Catch-up growth in autosomal dominant isolated growth hormone deficiency (IGHD type II)

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• 作者：G. Binder ; D.I. Iliev ; P.E. Mullis ; M.B. Ranke
• 关键词：Isolated growth hormone deficiency ; Dominant transmission ; Splice-site mutation ; Missense mutation
• 刊名：Growth Hormone & IGF Research
• 出版年：2007
• 出版时间：June 2007
• 年：2007
• 卷：17
• 期：3
• 页码：242-248
• 全文大小：393 K

文摘

Data on the GH-induced catch-up growth of severely GH-deficient children affected by monogenetic defects are missing.

Patients

Catch-up growth of 21 prepubertal children (6 females, 15 males) affected with IGHD type II was analyzed in a retrospective chart review. At start of therapy, mean age was 6.2 years (range, 1.6–15.0), mean height SDS was −4.7 (−7.6 to −2.2), mean IGF-I SDS was −6.2 (−10.1 to −2.2). GH was substituted using a mean dose of 30.5 μg/kg*d.

Results

Catch-up growth was characterized by a mean height gain of +0.92, +0.82, and +0.61 SDS after 1, 2, and 3 years of GH therapy, respectively. Mean height velocities were 10.7, 9.2 and 7.7 cm/year during the first three years. Mean duration of complete catch-up growth was 6 years (3–9). Mean height SDS reached was −0.97 (−2.3 to +1.1), which was within the range of the estimated target height of −0.60 SDS (−1.20 to −0.15). The younger and shorter the children were at start of therapy the better they grew during the first year independent of the dose. Mean bone age was delayed at start by 2.1 years and progressed by 2.5 years during the first two years of therapy. Incomplete catch-up growth was caused by late initiation or irregular administration of GH in four cases.

Conclusions

Our data suggest that GH-treated children with severe IGHD show a sustained catch-up growth over 6 years (mean) and reach their target height range. This response to GH is considered to be characteristic for young children with severe growth retardation due to IGHD.