Acute Effects of Blood Transfusion on Growth Hormone and Insulin-Like Growth Factor-1 Levels in Children with Thalassemia

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Abstract

Background/Aim: Children with β-thalassemia have chronic anemia and growth retardation. Impaired growth hormone (GH)-insulin-like growth factor-1 (IGF-1) axis function has been demonstrated. Growth improvement has been demonstrated following optimal blood transfusion. Whether correction of anemia by blood transfusion augments GH-IGF-1 axis function has not been established. Methods: Twenty children with thalassemia aged 11.7 years (5.3–16.3 years) were recruited. GH provocative tests were performed twice, before and 1 week after blood transfusion. IGF-1, IGF-binding protein-3 (IGFBP-3) and hematocrit were measured. Results: Median IGF-1 and IGFBP-3 concentrations were significantly increased at 1 week following transfusion: pre- versus posttransfused concentrations: 86.4 versus 143.5 ng/ml (p < 0.001) and 2.95 versus 3.75 mg/l (p < 0.001), respectively. However, median peak GH levels and areas under the curve of GH during GH testing between pre- and posttransfusion periods were not different. The pretransfused hematocrit level was correlated with pretransfused IGF-1 (r = 0.662, p < 0.001) and IGFBP-3 (r = 0.691, p < 0.001) levels . Conclusions: In thalassemics, correction of anemia by blood transfusion rapidly enhanced GH-mediated IGF-1 and IGFBP-3 secretion. This suggests thatanemia may be one of the factors causing partial GH insensitivity.

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Most cited references 19

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Beta-thalassemia.

Deborah Rund, Eliezer Rachmilewitz (2005)

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Reliability of provocative tests to assess growth hormone secretory status. Study in 472 normally growing children

E. Ghigo (1996)

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Growth and pubertal development in transfusion-dependent children and adolescents with thalassaemia major and sickle cell disease: a comparative study.

B Ansari, Haitham M. Amer, Nada Soliman … (1999)

Despite regular blood transfusion and desferrioxamine treatment, growth impairment and pubertal delay are commonly seen in children and adolescents with transfusion-dependent thalassaemia and sickle cell disease (SCD). We evaluated growth parameters and sexual maturation in a large cohort of children and adolescents with SCD (n = 110) and thalassaemia (n = 72) receiving nearly the same protocol of transfusion and chelation, and compared them with those for 200 normal age-matched children, 30 children with constitutional delay of growth (CSS), and 25 children with growth hormone deficiency (GHD). Before transfusion, haemoglobin concentration had not been less than 9 g/dl in the past 7 years; desferrioxamine was administered for 7-10 years, including by the intramuscular and subcutaneous routes, three times or more per week. The height standard deviation score (HtSDS), growth velocity (GV) (cm/yr), and growth velocity standard deviation score (GVDSD) of children and adolescents with thalassaemia and SCD were significantly decreased compared to normal children (p < 0.01). Forty-nine per cent of thalassaemic patients and 27 per cent of patients with SCD had HtSDS less than -2, and 83 per cent of thalassaemic patients and 67 per cent of SCD patients had HtSDS less than -1. Fifty-six per cent of thalassaemic children and 51 per cent of children with SCD had GVSDS less than -1. The GV of thalassaemic children was significantly slower than that for children with SCD. Children with thalassaemia and SCD had HtSDS and GVSDS comparable to those for children with CSS but higher than those for patients with GHD. Serum ferritin concentration was correlated significantly with the linear GV in all patients (r = 0.45, p < 0.001). The bone age delay did not differ among the three groups with thalassaemia, SCD and CSS, but the delay was significant in the group with GHD. The mid-arm circumference was significantly smaller in children with thalassaemia and SCD than in normal children. The triceps skin-fold thickness of patients with SCD was significantly decreased compared to thalassaemic and normal children. The upper/lower segment ratio was significantly lower in thalassaemic and SCD patients than in normal children. In thalassaemic patients between the ages of 13 and 21 years a complete lack of pubescent changes was present in 73 per cent of boys and 42 per cent of girls. Seventy-four per cent of the thalassaemic girls had primary amenorrhoea. Girls with SCD aged between 13 and 21 years had markedly delayed breast development and menarche. Twenty-five per cent of boys with SCD above the age of 14 years had absence of testicular development. Males with thalassaemia and SCD who had spontaneous testicular development had significantly smaller testicular volume than did normal controls. Short children with thalassaemia and SCD had significantly decreased serum insulin-like growth factor 1 (IGF-1) concentrations compared to children with CSS. Collectively, these data confirm the high prevalence of impaired growth and pubertal delay/failure in children and adolescents with thalassaemia and SCD. The aetiology of impaired growth includes the contributions of lack of pubertal growth spurt due to delayed/absent puberty, decreased synthesis of IGF-1 which might be secondary to a disturbed GH-IGF-1 axis and/or under nutrition, probably due to the hypermetabolic status of these children. It is suggested that newer protocols of treatment, in addition to optimization of transfusion and chelation requirements, should increase the caloric intake of these patients and properly manage their pubertal delay-failure in order to improve their adult height.

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Author and article information

Journal

Journal ID (publisher-id): HRP

Journal ID (nlm-ta): Horm Res Paediatr

Journal ID (doi): 10.1159/issn.1663-2818

Title: Hormone Research in Paediatrics

Publisher: S. Karger AG

ISSN (Print): 1663-2818

ISSN (Electronic): 1663-2826

Publication date Subscription-year: 2011

Publication date (Print): March 2011

Publication date (Electronic): 04 November 2010

Volume: 75

Issue: 4

Pages: 240-245

Affiliations

Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand

Author notes

*Pat Mahachoklertwattana, MD, Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Rama 6 Road, Bangkok 10400 (Thailand), Tel. +662 201 1394, Fax +662 201 1850, E-Mail rapmw@mahidol.ac.th

Article

Publisher ID: 321189 Other ID: Horm Res Paediatr 2011;75:240–245

DOI: 10.1159/000321189

PubMed ID: 21051865

SO-VID: a6ad9708-4ebb-43d9-83b6-cd2fa3aa41d7

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Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

History

Date received : 28 April 2010

Date accepted : 08 September 2010

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Figures: 2, Tables: 2, Pages: 6

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Acute Effects of Blood Transfusion on Growth Hormone and Insulin-Like Growth Factor-1 Levels in Children with Thalassemia

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Karger: Endocrinology

Most cited references 19

Beta-thalassemia.

Reliability of provocative tests to assess growth hormone secretory status. Study in 472 normally growing children

Growth and pubertal development in transfusion-dependent children and adolescents with thalassaemia major and sickle cell disease: a comparative study.

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