Treatment outcome in Turner syndrome.
Short stature is an almost universal feature of Turner Syndrome (TS) with patients having a mean adult height up to 20 cm less than that of the general female population. As a result a significant focus of medical management in TS is on growth promoting strategies. The aim of this study was to assess factors influencing final height in those with TS and evaluate the response to various treatments. A retrospective study was performed in 2 Dublin centres. The age of commencement, cessation, dose and duration of treatment was calculated for growth hormone, oestrogen and oxandrolone, either alone or in combination. Additional factors known to influence growth in TS were also examined. The mean final height achieved was 147.31 cm (SD 4.77), the mean predicted height was 142.81 (SD 5.63). Height gain was 4.28 cm (SD 4.93). All patients received growth hormone. 75% of patients received oestrogen and 35% received oxandrolone. The maximal height gain of 6.71 cm (SD 2.43) in the triple therapy group. Parental height was found to have a significant correlation with final height (r=0.591). Spontaneous menarche in TS was associated with a poor adult height (r=-0.522), mean height 144.03 cm (SD 3.23) whereas those with induced menarche had a mean height of 148.43 cm (SD 4.67). Growth hormone therapy improves the mean final height in TS. However, the degree of height gain is influenced by confounding variables of parental height and age and tempo of puberty and treatment variables.
Parvin
M
M
Department of Paediatrics, University of Dublin, Trinity College, Tallaght, Dublin 24. mparvin@eircom.net
Roche
E
E
Costigan
C
C
Hoey
H M C V
HM
D000293:Adolescent; D001827:Body Height; D001835:Body Weight; D002648:Child; D004305:Dose-Response Relationship, Drug; D004334:Drug Administration Schedule; D004359:Drug Therapy, Combination; D004967:Estrogens / Q000627:therapeutic use*; D005260:Female; D005500:Follow-Up Studies; D019382:Human Growth Hormone / Q000627:therapeutic use*; D006801:Humans; D007494:Ireland; D010074:Oxandrolone / Q000627:therapeutic use*; D012189:Retrospective Studies; D018570:Risk Assessment; D016896:Treatment Outcome; D014424:Turner Syndrome / Q000175:diagnosis* / Q000188:drug therapy*
D004967:Estrogens; D019382:Human Growth Hormone; D010074:Oxandrolone