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  • Title: Turner's syndrome.
    Author: Guarneri MP, Abusrewil SA, Bernasconi S, Bona G, Cavallo L, Cicognani A, Di Battista E, Salvatoni A, International Workshop on Management of Puberty for Optimum Auxological Results.
    Journal: J Pediatr Endocrinol Metab; 2001 Jul; 14 Suppl 2():959-65. PubMed ID: 11529402.
    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.
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