Use of dexamethasone in idiopathic, acute pediatric rhabdomyolysis

Maxwell L. Summerlin, Debra S. Regier, Jamie L. Fraser, Kimberly A. Chapman, Dariush Kafashzadeh, Charles Billington, Monisha Kisling, Angela Grochowsky, Nicholas Ah Mew, Natasha Shur

Research output: Contribution to journalArticlepeer-review

4 Scopus citations

Abstract

Current rhabdomyolysis treatment guidelines vary based on the etiology and diagnosis, yet many cases evade conclusive diagnosis. In these cases, treatment options remain largely limited to fluids and supportive therapy. We present two cases of acute rhabdomyolysis diagnosed in the emergency department: a 5-year-old boy with sudden onset bilateral flank pain, and a 13-year-old boy with 2–3 days of worsening pectoral and shoulder pain. Each patient had a prior similar episode requiring hospitalization in the past. The 5-year-old had no inciting trauma or trigger, medication use, or illness. The 13-year-old previously had an upper respiratory infection during the week prior and had been strenuously exercising at the time of onset. Genetic testing results were unknown for both patients during their hospitalizations, and insurance and other barriers led to delay. Later results for the first patient revealed a heterozygous deletion in intron 19 on the LPIN1 gene interpreted as a variant of unknown significance. During their hospitalizations, both children were started on intravenous (i.v.) fluids, and creatine kinase (CK) initially trended downward, but then began to rise or plateau. After reviewing the cases, prior literature, and anecdotal evidence of benefit from corticosteroid therapy in rhabdomyolysis with our consultant metabolic physicians, dexamethasone was initiated. In both patients, dexamethasone use correlated with relief of patient symptoms, significantly decreased CK value, and our ability to discharge these patients home quickly. Our cases, discussion, and literature review all lead to the consideration of the use of dexamethasone in conjunction with standard therapy for acute rhabdomyolysis.

Original languageEnglish (US)
Pages (from-to)500-507
Number of pages8
JournalAmerican Journal of Medical Genetics, Part A
Volume185
Issue number2
Early online dateDec 10 2020
DOIs
StatePublished - Dec 10 2020

Bibliographical note

Funding Information:
We would like to thank Marshall Summar, MD, from The Rare Disease Institute, and all our patients we serve.

Publisher Copyright:
© 2020 Wiley Periodicals LLC

Keywords

  • creatine kinase
  • dexamethasone
  • rhabdomyolysis
  • steroids

Fingerprint

Dive into the research topics of 'Use of dexamethasone in idiopathic, acute pediatric rhabdomyolysis'. Together they form a unique fingerprint.

Cite this