{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,2]],"date-time":"2025-11-02T16:12:32Z","timestamp":1762099952036},"reference-count":30,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2002,9,23]],"date-time":"2002-09-23T00:00:00Z","timestamp":1032739200000},"content-version":"vor","delay-in-days":22,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Eur J Clin Investigation"],"published-print":{"date-parts":[[2002,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p><jats:bold>Background<\/jats:bold> Congenital haemolytic anaemia may be associated with pseudoxanthoma elasticum (PXE)\u2010like clinical manifestations.<\/jats:p><jats:p><jats:bold>Methods<\/jats:bold> The cardiovascular system of 14 homozygous and double heterozygous \u03b2\u2010thalassaemia patients with skin and retinal vessel alterations similar to those in genetic PXE was analysed over a period of 12\u00a0years and compared with that of 13 relatives (five sets of parents, one single parent, two thalassaemic brothers), and that of the control group composed of 16, age\u2010 and sex\u2010matched, thalassaemic patients.<\/jats:p><jats:p><jats:bold>Results<\/jats:bold> All patients with clinical PXE\u2010like skin lesions exhibited, by light and electron microscopy, dermal alterations and mineralization of elastic fibres identical to those typical of inherited PXE. None of the relatives and none of the control group showed clinical or structural findings of PXE. The follow\u2010up started in 1988. After 12\u00a0years of clinical observation, six patients showed dramatic progression of skin involvement, angioid streaks had progressed in two subjects. One patient had recurrent gastrointestinal bleeding and underwent partial stomach removal for gastric artery aneurysm, one underwent colon resection for intestinal infarct, one patient had a transitory ischaemic attack, one died after an intracranial haemorrhage, two patients died from cardiovascular disease and one from neoplasia.<\/jats:p><jats:p><jats:bold>Conclusions<\/jats:bold> Thalassaemic patients with PXE\u2010like skin lesions also manifest PXE\u2010like vessel alterations that progress with time. Considering the severe outcome of these lesions, accurate monitoring should be routinely performed on the cardiovascular system of thalassaemic patients with PXE\u2010like skin manifestations.<\/jats:p>","DOI":"10.1046\/j.1365-2362.2002.01032.x","type":"journal-article","created":{"date-parts":[[2003,3,11]],"date-time":"2003-03-11T15:55:32Z","timestamp":1047398132000},"page":"700-706","source":"Crossref","is-referenced-by-count":27,"title":["Cardiovascular involvement in thalassaemic patients with Pseudoxanthoma elasticum\u2010like skin lesions: a long\u2010term follow\u2010up study"],"prefix":"10.1111","volume":"32","author":[{"given":"P.","family":"Cianciulli","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"F.","family":"Sorrentino","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L.","family":"Maffei","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S.","family":"Amadori","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M. 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