Nerve conduction study in children with thalassemia

Authors

  • Banita Negi Department of Pediatrics, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India
  • Parveen Bhardwaj Department of Pediatrics, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India
  • Minoo Sharma Department of Physiology, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India
  • Sudhir Sharma Department of Neurology, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India
  • Neelam Grover Department of Pediatrics, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India

DOI:

https://doi.org/10.18203/2320-6012.ijrms20182302

Keywords:

Deferasirox, Ferritin, Nerve conduction study, Thalassemia

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.

References

Cooley T, Lee P. A series of cases of splenomegaly in children with anemia and peculiar bone changes. Trans Am Pediatr. 1925;37:29.

Orsini A, Boyer G. Thalassemia in Marseille; data on the frequency and observations on some clinical therapeutic and assisitential aspects. The social problem of microcythemia and Cooley's disease. Rome, 1961.

Wolman LJ. Transfusion therapy in Cooley’s anemia. Growth and health as related to long range hemoglobin level. Ann N Y Acad Sci. 1964;119:736.

Wolman LJ, Ortolani M. Some clinical features of Cooley’s anemia patients as related to transfusion schedules. Ann N Y Acad Sci. 1969;105:407-14.

Piomelli S, Danoff SJ, Becker MH, Lipera MJ, Travis SF. Prevention of bone malformations and cardiomegaly in Cooley's anemia by early hypertransfusion regimen. Annals New York Academy Sciences. 1969 Nov 1;165(1):427-36.

Khan DI. Thalassemia: Still a challenge. Gomal J Med Sci. 2006;4:47-8.

Cunningham MJ, Shankaran VG, Nathan DG, orkin SH. The Thalassemia. In: Orkin SH, Nathan DG, Ginsberg D, Look AT. Nathan and Oski’s Hematology of infancy and childhood, 7th ed, Philadelphia: Sounders Elsevier;2009:1015-1109.

Moorjani JD, Chithira I. Neurotic manifestations in adolescents with thalassemia major. Indian J Pediatr. 2006;73:603-7.

Wong V, Li A, Lee A. Neurophysiological study of betathalassemia patients. J Child Neurol. 1993;8:330-5.

Zafeiriou DI, Kousi AA, Tsantali CT, Kontopoulos EE, Augoustidou-Savvopoulou PA. Neurophysiologic evaluation of long-term desferrioxamine therapy in beta-thalassemia patients. Pediatric Neurology. 1998;18:420-24.

Papanastasiou D, Papanikolaou D, Magiakau AM, Beratis NG, Tzebelikos E, Papapetropoulos T. Peripheral neuropathy in patients with b-thalassemia. J neurol Neurosurg Psychiatry. 1991;54:997-1000.

Stamboulis E, Vlachou N, Drossou M, Tsaftaridis P, Koutsi G, Katsaros N, Economou-Petersen E, et al. Axonal sensorimotor neuropathy in patients with beta-thalassemia. J Neurol Neurosurg Psychiatry. 2004;75:1483-6.

Sawaya RA, Zahed L, Taher A. Peripheral neuropathy in thalassaemia. Ann Saudi Med. 2006;26(5):358-63.

Downloads

Published

2018-05-25

How to Cite

Negi, B., Bhardwaj, P., Sharma, M., Sharma, S., & Grover, N. (2018). Nerve conduction study in children with thalassemia. International Journal of Research in Medical Sciences, 6(6), 2138–2142. https://doi.org/10.18203/2320-6012.ijrms20182302

Issue

Section

Original Research Articles