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Category: Volume 08 Issue 01 January 2020
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Title: Study of Growth Hormone Levels in Thalessemia Major Patients in Children

Authors: Shruti Dhale, Rasika Hatttewar, Poonam Patil, Abhinaya Vaidyanathan, Kuldip Shimpi

 DOI: https://dx.doi.org/10.18535/jmscr/v8i1.95

Abstract

Introduction: Thalassemia is an inherited disorder of hemoglobin synthesis. Regular blood transfusions and chelation therapy have notice ably prolonged survival in thalassemic patients.[1] Despite a significant increase in the life span of these patients, they suffer from multiple abnormalities probably due to iron over load, including endocrinal abnormalities such as hypogonadism, diabetes mellitus, hypothyroidism and hyperparathyroidism.[2] The pattern of levels of growth hormone levels among the patients with thalassemia major undergoing repeated blood transfusions remain unexplored. Very few studies have been undertaken among Indian population.

Methodology: It is a prospective study and was conducted at St George hospital, Mumbai, on diagnosed patients of thalassemia major admitted to the paediatric wards. Duration of the study was 3 months and 20 patients were included.

Inclusion Criteria

  1. All admitted patients of thalassemia major aged 18 years and below.

Exclusion Criteria

  1. Chronic haemolytic anemia apart from thalassemia major
  2. Those that was very sick
  3. Those suffering from malnutrition
  4. Those who were on supplementation of growth hormone.

Patients who were willing to participate and sign the inform consent were enrolled in the study.

Growth hormone levels were done using CLIA.

Results: The mean age of the studied thalassemia patients was 9.65 ± 4.23  years with no gender preponderance. Mean (SD) Growth hormone levels was  0.769 ng/dl ± 0.540 ng/dl and was not  found to be statistically significant (p>0.001).

Conclusion: It is evident from the present study that the levels of Growth Hormone are normal among β-thalassemia major patients on repeated blood transfusion. But GH supplementation can help these patients. Frequent monitoring and supplementation in deficient states is recommended.

Keywords: Beta Thalassemia major, Growth Hormone levels

References

  1. Ngim CF, Lai NM, Hong JY, et al. Growth hormone therapy for people with thalassaemia. Cochrane Database Syst Rev. 2017;9(9):CD012284. Published 2017 Sep 18. doi:10.1002/14651858.CD012284.pub2
  2. Ngim  CF, Lai  NM, Hong  JYH, Tan  SL, Ramadas  A, Muthukumarasamy  P, Thong  MK. Growth hormone therapy for people with thalassaemia. Cochrane Database of Systematic Reviews 2017, Issue 9. Art. No.: CD012284. DOI: 10.1002/14651858.CD012284.pub2.
  3. Shruti Dhale, Subash Valinjkar, Jyothi Janardhan, Sagar Walhekar. Study of Vitamin D levels in Thalessemia Major Patients in Childern. Volume07Issue07 July 2019.
  4. De Sanctis V, Soliman AT, Elsedfy H, et al. Growth and endocrine disorders in thalassemia: The international network on endocrine complications in thalassemia (I-CET) position statement and guidelines. Indian J Endocrinol Metab. 2013;17(1):8–18. doi:10.4103/2230-8210.107808.
  5. Vogiatzi MG, Macklin EA, Trachtenberg FL, et al. Differences in the prevalence of growth, endocrine and vitamin D abnormalities among the various thalassaemia syndromes in North America. Br J Haematol. 2009;146(5):546–556. doi:10.1111/j.1365-2141.2009.07793.x
  6. Raiola, Giuseppe & Galati, M & Sanctis, Vincenzo & Caruso-Nicoletti, Manuela & Pintor, C & Simone, M & Arcuri, Vincenzo & Anastasi, Salvatore. (2003). Growth and puberty in Thalassaemia major. Journal of pediatric endocrinology & metabolism : JPEM. 16 Suppl 2. 259-66.
  7. Karimi, Mehran & Karamifar, Hamd-Allah. (2004). Short stature in beta-thalassemia minor subjects. Medical science monitor : international medical journal of experimental and clinical research. 10. CR603-5.
  8. Low, Louis. (2005). Growth of children with β-thalassemia major. The Indian Journal of Pediatrics. 72. 159-164. 10.1007/BF02760702.
  9. PINTOR, S. G. CELLA, PAOLA MANSO, R. CORDA, C. DESSI, V. LOCATELLI, E. E. MÜLLER, Impaired Growth Hormone (GH) Response to GHReleasing Hormone in Thalassemia Major, The Journal of Clinical Endocrinology & Metabolism, Volume 62, Issue 2, 1 February 1986, Pages 263–267.
  10. Wu, Kang-Hsi & Tsai, Fuu-Jen & Peng, C. (2003). Growth hormone (GH) deficiency in patients with ?-thalassemia major and the efficacy of recombinant GH treatment. Annals of hematology. 82. 637-40. 10.1007/s00277-003-0712-3.
  11. Karamifar, H & Karimi, Mehran & Amirhakimi, G & Badiei, M. (2006). Endocrine Function in Thalassemia Intermedia. International journal of biomedical science : IJBS. 2. 236-240.
  12. Vidergor, G., Goldfarb, A.W., Glaser, B. et al. Growth hormone reserve in adult beta thalassemia patients. Endocr 31, 33–37 (2007) doi:10.1007/s12020-007-0018-7
  13. Fahim FM, Saad K, Askar EA, Eldin EN, Thabet AF. Growth Parameters and Vitamin D status in Children with Thalassemia Major in Upper Egypt. Int J Hematol Oncol Stem Cell Res. 2013;7(4):10–14.
  14. Kyriakou A, Skordis N. Thalassaemia and aberrations of growth and puberty. Mediterr J Hematol Infect Dis. 2009;1(1):e2009003. Published 2009 Jul 27. doi:10.4084/MJHID.2009.003.
  15. Aydinok, Yesim. (2002). Endocrine Complications in Patients with -thalassemia Major. Journal of Tropical Pediatrics - J TROP PEDIAT. 48. 50-54. 10.1093/tropej/48.1.50.
  16. Soliman AT, De Sanctis V, Elalaily R, Yassin M. Insulin-like growth factor- I and factors affecting it in thalassemia major. Indian J Endocrinol Metab. 2015;19 (2):245–251. doi:10.4103/2230-8210.131750.
  17. Soliman A, De Sanctis V, Yassin M, Abdelrahman MO. Growth hormone - insulin-like growth factor-I axis and bone mineral density in adults with thalassemia major. Indian J Endocrinol Metab. 2014;18 (1):32–38. doi:10.4103/2230-8210.126525.

Corresponding Author

Rasika Hattewar

M.D. Pediatrics, Department of Pediatrics, Grant Govt Medical College, Mumbai, India