Fibromuscular dysplasia of the internal carotid artery in a child with alpha-1-antitrypsin deficiency

Developmental Medicine and Child Neurology
B SölderW Jaschke

Abstract

Fibromuscular 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.

References

Nov 1, 1991·Stroke; a Journal of Cerebral Circulation·W I SchievinkD G Piepgras
Oct 1, 1990·The Journal of Surgical Research·J R CohenD Tilson
Feb 1, 1990·The Journal of Pediatrics·M WallA S Buist
Nov 1, 1988·Acta paediatrica Scandinavica·T Sveger
Apr 1, 1988·Stroke; a Journal of Cerebral Circulation·A Perez-HiguerasE Diez-Tejedor
Dec 1, 1966·The American Journal of Roentgenology, Radium Therapy, and Nuclear Medicine·A J PalubinskasT H Newton
May 28, 1994·Lancet·S CattanJ C Brouet
Nov 1, 1994·Mayo Clinic Proceedings·W I SchievinkU B Prakash
Feb 10, 1994·The New England Journal of Medicine·W I SchievinkW M O'Fallon
Feb 1, 1996·Developmental Medicine and Child Neurology·P Nicolaides, R E Appleton

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Citations

Apr 21, 2004·Current Treatment Options in Cardiovascular Medicine·Megan C. LearyLouis R. Caplan
May 20, 1999·Journal of Child Neurology·V Puri, G Riggs
Jan 27, 2009·Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology·Mine OzdilSait Albayram
Mar 15, 2006·Journal of Hypertension·Jérôme PerduXavier Jeunemaitre

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