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Pycnodysostosis Summary

Description: Rare autosomal recessive syndrome characterized by delayed closing of CRANIAL SUTURES, short stature, ACRO-OSTEOLYSIS of distal phalanges, dental and MAXILLOFACIAL ABNORMALITIES and an increase in bone density that results in frequent BONE FRACTURES. It is associated with BONE RESORPTION defect due to mutations in the lysosomal cysteine protease CATHEPSIN K.

Also Known As: Pycnodysostoses; Pyknodysostoses; Pyknodysostosis

Networked: 93 relevant articles (2 outcomes, 8 trials/studies) for this Disease

Key Drugs and Agents for Pycnodysostosis

Efficacy Chart >>
Drugs and Important Biological Agents (IBA) related to treatments:
  1. Growth Hormone (Somatotropin) : 1 outcome in 4 results : IBA
  2. Cathepsin K : 5 studies in 55 results : IBA
  3. Collagen : 1 study in 5 results : IBA
  4. Insulin-Like Growth Factor I (IGF-1) : 1 study in 3 results : IBA
  5. Tetracycline (Achromycin) : 1 study in 1 result : FDA 62 Generic
  6. Sclerosteosis : 1 study in 1 result : IBA
  7. hereditary expansile Polyostotic osteolytic dysplasia : 1 study in 1 result : IBA
  8. Cysteine Proteases (Cysteine Protease) : 8 results : IBA
  9. Proline (L-Proline) : 2 results : FDA 18
  10. Papain : 2 results : IBA
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Diseases Related to Pycnodysostosis

  1. Sleep Apnea Syndromes (Sleep Apnea)
  2. Malocclusion (Crossbite)
  3. Osteopetrosis
  4. Bone Resorption
  5. Osteoporosis
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Key Therapies for Pycnodysostosis

Efficacy Chart >>
  1. Distraction Osteogenesis : 1 outcome in 2 results
  2. Transplants (Transplant) : 3 results
  3. Sutures (Suture) : 3 results
  4. Arthroplasty : 3 results
  5. Aftercare (After-Treatment) : 2 results
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