In:
Acta Paediatrica, Wiley, Vol. 94, No. 9 ( 2005-09), p. 1232-1237
Abstract:
Aim: To assess whether children with Noonan syndrome on long‐term growth hormone (GH) therapy improve their final height to near mid‐parental height. Methods: Twenty‐five prepubertal children (13 girls) with Noonan syndrome (NS) were studied. A single clinician made the diagnosis based on clinical criteria. GH treatment started at an age ranging from 3.1 to 13.8 y and was continued for at least 2 y. Improvement or “gain” in final height (FH) was defined as either the difference between adult height SD scores (SDS) and pre‐treatment height SDS (the childhood component of the Swedish reference) or height SDS compared to the Noonan reference. Results: Ten children received a GH dose of 33 μg/kg/d (mean age at start 7.7±2.1 y, mean age at stop 17.6±1.7 y) and 15 received a dose of 66 μg/kg/d (mean age at start 8.6±3.3 y, mean age at stop 18.4±2.1 y). Eighteen out of 25 patients reached FH. A substantial improvement in FH of 1.7 SDS, equivalent to 10.4 cm compared to pre‐treatment height, was observed. No significant difference was seen between the two GH doses. Females gained a mean height of 9.8 cm and males 1–13 cm (FH 174.5±7.8 cm vs mean adult height of 162.5±5.4 cm for males with NS) at final height. Moreover, 60% reached a mid‐parental height of±1 SD. Conclusion: GH treatment improves final height in patients with Noonan syndrome, with a mean gain of 1.7 SDS. The prepubertal height gain is maintained to final height and the children achieve a height close to their mid‐parental height.
Type of Medium:
Online Resource
ISSN:
0803-5253
,
1651-2227
DOI:
10.1111/apa.2005.94.issue-9
DOI:
10.1111/j.1651-2227.2005.tb02081.x
Language:
English
Publisher:
Wiley
Publication Date:
2005
detail.hit.zdb_id:
1492629-5
detail.hit.zdb_id:
1501466-6
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