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Polyneuropathy Associated with Severe Iron Overload and Oxidative Stress in β-Thalassemia Patients

  • Mona H. El-Tagui
  • Khaled M. Salama
  • Mohamed H. El-Sabbagh
  • Eman R. Youness
  • Marwa Ragaey
  • Amina Abdel-SalamEmail author
Original Article
  • 28 Downloads

Abstract

To investigate the frequency of peripheral neuropathy in patients with β-thalassemia, and to assess its relation to iron overload and oxidative stress. Sixty β-thalassemia patients with mean age of 19 ± 4.9 years were recruited. Serum ferritin was quantitatively assessed by enzyme-linked immunoassay and biomarkers of oxidative stress were estimated calorimetrically. Electrophysiological studies using NEMUS 2, Galileu Software were carried out. The patients were separated into two groups: those with abnormal nerve conduction studies (NCS) {Group A; N = 38} and those with normal NCS {Group B; N = 22}. Thirty-eight (63.3%) patients had axonal motor neuropathy as evidenced by abnormal NCS (group A), they showed higher mean serum ferritin (p < 0.01), higher mean malondialdehyde (MDA) (p < 0.01), and lower mean nitrous oxide, total antioxidant capacity, paraoxonase-1 (PON1) (p < 0.01) compared to group B. Bivariate analysis of NCS data demonstrated that abnormal NCS were more frequent in splenectomized patients (p = 0.002), and poorly-chelated patients with serum ferritin ≥ 2000 ng/ml (p = 0.001). Significant variables associated with abnormal motor NCS were entered in stepwise regression analysis and only elevated serum ferritin (p = 0.01) was independently associated with abnormal motor NCS (p = 0.02; 95% CI 1.433–51.791). None of the studied patients had sensory neuropathy or myopathy. Peripheral motor neuropathy may occur in β-thalassemia patients at a high frequency, regardless of their age and gender. Severe iron overload may contribute to the pathogenesis of neuropathy. Other factors including chelation therapy, splenectomy, and oxidative stress might have an enhancing effect that couldn’t be proved in this study.

Keywords

Polyneuropathy Iron overload Oxidative stress-β-Thalassemia 

Notes

Acknowledgements

We are indebted to every patient included in this study and also their parents for their corporation and their trust. We wish them all the best of health and happiness.

Funding

This research received no specific grant from any funding agency in the public, commercial or not-for-profit sectors.

Compliance with Ethical Standards

Conflict of interest

All authors declare that they have no conflict of interest.

Statement of Human Rights

The current study have been approved by Cairo University research ethics committee (IRB) and have been performed in accordance with the ethical standards as laid down in the 1964 Declaration of Helsinki and its later amendments or comparable ethical standards.

Ethical Approval

All procedures performed in the current study were in accordance with the ethical standards of our institutional research committee and with the 1964 Helsinki declaration and its later amendments.

Informed Consent

Informed consent was obtained from all individual participants and/or the legal guardians of participants included in the study.

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Copyright information

© Indian Society of Hematology and Blood Transfusion 2018

Authors and Affiliations

  1. 1.Department of PediatricsCairo UniversityCairoEgypt
  2. 2.Department of Pediatrics and NeurophysiologyNational Institute of Neuromotor SystemCairoEgypt
  3. 3.Department of Medical BiochemistryNational Research Centre (NRC)GizaEgypt

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