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Fibromuseular dysplasia of the internal carotid artery in a child with alpha‐1‐antitrypsin deficiency
Author(s) -
Sölder Brigitte,
Streif Werner,
Ellemunter Helmut,
Mayr Udo,
Jaschke Werner
Publication year - 1997
Publication title -
developmental medicine and child neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.658
H-Index - 143
eISSN - 1469-8749
pISSN - 0012-1622
DOI - 10.1111/j.1469-8749.1997.tb07551.x
Subject(s) - medicine , fibromuscular dysplasia , pathogenesis , lesion , internal carotid artery , stroke (engine) , pathology , cardiology , renal artery , kidney , mechanical engineering , engineering
Fibromuseular dysplasia (FMD) is a non‐inflammatory segmental arteriopathy of unknown origin. Most often the renal arteries are affected, however, also mesenteric, lumbar, vertebral, or carotid arteries may be Involved. FMD has frequently been reported as a cause of stroke in adults, but very rarely in children. We report the case of an 11‐year‐old boy who presented with an ischaemic infarction in the anterior part of the territory of the left middle cerebral artery. Angiography demonstrated a'string of beads'lesion suggestive of FMD causing occlusion at the origin of the middle artery. Laboratory analyses revealed the protease inhibitor (Pi) phenotype SZ (PiSZ) of alpha‐1‐antitrypsin deficiency as well as decreased antioxidants and signs of enhanced lipid peroxidation. Such an imbalance may be associated with diminished resistance to oxidation, possibly causing direct cellular and tissue injury. Whether alpha‐1‐antitrypsin deficiency and an impaired status of antioxidants, as seen in our patient, might play a role in the pathogenesis of FMD is presently unclear.