Management of puberty for optimal auxological results in β-thalassaemia major

M. Caruso-Nicoletti, V. De Sanctis, L. Cavallo, G. Raiola, L. Ruggiero, N. Skordis, B. Wonke

Research output: Contribution to journalArticlepeer-review

Abstract

Short stature is present in a significant percentage of patients affected by β-thalassaemia major. Growth failure of patients with thalassaemia is multifactorial. The most important contribution is attributed to the toxic effect of desferrioxamine and to endocrine disorders, due to iron overload. The commonest endocrine complication is hypogonadism. The growth pattern of patients with thalassaemia is characterized by normal growth during childhood, a deceleration of growth velocity around age 9-10 years, and a reduced pubertal growth spurt. In addition, reduced growth of the trunk is often present. Short stature and short trunk are more evident at pubertal age. Hypogonadism is usually considered responsible for the pubertal growth failure, as well as the aggravation of body disproportion at pubertal age. However, data suggest that pubertal height gain and final height are reduced in both patients with spontaneous puberty and patients with induced puberty. It is concluded that several aspects of peripubertal growth in patients with thalassaemia remain to be clarified.

Original languageEnglish
Pages (from-to)939-944
Number of pages6
JournalJournal of Pediatric Endocrinology and Metabolism
Volume14
Issue numberSUPPL. 2
Publication statusPublished - 2001

Keywords

  • β-Thalassaemia
  • Consensus guidelines
  • Growth
  • Hypogonadism
  • Puberty
  • Short stature
  • Short trunk

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