Differences between the pediatric and adult presentation of fibromuscular dysplasia: results from the US Registry
- 670 Downloads
Fibromuscular dysplasia (FMD) is a non-inflammatory arteriopathy that causes significant morbidity in children.
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.
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.
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.
KeywordsHypertension Renovascular hypertension Fibromuscular dysplasia Pediatrics
Our research efforts would not be possible without coordination by the Michigan Clinical Outcomes Research and Reporting Program (MCORRP), the support of the Fibromuscular Dysplasia Society of America and the American Society of Nephrology Foundation for Kidney Research (RG), and the encouragement and motivation provided by our community of FMD patients.
Compliance with ethical standards
This work was supported by the American Society of Nephrology Foundation for Kidney Research and the Fibromuscular Dysplasia Society of America, a nonprofit organization.
Dr Meyers is a consultant for Novartis and Biomarin. Drs Gornik, Olin, and Meyers are volunteer (noncompensated) medical advisory board members to the Fibromuscular Dysplasia Society of America (FMDSA). Pamela Mace, RN, is a paid employee of FMDSA.
- 4.Persu A, Giavarini A, Touze E, Januszewicz A, Sapoval M, Azizi M, Barral X, Jeunemaitre X, Morganti A, Plouin PF, de Leeuw P, ESH working Group on Hypertension and the Kidney (2014) European consensus on the diagnosis and management of fibromuscular dysplasia. J Hypertens 32:1367–1378CrossRefPubMedGoogle Scholar
- 8.Leadbetter W, Burkland L (1938) Hypertension in unilateral renal disease. J Urol 39:611–626Google Scholar
- 10.Olin JW, Gornik HL, Bacharach JM, Biller J, Fine LJ, Gray BH, Gray WA, Gupta R, Hamburg NM, Katzen BT, Lookstein RA, Lumsden AB, Newburger JW, Rundek T, Sperati CJ, Stanley JC, American Heart Association Council on Peripheral Vascular Disease; American Heart Association Council on Clinical Cardiology; American Heart Association Council on Cardiopulmonary Critical Care Perioperative Resuscitation; American Heart Association Council on Cardiovascular Disease in the Young; American Heart Association Council on Cardiovascular Radiology and Intervention; American Heart Association Council on Epidemiology and Prevention; American Heart Association Council on Functional Genomics and Translational Biology; American Heart Association Council for High Blood Pressure Research; American Heart Association Council on the Kidney in Cardiovascular Disease; American Heart Association Stroke Council (2014) Fibromuscular dysplasia: state of the science and critical unanswered questions: a scientific statement from the American Heart Association. Circulation 129:1048–1078CrossRefPubMedGoogle Scholar
- 16.Bayazit AK, Yalcinkaya F, Cakar N, Duzova A, Bircan Z, Bakkaloglu A, Canpolat N, Kara N, Sirin A, Ekim M, Oner A, Akman S, Mir S, Baskin E, Poyrazoglu HM, Noyan A, Akil I, Bakkaloglu S, Soylu A (2007) Reno-vascular hypertension in childhood: a nationwide survey. Pediatr Nephrol 22:1327–1333CrossRefPubMedGoogle Scholar
- 18.Kirton A, Crone M, Benseler S, Mineyko A, Armstrong D, Wade A, Sebire G, Crous-Tsanaclis AM, deVeber G (2013) Fibromuscular dysplasia and childhood stroke. Brain 136:1846–1856Google Scholar
- 26.Kim ES, Olin JW, Froehlich JB, Gu X, Bacharach JM, Gray BH, Jaff MR, Katzen BT, Kline-Rogers E, Mace PD, Matsumoto AH, McBane RD, White CJ, Gornik HL (2013) Clinical manifestations of fibromuscular dysplasia vary by patient sex: a report of the United States registry for fibromuscular dysplasia. J Am Coll Cardiol 62:2026–2028CrossRefPubMedGoogle Scholar