TY - JOUR
T1 - The insulin‐like growth factor I generation test in the investigation of short stature
AU - Cotterill, AM
AU - Camacho‐Hübner, C.
AU - Woods, K.
AU - Martinelli, C.
AU - Duquesnoy, P.
AU - Savage, MO
PY - 1994/4
Y1 - 1994/4
N2 - Genotypic and phenotypic heterogeneity in patients with growth hormone (GH) insensitivity syndrome suggests that partial defects exist in the GH receptor. The insulin‐like growth factor I (IGF‐I) generation test was assessed as a means of identifying partial GH receptor defects in a heterogeneous group of 22 prepubertal children with short stature. In a subgroup of nine patients with peak GH levels of 63.7 ± 3.7 mU/l during a glucagon tolerance test, the response to the IGF‐I generation test was no different from that for the group as a whole (peak GH, 43.3 ± 4.5 mU/l), despite the fact that this subgroup exhibited a negative relationship between height SDS and peak GH and a positive relationship between height SDS and IGF binding protein‐3. This preliminary study therefore suggests that the IGF‐I generation test in its present form will not be useful as a primary screening test for partial GH insensitivity. Despite this, the IGF‐I generation test has been extremely useful in the confirmation of the diagnosis of GHIS and may therefore also prove useful in the confirmation of partial defects in the GH receptor. A subgroup of short children with peak GH levels above 40 mU/l had some characteristics of partial GH receptor deficiency. These children, to whom GH therapy would not normally be given, may respond better to recombinant human IGF‐I.
AB - Genotypic and phenotypic heterogeneity in patients with growth hormone (GH) insensitivity syndrome suggests that partial defects exist in the GH receptor. The insulin‐like growth factor I (IGF‐I) generation test was assessed as a means of identifying partial GH receptor defects in a heterogeneous group of 22 prepubertal children with short stature. In a subgroup of nine patients with peak GH levels of 63.7 ± 3.7 mU/l during a glucagon tolerance test, the response to the IGF‐I generation test was no different from that for the group as a whole (peak GH, 43.3 ± 4.5 mU/l), despite the fact that this subgroup exhibited a negative relationship between height SDS and peak GH and a positive relationship between height SDS and IGF binding protein‐3. This preliminary study therefore suggests that the IGF‐I generation test in its present form will not be useful as a primary screening test for partial GH insensitivity. Despite this, the IGF‐I generation test has been extremely useful in the confirmation of the diagnosis of GHIS and may therefore also prove useful in the confirmation of partial defects in the GH receptor. A subgroup of short children with peak GH levels above 40 mU/l had some characteristics of partial GH receptor deficiency. These children, to whom GH therapy would not normally be given, may respond better to recombinant human IGF‐I.
KW - Growth hormone insensitivity syndrome
KW - insulin‐like growth factor I generation test
KW - partial GH receptor defects
KW - short stature
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U2 - 10.1111/j.1651-2227.1994.tb13305.x
DO - 10.1111/j.1651-2227.1994.tb13305.x
M3 - Article
C2 - 7949597
AN - SCOPUS:0028415489
VL - 83
SP - 128
EP - 130
JO - Acta Paediatrica Scandinavica
JF - Acta Paediatrica Scandinavica
SN - 0803-5253
ER -