Transient impairment or delay of urinary trihydroxypregnanone (THS) response to metyrapone in boys with delayed adolescence and in patients with isolated growth hormone deficiency

1982 ◽  
Vol 99 (2) ◽  
pp. 166-173 ◽  
Author(s):  
M. Zachmann ◽  
D. Tassinari ◽  
W. Sorgo ◽  
G. U. Exner ◽  
B. Kempken ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Single Dose ◽  
Growth Hormone Deficiency ◽  
Bone Age ◽  
Urine Samples ◽  
Hormone Deficiency ◽  
Cortisol Response ◽  
Transient Impairment ◽  
Group A ◽  
Group B

Abstract. Twentythree boys with delayed adolescence (age 15.7 ± 2.0, bone age 12.4 ± 2.1 years) were studied. Their cortisol response to insulin was normal. After oral metyrapone (500 mg/m2 by mouth) one to three consecutive 12 h urine samples were collected for analysis of THS. Thirtyseven tests with 37 first, 21 second, and 11 third samples were carried out. The results could be divided into two main groups: 25 tests (group A) were subnormal in the first sample, 12 of them with a very weak (40 ± 8 μg/m2/12 h) and 13 with an insufficient (191 ± 16 μg/m2/12 h) THS response. Values in the second and third sample were higher, indicating a dealyed response. In 12 tests (group B), the results were normal (1016 ± 143 μg/m2/12 h) in the first and lower in the second and third samples. In three patients with repeated tests, there was improvement with increasing bone age. The THS-responses to metyrapone did not correlate with those of growth hormone, gonadotrophins, and TSH to stimuli. It is concluded that the THS-response to a single dose of metyrapone may be temporarily insufficient or delayed in delayed adolescence. We interpret this finding as showing transiently reduced or slow hypothalamic responsiveness.

scholarly journals Influence of biochemical diagnosis of growth hormone deficiency on replacement therapy response and retesting results at adult height

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Giulia Rodari ◽  
E. Profka ◽  
F. Giacchetti ◽  
I. Cavenaghi ◽  
M. Arosio ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Growth Hormone Deficiency ◽  
Adult Height ◽  
Initial Diagnosis ◽  
Stimulation Test ◽  
Hormone Deficiency ◽  
Group A ◽  
Group B ◽  
Term Response

AbstractIsolated growth hormone deficiency (IGHD) is the most frequent endocrinological disorder in children with short stature, however the diagnosis is still controversial due to the scarcity of reliable diagnostic criteria and pre-treatment predictive factors of long term-response. To evaluate recombinant growth hormone (rGH) long-term response and retesting results in three different groups of children divided in accordance with the biochemical criteria of initial diagnosis. Height gain (∆HT) at adult height (AH) and retesting results were evaluated in 57 rGH treated children (M = 34, 59.6%) divided into 3 groups according to initial diagnosis: Group A (n = 25) with max GH peak at stimulation test < 8 µg/L, Group B (n = 19) between 8 and 10 µg/L and Group C (n = 13) with mean overnight GH < 3 µg/L (neurosecretory dysfunction, NSD). Retesting was carried out in all patients after at least one month off therapy upon reaching the AH. 40/57 (70.2%) patients were pre-pubertal at diagnosis and showed ∆HT of 1.37 ± 1.00 SDS, with no significant differences between groups (P = 0.08). Nonetheless, 46% patients in Group B showed ∆HT < 1SDS (vs 13% and 12% in Group A and C, respectively) and 25% children failed to reach mid-parental height (vs 6% and 0% in Group A and C, respectively). At AH attainment, IGHD was reconfirmed in 28% (7/25) and 10% (2/19) in Group A and B, respectively. A reduction of diagnostic cut-off at GH stimulation tests could better discriminate between “good” and “poor responders” and predict the persistence of IGHD through transition. Group C response and the predictive value of baseline IGF-I SDS bring back to light NSD: should we consider an underlying hypothalamic derangement when the clinical presentation is strongly consistent with IGHD but pharmacological stimulation test is normal?

scholarly journals Síndrome de Klinefelter con deficiencia parcial de hormona de crecimiento.

2015 ◽  
Vol 10 (1) ◽  
pp. 38
Author(s):  
Carlos TORI TORI ◽  
Carlos ROE B.
Keyword(s):  
Growth Hormone ◽  
Growth Hormone Deficiency ◽  
Short Stature ◽  
Klinefelter Syndrome ◽  
Bone Age ◽  
Hormone Deficiency ◽  
Klinefelter’S Syndrome ◽  
Klinefelter's Syndrome ◽  
The Third ◽  
Rare Association

We present a case of Klinefelter’s syndrome and short stature due to partial growth hormone deficiency. His height was below the third percentile for age and his bone age lagged behind four years. Cases like this are generally due to the presence of a an isochromosome Xq or to an isolated partial or total deficiency of growth hormone, or to partial or panhypopituitarism. We wish de emphasize the rare association between Klinefelter syndrome and growth hormone deficiency.

Current Dosing of Growth Hormone in Children With Growth Hormone Deficiency: How Physiologic?

PEDIATRICS ◽  
1998 ◽  
Vol 102 (Supplement_3) ◽  
pp. 527-530
Author(s):  
Margaret H. MacGillivray ◽  
Sandra L. Blethen ◽  
John G. Buchlis ◽  
Richard R. Clopper ◽  
David E. Sandberg ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Adverse Events ◽  
Growth Hormone Deficiency ◽  
Growth Velocity ◽  
Bone Age ◽  
Early Initiation ◽  
Hormone Deficiency ◽  
Treatment Result ◽  
Recombinant Growth Hormone ◽  
Pituitary Growth Hormone

The current doses of recombinant growth hormone (rGH) are two to three times those used in the pituitary growth hormone era. These rGH doses (0.025 to 0.043 mg/kg/d) are similar to or moderately greater than the physiologic requirements. Growth velocity and height gains have been shown to be greater with 0.05 mg/kg/d of rGH than with 0.025 mg/kg/d. Larger doses of GH and early initiation of treatment result in greater heights at the onset of puberty and greater adult heights. Earlier onset of puberty and more rapid maturation, as indicated by bone age, were not observed in children who were given 0.18 to 0.3 mg/kg/wk of rGH. The frequency of adverse events is very low, but diligent surveillance of all children who are treated with rGH is essential.

Final height of patients with idiopathic growth hormone deficiency after long-term growth hormone treatment

1989 ◽  
Vol 120 (4) ◽  
pp. 409-415 ◽  
Author(s):  
Itsuro Hibi ◽  
Toshiaki Tanaka ◽  
_ _
Keyword(s):  
Growth Hormone ◽  
Standard Deviation ◽  
Growth Hormone Deficiency ◽  
Final Height ◽  
Standard Deviation Score ◽  
Hormone Deficiency ◽  
Chronological Age ◽  
Deviation Score ◽  
Idiopathic Growth Hormone Deficiency ◽  
Group A

Abstract. One hundred and 8 patients with idiopathic growth hormone deficiency with spontaneous pubertal maturation (group A), were followed until they reached their final height after hGH treatment lasting between 2 and 11.1 (average 5.5) years. Their standard deviation scores of final height averaged 3.3 sd below the population mean, which was significantly lower than 1.1 standard deviation below the mean in 29 GH deficient patients without spontaneous puberty (group B) who were treated with hGH and sex hormones. The presence of gonadal function was found to be unbeneficial for final height. The final height of the patients in both groups was not related to either 1) age at the start of hGH treatment, 2) age at the onset of puberty, or 3) the duration of hGH treatment. It was, however, significantly related to the standard deviation score of height at the start of hGH treatment in both groups and to that of height at the onset of puberty in group A. The findings demonstrated that earlier introduction of hGH treatment resulted in earlier onset of puberty in group A, because the chronological age at the onset of puberty was positively correlated to the chronological age at the start of hGH treatment. It was also confirmed that the later puberty began, the taller the final height in GH deficient patients who had the same degree of standard deviation score of height for chronological age before puberty.

The effects of treatment combining an agonist of gonadotropin-releasing hormone with growth hormone in pubertal patients with isolated growth hormone deficiency

1989 ◽  
Vol 120 (6) ◽  
pp. 795-799 ◽  
Author(s):  
Jean Edmond Toublanc ◽  
Claire Couprie ◽  
Philippe Garnier ◽  
Jean-Claude Job
Keyword(s):  
Growth Hormone ◽  
Growth Hormone Deficiency ◽  
Final Height ◽  
Bone Age ◽  
Hormone Deficiency ◽  
Gnrh Analogue ◽  
Isolated Growth Hormone Deficiency ◽  
One Year ◽  
Long Acting

Abstract. The final height of patients treated with growth hormone for isolated growth hormone deficiency has, up to now, been subnormal, with a mean below −2 sd in the series reported, an insufficient height at the onset of puberty and a more or less accelerated bone maturation during puberty being two important factors of the poor results. A long-acting analogue of gonadoliberin, Trp6-GnRH, has been given to GH-treated patients with isolated growth hormone deficiency at the time they reached pubertal stage 2, in combination with unchanged doses of GH, for one year in 11 and for two years in 7 of them. It resulted in an increase in the height age/bone age ratio and a reduction of the height insufficiency for bone age. The increase was slight but significant after one year, and fair after two years, in spite of a reduced annual growth rate. Post-analogue follow-up in 5 patients with continued GH treatment showed a good development of growth and of puberty. It is concluded that combination of the long-acting Trp6-GnRH analogue and GH for 1–2 years in patients with isolated growth hormone deficiency whose puberty starts with a very insufficient height may be an appropriate way to improve their growth parameters. Studies with increased doses of GH or increased frequency of injections could help to optimize the results. Several years of follow-up are needed for demonstrating the results on final height.

scholarly journals Adult height prediction by bone age determination in children with isolated growth hormone deficiency

2020 ◽  
Vol 9 (5) ◽  
pp. 370-378
Author(s):  
Thomas Reinehr ◽  
Martin Carlsson ◽  
Dionisios Chrysis ◽  
Cecilia Camacho-Hübner
Keyword(s):  
Growth Hormone ◽  
Growth Hormone Deficiency ◽  
Adult Height ◽  
Age Determination ◽  
Bone Age ◽  
Hormone Deficiency ◽  
Gh Treatment ◽  
Height Prediction ◽  
The Mean ◽  
Tw2 Method

Background The precision of adult height prediction by bone age determination in children with idiopathic growth hormone deficiency (IGHD) is unknown. Methods The near adult height (NAH) of patients with IGHD in the KIGS database was compared retrospectively to adult height prediction calculated by the Bayley–Pinneau (BP) prediction based on bone age by Greulich–Pyle (GP) in 315 children and based on the Tanner-Whitehouse 2 (TW2) method in 121 children. Multiple linear regression analyses adjusted for age at GH start, age at puberty, mean dose and years of of GH treatment, and maximum GH peak in stimulation test were calculated. Results The mean underestimation of adult height based on the BP method was at baseline 4.1 ± 0.7 cm in girls and 6.1 ± 0.6 cm in boys, at 1 year of GH treatment 2.5 ± 0.5 cm in girls and 0.9 ± 0.4 cm in boys, while at last bone age determination adult height was overestimated in mean by 0.4 ± 0.6 cm in girls and 3.8 ± 0.5 cm in boys. The mean underestimation of adult height based on the TW2 method was at baseline 5.3 ± 2.0 cm in girls and 7.9 ± 0.8 cm in boys, at 1 year of GH treatment adult height was overestimated in girls 0.1 ± 0.6 cm in girls and underestimated 4.1 ± 0.4 cm in boys, while at last bone age determination adult height was overestimated in mean by 3.1 ± 1.5 cm in girls and 3.6 ± 0.8 cm in boys. Conclusions Height prediction by BP and TW2 at onset of GH treatment underestimates adult height in prepubertal IGHD children, while in mean 6 years after onset of GH treatment these prediction methods overestimated adult height.

scholarly journals Isolated Growth Hormone Deficiency and Idiopathic Short Stature: Comparative Efficiency after Growth Hormone Treatment up to Adult Height

2021 ◽  
Vol 10 (21) ◽  
pp. 4988
Author(s):  
Ana-Belen Ariza-Jimenez ◽  
Isabel Leiva Gea ◽  
Maria Jose Martinez-Aedo Ollero ◽  
Juan Pedro Lopez-Siguero
Keyword(s):  
Growth Hormone ◽  
Short Stature ◽  
Hormone Treatment ◽  
Treated Group ◽  
Idiopathic Short Stature ◽  
Hormone Deficiency ◽  
Comparative Efficiency ◽  
Height Gain ◽  
Group A ◽  
Group B

Introduction: Treatment with growth hormone (GH) is not approved for idiopathic short stature (ISS) in Europe. Objectives: To compare the growth of children treated with isolated GH deficiency (IGHD) vs. ISS-treated and untreated children. Methods: A retrospective descriptive study of patients treated in the last 14 years for IGHD (Group A), in comparison with ISS-treated (Group B) and untreated (Group C) subjects. Results: Group A had 67 males, who showed a height gain of 1.24 SD. Group B had 30 boys, who showed a height gain of 1.47 SD. Group C had 42 boys, who showed an improvement of 0.37 SD. The final heights were −1.52 SD, −1.31 SD, and −2.03 SD, respectively. Group A and C did not reach their target heights (with differences of 0.27 SD and 0.59 SD, respectively). Group B surpassed their target height by 0.29 SD. Conclusions: The final heights of the IGHD and treated ISS are similar. Treated groups were taller than untreated groups.

Sign in / Sign up

Export Citation Format

Share Document