Diseases

Pycnodysostosis

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.

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

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.

MeSH Record

Classification

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MeSH Record

Synonyms

3 entry terms
  • Pyknodysostosis
  • Pycnodysostoses
  • Pyknodysostoses

MeSH Record

Aspects Covered

34 allowable subheadings

Indexed with the subheadings blood, cerebrospinal fluid, chemically induced, classification, complications, diagnosis, diagnostic imaging, diet therapy, drug therapy, economics, embryology, enzymology, epidemiology, ethnology, etiology, genetics, history, immunology, metabolism, microbiology, mortality, nursing, parasitology, pathology, physiopathology, prevention & control, psychology, radiotherapy, rehabilitation, surgery, therapy, urine, veterinary, virology.

MeSH Record

History Note

2011

MeSH Record

Previous Indexing

  • Dysostoses (1966-2010)

MeSH Hierarchy

Tree Numbers

AMA Style

References

  1. National Library of Medicine. Pycnodysostosis. Medical Subject Headings (MeSH). 2026. Unique ID D058631. http://id.nlm.nih.gov/mesh/2026/D058631
  2. Pycnodysostosis. In: Wikipedia. https://en.wikipedia.org/wiki/Pycnodysostosis
  3. Pycnodysostosis. In: Wikidata. https://www.wikidata.org/wiki/Q974928