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Short Stature in Noonan Syndrome: Demography and Response to Growth Hormone Treatment in KIGS Barto J Otten KIGS 10 Year Book, 1999.

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Presentation on theme: "Short Stature in Noonan Syndrome: Demography and Response to Growth Hormone Treatment in KIGS Barto J Otten KIGS 10 Year Book, 1999."— Presentation transcript:

1 Short Stature in Noonan Syndrome: Demography and Response to Growth Hormone Treatment in KIGS Barto J Otten KIGS 10 Year Book, 1999

2 B J Otten - KIGS 10 Year Book, 1999 Auxological characteristics of all patients with Noonan Syndrome at the start of GH treatment Ta, Tanner et al (1966); Ra, Ranke et al (1988) WHI, weight-for-height index; PAH, projected adult height

3 B J Otten - KIGS 10 Year Book, 1999 (1/2) Height of male patients (n=101) with Noonan syndrome at the start of GH treatment, prepubertal;, in puberty;, no information on puberty;, median;, + 2 SD;, -4 SD (Noonan- specific standards; Ranke et al 1988) Shaded area, normative standards (Tanner et al 1966) Chronological age (years) Height (cm)

4 B J Otten - KIGS 10 Year Book, 1999 (2/2) Height of female patients (n=42) with Noonan syndrome at the start of GH treatment, prepubertal;, in puberty;, no information on puberty;, median;, + 2 SD;, -4 SD (Noonan- specific standards; Ranke et al 1988) Shaded area, normative standards (Tanner et al 1966) Chronological age (years) Height (cm)

5 B J Otten - KIGS 10 Year Book, 1999 Growth response of 57 prepubertal patients with Noonan syndrome to 1 year of GH therapy compared with predicted model for first-year growth response in Turner syndrome Predicted height velocity (cm/years) Studentised residuals

6 Response to GH therapy of patients with Noonan Syndrome (cross-sectional and longitudinal data for boys and girls) B J Otten - KIGS 10 Year Book, 1999 (1/6)

7 B J Otten - KIGS 10 Year Book, 1999 Response to GH therapy of patients with Noonan Syndrome (cross-sectional and longitudinal data for boys and girls) (2/6)

8 B J Otten - KIGS 10 Year Book, 1999 Response to GH therapy of patients with Noonan Syndrome (cross-sectional and longitudinal data for boys and girls) (3/6)

9 B J Otten - KIGS 10 Year Book, 1999 Response to GH therapy of patients with Noonan Syndrome (cross-sectional and longitudinal data for boys and girls) (4/6)

10 B J Otten - KIGS 10 Year Book, 1999 Response to GH therapy of patients with Noonan Syndrome (cross-sectional and longitudinal data for boys and girls) (5/6)

11 B J Otten - KIGS 10 Year Book, 1999 Response to GH therapy of patients with Noonan Syndrome (cross-sectional and longitudinal data for boys and girls) (6/6)

12 Response to 3 years of GH therapy of 26 patients (boys, n = 20; girls, n = 6) with Noonan Syndrome B J Otten - KIGS 10 Year Book, 1999 Data are shown as box plots, with medians (rules), means (dots), 25th-75th percentiles (shaded boxes) and 10th-90th percentiles (whiskers) p <0.05 (n = 26) Start 1 2 3 Years on GH therapy Parental corrected height SDS

13 B J Otten - KIGS 10 Year Book, 1999 Auxological characteristics of all patients with Noonan Syndrome discontinued from KIGS Ta, Tanner et al (1966); Ra, Ranke et al (1988) (1/2)

14 B J Otten - KIGS 10 Year Book, 1999 Auxological characteristics of all patients with Noonan Syndrome discontinued from KIGS Ta, Tanner et al (1966); Ra, Ranke et al (1988) (2/2)

15 B J Otten - KIGS 10 Year Book, 1999 Characteristics of Noonan patients on GH therapy close to final height Ta, Tanner et al (1966); Ra, Ranke et al (1988) (1/2)

16 B J Otten - KIGS 10 Year Book, 1999 Characteristics of Noonan patients on GH therapy close to final height Ta, Tanner et al (1966); Ra, Ranke et al (1988) (2/2)


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