scholarly journals Assessing the Impact of Growth Hormone Deficiency and Treatment in Adults: Development of a New Disease-Specific Measure

2014 ◽  
Vol 99 (4) ◽  
pp. 1204-1212 ◽  
Author(s):  
Meryl Brod ◽  
Lise Højbjerre ◽  
Johan Erpur Adalsteinsson ◽  
Michael Højby Rasmussen
Keyword(s):  
Growth Hormone ◽  
Item Response Theory ◽  
Growth Hormone Deficiency ◽  
Item Response ◽  
Outcome Measure ◽  
Gh Deficiency ◽  
Hormone Deficiency ◽  
Concept Elicitation ◽  
Development And Validation ◽  
The Impact

Context: Approximately 50 000 adults in the United States are diagnosed with GH deficiency, which has negative impacts on cognitive functioning, psychological well-being, and quality of life. Objective: This paper presents development and validation of a patient-reported outcome measure (PRO), the Treatment-Related Impact Measure-Adult Growth Hormone Deficiency (TRIM-AGHD). The TRIM-AGHD was developed to measure the impact of GH deficiency and its treatment. Design and Settings: The development and validation of the TRIM-AGHD was conducted according to the Food and Drug Administration guidance on the development of PROs. Concept elicitation, conducted in three countries included interviews with patients, clinical experts, and literature review. Qualitative data were analyzed based on grounded theory principles, and draft items were cognitively debriefed. The measure underwent psychometric validation in a US clinic-based population. An a priori statistical analysis plan included assessment of the measurement model, reliability, and validity. Item functioning was reviewed using item response theory analyses. Patients or Other Participants: Forty-eight patients and six clinical experts participated in concept elicitation and 169 patients completed the validation study. Main Outcome Measure: TRIM-AGHD was measured. Results: Factor analysis resulted in four domains: energy level, physical health, emotional health, and cognitive ability. The item response theory confirmed adequate item fit and placement within their domain. Internal consistency ranged from 0.82 to 0.95 and test-retest ranged from 0.80 to 0.92. All prespecified hypotheses for convergent validity and all but two for discriminant validity were met. Conclusions: The final 26-item TRIM-AGHD can be considered a reliable and valid PRO of the impact of disease and treatment for adult GH deficiency.

GROWTH AND GROWTH HORMONE. IV. LIMITATIONS OF THE GROWTH HORMONE RESPONSE TO INSULIN AND ARGININE AND OF THE IMMUNOREACTIVE INSULIN RESPONSE TO ARGININE IN THE ASSESSMENT OF GROWTH HORMONE DEFICIENCY IN CHILDREN

PEDIATRICS ◽  
1969 ◽  
Vol 43 (6) ◽  
pp. 989-1004
Author(s):  
R. Youlton ◽  
S. L. Kaplan ◽  
M. M. Grumbach
Keyword(s):  
Growth Hormone ◽  
Growth Hormone Deficiency ◽  
Gh Deficiency ◽  
Hormone Deficiency ◽  
Insulin Administration ◽  
Arginine Infusion ◽  
Group 3 ◽  
Group 2 ◽  
Serum Gh ◽  
Group 1

The growth hormone (GH) response to insulin-induced hypoglycemia and to arginine infusion has been evaluated in 60 children with growth retardation. These children have been classified into three groups: Group 1-9 children had peak serum growth hormone values of 7 mµg/ml or greater to both stimuli, a normal growth hormone response. Group 2-18 children had peak GH values of ≤ 3 mµg/ml to both stimuli, an abnormal response indicating growth hormone deficiency. Group 3-6 children had a blunted GH response (> 3 < 7 mµg/ml) to both stimuli; 8 showed a normal rise in serum GH following arginine infusion (> 7 mµg/ml) but exhibited no rise, or a minimal one, following insulin administration; 9 children had minimal increase in serum GH concentration following arginine infusion but showed a normal GH response to insulin administration (> 7mµg/ml). Children included in Group 3 represent a heterogenous population. In some patients with a blunted response to both stimuli, evidence of partial or less severe form of GH deficiency was found, whereas in 17 of 18 children exhibiting a disparate response the impaired growth was not attributable to growth hormone deficiency. The blood glucose at all sampling periods was significantly lower following insulin administration in patients in Group 2 than that observed for children in Group 1 and 3. The blood glucose was significantly lower at 90 and 120 minutes following arginine infusion in Group 2 compared to values for patients in Group 1 and 3. Changes in serum insulin in response to the infusion of arginine did not provide a useful index of discrimination among these groups. Administration of diethylstilbestrol, 10 mg/day times 2 days, prior to testing can modify the GH response to both hypoglycemia and arginine; it is a useful ancillary procedure in children with blunted or disparate responses. These studies suggest that two types of stimulation tests are necessary to establish the diagnosis of isolated GH deficiency with a high degree of probability.

The impact of idiopathic childhood-onset growth hormone deficiency (GHD) on bone mass in subjects without adult GHD

2005 ◽  
Vol 62 (1) ◽  
pp. 18-23 ◽  
Author(s):  
Martin Lange ◽  
Jorn Muller ◽  
Ole Lander Svendsen ◽  
Knud William Kastrup ◽  
Anders Juul ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Bone Mass ◽  
Growth Hormone Deficiency ◽  
Hormone Deficiency ◽  
Childhood Onset ◽  
The Impact

New approach to the diagnosis of growth hormone deficiency in adults

1996 ◽  
Vol 134 (3) ◽  
pp. 352-356 ◽  
Author(s):  
Ezio Ghigo ◽  
Gianluca Aimaretti ◽  
Laura Gianotti ◽  
Jaele Bellone ◽  
Emanuela Arvat ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Growth Hormone Deficiency ◽  
Gh Deficiency ◽  
Serum Insulin ◽  
Normal Subjects ◽  
Hormone Deficiency ◽  
New Approach ◽  
Igf I ◽  
Ghrh Test ◽  
Gh Deficiency In Adults

Ghigo E, Aimaretti G, Gianotti L, Bellone J, Arvat E, Camanni F. New approach to the diagnosis of growth hormone deficiency in adults. Eur J Endocrinol 1996;134:352–6. ISSN 0804–4643 Pyridostigmine (PD), a muscarinic cholinergic agonist, and arginine (ARG) clearly increase the growth hormone (GH) response to growth hormone-releasing hormone (GHRH) in man. The current study was undertaken to investigate the value and safety of PD + GHRH and ARG + GHRH tests as well as the measurement of serum insulin-like growth factor I (IGF-I) in diagnosing GH deficiency in adults. Fifty-four patients considered GH deficient from extensive organic or idiopathic pituitary disease and 326 healthy adults were studied. The IGF-I concentrations were lower than the 3rd percentile of normal values in only 31 of the 54 (57.4%) patients with hypopituitarism. However, the IGF-I levels in hypopituitary patients and in normal subjects overlapped more frequently between 41 and 60 years (50%) and between 61 and 80 years (92.3%) as opposed to between 20 and 40 years (8.6%). In contrast to the IGF-I measurement, the ranges of peak GH responses to PD + GHRH and ARG + GHRH tests were clearly differentiated between the hypopituitary (0.2–6.8 and 0.1–9.5 μg/l, respectively) and normal subjects 17.7–114 and 16.1–119 μg/l, respectively). However, the PD + GHRH test was reliable only in subjects of 20–40 years of age. In conclusion, IGF-I measurement had no value in the diagnosis of GH deficiency in adults aged over 40 years, but is reliable enough when young adults of 20–40 years of age are considered. Both PD + GHRH and ARG + GHRH testing should be considered more reliable biochemical measurements of GH deficiency. In contrast to the PD + GHRH test, the ARG + GHRH test is reliable throughout the adult lifespan and appears to be the most appropriate for patient compliance and safety. F Camanni, Divisione di Endocrinologia, Ospedale Molinette, C.so Dogliotti 14, 10126 Torino, Italy

scholarly journals Assessment of the performance of the Brazilian Portuguese Nottingham Health Profile in adult growth hormone deficiency and pulmonary hypertension

F1000Research ◽  
2021 ◽  
Vol 9 ◽  
pp. 1399
Author(s):  
Alice Heaney ◽  
Rafael W. R. de Oliveira ◽  
Mariana Bizzi ◽  
Ricardo Amorim Correa ◽  
Monica Corso Pereira ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Pulmonary Hypertension ◽  
Growth Hormone Deficiency ◽  
Nottingham Health Profile ◽  
Hormone Deficiency ◽  
Health Profile ◽  
Adult Growth Hormone Deficiency ◽  
Adult Growth ◽  
The Impact ◽  
Disease Specific

Background: The Nottingham Health Profile (NHP) is a generic measure of perceived distress that has been used widely as an outcome measure in clinical practice and trials. The availability of two Brazilian datasets provided the opportunity to assess the psychometric performance of the NHP in different populations - adult growth hormone deficiency (GHD) and pulmonary hypertension (PH). The purpose of the study was to see how valuable the NHP could be in assessing outcomes in diseases where no disease-specific measures are available. Methods: Secondary analyses were performed with NHP data. Patients diagnosed with adult GHD or PH were administered the NHP during clinic visits on two occasions, two weeks apart. A disease-specific measure of quality of life (QoL) was also administered to the relevant sample of patients on each occasion. Results: The psychometric properties of the NHP were good for both disease groups. As expected, both samples reported high scores on energy level, the PH sample scored high on physical functioning and the GHD sample on emotional reactions. For both samples, most of the NHP sections were able to distinguish between groups of respondents with different ratings of perceived general health. While most sections of the NHP were relatively highly correlated with the QoL measures, pain and sleep did not seem to be important predictors of QoL in either of the samples. Conclusions: The use of the NHP in adult GHD and PH populations in Brazil is not recommended as there are high-quality disease-specific measures available for each disease. However, where no disease-specific measures are available, the NHP can provide good descriptive information of the impact of disease on different patient populations.

scholarly journals Growth hormone deficiency in adults. Introduction to the problem

1994 ◽  
Vol 40 (4) ◽  
pp. 65-81
Author(s):  
М. Bengt-Ake Bengtsson
Keyword(s):  
Growth Hormone ◽  
Growth Hormone Deficiency ◽  
Replacement Therapy ◽  
Gh Deficiency ◽  
Hormone Deficiency ◽  
Control Group ◽  
Objective Criterion ◽  
Double Blind ◽  
Gh Replacement ◽  
Severe Degree

Growth hormone deficiency (GH) for a long time was recognized only in childhood. Compelling evidence has been obtained showing that HR replacement therapy effectively stimulates growth and, in many cases, achieves normal end-points of physical development. More recently, it was shown that the most effective in this regard was the appointment of regular evening injections of the drug, which mimic the physiological secretion of GH during sleep. Despite the fact that the acceleration of linear growth is the most objective criterion for the effectiveness of therapy for GH, it is known that GH has a significant effect on body structure, causing a decrease in fat mass and an increase in muscle mass. Until recently, GH was not considered an important hormonal regulator in adults, and therefore, there was no study of GH deficiency and treatment of children with GH deficiency when they reached adulthood, as well as patients with hypopituitarism who became ill in adulthood. However, in 1989, as a result of two double-blind trials using placebo in the control group, the effectiveness of GH replacement therapy in adults with an abnormally low level of GH, up to a severe degree of GH deficiency, was revealed. Further studies showed the presence of violations of both physical and mental status in adults in whom GH deficiency develops as a result of the tumor process in the pituitary gland or its therapy. Most of these disorders, but not all, can be corrected as a result of GH replacement therapy, which confirms the significant effect of GH throughout life.

scholarly journals Partial Loss of Function of the GHRH Receptor Leads to Mild Growth Hormone Deficiency

2016 ◽  
Vol 101 (10) ◽  
pp. 3608-3615 ◽  
Author(s):  
Louise Cheryl Gregory ◽  
Kyriaki Sandy Alatzoglou ◽  
Mark James McCabe ◽  
Peter Christopher Hindmarsh ◽  
Jose William Saldanha ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Growth Hormone Deficiency ◽  
Gh Deficiency ◽  
Final Height ◽  
Fold Increase ◽  
Hormone Deficiency ◽  
Loss Of Function ◽  
Partial Loss ◽  
Mild Phenotype ◽  
Partial Activity

Objective: Recessive mutations in GHRHR are associated with severe isolated growth hormone deficiency (IGHD), with a final height in untreated patients of 130 cm ± 10 cm (−7.2 ± 1.6 SDS; males) and 114 ± 0.7 cm (−8.3 ± 0.1 SDS; females). Design: We hypothesized that a consanguineous Pakistani family with IGHD in three siblings (two males, one female) would have mutations in GH1 or GHRHR. Results: Two novel homozygous missense variants [c.11G>A (p.R4Q), c.236C>T (p.P79L)] at conserved residues were identified in all three siblings. Both were absent from control databases, aside from pR4Q appearing once in heterozygous form in the Exome Aggregation Consortium Browser. The brothers were diagnosed with GH deficiency at 9.8 and 6.0 years (height SDS: −2.24 and −1.23, respectively), with a peak GH of 2.9 μg/liter with low IGF-1/IGF binding protein 3. Their sister presented at 16 years with classic GH deficiency (peak GH <0.1 μg/liter, IGF-1 <3.3 mmol/liter) and attained an untreated near-adult height of 144 cm (−3.0 SDS); the tallest untreated patient with GHRHR mutations reported. An unrelated Pakistani female IGHD patient was also compound homozygous. All patients had a small anterior pituitary on magnetic resonance imaging. Functional analysis revealed a 50% reduction in maximal cAMP response to stimulation with GHRH by the p.R4Q/p.P79L double mutant receptor, with a 100-fold increase in EC50. Conclusion: We report the first coexistence of two novel compound homozygous GHRHR variants in two unrelated pedigrees associated with a partial loss of function. Surprisingly, the patients have a relatively mild IGHD phenotype. Analysis revealed that the pP79L mutation is associated with the compromise in function, with the residual partial activity explaining the mild phenotype.

Quadriceps and hand-grip strength in adults with childhood-onset growth hormone deficiency

1995 ◽  
Vol 132 (1) ◽  
pp. 37-41 ◽  
Author(s):  
Alessandro Sartorio ◽  
Marco Narici ◽  
Antonio Conti ◽  
Marco Monzani ◽  
Giovanni Faglia
Keyword(s):  
Growth Hormone ◽  
Growth Hormone Deficiency ◽  
Grip Strength ◽  
Gh Deficiency ◽  
Vertical Jump ◽  
Body Height ◽  
Hormone Deficiency ◽  
Childhood Onset ◽  
Hand Grip ◽  
Muscle Groups

Sartorio A, Narici M, Conti A, Monzani M, Faglia G. Quadriceps and hand-grip strength in adults with childhood-onset growth hormone deficiency. Eur J Endocrinol 1995;132:37–41. ISSN 0804–4643 The effects of chronic growth hormone (GH) deficiency on muscle size and strength of postural (quadriceps) and non-postural (hand-grip) muscle groups, as well as on vertical jump capacity, were evaluated in six adults with childhood-onset GH deficiency. Data obtained were compared to those recorded in an age-, sex- and exercise-matched healthy control group. Thigh muscle plus bone cross-sectional area (CSAM+B) of the dominant quadriceps was significantly lower (p < 0.001) than in controls, while the CSAM+B/Body height)2 ratio was similar to that of controls. The maximum voluntary contraction (MVC) of the quadriceps of patients was significantly lower (p < 0.002) than in controls, while no differences existed in the quadriceps force expressed per unit area (MVC/CSA) between patients and controls. As far as hand-grip was concerned, the CSAM+B of the dominant forearm was significantly lower (p < 0.003) than in controls, while the CSAM+B/Body height)2 ratio was no different. The hand-grip MVC of patients was significantly lower (p < 0.004) than in controls, while no differences existed in the MVC/CSA ratio. It is noteworthy also that no difference existed in the hand-grip to quadriceps MVC ratio of the two groups. Furthermore, no differences were found in the vertical jump capacity, because both Δ Height and Δ Height/Body weight of patients were not significantly different from those of controls. In conclusion, our study suggests that GH deficiency seems to reduce the size and strength of postural and non-postural muscle groups to the same extent. However, these findings are likely to be attributed to a simple dimensional scaling, because their CSA/ (Body height)2, MVC/CSA and vertical jump capacity were comparable to those of controls. Alessandro Sartorio, Laboratorio Sperimentale di Ricerche Endocrinologiche, Centro Auxologico Italiano, IRCCS, via Ariosto 13, 1-20145 Milan, Italy

scholarly journals Impact of IGF-1 Normative Datasets on Indication and Outcome of Growth Hormone Stimulation Testing

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A678-A679
Author(s):  
Prim de Bie ◽  
Annemieke C Heijboer ◽  
Martine M L Deckers
Keyword(s):  
Growth Hormone ◽  
Growth Hormone Deficiency ◽  
Stimulation Test ◽  
Hormone Deficiency ◽  
Growth Disorders ◽  
Z Score ◽  
Hormone Stimulation ◽  
Stimulation Tests ◽  
Z Scores ◽  
The Impact

Abstract In the Netherlands, the diagnosis of growth hormone deficiency in children follows the Dutch national guidelines for Triage and Diagnosis of Growth Disorders in Children. Initial biochemical evaluation includes an IGF-1 measurement as screening parameter for growth hormone deficiency. Based on the clinical probability of growth hormone deficiency and the IGF-1 Z-score, a growth hormone stimulation test is performed if serum IGF-1 Z-score is &lt; 0 SD in case of a high probability and if serum IGF-1 Z-score is &lt; -1 SD in case of low probability. An IGF-1 Z-score &gt; 0 SD virtually excludes a growth hormone deficiency disorder. The interpretation of growth hormone stimulation testing is dependent on both the peak growth hormone concentration, but also on the baseline IGF-1 Z-score, particularly in cases of partial deficiency. Although, nation wide, Dutch laboratories have harmonized their measurement for IGF-1 (as was previously done for growth hormone), a Dutch harmonized normative data set has not been widely adopted. Moreover a clinical evaluation of the implementation of this dataset based on dynamic testing has not been published. To assess the impact of choice of a particular normative dataset on the diagnosis of growth hormone deficiency we recalculated Z-scores of IGF-1 measurements between 2016 and 2019, using our home reference values based on de normative dataset by Elmlinger (E)1, and using the normative datasets defined by Bidlingmaier (B)2 and by the Dutch IGF-1 harmonization program (NL). Based on these three Z-scores, the outcomes of growth hormone stimulation tests performed in this period (n=86) were reassessed according to the interpretation described in the Dutch guideline. Using all three normative datasets the same 4 patients were identified as likely to have a growth hormone deficiency, whereas 10(E), 10(B), or 8(NL) patients were identified as possible partial growth hormone deficiency. In 70(E), 66(B) or 72(NL) patients the growth hormone stimulation test was unaffected. Using normative dataset B, 6 patients displayed a pattern associated with a possible growth hormone resistance, or of bio-inactive growth hormone syndromes, which based on its incidence would be unlikely for a secondary care setting. A striking observation was however, that of all patients with a normal stimulation test 9 (E)/16 (B) or 30 (NL) had a IGF-1 Z-score of &gt; 0 SD. This implies that, for the diagnosis of growth hormone deficiency, it is safe to implement the Dutch harmonized dataset, which in addition could result in a reduction in the number of growth hormone stimulation tests that have to be performed. References: 1. Elmlinger MW et al. Clin Chem Lab Med. 2004;42(6):654-64. 2. Bidlingmaier M et al. J Clin Endocrinol Metab. 2014 May;99(5):1712-21.

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