Growth, development, puberty and adult height before and during treatment in children with congenital isolated growth hormone deficiency. Issue 4 (August 2015)

• Record Type: Journal Article
• Authors: Smuel, Keren; Kauli, Rivka; Lilos, Pearl; Laron, Zvi
• Is Part Of: Growth hormone & IGF research. Volume 25:Issue 4(2015:Aug.)
• Page Start: 182
• Page End: 188
• Publication Date: 2015-08
• Subjects: Growth regulators -- Periodicals; Growth -- Regulation -- Periodicals; Somatomedin -- Periodicals; Somatomedins -- Periodicals; Growth Hormone -- Periodicals; Growth Substances -- Periodicals; Croissance -- Régulation -- Périodiques; Croissance -- Régulateurs -- Périodiques; Somatotrophine -- Périodiques; Somatomédine -- Périodiques; Growth -- Regulation; Growth regulators; Electronic journals; Periodicals; Electronic journals; 612.4
• DOI: 10.1016/j.ghir.2015.05.001
• Languages: English
• ISSNs: 1096-6374

Abstract

<abstract abstract-type="author" id="ab0010"> <title id="st0095">Abstract</title> <sec> <title id="st0100">Objective</title> <p id="sp0055">To describe the growth, development and puberty in children with congenital IGHD before and during hGH treatment.</p> </sec> <sec> <title id="st0105">Subjects</title> <p id="sp0060">Patients with cIGHD treated by hGH between the years 1958-1992.</p> </sec> <sec> <title id="st0110">Setting</title> <p id="sp0065">All patients were diagnosed, treated and followed in our clinic.</p> </sec> <sec> <title id="st0115">Participants</title> <p id="sp0070">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.</p> </sec> <sec> <title id="st0120">Results</title> <p id="sp0075">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 (35 μg/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).</p> </sec> <sec> <title id="st0125">Conclusion</title> <p id="sp0080">Early diagnosis and treatment of cIGHD enables normal or near normal growth, development and puberty.</p> </sec> </abstract>