Assessment of Growth parameters in Transfusion dependent Thalassemics at a tertiary care hospital
Keywords:
Thalassemia major, growth failure, percentile, chelationAbstract
Background: Thalassemia is a group of genetic blood disorders all of which involve under production of haemoglobin , and partial or complete failure of synthesis a specific type of globin chain . Thalassemia major is most common monogenic disorder in the world. Around 1,00,000 children are born each year with the severe homozygous state of the disease in India. The objectives of this study was to assess clinical data and the growth parameters of thalassemia major patients attending tertiary care hospital.Beta Thalassemia Major (BTM) is transfusion dependent state where physical growth is affected in majority of patients. This study will emphasize to assess iron overload and growth parameters of transfusion dependent BTM patients. This was undertaken as very few studies have been done in this region of the our country.
Methods: The study was done at a tertiary care teaching hospital from January 2014 to December 2014. Universal sampling method was used and 31 β thalassemia major patients who received blood transfusions at 2 to 4 weeks interval in the hospital were included in this study. Clinical details and blood transfusion record was collected on proforma for all patients and data interpreted. This study was done on 31 known diagnosed BTM patients with age 10 years and above. Height, weight, BMI along with hemoglobin and serum ferritin were estimated. IAP growth charts were plotted for various parameters. Appropriate statistical tests were used for analysis.
Result: Of 31 patients, 25 were males and 6 were females . Age range was 10 -18 years with mean age of 12.45 years. There were 20(64.5%) patients not taking chelation. Mean and SD of pretransfusion hemoglobin of all patients was 6.85±1.13 gm%. Mean and SD of ferritin of all patients, nonchelated and chelated patients was 3786 ±2382 ng/ml, 4505±2633 ng/ml and 2479 ± 963.6 ng/ml respectively. Weight for Age (W/A) charts of males and females showed 28% and 16.6 % respectively below 3rd percentile. Height for age (H/A) charts of males showed 40 % below 3rd percentile and none of the females below 3rd percentile. BMI of males and females showed 20% and 50% respectively below 3rd percentile. Most of the patients were of short stature and underweight indicating growth failure.
Conclusion: The present study describes the growth parameters of β thalassemia major patients attending the tertiary care hospital and emphasizes on maintenance of growth charts of these patients for better management. Growth failure in BTM is mainly due to iron overload and chronic anemia. This study emphasizes the need of screening for growth failure, regularly estimating iron overload and its control by chelation therapy.
DOI:10.21276/APALM.1833
References
2. Noguchi C, Butterwoth J,Karawajew L,Kupperrs R, Jacobsohn D. Haematologica 2004;89:1281-3.
3. Cao A, Galanello R. Beta thalassemia.Genet Med 2010;12:61-76.
4. Joint WHO-March of Dimes Meeting on Management of Birth Defects and Haemoglobin Disorders (2nd:2006:Geneva,Switzerland),World Health Organization, March of Dimes. Meeting goals. In:Management of birth defects and haemoglobin disorders. Geneva:World Health Organization;2006:5.
5. Sarnaik AS. Thalassemia and Related Hemoglobinopathies. Indian J Pediatr 2005;72(4):319-24.
6. De Sanctis V, Soliman AT, Elsedfy H, Skordis N, Kattamis C, Angastiniotis M, et al. Growth and endocrine disorders in thalassemia: The international network on endocrine complications in thalassemia (I-CET) position statement and guidelines. Indian J Endocr Metab 2013;17:8-18.
7. Olivieri NF, Brittenham GM. Iron-chelating therapy and the treatment of thalassemia. Blood 1997;89(3):739-61.
8. Grundy RG, Woods KA, Savage MO, Evans JP. Relationship of endocrinopathy to iron chelation status in young patients with thalassaemia major. Arch Dis Child 1994;71(2):128-32.
9. Kwan EY, Lee AC, Li AM, Tam SC, Chan CF, Lau YL, et al. A cross-sectional study of growth, puberty and endocrine function in patients with thalassaemia major in Hong Kong. J Paediatr Child Health 1995;31(2):83-7.
10. Borgna-Pigatti C, De Stefano P, Zonte L. Growth and puberty in thalassaemia major. An interim report. In: Sirchia G, Zanella A. Thalassaemia today: the Mediterranean experience. Milan: Centro Transfusionale Ospedale Maggiore Policlinico di Milano Editore.1987:88-92.
11.Soliman AT, El Zalabany MM, Amer M, Ansari BM. Growth and pubertal development in transfusion- dependent children and adolescents with thalassaemia major. Hemoglobin 2009;33:16–20.
12. Kazazian HH Jr. The thalassemia syndromes: molecular basis and prenatal diagnosis in 1990. Semin Hematol 1990;27:209-28.
13. Higgs DR, Thein SL,Woods WG. The Molecular Pathology of the Thalassemias. In: Weatherall DJ, Clegg B. The Thalassemia Syndromes.4th ed., Oxford: Blackwell Science; 2001:133–191.
14. Borgna-Pignatti C, Cappellini MD, De Stefano P, Del Vecchio GC, Forni GL, Gamberini MR, et al. Survival and complications in thalassemia. Ann N Y Acad Sci 2005;1054:40-7.
15. Chern JP, Su S, Lin KH, Chang SH, Lu MY, Jou ST, et al. Survival, mortality and complications in patients with beta thalassaemia major in northern Taiwan. Pediatr Blood Cancer 2006;47:432-7.
16. Khalifa AS, Salem M, Mounir E, El-Tawil MM, El-Sawy M, Abd Al-Aziz MM. Abnormal glucose tolerance in Egyptian beta-thalassemic patients: possible association with genotyping. Pediatr Diabetes 2004;5:126-32.
17. Najafipour F, Sorkhabi RS, Aghai NH, Zareizadeh M, Bahrami A. Importance of OGTT for diagnosis of Diabetes in thalassemia major patients. J Gorgan Uni Med Sci 2008;10(3):71-6.
18. Borgna-Pignatti C, De Stefano P, Zonta et al. Growth and sexual maturation in thalassemia Major. J Pediatr 1985;106:150-7.
19. George A, Bhaduri A, Ben S, Choudhry VP. Physical Growth Parameters in Thalassemic Children. Indian J Pediatr 1997; 64:861-71.
20. Hamidah A, Arini MI, Zarina AL, Zulkifli SZ, Jamal R. Growth velocity in transfusion dependent prepubertal thalassemia patients: results from a thalassemia center in Malaysia.Southeast Asian J Trop Med Public Health 2008;39(5):900-5.
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2018 Mallikarjun A Pattanashetti, Ganga S Pilli
This work is licensed under a Creative Commons Attribution 4.0 International License.
Authors who publish with this journal agree to the following terms:
- Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.
- Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.
- Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work (See The Effect of Open Access at http://opcit.eprints.org/oacitation-biblio.html).