Table 1 Proposed model of assessment and management for girls with Turner syndrome from infancy to 18 years.
Measurements and investigations | Treatment |
---|---|
4–6 monthly | Growth hormone |
Height and weight | Age at start |
Blood pressure | When height falls <−2 SD on a standard growth chart or |
Pubertal staging (from 10 years onwards) | When the family identifies short stature as becoming a problem or |
By 8 years of age | |
12–18 monthly | Dose: 10 mg/m2/week (≈0.3 mg/kg/week) by daily injection |
Thyroid function and IGF‐1 measurement | |
Bone age | |
Hearing assessment | |
3–5 yearly | Oestrogen for pubertal induction* |
DXA scan for bone density | Age at start: 13 years, unless GH started particularly late and in the absence of any patient/family preference for earlier or later |
Other assessments | Dose: Protocol adopted by UK Turner Study (see text for details): |
FSH levels and pelvic ultrasound scan prior to pubertal induction | Year 1: Ethinyloestradiol 2 μg daily |
Liver function tests, prior to (a) pubertal induction and (b) adult transfer | Year 2: Ethinyloestradiol 4 μg daily |
Renal imaging Ultrasound scan at diagnosis and at adult transfer. Additional imaging if indicated (e.g. ultrasound abnormal ± recurrent UTI) | Year 3: Ethinyloestradiol 6 μg daily, increasing every 4 months to 8 μg and then 10 μg daily Year 4: Adult replacement dose |
Cardiac assessment | |
Ultrasound scan at diagnosis, at pubertal induction, and at adult transfer | |
Eye assessment at diagnosis, follow up as required | Norethisterone 5 mg daily for the first 5 days of each calendar month when ethinyloestradiol reaches 10 μg daily, or when breakthrough bleeding occurs, whichever is the sooner |
Referral as required | Oxandrolone* |
ENT/Audiology | Age at start: from 9 years of age |
Podiatrist | Dose: 0.05 mg/kg/day, maximum 2.5 mg/day |
Dietician | |
Dermatologist | |
Psychologist | |
Ophthalmology |
*Currently under investigation in the UK Turner Study.