Deficiency of anterior pituitary hormones in Noonan syndrome and its impact on response to growth hormone therapy

Table 1 Clinical and biochemical parameters of the cohort with Noonan syndrome

Parameter	Median (IQR) or n (%) (No. of cases with NS = 11)
Age	10.5 (7.8, 13.0)
Height (cm)	117.5 (104.0, 124.5)
Ht-SDS	-3.43 (-4.18, -2.52)
Noonan specific Ht-SDS	-1.08 (-1.97, -0.61)
Bodyweight (kg)	18 (14, 25)
Bodyweight SDS	-2.93 (-4.38, -2.22)
BMI (kg/m2)	13.77 (12.69, 15.18)
BMI-SDS	-1.68 (-3.52, -1.00)
IGF-1 (ng/mL)	162 (135, 200)
IGF-1 SDS	-1.21 (-1.82, 0.22)
Height velocity in first year after GH	9.2 (7.5, 9.8)
Low IGF-1 (< -2 SDS)	4
GH deficiency seen on stimulation tests	5
IGF-1 SDS (%) increment in 1 year	75.21 (51.33, 83.85)
Genetic test ( n = 6)	PTPN11 mutation: 5
Cardiac defects (n = 8)	Pulmonary stenosis, n = 4
	ASD; n = 3
	HCM, n = 1
Chest deformity	9
Family history	1
Cryptorchidism	3/5 males
Mental retardation	2
Coagulation defects	1
Lymphatic dysplasia	3
Neuropsychological impairments, language delay	3

Data are expressed as median (interquartile range) and expressed as (25^th percentile, 75^th percentile) or as n (%). SDS: Standard deviation score; BMI: Body mass index; IGF-1: Insulin like growth factor 1; GH: Growth hormone; ASD: Atrial septal defect; IQR: Interquartile range; Ht: Height; HCM: Hypertrophic cardiomyopathy.