Nebenwirkungen · Procysbi
PROCYSBI 25 mg gastro-resistant hard capsules PROCYSBI 75 mg gastro-resistant hard capsules · Abschnitt 4.8 · PDF-Seite 8
HOS-215711 · Präparat HOS-155504
A few cases of Ehlers-Danlos-like syndrome on elbows have been reported in children chronically treated with high doses of different cysteamine preparations (cysteamine chlorhydrate or cystamine or cysteamine bitartrate) mostly above the maximal dose 1.95 g/m2/day. In some cases, these skin lesions were associated with skin striae and bone lesions first seen during an X-ray examination. Bone disorders reported were genu valgum, leg pain and hyperextensive joints, osteopenia, compression fractures, and scoliosis. In the few cases where histopathological examination of the skin was performed, the results suggested angioendotheliomatosis. One patient subsequently died of acute cerebral ischemia with marked vasculopathy. In some patients, the skin lesions on elbows regressed after immediate-release cysteamine dose reduction (see section 4.4).
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