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Juvenile chronic recurrent multifocal osteomyelitis

Orpha number ORPHA2778
Synonym(s) Juvenile CRMO
Prevalence -
Inheritance -
Age of onset -
ICD-10 -
OMIM -
UMLS -
MeSH -
MedDRA -
SNOMED CT -

Summary

Chronic recurrent multifocal osteomyelitis (CRMO) in children is an inflammatory disorder. It affects mainly the metaphyses of the long bones, in addition to the spine, the pelvis and the shoulder girdle. However, bone lesions can occur at any site of the skeleton. Even though this disease has been recognized as a clinical entity for almost three decades now, its origin and pathogenesis are not entirely clear. No apparent infectious agents are detectable at the site of the bone lesion. No epidemiological data on incidence and prevalence have been published so far. However, incidence might be something around 1:1,000,000, thus reflecting the number of patients followed-up. Clinical diagnosis in an affected child can be difficult because the clinical picture and course of disease may vary significantly. It has been shown that histological examination alone does not allow the distinction of CRMO from acute or subacute bacterial osteomyelitis. Therefore an extensive microbial workup of the tissue biopsy, including PCR-techniques, is essential in order to establish the diagnosis and decide as to the treatment. Non steroid anti-inflammatory drugs (NSAID) are the treatment of choice. In case of frequent relapses oral steroid treatment, bisphosphonates and azulfidine have been used and are reported to be beneficial.

Expert reviewer(s)

  • Pr Hermann GIRSCHICK

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