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Published on: January 14, 2014
Catch-up growth in autosomal dominant isolated growth hormone deficiency (IGHD type II)
G Binder1, D I Iliev, P E Mullis
1University-Children's Hospital, Pediatric Endocrinology Section, Hoppe-Seyler-Str. 1, 72076 Tuebingen, Germany. gerhard.binder@med.uni-tuebingen.de
Insights
Growth hormone (GH) therapy promotes sustained catch-up growth in children with severe idiopathic GH deficiency (IGHD). These children reach their target height range, especially when treatment begins early.
Area of Science:
- Pediatric Endocrinology
- Growth Hormone Therapy
- Monogenetic Disorders
Background:
- Severely GH-deficient children with monogenetic defects lack data regarding GH-induced catch-up growth.
- Idiopathic GH deficiency (IGHD) type II is a significant cause of childhood growth failure.
Purpose of the Study:
- To investigate the catch-up growth response in prepubertal children with IGHD type II receiving GH therapy.
- To determine the long-term efficacy of GH substitution in achieving target height.
Main Methods:
- Retrospective chart review of 21 prepubertal children with IGHD type II.
- Analysis of height SDS, IGF-I SDS, and height velocity during GH therapy.
- Evaluation of bone age progression and factors influencing catch-up growth.
Main Results:
- Mean height gain of 0.92 SDS in the first year, with sustained growth over 6 years.
- Mean height velocity of 10.7 cm/year in the first year, reaching target height range (-0.97 SDS).
- Younger, shorter children showed better initial growth; bone age advanced significantly during therapy.
Conclusions:
- GH therapy leads to sustained catch-up growth in children with severe IGHD, enabling them to reach their target height.
- Early initiation and consistent administration of GH are crucial for optimal outcomes.
- The response pattern suggests GH therapy is effective for severe growth retardation due to IGHD.
Objective:
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 30.5microg/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 7.7cm/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.
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