Relationship of endocrinopathy to iron chelation status in young patients with thalassaemia major

R. G. Grundy, Kathryn (Katie) Woods, M. O. Savage, J. P M Evans

Research output: Contribution to journalArticle

59 Citations (Scopus)

Abstract

Disturbances of growth and development in patients with thalassaemia receiving hypertransfusion programmes are well recognised and are most likely to be due to iron overload. The extent of endocrine dysfunction was investigated in a group of 18 patients thought to have been treated by acceptable modern standards, 11 of whom could be considered as well chelated. Assessment of growth and puberty showed a wide variation in height SD scores with five patients having significantly short stature. Most patients are progressing through puberty normally with the exception of two boys with marked pubertal delay. The most prominent finding was that growth hormone responses to glucagon stimulation were significantly impaired in all of the patients with iron overload. Basal endocrine assessment showed primary hypothyroidism in two patients aged 16.8 and 12.9 years with plasma thyroxine-concentrations of 86 and 59 nmol/l (normal range 65-165 nmol/l) and plasma thyroid stimulating hormone 10.2 and 30.3 mU/l (normal range 0.5-5 mU/l). One patient had diabetes mellitus. These results show that even when ideal management is sought a significant amount of endocrine damage occurs; surveillance of these patients is thus essential.

Original languageEnglish (US)
Pages (from-to)128-132
Number of pages5
JournalArchives of Disease in Childhood
Volume71
Issue number2
StatePublished - 1994
Externally publishedYes

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beta-Thalassemia
Iron
Iron Overload
Puberty
Reference Values
Thalassemia
Thyrotropin
Hypothyroidism
Glucagon
Thyroxine
Growth and Development
Growth Hormone
Diabetes Mellitus
Growth

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Relationship of endocrinopathy to iron chelation status in young patients with thalassaemia major. / Grundy, R. G.; Woods, Kathryn (Katie); Savage, M. O.; Evans, J. P M.

In: Archives of Disease in Childhood, Vol. 71, No. 2, 1994, p. 128-132.

Research output: Contribution to journalArticle

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