Renal dysfunction can occur in advanced-stage Duchenne muscular dystrophy

Muscle Nerve. 2020 Feb;61(2):192-197. doi: 10.1002/mus.26757. Epub 2019 Nov 28.

Abstract

Introduction: With improved treatments, patients with Duchenne muscular dystrophy (DMD) can survive far beyond adolescence. However, advanced-stage DMD patients are at risk of developing renal dysfunction. In this study, long-term renal function outcomes and associated risk factors in advanced stage DMD were analyzed.

Methods: Fifty-one patients were classified into three different age groups (<20, 20-29, and ≥30 years of age), and cystatin C (CysC) levels were compared among groups.

Results: Median serum CysC levels were 0.74 mg/L, 0.63 mg/L, and 0.76 mg/L in the age groups of <20, 20-29, and ≥30 years, respectively (P = .003). Five of the nine patients in the ≥30 years age group showed elevated serum CysC and decreased cardiac function compared with the other four in the group (P = .014).

Discussion: Our results indicate an association between cardiac and renal dysfunction in patients with advanced-stage DMD.

Keywords: Duchenne muscular dystrophy; advanced stage; cystatin C; ejection fraction; fractional shortening; renal dysfunction.

MeSH terms

  • Adolescent
  • Adult
  • Aging
  • Child
  • Child, Preschool
  • Cystatin C / blood
  • Disease Progression
  • Female
  • Heart Diseases / etiology
  • Heart Diseases / physiopathology
  • Heart Function Tests
  • Humans
  • Kidney Diseases / etiology*
  • Kidney Diseases / physiopathology
  • Kidney Function Tests
  • Male
  • Muscular Dystrophy, Duchenne / complications*
  • Muscular Dystrophy, Duchenne / physiopathology
  • Risk Factors
  • Young Adult

Substances

  • Cystatin C