title: Differences between the pediatric and adult presentation of fibromuscular dysplasia: results from the US Registry.
abstract: BACKGROUND
  Fibromuscular dysplasia (FMD) is a non-inflammatory arteriopathy that causes significant morbidity in children.
  
  
  METHODS
  The clinical features, presenting symptoms, and vascular beds involved are reviewed in the first 33 patients aged <18 years who are enrolled in the United States Registry for FMD from five registry sites and compared with 999 adult patients from 12 registry sites.
  
  
  RESULTS
  Mean age at diagnosis was 8.4 ± 4.8 years (16 days to 17 years). Compared with adults, pediatric FMD occurs in more males (42.4 vs 6 %, p < 0.001). Children with FMD have a stronger previous history of hypertension (93.9 vs 69.9 %, p = 0.002). Hypertension (100 %), headache (55 %), and abdominal bruits (10.7 %) were the most common presenting signs and symptoms. FMD affects renal vasculature in almost all children (97 vs 69.7 %, p = 0.003). The extra-cranial carotid vessels are less commonly involved in children (23.1 vs 73.3 %, p < 0.001). The mesenteric arteries (38.9 vs 16.2 %, p = 0.02) and aorta (26.3 vs 2.4 %, p < 0.001) are more commonly involved in children.
  
  
  CONCLUSIONS
  In the United States Registry for FMD, pediatric FMD affects children from infancy throughout childhood. All children presented with hypertension and many presented with headache and abdominal bruits. In children, FMD most commonly affects the renal vasculature, but also frequently involves the mesenteric arteries and abdominal aorta; the carotid vessels are less frequently involved.
authors.affiliation: Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA. greenrs@email.chop.edu.|The Children's Hospital of Philadelphia, Philadelphia, PA, USA. greenrs@email.chop.edu.|Division of General Pediatrics, The Children's Hospital of Philadelphia and University of Pennsylvania, 9NW63, 34th Street and Civic Center Boulevard, Philadelphia, PA, 19104-4399, USA. greenrs@email.chop.edu.
authors.forename: Rebecca
authors.initials: R
authors.lastname: Green
authors.affiliation: University of Michigan, Ann Arbor, MI, USA.
authors.forename: Xiaokui
authors.initials: X
authors.lastname: Gu
authors.affiliation: University of Michigan, Ann Arbor, MI, USA.
authors.forename: Eva
authors.initials: E
authors.lastname: Kline-Rogers
authors.affiliation: University of Michigan, Ann Arbor, MI, USA.
authors.forename: James
authors.initials: J
authors.lastname: Froehlich
authors.affiliation: Fibromuscular Dysplasia Society of America, Rocky River, OH, USA.
authors.forename: Pamela
authors.initials: P
authors.lastname: Mace
authors.affiliation: Greenville Health System, Greenville, SC, USA.
authors.forename: Bruce
authors.initials: B
authors.lastname: Gray
authors.affiliation: Miami Baptist Cardiac/Vascular Institute, Miami, FL, USA.
authors.forename: Barry
authors.initials: B
authors.lastname: Katzen
authors.affiliation: Icahn School of Medicine at Mount Sinai, New York, NY, USA.
authors.forename: Jeffrey
authors.initials: J
authors.lastname: Olin
authors.affiliation: Cleveland Clinic Foundation, Cleveland, OH, USA.
authors.forename: Heather L
authors.initials: HL
authors.lastname: Gornik
authors.affiliation: Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.|The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
authors.forename: Ann Marie
authors.initials: AM
authors.lastname: Cahill
authors.affiliation: Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.|The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
authors.forename: Kevin E
authors.initials: KE
authors.lastname: Meyers
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D001012:Aorta, Abdominal / Q000000981:diagnostic imaging*; D002339:Carotid Arteries / Q000000981:diagnostic imaging*; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D000072226:Computed Tomography Angiography; D005260:Female; D005352:Fibromuscular Dysplasia / Q000000981:diagnostic imaging* / Q000453:epidemiology / Q000628:therapy; D006261:Headache / Q000453:epidemiology; D006801:Humans; D006973:Hypertension / Q000453:epidemiology; D007223:Infant; D007231:Infant, Newborn; D018810:Magnetic Resonance Angiography; D008297:Male; D008638:Mesenteric Arteries / Q000000981:diagnostic imaging*; D008875:Middle Aged; D011237:Predictive Value of Tests; D011379:Prognosis; D012042:Registries; D012077:Renal Artery / Q000000981:diagnostic imaging*; D012189:Retrospective Studies; D012307:Risk Factors; D018084:Ultrasonography, Interventional; D014481:United States / Q000453:epidemiology; D055815:Young Adult
keywords: Fibromuscular dysplasia; Hypertension; Pediatrics; Renovascular hypertension