Turner’s Syndrome
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Maria Pia Guarneri
,
S.A.S. Abusrewil
Abstract
Turner syndrome (TS) is the most common sex-chromosome abnormality in females. Short stature and hypogonadism are the classical clinical findings. The spontaneous final height (FH) ranges between 139 and 147 cm, representing a growth deficit of about 20 cm with respect to the unaffected population. GH therapy improves FH and should be started during childhood at a high dose of about 1 IU/kg/week (range 0.6-2 IU/kg/week). Some authors advocate combined therapy with an anabolic steroid at various doses (e.g. oxandrolone 0.05-0.1 mg/kg/day). This treatment results in a significantly increased FH, .a large proportion of treated girls reaching a FH of more than 150 cm. Gonadal function is compromised during adolescence in about 80% of girls with TS, whilst in about 20% pubertal development occurs spontaneously. Oestrogen therapy should be started at the age of 13-14 years in hypogonadic patients; early onset of treatment (before 12 years) seems to compromise FH. Other concerns in these patients are fertility and osteopenia.
© 2016 by Walter de Gruyter Berlin/Boston
Artikel in diesem Heft
- 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
Artikel in diesem Heft
- 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
