Growth hormone, somatomedin levels and growth regulation in Turner's syndrome

1987 ◽  
Vol 116 (2) ◽  
pp. 305-313 ◽  
Author(s):  
Michael B. Ranke ◽  
Werner F. Blum ◽  
Frank Haug ◽  
Werner Rosendahl ◽  
Andrea Attanasio ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Growth Regulation ◽  
Bone Age ◽  
Serum Levels ◽  
Turner's Syndrome ◽  
Turner’S Syndrome ◽  
Normal Range ◽  
Cross Sectional ◽  
Gh Secretion ◽  
Igf I

Abstract. In a total of 56 children and adolescents with Turner's syndrome (41 with karyotype 45,X) basal serum levels of somatomedin bioactivity, Sm-C/IGF-I (RIA), IGF II (RIA), GH response to arginine and GHRH (GRF(1-29)NH2), and spontaneous GH secretion during 5.5 h of deep sleep were determined in a cross-sectional manner. GH responses to GRF and arginine as well as IGF-II levels were found to be in the normal range. Levels of somatomedin bioactivity were higher than normal before a bone age of 10 years, in the low-normal range thereafter, and below normal in some patients. Levels of Sm-C/IGF-I were found normal before and low-normal after a bone age of ten years. There was a trend towards increasing Sm-C/IGF-I levels with age. In contrast to the normal pattern, spontaneous sleep-related GH secretion was declining with age and did not show the puberty-associated rise. These findings suggest a normally functioning growth hormone-somatomedin axis in Turner's syndrome with alterations of its functioning level occurring secondarily as a result of absent gonadal activation. In single patients abnormally low growth hormone and/or somatomedin secretion may be present.

Statural development parallels IGFI levels in subjects of constitutionally variant stature

1987 ◽  
Vol 114 (4) ◽  
pp. 524-530 ◽  
Author(s):  
Michel Binoux ◽  
Micheline Gourmelen
Keyword(s):  
Growth Rate ◽  
Children And Adolescents ◽  
Normal Growth ◽  
Bone Age ◽  
Serum Levels ◽  
Normal Children ◽  
Gh Secretion ◽  
Stage Of Development ◽  
Igf I ◽  
The Individual

Abstract. Using a protein-binding assay which measures mainly IGF I, serum levels of insulin-like growth factor (IGF) were determined in 263 children and adolescents of constitutionally variant stature but with normal GH secretion following provocative stimuli. A positive correlation was found between height age and the logarithm of IGF levels (r = 0.67, P < 0.001). Furthermore, a correlation was found in the tall and short subjects as a group between the ratio of IGF/normal IGF for age and between the ratio of growth rate/normal growth rate for age (r = 0.53, P < 0.001). Subjects were compared at the same stage of development. At a given bone age or stage of puberty, tall subjects had significantly higher IGF levels than short subjects (P < 0.005). After the completion of growth, IGF levels in both short and tall subjects stabilized within the normal range. Nevertheless, their mean levels remained significantly different (P < 0.001). Our results suggest that in normal children and adolescents, differences in IGF I secretion may be at least partially responsible for the individual differences in growth.

Growth hormone secretion in patients with Turner's syndrome as determined by time series analysis

1992 ◽  
Vol 127 (1) ◽  
pp. 7-12 ◽  
Author(s):  
Jan M Wit ◽  
Albert A Massarano ◽  
Gerdine A Kamp ◽  
Peter C Hindmarsh ◽  
An van Es ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Ethinyl Estradiol ◽  
Height Velocity ◽  
Breast Development ◽  
Turner's Syndrome ◽  
Turner’S Syndrome ◽  
Estradiol Treatment ◽  
Gh Secretion ◽  
Short Children ◽  
Significant Difference

Twenty-four-hour growth hormone (GH) profiles in 26 girls with Turner's syndrome were compared with those of 26 normally growing short children and 24 slowly growing short children. All children were prepubertal and below 12 years of age. A subgroup of 13 girls was treated with ethinyl estradiol and a 24-h GH profile was reassessed. In an additional group of 45 girls with Turner's syndrome (aged 6.7–18.9 years) the effect of age, spontaneous breast development and ethinyl estradiol treatment was studied. The profiles were assessed by Fourier analysis. The oscillatory activity and the mean 24-h GH concentration were similar in children with Turner's syndrome and the normally growing short children, in contrast to lower levels in the slowly growing short children. The periodicity of GH secretion was similar in all groups. In the longitudinal study, ethinyl estradiol treatment resulted in a significant increase in pulse amplitude, but not in periodicity. In the cross-sectional study there was no significant difference between the subgroups of girls with either presence or absence of breast development or ethinyl estradiol treatment. GH secretion was not significantly related to age, height in standard deviation score or height velocity. These data imply that there is no abnormality in GH secretion in girls with Turner's syndrome.

Measurement of nocturnal urinary growth hormone values

1989 ◽  
Vol 121 (2) ◽  
pp. 290-296 ◽  
Author(s):  
Izumi Sukegawa ◽  
Naomi Hizuka ◽  
Kazue Takano ◽  
Kumiko Asakawa ◽  
Reiko Horikawa ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Gh Deficiency ◽  
Turner's Syndrome ◽  
Turner’S Syndrome ◽  
Gh Secretion ◽  
Short Children ◽  
The Mean ◽  
Plasma Gh ◽  
Normal Adults ◽  
Collection Time

Abstract. Nocturnal urinary growth hormone values were measured by a sensitive enzyme immunoassay in normal adults, patients with GH deficiency, patients with Turner's syndrome, normal but short children who had normal plasma GH responses to provocative tests, and patients with acromegaly. The mean nocturnal urinary GH values in patients with acromegaly were significantly greater than those in normal adults (1582.3 ± 579.8 vs 53.5 ± 8.6 pmol/mmol creatinine (± sem); p < 0.05). In the normal but short children and patients with Turner's syndrome, the mean nocturnal urinary GH values were 83.1 ± 5.2 and 79.8 ± 29.5 pmol/mmol creatinine, respectively. In patients with GH deficiency, the nocturnal urinary GH values were undetectable (< 5.3 pmol/mmol creatinine) except in one patient where the value was 6.3 pmol/mmol creatinine. The nocturnal urinary GH values of the patients with GH deficiency were significantly lower than those of the other groups (p < 0.05). In normal but short children, the nocturnal urinary GH values correlated significantly with mean plasma nocturnal GH concentrations (r = 0.76, p < 0.001), and 24-hour urinary GH values (r = 0.84, p < 0.001), respectively. In 4 patients with GH deficiency who had circulating anti-hGH antibody, the urinary GH values were also undectable. These data indicate that nocturnal urinary GH value reflects endogenous GH secretion during collection time, and that measurement of the nocturnal urinary GH values is a useful method for screening of patients with GH deficiency and acromegaly.

Influence of estrogen administration on growth hormone response to GHRH and L-Dopa in patients with Turner's syndrome

1989 ◽  
Vol 120 (4) ◽  
pp. 442-446 ◽  
Author(s):  
E. Schober ◽  
H Frisch ◽  
F. Waldhauser ◽  
Ch. Bieglmayr
Keyword(s):  
Growth Hormone ◽  
Priming Effect ◽  
Ovarian Failure ◽  
Estrogen Deficiency ◽  
Growth Hormone Response ◽  
Turner's Syndrome ◽  
Hormone Response ◽  
Turner’S Syndrome ◽  
Gh Secretion ◽  
Estrogen Administration

Abstract. The modulating effect of estrogen on GH secretion was studied in 22 patients with Turner's syndrome. Estrogen administration (0.5 μg/kg ethinylestradiol) for a period of 4 weeks resulted in a significant increase in basal GH concentrations (2.6 vs 4.8 μg/l, P< 0.01). The L-Dopa-stimulated GH concentrations were also significantly increased (P< 0.01), whereas no effect of estrogen substitution on GH responses to GHRH (1–44) and Sm-C levels was seen. Our findings demonstrate a priming effect of estrogen on GH secretion in patients with Turner's syndrome. These patients generally lack the puberty-associated rise in GH secretion, which might be due to ovarian failure and the concomitant estrogen deficiency.

Serum α-klotho levels are not informative for the evaluation of growth hormone secretion in short children

2017 ◽  
Vol 30 (10) ◽  
Author(s):  
Cristina Meazza ◽  
Heba H. Elsedfy ◽  
Randa I. Khalaf ◽  
Fiorenzo Lupi ◽  
Sara Pagani ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Transmembrane Protein ◽  
Hormone Secretion ◽  
Growth Hormone Secretion ◽  
Serum Levels ◽  
Tolerance Test ◽  
Gh Secretion ◽  
Short Children ◽  
Egyptian Children ◽  
Igf I

AbstractBackground:α-Klotho is a transmembrane protein that can be cleaved and act as a circulating hormone (s-klotho). s-Klotho serum levels seem to reflect growth hormone (GH) secretory status. We investigated the role of s-klotho as a reliable marker of GH secretion in short children and the factors influencing its secretion.Methods:We enrolled 40 short Egyptian children (20 GH deficiency [GHD] and 20 idiopathic short stature [ISS]). They underwent a pegvisomant-primed insulin tolerance test (ITT) and were accordingly reclassified as 16 GHD and 24 ISS. The samples obtained before and 3 days after pegvisomant administration, prior to the ITT, were used for assaying insulin-like growth factor (IGF)-I and s-klotho.Results:IGF-I and s-klotho serum levels were not significantly different (p=0.059 and p=0.212, respectively) between GHD and ISS. After pegvisomant, a significant reduction in IGF-I and s-klotho levels was found in both groups. s-Klotho significantly correlated only with IGF-I levels in both groups.Conclusions:s-Klotho mainly reflects the IGF-I status and cannot be considered a reliable biomarker for GH secretion in children.

Evaluation of acromegaly by measurement of 24-hourly urinary growth hormone excretion

1993 ◽  
Vol 128 (1) ◽  
pp. 9-14 ◽  
Author(s):  
Maryse Pholséna ◽  
Yves Le Bouc ◽  
Elisabeth Rousseau ◽  
Rémy Christol ◽  
Pascal Birman ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Clinical Signs ◽  
Normal Range ◽  
Gh Secretion ◽  
Igf I ◽  
Clear Differentiation ◽  
Plasma Gh ◽  
Hormone Excretion ◽  
Normal Adults ◽  
Accurate Indicator

Twenty-four hourly urinary growth hormone excretion (24-h uGH) has been quantified using a combination of ultrafiltration and conventional immunoradiometric assay. Twenty-four hourly uGH was measured in 20 normal adults and in 42 patients with acromegaly (9 untreated, 28 treated but with above-normal IGF-I levels, and 5 treated and cured). The means and ranges were as follows: 3.7 (1–9) ng/24 h for normals and 160(40–540), 66(2–380) and 5.2 (4–8) ng/24 h for the three groups of acromegalic patients, respectively. Ten patients with pituitary adenomas without acromegaly had 24-h uGH within the normal range. Twenty-four hourly uGH therefore gives a clear differentiation between controls and untreated patients. Log-transformed values for subjects with acromegaly showed significant correlations between 24-h uGH and levels of IGF-I (r=0.63, p<0.01), fasting plasma GH (r=0.92, p<0.001) and plasma GH after glucose loading (r=0.85, p<0.001). Twenty-four hourly uGH was also determined in three acromegalic patients before and during SMS 201–995 therapy. Twenty-four hourly uGH reflected the corresponding changes in mean levels for hourly sampling over 12 h of plasma GH and IGF-I and in clinical signs after 3–6 months of therapy. The results of this study indicate that 24-h uGH is an accurate indicator of GH secretion in acromegalic patients and could therefore be used both in diagnosis and in monitoring the progress of therapy in these patients.

Growth and development in girls with Turner's syndrome during early therapy with low doses of estradiol

1986 ◽  
Vol 113 (4_Suppl) ◽  
pp. S157-S163 ◽  
Author(s):  
K.W. KASTRUP ◽  
_ _
Keyword(s):  
Growth Velocity ◽  
Final Height ◽  
Bone Age ◽  
Growth Spurt ◽  
First Year ◽  
Turner's Syndrome ◽  
Turner’S Syndrome ◽  
Early Therapy ◽  
Group I ◽  
Group Ii

Abstract Early therapy with a low dose of estrogen (estradiol-17β) was given to 33 girls with Turner's syndrome (T.s.) for a period of 4 years. The dose (0.25-2 mg/day) was adjusted every 3 months to maintain plasma estradiol in the normal concentration range for bone age. Growth velocity was compared with that of untreated girls with T.s. All girls were above age 10 years. Bone age was below 10 years in 11 girls (group I) and above 10 years in 22 girls (group II). Growth velocity in the first year of treatment in group I 7.5 ± 1.3 cm (SD) with mean SD score (SDS) of +4.3 and in group II 4.9 ± 1.3 with mean SDS of +3.5. Growth velocity decreased in the following years to 1.6 ± 1.0 cm, SDS -1.44 in group I and 0.9 ± 0.6cm, SDS -2.34 in group II during the fourth year. Withdrawal bleeding occurred in 16 girls of group II after the mean of 23 (range 15-33) months and in 3 girls of group I after 15 to 51 months of treatment. The treatment did not cause an inappropriate acceleration of pubertal development. Breast development appeared in most girls by 3 months of treatment. Pubic hair appeared by 12 months of treatment in group I; it was present in most girls in group II at start of treatment. Final height is known for 12 girls of group II; it was 144.2 ± 4.5 cm. The final height as predicted at the start of therapy was 142.2 ± 5.3 cm. Bone age advanced in the first year of treatment by 2 years. Early treatment with small doses of estrogens induces a growth spurt and normalizes the events of puberty. This will presumably decrease the psychological risks associated with abnormally delayed development.

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.

Efficacy of long-term growth hormone therapy in short non-growth hormone-deficient children

2017 ◽  
Vol 30 (2) ◽  
Author(s):  
Lucia Schena ◽  
Cristina Meazza ◽  
Sara Pagani ◽  
Valeria Paganelli ◽  
Elena Bozzola ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Final Height ◽  
Standard Deviation Score ◽  
Bone Age ◽  
Gh Treatment ◽  
Short Children ◽  
Igf I ◽  
Height Gain ◽  
The Cost

AbstractBackground:In recent years, several studies have been published showing different responses to growth hormone (GH) treatment in idiopathic short stature children. The aim of the present study was to investigate whether non-growth-hormone-deficient (non-GHD) short children could benefit from long-term GH treatment as GHD patients.Methods:We enrolled 22 prepubertal children and 22 age- and sex-matched GHD patients, with comparable height, body mass index (BMI), bone age, and insulin-like growth factor 1 (IGF-I) circulating levels. The patients were treated with recombinant human GH (rhGH) and followed until they reach adult height.Results:During GH treatment, the two groups grew in parallel, reaching the same final height-standard deviation score (SDS) and the same height gain. On the contrary, we found significantly lower IGF-I serum concentrations in non-GHD patients than in GHD ones, at the end of therapy (p=0.0055).Conclusions:In our study, the response to GH treatment in short non-GHD patients proved to be similar to that in GHD ones. However, a careful selection of short non-GHD children to be treated with GH would better justify the cost of long-term GH therapy.

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