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Differences between the pediatric and adult presentation of fibromuscular dysplasia: results from the US Registry

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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.

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Acknowledgements

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.

Author information

Authors and Affiliations

  1. Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA

    Rebecca Green, Ann Marie Cahill & Kevin E. Meyers

  2. The Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Rebecca Green, Ann Marie Cahill & Kevin E. Meyers

  3. University of Michigan, Ann Arbor, MI, USA

    Xiaokui Gu, Eva Kline-Rogers & James Froehlich

  4. Fibromuscular Dysplasia Society of America, Rocky River, OH, USA

    Pamela Mace

  5. Greenville Health System, Greenville, SC, USA

    Bruce Gray

  6. Miami Baptist Cardiac/Vascular Institute, Miami, FL, USA

    Barry Katzen

  7. Icahn School of Medicine at Mount Sinai, New York, NY, USA

    Jeffrey Olin

  8. Cleveland Clinic Foundation, Cleveland, OH, USA

    Heather L. Gornik

  9. 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

    Rebecca Green

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  1. Rebecca Green
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Corresponding author

Correspondence to Rebecca Green.

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Funding Sources

This work was supported by the American Society of Nephrology Foundation for Kidney Research and the Fibromuscular Dysplasia Society of America, a nonprofit organization.

Disclosures

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.

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Green, R., Gu, X., Kline-Rogers, E. et al. Differences between the pediatric and adult presentation of fibromuscular dysplasia: results from the US Registry. Pediatr Nephrol 31, 641–650 (2016). https://doi.org/10.1007/s00467-015-3234-z

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  • DOI: https://doi.org/10.1007/s00467-015-3234-z

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