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CAMURATI-ENGELMANN DISEASE [1 record]

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Record 1 2001-06-04

English

Subject field(s)
  • Symptoms (Medicine)
  • Nervous System
CONT

Progressive diaphyseal dysplasia (Camurati-Engelmann disease). This developmental disorder affects all races and is transmitted as an autosomal dominant trait with variable penetrance. New bone formation gradually involves both the periosteal and endosteal surface of long bone diaphyses. With severe disease, osteosclerosis also affects the axial skeleton.

French

Domaine(s)
  • Symptômes (Médecine)
  • Système nerveux
CONT

Maladie osseuses constitutionnelles [transmise selon le mode dominant], cette affection est habituellement découverte au cours de la seconde enfance, mais elle peut parfois l'être beaucoup plus tardivement. Ce sont généralement des douleurs des membres inférieurs qui attirent l'attention, plus rarement des troubles de la marche ou une fatigabilité excessive. Cliniquement, le fait le plus frappant est la disproportion entre le tronc et les membres qui paraissent anormalement longs. [...] L'examen radiologique montre un épaississement en fuseau de la corticale diaphysaire des os longs dont les contours légèrement ondulés.

Key term(s)
  • syndrome de Camurati-Engelmann

Spanish

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