Short stature with normal growth hormone and elevated IGF-I

1992 ◽  
Vol 151 (5) ◽  
pp. 321-325 ◽  
Author(s):  
T. Momoi ◽  
C. Yamanaka ◽  
M. Kobayashi ◽  
T. Haruta ◽  
H. Sasaki ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Short Stature ◽  
Normal Growth ◽  
Igf I

scholarly journals Differential Diagnosis of the Short IGF-I-Deficient Child with Apparently Normal Growth Hormone Secretion

2021 ◽  
pp. 1-24
Author(s):  
Jan M. Wit ◽  
Sjoerd D. Joustra ◽  
Monique Losekoot ◽  
Hermine A. van Duyvenvoorde ◽  
Christiaan de Bruin
Keyword(s):  
Growth Hormone ◽  
Differential Diagnosis ◽  
Growth Hormone Secretion ◽  
Normal Growth ◽  
Stimulation Test ◽  
Healthy Children ◽  
Genetic Causes ◽  
Gh Secretion ◽  
Igf I

The current differential diagnosis for a short child with low insulin-like growth factor I (IGF-I) and a normal growth hormone (GH) peak in a GH stimulation test (GHST), after exclusion of acquired causes, includes the following disorders: (1) a decreased spontaneous GH secretion in contrast to a normal stimulated GH peak (“GH neurosecretory dysfunction,” GHND) and (2) genetic conditions with a normal GH sensitivity (e.g., pathogenic variants of <i>GH1</i> or <i>GHSR</i>) and (3) GH insensitivity (GHI). We present a critical appraisal of the concept of GHND and the role of 12- or 24-h GH profiles in the selection of children for GH treatment. The mean 24-h GH concentration in healthy children overlaps with that in those with GH deficiency, indicating that the previously proposed cutoff limit (3.0–3.2 μg/L) is too high. The main advantage of performing a GH profile is that it prevents about 20% of false-positive test results of the GHST, while it also detects a low spontaneous GH secretion in children who would be considered GH sufficient based on a stimulation test. However, due to a considerable burden for patients and the health budget, GH profiles are only used in few centres. Regarding genetic causes, there is good evidence of the existence of Kowarski syndrome (due to <i>GH1</i> variants) but less on the role of <i>GHSR</i> variants. Several genetic causes of (partial) GHI are known (<i>GHR</i>, <i>STAT5B</i>, <i>STAT3</i>, <i>IGF1</i>, <i>IGFALS</i> defects, and Noonan and 3M syndromes), some responding positively to GH therapy. In the final section, we speculate on hypothetical causes.

Short Children and Growth Hormone

PEDIATRICS ◽  
1984 ◽  
Vol 73 (1) ◽  
pp. 112-113
Author(s):  
KENNETH C. COPELAND
Keyword(s):  
Growth Hormone ◽  
Short Stature ◽  
Growth Rates ◽  
Plasma Concentrations ◽  
Normal Growth ◽  
Somatomedin C ◽  
Short Children ◽  
Provocative Testing

To the Editor.— The article by Bright et al1 was a provocative description of two subjects with short stature, normal growth hormone (GH) responses to provocative testing, and low somatomedin-C (SM-C) concentrations, which increased after administration of GH. The authors conclude that the short stature in these individuals may be due to a biologically inactive GH molecule or to decreased dose responsiveness to GH of SM-producing cells. Their data also seem compatible with a third possibility: normal short children respond to GH administration with increases in SM-C plasma concentrations and growth rates.

Growth hormone and insulin-like growth factor regulate insulin-like growth factor-binding protein-1 in Laron type dwarfism, growth hormone deficiency and constitutional short stature

1992 ◽  
Vol 127 (4) ◽  
pp. 351-358 ◽  
Author(s):  
Zvi Laron ◽  
Anne-Maria Suikkari ◽  
Beatrice Klinger ◽  
Aviva Silbergeld ◽  
Athalia Pertzelan ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Growth Factor ◽  
Growth Hormone Deficiency ◽  
Short Stature ◽  
Hormone Deficiency ◽  
Pituitary Hormone ◽  
Healthy Children ◽  
Insulin Like Growth Factor ◽  
Factor Binding ◽  
Igf I

Insulin-like growth factors (IGFs) mediate the effects of growth hormone (GH), and the insulin-like growth factor-binding proteins (IGFBPs) modulate the actions of IGFs in tissues. We studied the circulating levels of IGFBP-1 in 6 children and 9 adults with Laron type dwarfism (LTD), in 11 children and 21 adults with growth hormone deficiency (GHD), and in 8 children with constitutional short stature. Compared with the situation in healthy children, the basal serum IGFBP-1 concentration was 5.4-fold higher in LTD children, 4.1-fold higher in GHD children, and 3.8-fold higher in children with short stature (p<0.02 vs controls in all groups). In adult patients with multiple pituitary hormone deficiency (MPHD), the IGFBP-1 concentration was 2-fold elevated, but it was normal in adult LTD patients. Intravenous (N= 10) or subcutaneous (N=9) administration ofIGF-I (75 μg·kg−1 and 150 μg·kg−1, respectively) in LTD children resulted in a rapid 50–60% fall in serum insulin (p<0.02), a decline in blood glucose and a concomitant 40–60% rise of IGFBP-1 levels (p<0.05). Treatment for seven days with IGF-I (150 μg·kg−1·d−1) resulted in a decrease by 34% and 44% of serum IGFBP-1 level in two out of three children with LTD. After prolonged GH therapy, the IGFBP-1 level fell in GHD children by 29% (p<0.05), in GHD adults by 52% (p<0.02) and in children with constitutional short stature by 17% (p<0.02). IGFBP-1 and insulin concentrations were inversely related in patients with GHD (r= −0.66, p<0.001) or with LTD (r= −0.57, p<0.05). Our data suggest that: (a) increased IGFBP-1 concentration in LTD, GHD and constitutional short children may, at least in part, be accounted for by an IGF-I deficiency; (b) both the rise in IGF-I and a fall in insulin contributed to the rise in IGFBP-1 after acute IGF-I administration; (c) prolonged IGF-I or GH treatment causes a persistent decline in IGFBP-1 concentration. In conclusion, IGF-I and GH may regulate IGFBP-1 secretion either directly or via insulin.

Standard and low-dose IGF-I generation tests and spontaneous growth hormone secretion in children with idiopathic short stature

2004 ◽  
Vol 60 (2) ◽  
pp. 163-168 ◽  
Author(s):  
J. C. Blair ◽  
C. Camacho-Hübner ◽  
F. Miraki Moud ◽  
S. Rosberg ◽  
C. Burren ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Short Stature ◽  
Low Dose ◽  
Hormone Secretion ◽  
Growth Hormone Secretion ◽  
Idiopathic Short Stature ◽  
Spontaneous Growth ◽  
Igf I

Long-term results of growth hormone therapy in children with short stature, subnormal growth rate and normal growth hormone response to secretagogues

1995 ◽  
Vol 42 (4) ◽  
pp. 365-372 ◽  
Author(s):  
Jan-Maarten Wit ◽  
Bart Boersma ◽  
Sabine M. P. F. Muinck Keizer-Schrama ◽  
Henrlët E. Nienhuls ◽  
Wilma Oostdijk ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Growth Rate ◽  
Hormone Therapy ◽  
Short Stature ◽  
Growth Hormone Therapy ◽  
Normal Growth ◽  
Growth Hormone Response ◽  
Long Term Results ◽  
Hormone Response

scholarly journals 16 YEARS OF NORMAL GROWTH WITHOUT GROWTH HORMONE(GH), WITHOUT IGF-I AND IGFBP-3 AND WITHOUT OBESITY

1993 ◽  
Vol 33 ◽  
pp. S62-S62 ◽  
Author(s):  
W V Petrykowski ◽  
W F Blum ◽  
M B Ranke ◽  
C M Niemeyer
Keyword(s):  
Growth Hormone ◽  
Normal Growth ◽  
Igf I ◽  
Igfbp 3
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