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Growth curves for Laron syndrome
1Institute of Pediatric and Adolescent Endocrinology Children's Medical Center, Beilinson Campus, Sackler Faculty of Medicine, Petah Tikva, Israel.
Insights
Growth curves for children with Laron syndrome reveal significant growth retardation from birth, with no clear pubertal spurt. Final heights were notably shorter than average, establishing a model for insulin-like growth factor-I deficiency.
Area of Science:
- Pediatrics
- Endocrinology
- Genetics
Background:
- Laron syndrome is a rare genetic disorder characterized by primary insulin-like growth factor-I (IGF-I) deficiency.
- Growth retardation is a hallmark feature, evident from birth and persisting throughout childhood and adolescence.
Purpose of the Study:
- To construct detailed growth curves for children with Laron syndrome.
- To establish a reference model for growth patterns in primary and secondary IGF-I deficiencies.
- To provide a basis for monitoring treatment outcomes in Laron syndrome.
Main Methods:
- Longitudinal growth measurements (height) were collected from 24 patients (10 boys, 14 girls) with Laron syndrome.
- Growth data were analyzed across infancy, childhood, and puberty.
- Upper to lower body segment ratios were assessed.
Main Results:
- Growth retardation was observed from birth (42-46 cm).
- Postnatal growth curves deviated significantly from normal trajectories.
- No distinct pubertal growth spurt was identified in either sex.
- Final mean height for girls was 119 cm (16-19 years), and for boys was 124 cm (beyond 20 years).
- Upper to lower body segment ratio exceeded 2 SD above the normal mean.
Conclusions:
- The constructed growth curves serve as a valuable model for Laron syndrome and other IGF-I deficiencies.
- These curves are essential for tracking growth in untreated patients and evaluating the efficacy of recombinant IGF-I therapy.
Abstract:
Growth curves for children with Laron syndrome were constructed on the basis of repeated measurements made throughout infancy, childhood, and puberty in 24 (10 boys, 14 girls) of the 41 patients with this syndrome investigated in our clinic. Growth retardation was already noted at birth, the birth length ranging from 42 to 46 cm in the 12/20 available measurements. The postnatal growth curves deviated sharply from the normal from infancy on. Both sexes showed no clear pubertal spurt. Girls completed their growth between the age of 16-19 years to a final mean (SD) height of 119 (8.5) cm whereas the boys continued growing beyond the age of 20 years, achieving a final height of 124 (8.5) cm. At all ages the upper to lower body segment ratio was more than 2 SD above the normal mean. These growth curves constitute a model not only for primary, hereditary insulin-like growth factor-I (IGF-I) deficiency (Laron syndrome) but also for untreated secondary IGF-I deficiencies such as growth hormone gene deletion and idiopathic congenital isolated growth hormone deficiency. They should also be useful in the follow up of children with Laron syndrome treated with biosynthetic recombinant IGF-I.
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