Hereditary idiopathic osteolysis type VII Torg

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Classification according to ICD-10
M89.5 Osteolysis
Q87.8 Other specified congenital malformation syndromes, not elsewhere classified
ICD-10 online (WHO version 2019)

The Hereditary idiopathic osteolysis Type VII Torg is a very rare congenital disease with the main features of a karpotarsalen osteolysis .

Synonyms are: hereditary multicenter osteolysis; Torg-Winchester Syndrome; English Torg Syndrome; Nodulosis-arthropathy-osteolysis syndromes; NAO syndromes; Al-Aqeel Sewairi Syndrome; Osteolysis, Hereditary Multicentrile; Torg-Winchester Syndrome (obsolete)

The disease is regarded as part of the MONA spectrum , for more see p. there.

The name refers to the first author of the first description from 1969 by the American orthopedic surgeon JS Torg and coworkers and the Saudi Arabian pediatrician Aida al Aqeel and coworkers.

Individual evidence

  1. Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .
  2. MONTH.  In: Online Mendelian Inheritance in Man . (English)
  3. JS Torg, AM DiGeorge, JA Kirkpatrick, MM Trujillo: Hereditary multicentric osteolysis with recessive transmission: a new syndrome. In: The Journal of pediatrics. Volume 75, Number 2, August 1969, pp. 243-252, PMID 5795345 .
  4. ^ A. Al Aqeel, W. Al Sewairi, B. Edress, RJ Gorlin, RJ Desnick, JA Martignetti: Inherited multicentric osteolysis with arthritis: a variant resembling Torg syndrome in a Saudi family. In: American journal of medical genetics. Volume 93, Number 1, July 2000, pp. 11-18, PMID 10861676 .