Prevalence of Primary Insulin-like Growth Factor-I deficiency in prepubertal children with short sta...
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Prevalence of Primary Insulin-like Growth Factor-I deficiency in prepubertal children with short stature. Mäithé Tauber and Thomas Edouard Toulouse, France. Barcelona May 2009. Aims of the study.

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Prevalence of Primary Insulin-like Growth Factor-I deficiency in prepubertal children with short stature

MäithéTauber and Thomas Edouard

Toulouse, France

Barcelona May 2009


Aims of the study
Aims of the study deficiency in prepubertal children with short stature

To review all children with SS seen in our Paediatric Endocrinology Unit from January 2005 to December 2007

To evaluate the prevalence of non-GH deficient IGFD in prepubertal children with SS and to describe this population


Inclusion criteria
Inclusion criteria: deficiency in prepubertal children with short stature

Short stature with current height SDS below or equal to -2.5

Age  2 years

Prepubertal status

Exclusion criteria:

Identified cause of short stature

Current or past therapy with rhGH


Screening process to identify patients with primary igfd
Screening process to identify patients with deficiency in prepubertal children with short statureprimary IGFD

In 2005, 2006, 2007short stature < -2 SDS

Girls: 2-13 years, boys: 2-14 years

n=362

Height> -2.5 SDS (n=217)

Pubertal patients (S2 or G2) (n=54)

Syndromes or chromosomal defects (n=17)

Bone diseases (n=7)

Severe neurological impairment (n=10)

Severe chronic illness (n=5)

Prepubertal children withshort stature < -2.5 SDS (n=65)

n=65


Screening process to identify patients with primary igfd1
Screening process to identify patients with deficiency in prepubertal children with short statureprimary IGFD

Prepubertal children withshort stature < -2.5 SDS (n=65)

n=65

Growth hormone deficiency (n=19)

IGF-I > -2SDS

Primary IGFD (n=13)

AGA n=9 and SGA n=4

20% PIGFD


Frequency of primary igfd in 65 children with ss
Frequency of primary IGFD in 65 children deficiency in prepubertal children with short staturewith SS

GHD

SGA

IGFD

IGFD

ISS

GHD

19

29%

ISS34%

13

20%

SGA37%

17% PIGFD

20

31%

40% PIGFD

9

14%

4

6%

PIGFD20%


Classification and frequency of gh igf i axis abnormalities
Classification and frequency of GH-IGF-I deficiency in prepubertal children with short statureaxis abnormalities

Normal 51%


Auxological hormonal characteristics and complementary studies of the 13 children with pigfd
Auxological, hormonal characteristics and complementary studies of the 13 children with PIGFD

IGF generation testa

* Molecular studies of GHR were undertaken in three patients (number 2, 6 and 8) and were normal.


Conclusion
Conclusion studies of the 13 children with PIGFD

The prevalence of primary IGFD was 20% in the children with short stature

30% of them are born SGA

Need for extensive multicentre studies to identify patients with Primary IGFD

 EPIGROW


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