International Journal of Research in Medical Sciences | June 2018 | Vol 6 | Issue 6 Page 2138
International Journal of Research in Medical Sciences
Negi B et al. Int J Res Med Sci. 2018 Jun;6(6):2138-2142
www.msjonline.org pISSN 2320-6071 | eISSN 2320-6012
Original Research Article
Nerve conduction study in children with thalassemia
Banita Negi
1
, Parveen Bhardwaj
1
, Minoo Sharma
2
*, Sudhir Sharma
3
, Neelam Grover
1
INTRODUCTION
Beta- thalassemia is an inherited disorder of hemoglobin
characterized by an absence or reduced synthesis of beta
globin chain. The net result is an excess of alfa chains,
which precipitate and destroy the red cell precursors,
leading to anemia, skeletal changes, splenomegaly and
numerous other complication. Beta thalassemia major
was first described in 1925 by Thomas Cooley and Lee.
1
In those days, thalassemia major patients rarely used to
survive the first decade of life. Following the introduction
of regular transfusion regimens in the 1960’s, initially by
Orsini, and later by Wolman and Piomelli, thalassemics
survived into 2nd and 3rd decades.
2-5
As a result of this
improved survival due to transfusion therapy, the
problems of transfusional hemosiderosis became
conspicuous. The combination of transfusion and
chelation therapy has dramatically extended the life
expectancy of these patients, thus transforming
thalassemia from a rapidly fatal disease of childhood to a
chronic illness compatible with a prolonged life.
6
Heart failure, arrhythmias, osteoporosis, bone pain, and
bone changes, bile stone formation, increased risk of viral
hepatitis, cirrhosis, delayed puberty, growth retardation;
1
Department of Pediatrics,
2
Department of Physiology,
3
Department of Neurology, Indira Gandhi Medical College,
Shimla, Himachal Pradesh, India
Received: 10 April 2018
Accepted: 01 May 2018
*Correspondence:
Dr. Minoo Sharma,
E-mail: minoo01sharma@gmail.com
Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under
the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial
use, distribution, and reproduction in any medium, provided the original work is properly cited.
ABSTRACT
Background: Regular blood transfusion and iron chelation therapy has increased life span of children with
Thalassemia and with prolongation of life expectancy the toxic effect of iron on nervous system are being
increasingly reported. Aim was to study nerve conduction study in thalassemia children and effect of iron overload on
NCV.
Methods: 30 children with thalassemia on regular transfusions and iron chelation therapy and 30 healthy age and sex
matched controls were subjected to nerve conduction study on three motor and sensory nerves. Statistical analysis
used: means of quantitative variables were calculated in two groups and compared with student t- test. A p-value of
<0.05 was taken as significant.
Results: On comparing the results the between cases and controls, we found that, there was no significant difference
in the distal latency, amplitude and nerve conduction velocity of all three motor nerves and sensory nerves which
were evaluated. Cases were further divided in to two groups depending upon serum ferritin level of < and
>1000ng/ml. On comparing these two groups it was noted that distal latency was increased, nerve conduction velocity
was slow in all the motor nerves (i.e. median, ulnar and tibial nerves) and sensory nerves (i.e. median, ulnar and sural
nerves) in group with serum ferritin level >1000ng/ml and these findings were statistically significant.
Conclusions: We concluded that in children with thalassemia on regular transfusion and Iron chelation regime, nerve
conduction study is normal in comparison to normal control but with progressive increase in serum ferritin level, the
latency and conduction velocity is decreased and is statistically significant.
Keywords: Deferasirox, Ferritin, Nerve conduction study, Thalassemia
DOI: http://dx.doi.org/10.18203/2320-6012.ijrms20182302