Rebecca Green1,2,3, Xiaokui Gu4, Eva Kline-Rogers4, James Froehlich4, Pamela Mace5, Bruce Gray6, Barry Katzen7, Jeffrey Olin8, Heather L Gornik9, Ann Marie Cahill10,11, Kevin E Meyers10,11. 1. Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA. greenrs@email.chop.edu. 2. The Children's Hospital of Philadelphia, Philadelphia, PA, USA. greenrs@email.chop.edu. 3. 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. 4. University of Michigan, Ann Arbor, MI, USA. 5. Fibromuscular Dysplasia Society of America, Rocky River, OH, USA. 6. Greenville Health System, Greenville, SC, USA. 7. Miami Baptist Cardiac/Vascular Institute, Miami, FL, USA. 8. Icahn School of Medicine at Mount Sinai, New York, NY, USA. 9. Cleveland Clinic Foundation, Cleveland, OH, USA. 10. Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA. 11. The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
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.
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.
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