Nebenwirkungen · Cystagon
CYSTAGON 50 mg hard capsules · Abschnitt 4.8 · PDF-Seite 5
HOS-086121 · Präparat HOS-083270
Cases of Ehlers-Danlos like syndrome and vascular disorders 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 vascular proliferation, 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 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 CYSTAGON dose reduction. Cysteamine mechanism of action by interfering with the cross-linking of collagen fibers has been postulated (see section 4.4).
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