Zvi Laron1, Moshe Iluz, Rivka Kauli. 1. Endocrinology and Diabetes Research Unit, Schneider Children's Medical Center, Petah Tikva 49202, Israel. laronz@clalit.org.il
Abstract
BACKGROUND: Head circumference (HC) is a simple and practical measure of brain size, development and longitudinal measurements of the HC in childhood are an index of brain growth. OBJECTIVE: To determine the effects of long IGF-I deficiency and treatment on HC in patients with Laron syndrome (LS). PATIENTS: 20 untreated adult LS patients, aged 48.4±11.2 years and 13 LS patients treated between ages of 5.6±4 to 11.3±3 years were studied. 15 patients with congenital IGHD treated between age 6.1±4 and 13±4 by hGH served as controls. METHODS: HC was expressed as standard deviation (SD) and Ht as SDS. HC was measured and plotted on Nellhaus charts. Linear height (Ht) was measured by a Harpenden Stadiometer. CONCLUSIONS: The mean HC deficit of the adult untreated LS males was -2.9±0.6 SD compared to a Ht deficit of -7.0±1.7 SDS. The HC of the LS adult females was -3.6±1 SD compared to a Ht SDS of -6.9±1.5 (p<0.001). IGF-I treatment (150-200 μg/kg once daily) increased the HC from -3.3±0.9 (m±SD) to normal values (0.87±1.8 SD) (p<0.001) in 11/13 children. The Ht SDS deficit decreased only by 1.5 SDS. hGH treatment of cIGHD children increased the HC from -2.0±1.8 to 0.3±1.2 SD and the Ht SDS from -4.8±1.6 to 1.6±1.0.
BACKGROUND: Head circumference (HC) is a simple and practical measure of brain size, development and longitudinal measurements of the HC in childhood are an index of brain growth. OBJECTIVE: To determine the effects of long IGF-I deficiency and treatment on HC in patients with Laron syndrome (LS). PATIENTS: 20 untreated adult LS patients, aged 48.4±11.2 years and 13 LS patients treated between ages of 5.6±4 to 11.3±3 years were studied. 15 patients with congenital IGHD treated between age 6.1±4 and 13±4 by hGH served as controls. METHODS: HC was expressed as standard deviation (SD) and Ht as SDS. HC was measured and plotted on Nellhaus charts. Linear height (Ht) was measured by a Harpenden Stadiometer. CONCLUSIONS: The mean HC deficit of the adult untreated LS males was -2.9±0.6 SD compared to a Ht deficit of -7.0±1.7 SDS. The HC of the LS adult females was -3.6±1 SD compared to a Ht SDS of -6.9±1.5 (p<0.001). IGF-I treatment (150-200 μg/kg once daily) increased the HC from -3.3±0.9 (m±SD) to normal values (0.87±1.8 SD) (p<0.001) in 11/13 children. The Ht SDS deficit decreased only by 1.5 SDS. hGH treatment of cIGHD children increased the HC from -2.0±1.8 to 0.3±1.2 SD and the Ht SDS from -4.8±1.6 to 1.6±1.0.
Authors: Jürgen Klammt; David Neumann; Evelien F Gevers; Shayne F Andrew; I David Schwartz; Denise Rockstroh; Roberto Colombo; Marco A Sanchez; Doris Vokurkova; Julia Kowalczyk; Louise A Metherell; Ron G Rosenfeld; Roland Pfäffle; Mehul T Dattani; Andrew Dauber; Vivian Hwa Journal: Nat Commun Date: 2018-05-29 Impact factor: 14.919
Authors: Thais R Villela; Bruna L Freire; Nathalia T P Braga; Rodrigo R Arantes; Mariana F A Funari; Jorge A L Alexander; Ivani N Silva Journal: Genet Mol Biol Date: 2020-01-20 Impact factor: 1.771