Peripheral neuropathy in cystic fibrosis: a prevalence study

J Cyst Fibros. 2013 Dec;12(6):754-60. doi: 10.1016/j.jcf.2013.01.005. Epub 2013 Feb 5.

Abstract

Background: Information on peripheral neuropathy in children with cystic fibrosis is scanty. The etiology can be multifactorial (micronutrient deficiency, chronic hypoxia, impaired glucose tolerance, immunological, vasculopathic, critical illness).

Methods: Forty five cystic fibrosis children aged 1-18 years on vitamin E supplementation for at least 6 months underwent detailed neurological examination, serum vitamin E, vitamin B12, folate, copper levels and detailed nerve conduction studies.

Results: The mean age of the study population was 8.35 years (±4.9 years) with 62.2% being males. Overall 22 out of 45 (48.88%,CI: 33.7-64.2) had electrophysiological evidence of peripheral neuropathy which was predominantly axonal (86.4%), sensory (50%), and polyneuropathy (95.45%). There was no significant association between status of serum micronutrients and electrophysiological evidence of peripheral neuropathy.

Conclusion: Patients with cystic fibrosis have electrophysiological evidence of peripheral neuropathy (predominantly axonal, sensory and polyneuropathy). There is significant association of higher chronological age with occurrence of peripheral neuropathy.

Keywords: Axonal; Cystic fibrosis; Micronutrients; Polyneuropathy; Sensory.

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Comorbidity
  • Copper / blood
  • Cystic Fibrosis / blood
  • Cystic Fibrosis / epidemiology*
  • Female
  • Folic Acid / blood
  • Humans
  • Infant
  • Male
  • Peripheral Nervous System Diseases / blood
  • Peripheral Nervous System Diseases / epidemiology*
  • Prevalence
  • Vitamin B 12 / blood
  • Vitamin E / blood

Substances

  • Vitamin E
  • Copper
  • Folic Acid
  • Vitamin B 12