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

Short Stature in Noonan Syndrome:Demography and Response to

Growth Hormone Treatment in KIGS

Barto J Otten

KIGS 10 Year Book, 1999

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

Variable Boys GirlsPercentile Percentile

(n) (median) (10th) (90th) (n) (median) (10th) (90th)

Mid-parental height (SDS) 95 -0.4 -2.4 0.9 41 -0.5 -1.7 0.8

Birth weight (SDS) 85 -0.4 -2.1 0.9 41 -0.7 -2.4 1.1Chronological age (yrs) 101 9.7 5.4 15.0 42 9.9 3.5 14.4

Bone age (yrs) 43 7.2 3.5 11.8 13 7.0 2.5 10.0

Height (Ta) (SDS) 101 -3.0 -4.3 -2.2 42 -3.1 -4.4 -2.1Height (Ra) (SDS) 100 -1.3 -2.7 -0.5 41 -1.0 -2.7 -0.1

Weight (SDS) 101 -2.8 -4.3 -1.4 42 -2.5 -4.5 -1.5WHI (%) 101 100 90 115 42 99 86 114Height velocity (cm/yr) 57 4.3 2.9 6.2 23 4.8 2.7 7.8

PAH (Ra) (cm) 100 152.2 141.5 158.1 41 143.9 132.8 150.4GH dose (IU/kg/week) 101 0.64 0.50 0.97 42 0.72 0.45 9.97Injections (n/week) 101 6.0 5.0 7.0 42 6.5 5.0 7.0


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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)

Hei

ght (

cm)


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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)

Hei

ght (

cm)


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)

Stud

enti s

e d re

sidu

a ls

Response to GH therapy of patients with Noonan Syndrome(cross-sectional and longitudinal data for boys and girls)


Variable Cross-sectional data Longitudinal data

Boys Girls Boys Girls

Percentile Percentile (median) (median)

(n) (median) (10th) (90th) (n) (median) (10th) (90th) (n=20) (n=6)

At start

Chronological age (yrs) 101 9.7 5.4 15.0 42 9.9 3.5 14.4 8.8 3.2

Height (Ta) (SDS) 101 -3.0 -4.3 -2.2 42 -3.1 -4.4 -2.1 -2.9 -3.8

Height (Ra) (SDS) 100 -1.3 -2.7 -0.5 41 -1.0 -2.7 -0.1 -1.2 -1.5

Height velocity (cm/yr) 57 4.3 3.9 6.2 23 4.8 2.7 7.8

Projected height (cm) 100 152.2 141.5 158.1 144 143.9 132.8 150.4 152.4 141.2

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1st Year

Height (Ta) (SDS) 76 -2.7 -4.0 -1.9 31 -2.7 -4.5 -1.8 -2.6 -2.9

Height (Ra) (SDS) 76 -0.9 -2.2 0.2 31 -0.5 -2.5 0.4 -0.8 -1.0



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2nd Year

Height (Ta) (SDS) 59 -2.5 -3.7 -1.6 27 -2.4 -3.8 -1.7 -2.4 -2.8

Height (Ra) (SDS) 57 -0.7 -1.9 0.8 26 -0.4 -2.2 0.4 -0.7 -1.0



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3rd Year

Height (Ta) (SDS) 49 -2.6 -4.3 -1.2 14 -2.5 -3.7 -1.6 -2.5 -2.5

Height (Ra) (SDS) 43 -0.5 -2.2 1.0 14 0.1 -2.1 0.7 -0.7 -0.4



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4th Year

Height (Ta) (SDS) 37 -2.3 -3.6 -0.9 12 -2.4 -3.6 -1.3

Height (Ra) (SDS) 30 0.2 -2.3 1.3 11 0.0 -2.0 0.6


Projected height (cm) 30 162.8 144.5 171.1 11 151.1 137.5 155.2

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5th Year

Height (Ta) (SDS) 24 -2.0 -3.6 -0.7 8 -2.3 -4.8 0.0

Height (Ra) (SDS) 19 0.5 -2.1 1.9 8 0.0 -2.7 1.9


Projected height (cm) 19 165.0 146.1 175.1 8 151.1 132.8 164.0

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Response to 3 years of GH therapy of 26 patients (boys, n = 20; girls, n = 6) with Noonan Syndrome


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) (n = 26) (n = 26) (n = 26)

Start 1 2 3 Years on GH therapy

Pare

ntal

cor

rect

ed h

eigh

t SD

S


Auxological characteristics of all patients with Noonan Syndrome discontinued from KIGS

Ta, Tanner et al (1966); Ra, Ranke et al (1988)

Variable Boys Girls

Patients Percentile Patients Percentile


At start of GH therapy

Chronological age (years) 36 12.0 5.8 15.9 16 12.7 7.5 14.9

Height (Ta) (SDS) 36 -3.4 -4.4 -2.5 16 -3.2 -4.1 -2.8

Height (Ra) (SDS) 36 -1.5 -2.7 -0.7 16 -1.0 -2.1 -0.1

Height velocity (1st year)(cm/yr)

31 6.4 5.5 9.1 13 7.0 3.8 8.0

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Auxological characteristics of all patients with Noonan Syndrome discontinued from KIGS


Variable Boys Girls

Patients Percentile Patients Percentile


At discontinuation fromKIGSChronological age (years) 36 15.6 9.4 19.1 16 15.8 12.3 17.0

Height (Ta) (SDS) 36 -2.9 -3.8 -1.8 16 -2.4 -3.7 -1.7

Height (Ra) (SDS) 36 -0.8 -2.3 -0.7 16 -0.3 -1.8 0.3

Duration of GH (years) 36 2.7 1.0 6.3 16 2.5 1.0 6.0

Height velocity duringpreceding year (cm/year)

36 4.3 2.7 6.5 16 3.2 0.7 4.7

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Characteristics of Noonan patients on GH therapy close to final height


Patients Percentile(n) (median) (10th) (90th)

At start of therapyMid-parental height (SDS) 18 0.1 -1.4 1.3Birth weight (SDS) 16 0.1 -1.9 1.3

Chronological age (years) 18 14.1 12.1 15.9

Height (Ta) (SDS) 18 -3.7 -4.9 -2.8Height (Ra) (SDS) 18 -1.5 -2.7 -0.4

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Characteristics of Noonan patients on GH therapy close to final height


Patients Percentile(n) (median) (10th) (90th)

Close to final heightChronological age (years) 18 18.8 17.0 20.9Height (Ta) (SDS) 18 -2.1 -3.0 -0.7Height (Ra) (SDS) 18 0.9 -0.8 3.2Height velocity (cm/year) 18 1.9 0.7 4.5Duration of GH therapy (years) 18 4.7 2.5 7.4Height (Ta) (SDS) 18 1.5 0.2 2.7Height (Ra) (SDS) 18 2.4 0.9 5.0

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Documents

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