Management of Puberty for Optimal Auxological Results in β-Thalassaemia Major
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Manuela Caruso-Nicoletti
,
V. De Sanctis
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 desferrioxamine and to endocrine disorders, due to iron overload. The commonest endocrine complication is hypogonadism. The growth pat- tern 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.
© 2016 by Walter de Gruyter Berlin/Boston
Articles in the same Issue
- Titelei
- Table of Contents
- Introduction
- Opening Remarks
- Factors Determining Final Height in Congenital Adrenal Hyperplasia
- Management of Puberty for Optimal Auxological Results in β-Thalassaemia Major
- Unresolved Problems Concerning Optimal Therapy of Puberty in Children with Chronic Renal Diseases
- Management of Puberty in Constitutional Delay of Growth and Puberty
- Turner’s Syndrome
- Idiopathic Short Stature
- Current Concepts in Tall Stature and Overgrowth Syndromes
- Optimal Therapy of Pubertal Disorders in Precocious/Early Puberty
- Unresolved Problems in Optimal Therapy of Pubertal Disorders in Oncological and Bone Marrow Transplanted Patients
- Isolated Growth Hormone Deficiency in Children and Adolescents
- Multiple Pituitary Hormone Deficiency: Management of Puberty for Optimal Auxological Results
- Concluding Remarks
Articles in the same Issue
- Titelei
- Table of Contents
- Introduction
- Opening Remarks
- Factors Determining Final Height in Congenital Adrenal Hyperplasia
- Management of Puberty for Optimal Auxological Results in β-Thalassaemia Major
- Unresolved Problems Concerning Optimal Therapy of Puberty in Children with Chronic Renal Diseases
- Management of Puberty in Constitutional Delay of Growth and Puberty
- Turner’s Syndrome
- Idiopathic Short Stature
- Current Concepts in Tall Stature and Overgrowth Syndromes
- Optimal Therapy of Pubertal Disorders in Precocious/Early Puberty
- Unresolved Problems in Optimal Therapy of Pubertal Disorders in Oncological and Bone Marrow Transplanted Patients
- Isolated Growth Hormone Deficiency in Children and Adolescents
- Multiple Pituitary Hormone Deficiency: Management of Puberty for Optimal Auxological Results
- Concluding Remarks
