Turner Syndrome and Genotropin (somatropin, rbe)

Turner syndrome is a chromosomal disorder characterised by the complete or partial lack of one X chromosome in girls. The two most common clinical features are short stature and ovarian failure.

Not all girls with Turner syndrome will need treatment with Genotropin (somatropin, rbe), but if the condition is untreated when indicated, girls with Turner syndrome will have a final adult height of 136–147 cm which on average is 20 cm shorter than other adult women.

Turner syndrome occurs in between 1 in 1500 and 1 in 2500 live female births.

Efficacy of Genotropin (somatropin) in the treatment of Turner Syndrome

Normalisation of height with induction of puberty at an appropriate time1,2

Significant improvement in height standard deviation score (SDS) relative to TS-specific height references (p < 0.05), in girls with TS treated to near adult height (NAH).

Improve standard deviation score relative to TS-specific height references

 

Maximisation of height with the early introduction of growth hormone (GH) therapy allows for the timely induction of puberty with oestrogen in patients with TS 1,2

Study methods

Height SDS data reported at GH start (mean age 9.7 years), 1st year and puberty from 1,146 TS girls from published data, treated to near final height (mean dose of 0.28 mg/kg body weight/week).

Adapted from Ranke et al, 2007 1

Genotropin dosage for treatment of Turner Syndrome

A Genotropin (somatropin) dose of 0.045 - 0.050 mg/kg body weight per day or 1.4 mg/m2 body surface area per day is recommended for the treatment of Turner Syndrome.

Adverse events in the long-term treatment of growth disturbance due to Turner syndrome

 

Long-term Treatment of Children with Growth Disturbance due to Turner syndrome

System Organ Class

Very Common

≥1/10

Common

≥ 1/100 to <1/10

Uncommon ≥ 1/1,000 to <1/100

Rare

≥ 1/10,000 to <1/1,000

Very Rare

<1/10,000

Not Known (cannot be estimated from available data)

Neoplasms Benign, Malignant and Unspecified (including cysts and polyps)

 

 

 

 

 

Leukaemia†

Metabolism and Nutrition Disorders

 

 

 

 

 

Type 2 diabetes mellitus

Nervous System Disorders

 

 

 

 

 

Paraesthesia*

Benign intracranial hypertension

Musculoskeletal, Connective Tissue and Bone Disorders

Arthralgia*

 

 

 

 

Myalgia*

Musculoskeletal stiffness*

General Disorders and Administration Site Conditions

 

 

 

 

 

Oedema peripheral*

Injection site reaction$

Investigations

 

 

 

 

 

Blood cortisol decreased

 

*In general, these adverse effects are mild to moderate, arise within the first months of treatment, and subside spontaneously or with dose-reduction. The incidence of these adverse effects is related to the administered dose, the age of the patients, and possibly inversely related to the age of the patients at the onset of growth hormone deficiency.

$ Transient injection site reactions in children have been reported.

‡ Clinical significance is unknown

† Reported in growth hormone deficient children treated with somatropin, but the incidence appears to be similar to that in children without growth hormone deficiency.

References:

1. Ranke MB, Price DA, Reiter EO (eds): Growth Hormone Therapy in Pediatrics – 20 Years of KIGS. Basel, Karger, 2007, pp 332–339.

2. Ranke MB, Price DA, Reiter EO (eds): Growth Hormone Therapy in Pediatrics – 20 Years of KIGS. Basel, Karger, 2007, pp 326–331.

Abbreviations: TS, turner syndrome; SDS, standard deviation score; NAH, near adult height; GH, growth hormone.

 

PP-GEN-GBR-0471. July 2018