Turner's syndrome.
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.
Guarneri
M P
MP
Paediatric Department-Endocrine Unit Scientific Institute H San Raffaele, Milan, Italy. guarneri.mariapia@hsr.it
Abusrewil
S A
SA
Bernasconi
S
S
Bona
G
G
Cavallo
L
L
Cicognani
A
A
Di Battista
E
E
Salvatoni
A
A
D000293:Adolescent; D002648:Child; D005260:Female; D012739:Gonadal Steroid Hormones / Q000008:administration & dosage / Q000627:therapeutic use; D006130:Growth Disorders / Q000209:etiology / Q000628:therapy*; D013006:Growth Hormone / Q000008:administration & dosage / Q000627:therapeutic use; D006801:Humans; D008297:Male; D011627:Puberty / Q000187:drug effects / Q000502:physiology*; D014424:Turner Syndrome / Q000188:drug therapy*
D012739:Gonadal Steroid Hormones; D013006:Growth Hormone