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Indian Pediatrics

, Volume 56, Issue 1, pp 41–44 | Cite as

Wrist Deformity in Children and Adolescents with β-thalassemia on Oral Iron Chelation Therapy

  • Rashid H. Merchant
  • Ameya Kulkarni
  • Pezad N. DoctorEmail author
  • Amitkumar Choudhari
  • Deepak P. Patkar
Resecrch Paper
  • 3 Downloads

Abstract

Objective

To describe a novel wrist deformity in β-thalassemia major patients, and their radiographic and magnetic resonance imaging findings.

Methods

30 patients with β-thalassemia major who were noticed to have ulnar deviation at wrist joint were evaluated for previous history of medications, serum ferritin levels, presence of pain and swelling at the wrist joint, and the duration of iron chelation therapy. Radiographs of wrist and limited magnetic resonance imaging (MRI) sequences were obtained in 30 and 15 patients, respectively.

Results

Radiographs revealed varying severity of distal ulnar shortening, distal radial slanting and presence of soft tissue distal to the ulna. MRI showed similar deformities along with abnormal marrow signal at distal ulnar ends; in 8 patients, a soft tissue distal to the distal end of ulna was noted.

Conclusions

Varying severity of radiological abnormalities, predominantly affecting the distal ulna, are present in children and adolescents with β-thalassemia receiving oral chelation therapy.

Keywords

Arthropathy Bone deformity Deferiprone Growth plate 

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References

  1. 1.
    Cohen AR, Galanello R, Piga A. Safety profile of the oral iron chelator deferiprone: A multi–centre study. Br J Haematol. 2000;108:305–12.CrossRefGoogle Scholar
  2. 2.
    Naithani R, Chandra J, Sharma S. Safety of oral iron chelator deferiprone in young thalassaemics. Eur J Haematol. 2005;74:217–20.CrossRefGoogle Scholar
  3. 3.
    Al–Refaie FN, Hershko C, Hoffbrand AV, Kosaryan M, Olivieri NF, Tondury P, et al. Results of long–term deferiprone (L1) therapy: A report by the International study group on oral iron chelators. Br J Haematol. 1995;91:224–9.CrossRefGoogle Scholar
  4. 4.
    Kellenberger CJ, Schmugge M, Saurenmann T, Gennaro LD, Eber SW, Willi UV, et al. Radiographic, MRI features of deferiprone–related arthropathy of the knees in patients with β–thalassemia. Am J Radiol. 2004;183:989–94.Google Scholar
  5. 5.
    Sharma R, Anand R, Chandra J, Seth A, Pemde H, Singh V. Distal ulnar changes in children with thalassemia and deferiprone related arthropathy. Pediatr Blood Cancer. 2013;60:1957–62.CrossRefGoogle Scholar
  6. 6.
    Frankel VH. The Terry–Thomas sign. Clin Orthop Relat Res. 1977;129:321–2.CrossRefGoogle Scholar
  7. 7.
    Chan YL, Pang LM, Chik KW, Cheng JC, Li CK. Patterns of bone disease in transfusion dependent homozygous thalassemia major: Predominance of osteoporosis and deferoxamine–induced bone dysplasia. Pediatr Radiol. 2002;32:492–7.CrossRefGoogle Scholar
  8. 8.
    Chan YL, Li CK, Pang LM, Chik KW. Deferoxamine induced long bone changes in patients–radiographic features, prevalence and relations with growth. Clin Radiol. 2000;55:610–4.CrossRefGoogle Scholar
  9. 9.
    Berdoukas V, Bentley P, Frost H, Schnebli HP. Toxicity of oral iron chelator L1. Lancet. 1993;341:1088.CrossRefGoogle Scholar
  10. 10.
    Diav–Citrin O, Koren G. Oral iron chelation with deferiprone. Pediatr Clin North Am. 1997;44:235–47.CrossRefGoogle Scholar
  11. 11.
    Berkovitch M, Laxer RM, Inman R, Koren G, Pritzker KP, Fritzler MJ, et al. Arthropathy in thalassemia patients receiving deferiprone. Lancet. 1994;343:1471–2.CrossRefGoogle Scholar
  12. 12.
    Greulich WW, Pyle SI. Radiographic atlas of skeletal development of the hand and wrist. 2nd edition. Stanford, California: Stanford University Press. October 1959. p. 513.CrossRefGoogle Scholar

Copyright information

© Indian Academy of Pediatrics 2019

Authors and Affiliations

  • Rashid H. Merchant
    • 1
  • Ameya Kulkarni
    • 2
  • Pezad N. Doctor
    • 1
    Email author
  • Amitkumar Choudhari
    • 2
  • Deepak P. Patkar
    • 2
  1. 1.Department of PediatricsNanavati Super Speciality HospitalMumbaiIndia
  2. 2.Department of RadiologyNanavati Super Speciality HospitalMumbaiIndia

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