DOI: http://dx.doi.org/10.18203/2349-3933.ijam20171587

Prevalence of hypothyroidism, diabetes mellitus and delayed puberty in patients of thalassemia major in a tertiary care center of Jammu province, Jammu Kashmir, India

Sushil Sharma, Neeti Dutt, Meena Sidhu, Sanjeev Digra, Renu Meenia

Abstract


Background: Thalassemia is a common genetic disorder which is associated with a lot of complications. Frequent blood transfusions result in increased iron deposition in various tissues leading to dysfunction of many organs of our body. Endocrine disorders constitute a major part of such complications increasing the morbidity of thalassemia manifold in the affected patients.

Methods: This is a prospective study carried out in 64 thalassemia major patients attending thalassemia day care centre at SMGS Hospital Jammu from December 2014 to November 2015. Patients were examined and investigated for presence of one or more endocrine disorders including diabetes mellitus, hypothyroidism and delayed puberty.

Results: Endocrine disorders were detected in a total of 22 patients. Diabetes mellitus was detected in 4.7% (n=3) patients, hypothyroidism in 4.7% (n=3) patients and delayed puberty was found in 26.6% (n=17) patients. Mean serum ferritin level was found to be 2885.5 ng/ml and there was no significant difference in patients affected with endocrine disorder and those without any endocrine disorder.

Conclusions: Endocrine complications occur commonly in patients of thalassemia major. Increasing life span of thalassemia patients has increased the number of patients living with these disorders. A lot of morbidity occurs due to the presence of one or more of these disorders. Hence timely detection of these disorders by screening in all patients of thalassemia should be done to initiate treatment at the earliest so as to limit the morbidity caused by these disorders.


Keywords


Endocrine disorders, Serum ferritin, Thalassemia

Full Text:

PDF

References


Shamshirsaz AA, Bekheirnia MR, Kamgar M, Pourzahedgilani N, Bouzari N, Habibzadeh M, et al. Metabolic and endocrinologic complications in beta-thalassemia major: a multicenter study in Tehran. BMC endocrine disorders. 2003;3(1):4.

Al-Elq AH, Al-Saeed HH. Endocrinopathies in patients with thalassemias. Saudi Med J. 2004;25(10):1347-51.

Bannerman RM, Keusch G, Kreimer-Birnbaum M, Vance VK, Vaughan S. Thalassemia intermedia, with iron overload, cardiac failure, diabetes mellitus, hypopituitarism and porphyrinuria. The Am J Med. 1967;42(3):476-86.

Flynn D, Fairney A, Jackson D, Clayton BE. Hormonal changes in thalassaemia major. Arch Dis Child. 1976;51:828-36.

Masala A, Meloni T, Gallisai D, Alagna S, Rovasio PP, Rassu S et al. Endocrine functioning in multitransfused prepubertal patients with homozygous 1-thalassaemia. Jf Clin Endocrinol Metab. 1984;58:667-70.

Abdulzahra MS, Al-Hakeim HK, Ridha MM. Study of the effect of iron overload on the function of endocrine glands in male thalassemia patients. Asian J Transfus Sci. 2011;5:127-31.

Mahmoodi M, De Sanctis V, Karimi M. Diffuse intracerebral calcification in a beta-thalassaemia major patient with hypoparathyroidism: a case report. Pediatr Endocrinol Rev. 2011;8:331-3.

Tiosano D, Hochberg Z. Endocrine Complications of Thalassaemia. J Endocrinol Invest. 2001;24(9):716-23.

Gulati R, Bhatia V, Agarwal SS. Early onset of endocrine abnormalities in ß-thalassemia major in a developing country. J Pediatr Endocrinol Metab. 2000;13:651-6.

Kuo B, Zaino E, Roginsky MS. Endocrine function in thalassemia major. J Clin Endocrinol Metab. 1968;28(6):805-8.

Anoussakis C, Alexiou D, Abatzis D, Bechrakis G. Endocrinological Investigation of Pituitary Gonadal Axis in Thalassemia Majo. Acta Paediatr Scand. 1977;66(1):49-51.

Landau H, Spitz IM, Cividalli G, Rachmilewitz EA. Gonadotropin, Thyrotropin and Prolactin Reserve in Beta Thalassemia. Clin Endocrinol (Oxf). 1978;9(2):163-73.

Kletzky OA, Costin G, Marrs RP, Bernstein G, March CM, Mishell DR Jr. “Gonadotropin Insufficiency in Patients with Thalassemia Major. J Clin Endocrinol Metab. 1979;48(6):901-5.

Hafez M, Youssry I, El-Hamed FA, Ibrahim A. Abnormal glucose tolerance in beta-thalassemia: assessment of risk factors. Hemoglobin. 2009;33:101-8.

Toumba M, Sergis A, Kanaris C, Skordis N. Endocrine complications in patients with thalassemia major. Pediatr Endocrinol Rev. 2007;5:642-8.

Chern JP, Lin KH, Lu MY, Lin DT, Lin KS, Chen JD et al. Abnormal glucose tolerance in transfusion-dependent beta-thalassemic patients. Diabetes Care. 2001;24: 850-4.

Monge L, Pinach S, Caramelling L, Bertero MT, Dallomo A, Carta Q. The possible role of autoimmunity in the pathogenesis of diabetes in B-thalassemia major. Diabetes Metab. 2001;27:149-54.

Cavallo-Perin P, Pacini G, Cerutti F, Bessone A, Condo C, Sacchetti L, Piga A, Pagano G. Insulin resistance and hyperinsulinemia in homozygous β-thalassemia. Metabolism. 1995;44(3):281-6.

Landau H, Matoth I, Landau‐Cordova Z, Goldfarbs A, Rachmilewitz EA, Glaser B. Cross‐sectional and longitudinal study of the pituitary‐thyroid axis in patients with thalassaemia major. Clinical endocrinology. 1993;38(1):55-61.

Italian Working Group on Endocrine Complications in Non-Endocrine Disease, “Multicentre Study on Prevalence of Endocrine Complications in Thalassemia Major,” Clinical Endocrinology (Oxf). 1995;42(6):581-6.

Modell B, Letsky EA, Flynn DM, Peto R, Weatherall DJ. Survival and desferioxamine in thalassaemia major. BMJ. 1982;284:1081-4.

Vanorden HE, Hagemann TM. Deferasirox an oral agent for chronic iron overload. Ann Pharmacother. 2006;40(6):1110-17.

Cappellini N, Cohen A, Eleftheriou A, Piga A, Porter J. Guidelines for the Clinical Management of Thalassemia. Thalassemia International Federation, Strovolos. 2000;41-9.

Hafez M, Youssry I, El-Hamed FA, Ibrahim A. Abnormal glucose tolerance in beta-thalassemia: assessment of risk factors. Hemoglobin. 2009;33:101-8.

Toumba M, Sergis A, Kanaris C, Skordis N. Endocrine complications in patients with thalassemia major. Pediatr Endocrinol Rev. 2007;5:642-8.

Ong CK, Lim SL, Tan WC, Ong EE, Goh AS. Endocrine complications in transfusion dependent thalassemia in Penang Hospital. Med J Malaysia. 2008;63:109-12.

Jaruratanasirikul S, Chareonmuang R, Wongcharnchailert M, Laosombat V, Sangsupavanich P, Leetanaporn K. Prevalence of impaired glucose metabolism in beta-thalassemic children receiving hypertransfusions with a suboptimal dosage of iron-chelating therapy. Eur J Pediatr. 2008;167:873-6.

Najafipour F, Aliasgarzadeh A, Aghamohamadzadeh N, Bahami A, Mobasri M, Niafar M et al. A cross-sectional study of metabolic and endocrine complications in beta-thalassemia major. Ann Saudi Med. 2008;28:361-6.

Zervas A, Katapodi A, Protonotariou A. Assessment of thyroid function in two hundred patients with beta-thalassemia major. Thyroid. 2002;12:151-4.

Karamifar H, Shahriari M, Sadjadian N. Prevalence of endocrine complications in beta-thalassemia major in the Islamic Republic of ıran. East Mediterr Health J. 2003;9:55-60.

Luca FD, Melluso R, Sobbrio G, Canfora G, Trimarchi F. Thyroid function in thalassemia major. Arch Dis Child. 1980;55:389-92.

Lassman MN, O'brien RT, Pearson HA, Wise JK, Donabedian RK, Felig P, Genel M. Endocrine evaluation in thalassemia major. Ann N Y Acad Sci. 1974;232(0):226-37.