scholarly journals Inclusion and Withdrawal Criteria for Growth Hormone (GH) Therapy in Children with Idiopathic GH Deficiency—Towards Following the Evidence but Still with Unresolved Problems

Author(s):  
Joanna Smyczyńska

According to current guidelines, growth hormone (GH) therapy is strongly recommended in children and adolescents with GH deficiency (GHD) in order to accelerate growth rate and attain normal adult height. The diagnosis of GHD requires demonstration of decreased GH secretion in stimulation tests, below the established threshold value. Currently, GHD in children is classified as secondary insulin-like growth factor-1 (IGF-1) deficiency. Most of children diagnosed with isolated GHD presents with normal GH secretion at the attainment of near-final height or even in mid-puberty. The most important clinical problems, related to the diagnosis of isolated GHD in children and to optimal duration of rhGH therapy include: arbitrary definition of subnormal GH peak in stimulation tests, disregarding factors influencing GH secretion, insufficient diagnostic accuracy and poor reproducibility of GH stimulation tests, discrepancies between spontaneous and stimulated GH secretion, clinical entity of neurosecretory dysfunction, discrepancies between IGF-1 concentrations and results of GH stimulation tests, significance of IGF-1 deficiency for the diagnosis of GHD, a need for validation IGF-1 reference ranges. Many of these issues have remained unresolved for 25 years or even longer. It seems that finding solutions to them should optimize diagnostics and therapy of children with short stature.

2020 ◽  
Vol 9 (1) ◽  
pp. 240
Author(s):  
Joanna Smyczyńska ◽  
Urszula Smyczyńska ◽  
Maciej Hilczer ◽  
Renata Stawerska ◽  
Andrzej Lewiński

Primary insulin-like growth factor-I (IGF-I) deficiency is a synonym of growth hormone (GH) insensitivity (GHI), however the necessity of direct confirmation of GH resistance by IGF-I generation test (IGF-GT) is discussed. GHI may disturb intrauterine growth, nevertheless short children born small for gestational age (SGA) are treated with GH. We tested the hypothesis that children with appropriate birth size (AGA), height standard deviation score (SDS) <−3.0, GH peak in stimulation tests (stimGH) ≥10.0 µg/L, IGF-I <2.5 centile, and excluded GHI may benefit during GH therapy. The analysis comprised 21 AGA children compared with 6 SGA and 20 GH-deficient ones, with height SDS and IGF-I as in the studied group. All patients were treated with GH up to final height (FH). Height velocity, IGF-I, and IGF binding protein-3 (IGFBP-3) concentrations before and during first year of treatment were assessed. Effectiveness of therapy was better in GHD than in IGF-I deficiency (IGFD), with no significant difference between SGA and AGA groups. All but two AGA children responded well to GH. Pretreatment IGF-I and increase of height velocity (HV) during therapy but not the result of IGF-GT correlated with FH. As most AGA children with apparent severe IGFD benefit during GH therapy, direct confirmation of GHI seems necessary to diagnose true primary IGFD in them.


2003 ◽  
Vol 59 (1) ◽  
pp. 7-15 ◽  
Author(s):  
M. Thomas ◽  
G. Massa ◽  
M. Maes ◽  
D. Beckers ◽  
M. Craen ◽  
...  

2004 ◽  
pp. S61-S65 ◽  
Author(s):  
MO Savage ◽  
WM Drake ◽  
PV Carroll ◽  
JP Monson

While the benefits of growth hormone (GH) therapy in adult hypopituitary patients with GH deficiency (GHD) are established, the role of continued GH therapy after final height in adolescent GH-deficient patients remains unclear. Preliminary data suggest that cessation of GH on completion of linear growth may be associated with impairment of somatic development and adverse changes in body composition. For the present time, the decision whether to continue GH treatment in adolescent patients with GHD is best made on an individual basis. For such patients, continuity of care is crucial. Children and adults with GHD are usually managed by physicians in separate departments, who may focus on different aspects of treatment and care. Close collaboration between paediatric and adult physicians is essential to ensure smooth transition and to minimize the drop-out rate from follow-up. Given the previous period of treatment during childhood, paediatric physicians should be best placed to discuss the potential benefits of continuing GH therapy and instigate retesting of GH secretion. Many children with isolated idiopathic GHD will produce normal GH responses if retested at adult height. Patients with multiple pituitary hormone deficits are more likely to have ongoing GHD, as are patients who have received CNS irradiation. Quality of life does not appear to be decreased in adolescents with GHD who stop treatment, so achievement of satisfactory bone mass is a major determinant of the decision whether to continue therapy.


1991 ◽  
Vol 12 (12) ◽  
pp. 355-363
Author(s):  
Paul Saenger

Growth hormone (GH) was isolated approximately 30 years ago. Since then, we have witnessed major advances in understanding the regulation and pattern of GH secretion and its many metabolic actions.1 With the advent of recombinant DNA-generated growth hormone (rhGH) in 1985, the opportunities to treat children who have organic or idiopathic GH deficiencies are now, for the first time, unlimited. DEFINITION OF GROWTH HORMONE DEFICIENCY Although a role for GH in the treatment of certain children deficient in GH is established, the definition of GH deficiency remains controversial. The controversy surrounding the diagnosis of GH deficiency was highlighted recently when it was shown that, among the many commercial assay kits available, the same serum sample yielded GH concentrations that differed by as much as 100% for the same sample. Using different GH assay kits, a child who has short stature could be determined to be deficient in GH in the view of one endocrinologist but to have sufficient GH (ie, to be normal) by another endocrinologist.2,3 Despite these obvious limitations, GH testing has an important role in the clinical evaluation of the child who is short. When a child fails a simple screening test (ie, serum GH level of &lt;10 ng/mL following vigorous exercise, such as running for 20 minutes), more definitive GH testing is the next step.


2008 ◽  
Vol 21 (6) ◽  
pp. 569-580
Author(s):  
Monika Obara-Moszynska ◽  
Andrzej Kctdzia ◽  
Eugeniusz Korman ◽  
Marek Niedziela

Abstract The diagnosis of growth hormone (GH) deficiency (GHD) is still problematic for the clinician. There is no gold standard for estimating GH secretion. The aim of this study was to compare the diagnostic usefulness of spontaneous GH secretion test, pharmacological tests with insulin, clonidine, L-dopa, and glucagon, and IGF-1 measurement in GHD. We studied 180 prepubertal, short children. Predictive values were calculated for different GH cutoff levels for each diagnostic test. ROC curves were used to estimate the diagnostic usefulness of the tests. The results show that sleep is the strongest stimulatory agent for GH secretion. The estimation of GH secretion after onset of sleep can be used as a screening test in GHD diagnosis. The insulin test has the highest discrimination. A combination of insulin test with another provocative test allows high discrimination and accuracy for standard cut-off GH level. Measurement of IGF -I is characterized by low predictive values. IGF-1 level below the mean according to age indicates high probability of GHD. Auxological parameters should be the most important factor in diagnosing GHD.


2005 ◽  
Vol 15 (2) ◽  
pp. 156-164 ◽  
Author(s):  
A. Minczykowski ◽  
M. Gryczynska ◽  
K. Ziemnicka ◽  
R. Czepczynski ◽  
J. Sowinski ◽  
...  

2008 ◽  
Vol 93 (3) ◽  
pp. 974-980 ◽  
Author(s):  
Barbara Räz ◽  
Marco Janner ◽  
Vibor Petkovic ◽  
Didier Lochmatter ◽  
Andrée Eblé ◽  
...  

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