Clinical evaluation of recombinant human growth hormone in Noonan syndrome.
نویسندگان
چکیده
The objective of this study was to investigate the effect of administration of recombinant human growth hormone (hGH) in patients with Noonan syndrome. hGH was administered (0.5 IU/kg/week) to 15 patients with Noonan syndrome over a 2 year period. Average patient age prior to therapy was 7.5 +/- 2.5 (mean +/- SD) yr, the height SD score was -2.8 +/- 0.7, and the pretreatment height velocity and bone age were 4.8 +/- 1.0 cm/yr and 5.8 +/- 2.1 yr, respectively. The height velocity in the year prior to treatment, and 0-12 and 12-24 months after commencing treatment was 4.8 +/- 1.0 cm/yr, 7.0 +/- 1.2 cm/yr, and 5.5 +/- 0.6 cm/yr, respectively. The height velocity in the first year of treatment was significantly greater (P = 0.0001, n = 14) than the pretreatment value, but there was no significant difference in the second year. The height SD scores at the commencement of treatment, and after 12 and 24 months of treatment were -2.8 +/- 0.7, -2.4 +/- 0.7, and -2.2 +/- 0.5, respectively. Bone age advanced by 1.1 +/- 0.5 yr in the 12 months after commencing treatment. We conclude that the use of hGH may be beneficial in the treatment of Noonan syndrome, although further research is required.
منابع مشابه
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سندرم نونان (گزارش یک مورد)
Noonan syndrome is a genetic condition inherited by an autosomally dominant manner, characterised by congenital heart disease, short stature, abnormal facies and the somatic feature of Turner's syndrome, but a normal karyotype. Noonan syndrome affects approximately 1 in 1500 live births. Congenital heart disease occurs in 35-50% of patients diagnosed with noonan syndrome. The most common cardia...
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Objective- To determine the effects of growth hormone (GH) therapy on bone density in controlled conditions in healthy adult rabbits. Design- Experimental in vivo study. Animal- 20 healthy, adult New Zealand White rabbits. Procedures- The rabbits were divided into 2 groups; receiving Human GH (0.006 mg/kg/d) and controls; receiving placebo for 3 months. The density of radius and tibia was m...
متن کاملThe Impact of Growth Hormone Therapy on Adult Height in Noonan Syndrome: A Systematic Review
Background: Recombinant human growth hormone (rhGH) is being used to promote linear growth in short children with Noonan syndrome. However, its efficacy is still controversial. Aims: To systematically determine the impact of rhGH therapy on adult height in children with Noonan syndrome. Methods: We searched the Cochrane Central Register of Controlled Trials, ISI Web of Science, MEDLINE, and the...
متن کاملThe Impact of Growth Hormone Therapy on Adult Height in Noonan Syndrome: A Systematic Review
Background: Recombinant human growth hormone (rhGH) is being used to promote linear growth in short children with Noonan syndrome. However, its efficacy is still controversial. Aims: To systematically determine the impact of rhGH therapy on adult height in children with Noonan syndrome. Methods: We searched the Cochrane Central Register of Controlled Trials, ISI Web of Science, MEDLINE, and the...
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ورودعنوان ژورنال:
- Endocrine journal
دوره 51 1 شماره
صفحات -
تاریخ انتشار 2004