Skip to main content
Erschienen in: Indian Journal of Pediatrics 6/2011

01.06.2011 | Original Article

Evaluation of Growth, Puberty and Endocrine Dysfunctions in Relation to Iron Overload in Multi Transfused Indian Thalassemia Patients

verfasst von: Rashid H. Merchant, Amruta Shirodkar, Javed Ahmed

Erschienen in: Indian Journal of Pediatrics | Ausgabe 6/2011

Einloggen, um Zugang zu erhalten

Abstract

Objective

To determine the prevalence of growth abnormality and endocrine dysfunction in a group of multi transfused thalassemic children and to correlate these with their body iron stores.

Methods

This cross sectional study included 35 Thalassemia Major patients, aged 13 to 24 years. Growth and puberty were assessed clinically and the laboratory values of various hormone levels were stratified with their age and sexual maturity.

Results

57.14% patients were short, 60% had not attained puberty, and 87.5% of the girls had primary amenorrhea. 14.29% had low FSH and 2.86% low LH levels. 89.47% of the boys had low free testosterone and 43.75% of the girls had low estradiol levels. While 20% had high TSH levels, 40% had high PTH levels, of which 92.8% had low levels of Vitamin D. Low levels of IGF-1 were noted in 51.43%.

Conclusions

In this study analysis, short stature and hypogonadism were frequent findings. These results support the need for vigilant clinical evaluation of growth and puberty, as well as appropriate hormonal evaluation in poly transfused thalassemic children in order to detect and treat endocrine dysfunction early. The authors also recommend aggressive and adequate chelation from early life so that permanent damage to the endocrine glands can be prevented.
Literatur
1.
Zurück zum Zitat Cunningham MJ, Macklin EA, Neufeld EJ, Cohen AR. Complications of β-thalassemia major in North America. Blood. 2004;104:34–9.PubMedCrossRef Cunningham MJ, Macklin EA, Neufeld EJ, Cohen AR. Complications of β-thalassemia major in North America. Blood. 2004;104:34–9.PubMedCrossRef
2.
Zurück zum Zitat Aydinok Y, Darcan S, Polat A, et al. Endocrine complications in patients with β-thalassemia major. J Trop Pediatr. 2002;48:50–4.PubMedCrossRef Aydinok Y, Darcan S, Polat A, et al. Endocrine complications in patients with β-thalassemia major. J Trop Pediatr. 2002;48:50–4.PubMedCrossRef
3.
Zurück zum Zitat Lo L, Singer ST. Thalassemia: Current approach to an old disease. Pediatr Clin North Am. 2002;49:1165–91.PubMedCrossRef Lo L, Singer ST. Thalassemia: Current approach to an old disease. Pediatr Clin North Am. 2002;49:1165–91.PubMedCrossRef
4.
Zurück zum Zitat Borgna-Pignatti C, De Stefano P, Zonta L, et al. Growth and sexual maturation in thalassemia major. J Pediatr. 1985;106:150–5.PubMedCrossRef Borgna-Pignatti C, De Stefano P, Zonta L, et al. Growth and sexual maturation in thalassemia major. J Pediatr. 1985;106:150–5.PubMedCrossRef
5.
6.
Zurück zum Zitat Kattamis C, Liakopoulou T, Kattamis A. Growth and development in children with thalassaemia major. Acta Paediatr Scand Suppl. 1990;366:111–7.PubMedCrossRef Kattamis C, Liakopoulou T, Kattamis A. Growth and development in children with thalassaemia major. Acta Paediatr Scand Suppl. 1990;366:111–7.PubMedCrossRef
7.
Zurück zum Zitat Low LC. Growth, puberty and endocrine function in beta-thalassaemia major. J Pediatr Endocrinol Metab. 1997;10:175–84.PubMedCrossRef Low LC. Growth, puberty and endocrine function in beta-thalassaemia major. J Pediatr Endocrinol Metab. 1997;10:175–84.PubMedCrossRef
8.
Zurück zum Zitat George A, Bhaduri A, Sen S, Choudhry VP. Physical growth parameters in thalassemic children. Indian J Pediatr. 1997;64:861–71.PubMedCrossRef George A, Bhaduri A, Sen S, Choudhry VP. Physical growth parameters in thalassemic children. Indian J Pediatr. 1997;64:861–71.PubMedCrossRef
9.
Zurück zum Zitat De Virgiliis S, Cossu P, Toccafondi C, et al. Effect of subcutaneous desferrioxamine on iron balance in young thalassemia major patients. Am J Pediatr Hematol Oncol. 1983;5:73–7.PubMed De Virgiliis S, Cossu P, Toccafondi C, et al. Effect of subcutaneous desferrioxamine on iron balance in young thalassemia major patients. Am J Pediatr Hematol Oncol. 1983;5:73–7.PubMed
10.
Zurück zum Zitat Fargion S, Taddei MT, Gabutti V, et al. Early iron overload in beta-thalassaemia major: when to start chelation therapy? Arch Dis Child. 1982;57:929–33.PubMedCrossRef Fargion S, Taddei MT, Gabutti V, et al. Early iron overload in beta-thalassaemia major: when to start chelation therapy? Arch Dis Child. 1982;57:929–33.PubMedCrossRef
11.
Zurück zum Zitat Wu KH, Tsai FJ, Peng CT. Growth hormone (GH) deficiency in patients with beta-thalassemia major and the efficacy of recombinant GH treatment. Ann Hematol. 2003;82:637–40.PubMedCrossRef Wu KH, Tsai FJ, Peng CT. Growth hormone (GH) deficiency in patients with beta-thalassemia major and the efficacy of recombinant GH treatment. Ann Hematol. 2003;82:637–40.PubMedCrossRef
12.
Zurück zum Zitat Karydis I. Karagiorga-Lagana M, Nounopoulos C, Tolis G. Basal and stimulated levels of growth hormone, insulin-like growth factor-I (IGF-I), IGF-I binding and IGF-binding proteins in beta-thalassemia major. J Pediatr Endocrinol Metab. 2004;17:17–25.PubMedCrossRef Karydis I. Karagiorga-Lagana M, Nounopoulos C, Tolis G. Basal and stimulated levels of growth hormone, insulin-like growth factor-I (IGF-I), IGF-I binding and IGF-binding proteins in beta-thalassemia major. J Pediatr Endocrinol Metab. 2004;17:17–25.PubMedCrossRef
13.
Zurück zum Zitat Low LC, Postel-Vinay MC, Kwan EY, Cheung PT. Serum growth hormone (GH) binding protein, IGF-I and IGFBP-3 in patients with beta-thalassaemia major and the effect of GH treatment. Clin Endocrinol (Oxf). 1998;48:641–6.CrossRef Low LC, Postel-Vinay MC, Kwan EY, Cheung PT. Serum growth hormone (GH) binding protein, IGF-I and IGFBP-3 in patients with beta-thalassaemia major and the effect of GH treatment. Clin Endocrinol (Oxf). 1998;48:641–6.CrossRef
14.
Zurück zum Zitat Ghosh S, Bandyopadhyay SK, Bandyopadhyay R, Roy D, Maisnam I, Ghosh MK. A study on endocrine dysfunction in thalassaemia. J Indian Med Assoc. 2008;106:655–9.PubMed Ghosh S, Bandyopadhyay SK, Bandyopadhyay R, Roy D, Maisnam I, Ghosh MK. A study on endocrine dysfunction in thalassaemia. J Indian Med Assoc. 2008;106:655–9.PubMed
15.
Zurück zum Zitat Flynn DM, Hoffbrand AV, Politis D. Subcutaneous desferrioxamine: the effect of three years’ treatment on liver, iron, serum ferritin, and comments on echocardiography. Birth Defects Orig Artic Ser. 1982;18:347–53.PubMed Flynn DM, Hoffbrand AV, Politis D. Subcutaneous desferrioxamine: the effect of three years’ treatment on liver, iron, serum ferritin, and comments on echocardiography. Birth Defects Orig Artic Ser. 1982;18:347–53.PubMed
16.
Zurück zum Zitat Agarwal MB, Shah S, Vishwanathan C, et al. Thyroid dysfunction in multi-transfused iron loaded thalassemia patients. Indian Pediatr. 1992;29:997–1002.PubMed Agarwal MB, Shah S, Vishwanathan C, et al. Thyroid dysfunction in multi-transfused iron loaded thalassemia patients. Indian Pediatr. 1992;29:997–1002.PubMed
17.
Zurück zum Zitat Hader A, Bashir N, Hasan Z, Khatib S. Thyroid function in children with beta-thalassemia major in north Jordan. J Trop Pediatr. 1993;39:107–10. Hader A, Bashir N, Hasan Z, Khatib S. Thyroid function in children with beta-thalassemia major in north Jordan. J Trop Pediatr. 1993;39:107–10.
18.
Zurück zum Zitat Cavallo L, Licci D, Acquafredda A, et al. Endocrine involvement in children with beta–thalassaemia major. Transverse and longitudinal studies. I. Pituitary–thyroidal axis function and its correlation with serum ferritin levels. Acta Endocrinol (Copenh). 1984;107:49–53. Cavallo L, Licci D, Acquafredda A, et al. Endocrine involvement in children with beta–thalassaemia major. Transverse and longitudinal studies. I. Pituitary–thyroidal axis function and its correlation with serum ferritin levels. Acta Endocrinol (Copenh). 1984;107:49–53.
19.
Zurück zum Zitat Filosa A, Di Maio S, Aloj G, Acampora C. Longitudinal study on thyroid function in patients with thalassemia major. J Pediatr Endocrinol Metab. 2006;19:1397–404.PubMedCrossRef Filosa A, Di Maio S, Aloj G, Acampora C. Longitudinal study on thyroid function in patients with thalassemia major. J Pediatr Endocrinol Metab. 2006;19:1397–404.PubMedCrossRef
20.
Zurück zum Zitat Gulati R, Bhatia V, Agarwal SS. Early onset of endocrine abnormalities in beta-thalassemia major in a developing country. J Pediatr Endocrinol Metab. 2000;13:651–6.PubMedCrossRef Gulati R, Bhatia V, Agarwal SS. Early onset of endocrine abnormalities in beta-thalassemia major in a developing country. J Pediatr Endocrinol Metab. 2000;13:651–6.PubMedCrossRef
21.
Zurück zum Zitat Italian Working Group on Endocrine Complications in Non–Endocrine Diseases. Multicentre study on prevalence of endocrine complications in thalassaemia major. Clin Endocrinol (Oxf). 1995;42:581–6.CrossRef Italian Working Group on Endocrine Complications in Non–Endocrine Diseases. Multicentre study on prevalence of endocrine complications in thalassaemia major. Clin Endocrinol (Oxf). 1995;42:581–6.CrossRef
22.
Zurück zum Zitat Chern JP, Lin KH. Hypoparathyroidism in transfusion-dependent patients with beta-thalassemia. J Pediatr Hematol Oncol. 2002;24:291–3.PubMedCrossRef Chern JP, Lin KH. Hypoparathyroidism in transfusion-dependent patients with beta-thalassemia. J Pediatr Hematol Oncol. 2002;24:291–3.PubMedCrossRef
23.
Zurück zum Zitat Srivatsa A, Marwaha RK, Muralidharan R. Trehan A. Assessment of Adrenal Endocrine Function in Asian Thalassemics: Indian Pediatrics. 2005;42:31–5.PubMed Srivatsa A, Marwaha RK, Muralidharan R. Trehan A. Assessment of Adrenal Endocrine Function in Asian Thalassemics: Indian Pediatrics. 2005;42:31–5.PubMed
24.
Zurück zum Zitat Tyagi S, Kabra M, Tandon N, Saxena R, Pati H, Choudhry VP. linico haematological profile of thalassemia Intermedia patients. Int. J Hum Genet. 2003;3:251–8. Tyagi S, Kabra M, Tandon N, Saxena R, Pati H, Choudhry VP. linico haematological profile of thalassemia Intermedia patients. Int. J Hum Genet. 2003;3:251–8.
25.
Zurück zum Zitat Jain M, Sinha RS, Chellani H, Anand NK. Assessment of thyroid functions and its role in body growth in thalassemia major. Indian Pediatr. 1995;32:213–9.PubMed Jain M, Sinha RS, Chellani H, Anand NK. Assessment of thyroid functions and its role in body growth in thalassemia major. Indian Pediatr. 1995;32:213–9.PubMed
26.
Zurück zum Zitat Agarwal MB. Deferasirox: oral, once daily iron chelator–an expert opinion. Indian J Pediatr. 2010;77:185–91.PubMedCrossRef Agarwal MB. Deferasirox: oral, once daily iron chelator–an expert opinion. Indian J Pediatr. 2010;77:185–91.PubMedCrossRef
27.
28.
Zurück zum Zitat Choudhry VP, Naithani R. Current status of iron overload and chelation with deferasirox. Indian J Pediatr. 2007;74:759–64.PubMedCrossRef Choudhry VP, Naithani R. Current status of iron overload and chelation with deferasirox. Indian J Pediatr. 2007;74:759–64.PubMedCrossRef
Metadaten
Titel
Evaluation of Growth, Puberty and Endocrine Dysfunctions in Relation to Iron Overload in Multi Transfused Indian Thalassemia Patients
verfasst von
Rashid H. Merchant
Amruta Shirodkar
Javed Ahmed
Publikationsdatum
01.06.2011
Verlag
Springer-Verlag
Erschienen in
Indian Journal of Pediatrics / Ausgabe 6/2011
Print ISSN: 0019-5456
Elektronische ISSN: 0973-7693
DOI
https://doi.org/10.1007/s12098-010-0351-3

Weitere Artikel der Ausgabe 6/2011

Indian Journal of Pediatrics 6/2011 Zur Ausgabe

Scientific Letters to the Editor

Splenic Lesions in Visceral Leishmaniasis

Scientific Letters to the Editor

Hyperekplexia Masquerading as Epilepsy

Neuer Typ-1-Diabetes bei Kindern am Wochenende eher übersehen

23.04.2024 Typ-1-Diabetes Nachrichten

Wenn Kinder an Werktagen zum Arzt gehen, werden neu auftretender Typ-1-Diabetes und diabetische Ketoazidosen häufiger erkannt als bei Arztbesuchen an Wochenenden oder Feiertagen.

Neue Studienergebnisse zur Myopiekontrolle mit Atropin

22.04.2024 Fehlsichtigkeit Nachrichten

Augentropfen mit niedrig dosiertem Atropin können helfen, das Fortschreiten einer Kurzsichtigkeit bei Kindern zumindest zu verlangsamen, wie die Ergebnisse einer aktuellen Studie mit verschiedenen Dosierungen zeigen.

Spinale Muskelatrophie: Neugeborenen-Screening lohnt sich

18.04.2024 Spinale Muskelatrophien Nachrichten

Seit 2021 ist die Untersuchung auf spinale Muskelatrophie Teil des Neugeborenen-Screenings in Deutschland. Eine Studie liefert weitere Evidenz für den Nutzen der Maßnahme.

Fünf Dinge, die im Kindernotfall besser zu unterlassen sind

18.04.2024 Pädiatrische Notfallmedizin Nachrichten

Im Choosing-Wisely-Programm, das für die deutsche Initiative „Klug entscheiden“ Pate gestanden hat, sind erstmals Empfehlungen zum Umgang mit Notfällen von Kindern erschienen. Fünf Dinge gilt es demnach zu vermeiden.

Update Pädiatrie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.