Surgery and Surgery, University of Miami, Leonard M Miller School of Medicine

Harris Gellman, MD is a member of the following medical societies: American Academy of Medical Acupuncture, American Academy of Orthopaedic Surgeons, American Orthopaedic Association, American Society for Surgery of the Hand, and Arkansas Medical Society

Disclosure: Nothing to disclose.

Additional Contributors

Peter R Calder, MBBS, FRCS(Eng), FRCS (Tr&Orth) Consulting Surgeon, Department of Pediatric Orthopedic Surgery, The Royal National Orthopaedic Hospital, UK

Peter R Calder, MBBS, FRCS(Eng), FRCS (Tr&Orth) is a member of the following medical societies: British Medical Association

Disclosure: Nothing to disclose.

Ian D Dickey, MD, FRCSC Adjunct Professor, Department of Chemical and Biological Engineering, University of Maine; Consulting Staff, Adult Reconstruction, Orthopedic Oncology, Department of Orthopedics, Eastern Maine Medical Center

Ian D Dickey, MD, FRCSC is a member of the following medical societies: American Academy of Orthopaedic Surgeons, British Columbia Medical Association, Canadian Medical Association, and Royal College of Physicians and Surgeons of Canada

Disclosure: Stryker Orthopaedics Consulting fee Consulting; Cadence Honoraria Speaking and teaching

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

References

  1. Smith R, Francis MJ, Houghton GR. The brittle bone syndrome. In: Osteogenesis Imperfecta. London: Butterworth. 1983.

  2. Brusin JH. Osteogenesis imperfecta. Radiol Technol. Jul-Aug 2008;79(6):535-48. [Medline].

  3. Cole WG. The Nicholas Andry Award-1996. The molecular pathology of osteogenesis imperfecta. Clin Orthop. Oct 1997;235-48. [Medline].

  4. Cole WG. Advances in osteogenesis imperfecta. Clin Orthop. Aug 2002;6-16. [Medline].

  5. Cole WG. Bone, cartilage and fibrous tissue disorders. In: Benson MKD, Fixsen JA, MacNicol MF, Parch K, eds. Children's Orthopaedics. 2002: 67-92.

  6. Baujat G, Lebre AS, Cormier-Daire V, Le Merrer M. [Osteogenesis imperfecta, diagnosis information (clinical and genetic classification)]. Arch Pediatr. Jun 2008;15(5):789-91. [Medline].

  7. Alanay Y, Avaygan H, Camacho N, Utine GE, Boduroglu K, Aktas D, et al. Mutations in the gene encoding the RER protein FKBP65 cause autosomal-recessive osteogenesis imperfecta. Am J Hum Genet. Apr 9 2010;86(4):551-9. [Medline]. [Full Text].

  8. Sillence D. Osteogenesis imperfecta: an expanding panorama of variants. Clin Orthop. Sep 1981;11-25. [Medline].

  9. Sillence DO, Senn A, Danks DM. Genetic heterogeneity in osteogenesis imperfecta. J Med Genet. Apr 1979;16(2):101-16. [Medline].

  10. Labuda M, Morissette J, Ward LM. Osteogenesis imperfecta type VII maps to the short arm of chromosome 3. Bone. Jul 2002;31(1):19-25. [Medline].

  11. Ward LM, Rauch F, Travers R. Osteogenesis imperfecta type VII: an autosomal recessive form of brittle bone disease. Bone. Jul 2002;31(1):12-8. [Medline].

  12. Duro Friedl EA, Ferrari Mayans L, Desalvo Portal LN, Ferrari Ruiz P, Bidondo Horno MP, Astraldi Tellechea MM. [Bruck syndrome: Osteogenesis imperfecta with congenital joint contractures.]. An Pediatr (Barc). Jul 2008;69(1):90-1. [Medline].

  13. [Guideline] Kellogg ND. Evaluation of suspected child physical abuse. Pediatrics. Jun 2007;119(6):1232-41. [Medline]. [Full Text].

  14. Francis MJ, Smith R, Bauze RJ. Instability of polymeric skin collagen in osteogenesis imperfecta. Br Med J. Mar 9 1974;1(905):421-4. [Medline].

  15. Rauch F, Travers R, Parfitt AM, Glorieux FH. Static and dynamic bone histomorphometry in children with osteogenesis imperfecta. Bone. Jun 2000;26(6):581-9. [Medline].

  16. Jones D, Hosalkar H, Jones S. The orthopaedic management of osteogenesis imperfecta. Clin Orthop. 2002;16:374-88.

  17. Zeitlin L, Fassier F, Glorieux FH. Modern approach to children with osteogenesis imperfecta. J Pediatr Orthop B. Mar 2003;12(2):77-87. [Medline].

  18. Forin V. [Paediatric osteogenesis imperfecta: medical and physical treatment]. Arch Pediatr. Jun 2008;15(5):792-3. [Medline].

  19. Esposito P, Plotkin H. Surgical treatment of osteogenesis imperfecta: current concepts. Curr Opin Pediatr. Feb 2008;20(1):52-7. [Medline].

  20. Sofield HA, Page MA, Mead NC. Multiple osteotomies and metal-rod fixation for osteogenesis imperfecta. J Bone Joint Surg. 1952;34A:500-2.

  21. Glorieux FH, Bishop NJ, Plotkin H, et al. Cyclic administration of pamidronate in children with severe osteogenesis imperfecta. N Engl J Med. Oct 1 1998;339(14):947-52. [Medline].

  22. Salehpour S, Tavakkoli S. Cyclic pamidronate therapy in children with osteogenesis imperfecta. J Pediatr Endocrinol Metab. Jan-Feb 2010;23(1-2):73-80. [Medline].

  23. Rauch F, Munns C, Land C, Glorieux FH. Pamidronate in children and adolescents with osteogenesis imperfecta: effect of treatment discontinuation. J Clin Endocrinol Metab. Apr 2006;91(4):1268-74. [Medline].

  24. Shapiro JR, Thompson CB, Wu Y, Nunes M, Gillen C. Bone Mineral Density and Fracture Rate in Response to Intravenous and Oral Bisphosphonates in Adult Osteogenesis Imperfecta. Calcif Tissue Int. Jun 11 2010;[Medline].

  25. Gallego L, Junquera L, Pelaz A, Costilla S. Pathological mandibular fracture after simple molar extraction in a patient with osteogenesis imperfecta treated with alendronate. Med Oral Patol Oral Cir Bucal. Jun 1 2010;[Medline].

  26. Castillo H, Samson-Fang L. Effects of bisphosphonates in children with osteogenesis imperfecta: an AACPDM systematic review. Dev Med Child Neurol. Jan 2009;51(1):17-29. [Medline].

  27. Bargman R, Huang A, Boskey AL, Raggio C, Pleshko N. RANKL inhibition improves bone properties in a mouse model of osteogenesis imperfecta. Connect Tissue Res. Apr 2010;51(2):123-31. [Medline]. [Full Text].

  28. Wekre LL, Frøslie KF, Haugen L, Falch JA. A population-based study of demographical variables and ability to perform activities of daily living in adults with osteogenesis imperfecta. Disabil Rehabil. 2010;32(7):579-87. [Medline].

 
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Acute fractures are observed in radius and ulna. Multiple fractures can be seen in ribs. Old healing humeral fracture with callus formation is observed.
Beaded ribs. Multiple fractures are seen in long bones of upper extremities.
Wormian bones are present in skull.
Newborn has bilateral femoral fractures.
Table. Adapted Sillence Classification of Osteogenesis Imperfecta
Type Genetic Teeth Bone Fragility Bone Deformity Sclera Spine Skull Prognosis
IAAD*NormalVariable but less severe than other typesModerateBlue20% scoliosis and kyphosisWormian bonesFair
IBADDentinogenesis imperfectaNANANANANANA
IIADUnknownVery severeMultiple fracturesBlueNAWormian bones with absence of ossificationPerinatal death
IIIADDentinogenesis imperfectaSevereProgressive bowing of long bones and spineBluish at birth but white in adultsKyphoscoliosisHypoplastic wormian bonesWheelchair-bound, not ambulatory
IVAADNormalModerateModerateWhiteKyphoscoliosisHypoplastic wormian bonesFair
IVBADDentinogenesis imperfectaNANANANANANA
* AD = autosomal dominant; NA = not applicable.
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