[Butterfly vertebrae and scoliosis: Alagille syndrome].

doi:10.1055/s-2002-31538

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[Butterfly vertebrae and scoliosis: Alagille syndrome].

Zeitschrift fur Orthopadie und ihre Grenzgebiete - 1 Jan 2000

Przkora R, Schmitz A, Perlick L, Koenig R, Jaeger U, Schmitt O

Abstract excerpt

INTRODUCTION: Alagille syndrome is a dominantly inherited disorder affecting the liver (arteriohepatic dysplasia), the heart, the eyes and the face. Butterfly vertebrae in the thoracic and lumbar spine are detectable in 50 to 80 per cent of the patients; most of them remain asymptomatic. The management of progressive cholestasis in early childhood is the main aspect of therapy. CASE: A 14-year-old female patient...

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