FIBROMUSCULAR DYSPLASIA OF THE INTERNAL CAROTID-ARTERY IN A CHILD WITH ALPHA-1-ANTITRYPSIN DEFICIENCY

Fibromuscular dysplasia (FMD) is a non-inflammatory segmental arteriop athy of unknown origin. Most often the renal arteries are affected, ho wever, also mesenteric, lumbar, vertebral, or carotid arteries may be involved. FMD has frequently been reported as a cause of stroke in adu lts, 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 o f the territory of the left middle cerebral artery. Angiography demons trated a 'string of beads' lesion suggestive of FMD causing occlusion at the origin of the middle artery, Laboratory analyses revealed the p rotease inhibitor (Pi) phenotype SZ (PiSZ) of alpha-1-antitrypsin defi ciency as well as decreased antioxidants and signs of enhanced lipid p eroxidation. Such an imbalance may be associated with diminished resis tance to oxidation, possibly causing direct cellular and tissue injury , Whether alpha-1-antitrypsin deficiency and an impaired status of ant ioxidants, as seen in our patient, might play a role in the pathogenes is of FMD is presently unclear.