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A Walk Like Charlie Chaplin

  • Hermann Girschick

摘要

Clinically, the rare hypophosphatasia was traditionally assigned to endocrinology, although since the 1990s there has been increasing evidence for an inflammatory pathophysiology within this bone metabolism disease. Recent data show that the stimulation of the inflammasome and the consequent formation of, for example, interleukin-1, alongside hyperprostaglandinism, plays a role. Clinically, this manifests as a multifocal painful musculoskeletal system, with whole-body MRI showing signs of bone edema. The latter mimics the clinical disease pattern of CRMO. Therefore, hypophosphatasia is now an important CNO differential diagnosis. Anti-inflammatory therapy strategies have proven to be helpful.