Growth and puberty in Turner's syndrome.
Turner's syndrome is the commonest sex chromosome abnormality in females, resulting from the absence of an X chromosome or the presence of a structurally abnormal X chromosome. Short stature and ovarian failure are the most consistent clinical features and require specific and co-ordinated medical approaches in order to improve final height and well-being in adulthood. High doses of growth hormone (GH) are able to improve adult height in comparison with untreated patients. GH therapy should be started during childhood as soon as the growth curve declines below the 5th percentile and doses adjusted according to clinical response. Some studies reported that combined therapy with GH and an anabolic steroid--oxandrolone--is also beneficial at lower GH doses. Ovarian failure should be treated by appropriate substitutive estrogen therapy. Since estrogen administration may impair growth of patients with Turner's syndrome, the age at beginning of therapy should be individualized taking into consideration potential growth, the need for feminization, bone mineral density and the psychological well-being of each patient. Well co-ordinated endocrine interventions can permit better long-term outcome in adulthood.
Bertelloni
Silvano
S
Pediatric Endocrinology and Adolescent Medicine, Division of Paediatrics, University of Pisa, Santa Chiara Hospital, Pisa, Italy. s.bertelloni@clp.med.unipi.it
Baroncelli
Giampiero I
GI
Fruzzetti
Franca
F
Spinelli
Claudio
C
Simi
Paolo
P
Saggese
Giuseppe
G
D045930:Anabolic Agents / Q000627:therapeutic use; D015914:Estrogen Replacement Therapy; D005260:Female; D006128:Growth; D013006:Growth Hormone / Q000627:therapeutic use; D006801:Humans; D010074:Oxandrolone / Q000627:therapeutic use; D016649:Primary Ovarian Insufficiency / Q000209:etiology; D011627:Puberty; D014424:Turner Syndrome / Q000150:complications / Q000188:drug therapy / Q000503:physiopathology*
D045930:Anabolic Agents; D010074:Oxandrolone; D013006:Growth Hormone