Hormonal changes in thalassaemia major.
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Journal: 1977/February - Archives of Disease in Childhood
ISSN: 1468-2044
PUBMED: 1008588
Abstract:
Patients with severe thalassaemia major suffer endocrine and other abnormalities before their eventual death from iron overload due to repeated blood transfusions. The endocrine status of 31 thalassaemic patients aged 2-5 to 23 years was investigated. Exact data were available on the rate and duration of blood transfusion in all of them and in many the liver iron concentration was also known. Although the patients were euthyroid, the mean serum thyroxine level was significantly lower, and the mean thyrotrophic hormone level significantly higher, compared with the values found in normal children. Forty oral glucose tolerance tests with simultaneous insulin levels were performed in 19 children, of whom 5 developed symptomatic diabetes and one had impaired tolerance. Previous tests on all 6 patients were available and some showed raised insulin levels possibly due to insulin resistance. 2 patients had clinical hypoparathyroidism and are described. The parathyroid hormone levels determined by radioimmunoassay in 25 patients were below the mean for the age group in all and outside the reference range in 16. Nonfasting plasma calcium levels were not reduced. Puberty was delayed in some patients. Concentrations of luteinizing hormone (LH) and follicle-stimulating hormone (FSH) measured in urine from 7 girls and 5 boys showed considerable variation. In the boys there was an overall tendency for FSH and LH excretion to be low with regard to age, but with respect to puberty rating FSH exretions were normal or low and LH normal or raised. The girls showed a tendency for LH but not FSH excretion to be raised in relation to puberty rating. The severity of the endocrine changes was related to the degree of iron loading and is discussed in relation to previous work in which the iron loading has rarely been accurately indicated nor parathyroid status assessed.
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Arch Dis Child 51(11): 828-836
PMID: 1008588

Hormonal changes in thalassaemia major.

Abstract

Patients with severe thalassaemia major suffer endocrine and other abnormalities before their eventual death from iron overload due to repeated blood transfusions. The endocrine status of 31 thalassaemic patients aged 2-5 to 23 years was investigated. Exact data were available on the rate and duration of blood transfusion in all of them and in many the liver iron concentration was also known. Although the patients were euthyroid, the mean serum thyroxine level was significantly lower, and the mean thyrotrophic hormone level significantly higher, compared with the values found in normal children. Forty oral glucose tolerance tests with simultaneous insulin levels were performed in 19 children, of whom 5 developed symptomatic diabetes and one had impaired tolerance. Previous tests on all 6 patients were available and some showed raised insulin levels possibly due to insulin resistance. 2 patients had clinical hypoparathyroidism and are described. The parathyroid hormone levels determined by radioimmunoassay in 25 patients were below the mean for the age group in all and outside the reference range in 16. Nonfasting plasma calcium levels were not reduced. Puberty was delayed in some patients. Concentrations of luteinizing hormone (LH) and follicle-stimulating hormone (FSH) measured in urine from 7 girls and 5 boys showed considerable variation. In the boys there was an overall tendency for FSH and LH excretion to be low with regard to age, but with respect to puberty rating FSH exretions were normal or low and LH normal or raised.The girls showed a tendency for LH but not FSH excretion to be raised in relation to puberty rating. The severity of the endocrine changes was related to the degree of iron loading and is discussed in relation to previous work in which the iron loading has rarely been accurately indicated nor parathyroid status assessed.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.
  • Arnaud CD, Goldsmith RS, Bordier PJ, Sizemore GW. Influence of immunoheterogeneity of circulating parathyroid hormone on results of radioimmunoassays of serum in man. Am J Med. 1974 Jun;56(6):785–793. [PubMed] [Google Scholar]
  • Barry M, Sherlock S. Measurement of liver-iron concentration in needle-biopsy specimens. Lancet. 1971 Jan 16;1(7690):100–103. [PubMed] [Google Scholar]
  • Barry M, Flynn DM, Letsky EA, Risdon RA. Long-term chelation therapy in thalassaemia major: effect on liver iron concentration, liver histology, and clinical progress. Br Med J. 1974 Apr 6;2(5909):16–20.[PMC free article] [PubMed] [Google Scholar]
  • Buckler JM, Clayton BE. Output of luteinizing hormone in the urine of normal children and those with advanced sexual development. Arch Dis Child. 1970 Aug;45(242):478–484.[PMC free article] [PubMed] [Google Scholar]
  • Canale VC, Steinherz P, New M, Erlandson M. Endocrine function in thalassemia major. Ann N Y Acad Sci. 1974;232(0):333–345. [PubMed] [Google Scholar]
  • CHAPTAL J, JEAN R, PAGES A, BONNET H, LLORET R, ROUSTAN J. H'EMOCHROMATOSE SECONDAIRE DE LA MALADIE DE COOLEY. ETUDE CLINIQUE, BIOLOGIQUE ET ANATOMIQUE. Pediatrie. 1964 Sep;19:677–693. [PubMed] [Google Scholar]
  • ELLIS JT, SCHULMAN I, SMITH CH. Generalized siderosis with fibrosis of liver and pancreas in Cooley's (Mediterranean) anemia; with observations on the pathogenesis of the siderosis and fibrosis. Am J Pathol. 1954 Mar-Apr;30(2):287–309.[PMC free article] [PubMed] [Google Scholar]
  • ENGLE MA. CARDIAC INVOLVEMENT IN COOLEY'S ANEMIA. Ann N Y Acad Sci. 1964 Oct 7;119:694–702. [PubMed] [Google Scholar]
  • Fairney A, Jackson D, Clayton BE. Measurement of serum parathyroid hormone, with particular reference to some infants with hypocalcaemia. Arch Dis Child. 1973 Jun;48(6):419–424.[PMC free article] [PubMed] [Google Scholar]
  • FINK H. TRANSFUSION HEMOCHROMATOSIS IN COOLEY'S ANEMIA. Ann N Y Acad Sci. 1964 Oct 7;119:680–685. [PubMed] [Google Scholar]
  • Gabriele OF. Hypoparathyroidism associate with thalassemia. South Med J. 1971 Jan;64(1):115–116. [PubMed] [Google Scholar]
  • Grant DB. Serum-insulin levels in children during glucose tolerance tests. Acta Paediatr Scand. 1968 Jul;57(4):297–299. [PubMed] [Google Scholar]
  • Hall R, Amos J, Ormston BJ. Radioimmunoassay of human serum thyrotrophin. Br Med J. 1971 Mar 13;1(5749):582–585.[PMC free article] [PubMed] [Google Scholar]
  • Jackson D, Grant DB, Clayton BE. A simple oral test of growth-hormone secretion in children. Lancet. 1968 Aug 17;2(7564):373–375. [PubMed] [Google Scholar]
  • Kuo B, Zaino E, Roginsky MS. Endocrine function in thalassemia major. J Clin Endocrinol Metab. 1968 Jun;28(6):805–808. [PubMed] [Google Scholar]
  • Lassman MN, Genel M, Wise JK, Hendler R, Felig P. Carbohydrate homeostasis and pancreatic islet cell funtion in thalassemia. Ann Intern Med. 1974 Jan;80(1):65–69. [PubMed] [Google Scholar]
  • 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):226–237. [PubMed] [Google Scholar]
  • Letsky EA, Miller F, Worwood M, Flynn DM. Serum ferritin in children with thalassaemia regularly transfused. J Clin Pathol. 1974 Aug;27(8):652–655.[PMC free article] [PubMed] [Google Scholar]
  • McIntosh N. Pituitary-adrenal function in thalassaemia major. Arch Dis Child. 1973 Aug;48(8):653–653.[PMC free article] [PubMed] [Google Scholar]
  • Milner AD. Blood glucose and serum insulin levels in children with cystic fibrosis. Arch Dis Child. 1969 Jun;44(235):351–355.[PMC free article] [PubMed] [Google Scholar]
  • Risdon RA, Barry M, Flynn DM. Transfusional iron overload: the relationship between tissue iron concentration and hepatic fibrosis in thalassaemia. J Pathol. 1975 Jun;116(2):83–95. [PubMed] [Google Scholar]
  • Ryness J. The measurement of serum thyroxine in children. J Clin Pathol. 1972 Aug;25(8):726–729.[PMC free article] [PubMed] [Google Scholar]
  • Segre GV, Niall HD, Habener JF, Potts JT., Jr Metabolism of parathyroid hormone: physiologic and clinical significance. Am J Med. 1974 Jun;56(6):774–784. [PubMed] [Google Scholar]
  • Toccafondi R, Maioli M, Meloni T. Plasma insulin response to oral carbohydrate in Cooley's anaemia. Riv Crit Clin Med. 1970;70(1):96–101. [PubMed] [Google Scholar]
  • Zaino EC, Kuo B, Roginsky MS. Growth retardation in thalassemia major. Ann N Y Acad Sci. 1969 Nov 20;165(1):394–399. [PubMed] [Google Scholar]
Copyright notice
Abstract
Patients with severe thalassaemia major suffer endocrine and other abnormalities before their eventual death from iron overload due to repeated blood transfusions. The endocrine status of 31 thalassaemic patients aged 2-5 to 23 years was investigated. Exact data were available on the rate and duration of blood transfusion in all of them and in many the liver iron concentration was also known. Although the patients were euthyroid, the mean serum thyroxine level was significantly lower, and the mean thyrotrophic hormone level significantly higher, compared with the values found in normal children. Forty oral glucose tolerance tests with simultaneous insulin levels were performed in 19 children, of whom 5 developed symptomatic diabetes and one had impaired tolerance. Previous tests on all 6 patients were available and some showed raised insulin levels possibly due to insulin resistance. 2 patients had clinical hypoparathyroidism and are described. The parathyroid hormone levels determined by radioimmunoassay in 25 patients were below the mean for the age group in all and outside the reference range in 16. Nonfasting plasma calcium levels were not reduced. Puberty was delayed in some patients. Concentrations of luteinizing hormone (LH) and follicle-stimulating hormone (FSH) measured in urine from 7 girls and 5 boys showed considerable variation. In the boys there was an overall tendency for FSH and LH excretion to be low with regard to age, but with respect to puberty rating FSH exretions were normal or low and LH normal or raised.The girls showed a tendency for LH but not FSH excretion to be raised in relation to puberty rating. The severity of the endocrine changes was related to the degree of iron loading and is discussed in relation to previous work in which the iron loading has rarely been accurately indicated nor parathyroid status assessed.
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