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Volume 10
Issue 8
10.3390/children10081282
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Article

Clinical Characterization of Pediatric Erythromelalgia: A Single-Center Case Series

by
Jenny Sun
1,
Don Daniel Ocay
1,2,
Meghan Halpin
1,
Kimberly Lobo
1,
Dafni F. T. Frohman
1,
Carolina Donado
1,2,
Catherine A. Brownstein
3,
Casie A. Genetti
3,
Anna Madden
3 and
Charles B. Berde
1,2,*
1
Department of Anesthesiology, Critical Care, and Pain Medicine, Boston Children’s Hospital, Boston, MA 02115, USA
2
Department of Anaesthesia, Harvard Medical School, Boston, MA 02115, USA
3
Manton Center for Orphan Disease Research, Division of Genetics and Genomics, Boston Children’s Hospital, Boston, MA 02115, USA
*
Author to whom correspondence should be addressed.
Children 2023, 10(8), 1282; https://doi.org/10.3390/children10081282
Submission received: 23 June 2023 / Revised: 18 July 2023 / Accepted: 21 July 2023 / Published: 26 July 2023
(This article belongs to the Section Pediatric Anesthesiology, Perioperative and Pain Medicine)
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Abstract

Erythromelalgia is a descriptive term for severe burning pain and erythema in the distal extremities relieved by cold and exacerbated by heat. Pediatric case series to date are relatively small. We extracted and analyzed medical record data for 42 pediatric patients to describe clinical characteristics, associated conditions, and responses to treatments. Informed consent was obtained according to an IRB-approved protocol that included gene discovery. Three patients had confirmed Nav1.7 sodium channelopathies, with six additional patients under investigation with novel gene candidates. There was a female predominance (2.5:1), and the median onset age was 12 years (IQR = 3–14). Patients saw a median of three specialists (IQR = 2–3) for a diagnosis. The majority (90%) reported bilateral symptoms. Cooling methods usually provided partial relief, while heat and exercise exacerbated pain. No medication appeared to be consistently effective; commonly prescribed medications included sodium channel blockers (n = 37), topical analgesics (n = 26), gabapentin (n = 22), and aspirin (n = 15). Based on the currently published literature, we believe this cohort is the largest pediatric study of erythromelalgia to date. Many findings are consistent with those of previously published case series. Work is in progress to establish a prospective cohort and multi-center registry.
Keywords: erythromelalgia; sodium channelopathy; small-fiber neuropathy erythromelalgia; sodium channelopathy; small-fiber neuropathy

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MDPI and ACS Style

Sun, J.; Ocay, D.D.; Halpin, M.; Lobo, K.; Frohman, D.F.T.; Donado, C.; Brownstein, C.A.; Genetti, C.A.; Madden, A.; Berde, C.B. Clinical Characterization of Pediatric Erythromelalgia: A Single-Center Case Series. Children 2023, 10, 1282. https://doi.org/10.3390/children10081282

AMA Style

Sun J, Ocay DD, Halpin M, Lobo K, Frohman DFT, Donado C, Brownstein CA, Genetti CA, Madden A, Berde CB. Clinical Characterization of Pediatric Erythromelalgia: A Single-Center Case Series. Children. 2023; 10(8):1282. https://doi.org/10.3390/children10081282

Chicago/Turabian Style

Sun, Jenny, Don Daniel Ocay, Meghan Halpin, Kimberly Lobo, Dafni F. T. Frohman, Carolina Donado, Catherine A. Brownstein, Casie A. Genetti, Anna Madden, and Charles B. Berde. 2023. "Clinical Characterization of Pediatric Erythromelalgia: A Single-Center Case Series" Children 10, no. 8: 1282. https://doi.org/10.3390/children10081282

APA Style

Sun, J., Ocay, D. D., Halpin, M., Lobo, K., Frohman, D. F. T., Donado, C., Brownstein, C. A., Genetti, C. A., Madden, A., & Berde, C. B. (2023). Clinical Characterization of Pediatric Erythromelalgia: A Single-Center Case Series. Children, 10(8), 1282. https://doi.org/10.3390/children10081282

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