Keren Smuel1, Rivka Kauli1, Pearl Lilos1, Zvi Laron2. 1. Endocrinology and Diabetes Research Unit, Schneider Children's Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Israel. 2. Endocrinology and Diabetes Research Unit, Schneider Children's Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Israel. Electronic address: laronz@clalit.org.il.
Abstract
OBJECTIVE: To describe the growth, development and puberty in children with congenital IGHD before and during hGH treatment. SUBJECTS: Patients with cIGHD treated by hGH between the years 1958-1992. SETTING: All patients were diagnosed, treated and followed in our clinic. PARTICIPANTS: Data were found in 37/41 patients (21 m, 16 f). 34 had hGH-1A deletions, 7 GHRH-R mutations. Patients, referred after age 25, were excluded. RESULTS: The birth length of 10/37 neonates was 48.29±2.26 (44-50) cm. Birth weight of 28/37 neonates was 3380±370 g (m), 3230±409 g (f). Neuromotor milestones were variable. Age at referral was 5.7±4.2 y (m) and 5.6±3.8 y (f). Initiation of hGH treatment (35μg/kg/d) was 7.5±4.8, (0.8-15.08) y (m) and 6.8±4.36 (0.8-16.5) y (f). Height SDS increased from -4.3 to -1.8 (m) and from -4.5 to -2.6 (f). Head circumference increased from -2.6 to -1.3 (m) and from -2.7 to -2.3 (f). BMI increased from 15.8 to 20.6 (m) and from 15.5 to 20.4 (f). There was a negative correlation between age of hGH initiation and change in height SDS (r=-0.66; ρ<0.01), same for bone age (r=-0.69; ρ<0.01). Upper/lower body ratio decreased from 2.5±2.1 (m±SD) to 1.08±0.1 (ρ<0.0005). Puberty was delayed in boys, less so in girls. Mean age of 1st ejaculation (14 m) was 17.6±2.2 y and of menarche (14 f. was 13.7±1.2 y. In both genders there was a positive correlation between age at start of hGH and age at onset of puberty (r=0.57; ρ<0.01). All reached full sexual development but the penile and testicular sizes were below normal. There was a positive correlation between length of hGH treatment and final testicular volume (r=0.597, ρ=0.05) and a negative correlation between the age at initiation of hGH treatment and final testicular volume(r=-0.523, ρ=0.018). All were obese and hGH treatment increased the adiposity progressively (r=0.418, ρ=0.013). CONCLUSION: Early diagnosis and treatment of cIGHD enables normal or near normal growth, development and puberty.
OBJECTIVE: To describe the growth, development and puberty in children with congenital IGHD before and during hGH treatment. SUBJECTS:Patients with cIGHD treated by hGH between the years 1958-1992. SETTING: All patients were diagnosed, treated and followed in our clinic. PARTICIPANTS: Data were found in 37/41 patients (21 m, 16 f). 34 had hGH-1A deletions, 7 GHRH-R mutations. Patients, referred after age 25, were excluded. RESULTS: The birth length of 10/37 neonates was 48.29±2.26 (44-50) cm. Birth weight of 28/37 neonates was 3380±370 g (m), 3230±409 g (f). Neuromotor milestones were variable. Age at referral was 5.7±4.2 y (m) and 5.6±3.8 y (f). Initiation of hGH treatment (35μg/kg/d) was 7.5±4.8, (0.8-15.08) y (m) and 6.8±4.36 (0.8-16.5) y (f). Height SDS increased from -4.3 to -1.8 (m) and from -4.5 to -2.6 (f). Head circumference increased from -2.6 to -1.3 (m) and from -2.7 to -2.3 (f). BMI increased from 15.8 to 20.6 (m) and from 15.5 to 20.4 (f). There was a negative correlation between age of hGH initiation and change in height SDS (r=-0.66; ρ<0.01), same for bone age (r=-0.69; ρ<0.01). Upper/lower body ratio decreased from 2.5±2.1 (m±SD) to 1.08±0.1 (ρ<0.0005). Puberty was delayed in boys, less so in girls. Mean age of 1st ejaculation (14 m) was 17.6±2.2 y and of menarche (14 f. was 13.7±1.2 y. In both genders there was a positive correlation between age at start of hGH and age at onset of puberty (r=0.57; ρ<0.01). All reached full sexual development but the penile and testicular sizes were below normal. There was a positive correlation between length of hGH treatment and final testicular volume (r=0.597, ρ=0.05) and a negative correlation between the age at initiation of hGH treatment and final testicular volume(r=-0.523, ρ=0.018). All were obese and hGH treatment increased the adiposity progressively (r=0.418, ρ=0.013). CONCLUSION: Early diagnosis and treatment of cIGHD enables normal or near normal growth, development and puberty.
Authors: Tabata M Bohlen; Thais T Zampieri; Isadora C Furigo; Pryscila Ds Teixeira; Edward O List; John Kopchick; Jose Donato; Renata Frazao Journal: J Endocrinol Date: 2019-08-01 Impact factor: 4.286
Authors: David Garcia-Galiano; Alexandra L Cara; Zachary Tata; Susan J Allen; Martin G Myers; Ernestina Schipani; Carol F Elias Journal: J Neurosci Date: 2020-11-06 Impact factor: 6.167