Assessment of serum IGF-I concentrations in the diagnosis of isolated childhood-onset GH deficiency: A proposal of the Italian Society for Pediatric Endocrinology and Diabetes (SIEDP/ISPED)

2006 ◽  
Vol 29 (8) ◽  
pp. 732-737 ◽  
Author(s):  
G. Federico ◽  
◽  
M. E. Street ◽  
M. Maghnie ◽  
M. Caruso-Nicoletti ◽  
...  
Keyword(s):  
Gh Deficiency ◽  
Italian Society ◽  
Pediatric Endocrinology ◽  
Childhood Onset ◽  
Igf I

scholarly journals Subnormal Serum Insulin-Like Growth Factor-I Levels in Young Adults with Childhood-Onset Nonacquired Growth Hormone (GH) Deficiency Who Recover Normal GH Secretion May Indicate Less Severe but Persistent Pituitary Failure

2007 ◽  
Vol 92 (10) ◽  
pp. 3788-3795 ◽  
Author(s):  
Georges Gelwane ◽  
Catherine Garel ◽  
Didier Chevenne ◽  
Priscilla Armoogum ◽  
Dominique Simon ◽  
...  
Keyword(s):  
Young Adults ◽  
Anterior Pituitary ◽  
Gh Deficiency ◽  
Childhood Onset ◽  
Mri Findings ◽  
Gh Secretion ◽  
Structural Abnormalities ◽  
Igf I ◽  
Ectopic Neurohypophysis

Abstract Context: The unexpected observation of a normal GH peak in 22% of young adults with childhood-onset GH deficiency (GHD) and ectopic neurohypophysis has raised questions about the criteria defining GHD in young adults and whether patients with subsequent increases in GH secretion nonetheless have a subtle form of GHD. Objective: Our objective was to determine the characteristics of patients with childhood-onset nonacquired GHD who recover normal peak GH secretion when adult height has been achieved. Design and Setting: We conducted a university hospital-based observational follow-up study. Participants: Sixty-two patients with ectopic neurohypophysis (n = 24), isolated hypoplastic anterior pituitary (n = 14), or normal hypothalamic pituitary area (n = 24) on magnetic resonance imaging (MRI) at the time of GHD diagnosis underwent reevaluation of the GH-IGF-I axis at a mean age of 16.8 ± 1.6 yr. Main Outcome Measures: Outcome measures included clinical and MRI findings and serum IGF-I and peak GH levels. Results: On retesting, peak GH exceeded 10 μg/liter in 31 patients (50%): six (20%) patients with ectopic neurohypophysis, 10 (32%) patients with initially isolated hypoplastic anterior pituitary, and 15 (48%) patients with normal MRI findings. Among these patients, serum IGF-I levels were significantly lower in patients with ectopic neurohypophysis than in those without structural abnormalities of the hypothalamic pituitary axis (n = 25), but patients without structural abnormalities also had significantly lower serum IGF-I levels than control subjects, after controlling for age, sex, and body mass index (mean serum IGF-I levels of 374 ± 83 vs. 446 ± 108 μg/liter; β-coefficient = −72; P = 0.003). Conclusions: The severity of the disease seems to have decreased over time in these patients, who may nonetheless present persistent pituitary failure. The natural history and clinical implications of these findings remain to be clarified. The possibility of a deterioration in the secretion of GH and other pituitary hormones later in life in a subset of these patients warrants the careful long-term follow-up of this population.

scholarly journals Discontinuation of Growth Hormone (GH) Treatment during the Transition Phase Is an Important Factor Determining the Phenotype of Young Adults with Nonidiopathic Childhood-Onset GH Deficiency

2010 ◽  
Vol 95 (6) ◽  
pp. 2646-2654 ◽  
Author(s):  
Maria Kołtowska-Häggström ◽  
Mitchell E. Geffner ◽  
Peter Jönsson ◽  
John P. Monson ◽  
Roger Abs ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Gh Deficiency ◽  
Transition Phase ◽  
Childhood Onset ◽  
Future Health ◽  
Gh Treatment ◽  
Total Cohort ◽  
Igf I ◽  
The Impact

Abstract Context: Little is known about the impact of childhood-onset GH deficiency (GHD), in particular the duration of GH cessation during the transition phase, on adult phenotype. Objective: We investigated the association between the manifestations and management of GHD during childhood/adolescence and the clinical features of GHD in adulthood. Design/Setting/Patients/Intervention: Patients with reconfirmed childhood-onset GHD who resumed GH treatment as adults were identified from two sequential databases (n = 313). The cohort was followed up longitudinally from GH start in childhood to reinitiation of treatment in adulthood and 1 yr beyond. Analyses were performed in the total cohort and in subgroups of patients with idiopathic GHD (IGHD) and non-IGHD. The cohorts were stratified based on duration of GH cessation (short, ≤2 yr; long, >2 yr). Main Outcome Measures: Regimen of pediatric GH administration, duration of GH interruption, IGF-I sd score, lipid concentrations, and quality of life were measured. Results: Mean duration of GH interruption was 4.4 yr. IGF-I sd score in adulthood was related to severity of childhood GHD. In non-IGHD patients, a longer duration of GH interruption was associated with a worse lipid profile (P < 0.0001). Non-IGHD patients who gained more height during childhood GH treatment reported better quality of life than those who gained less height (P < 0.05). Conclusions: Pediatricians should tailor GH treatment, not only for its beneficial effect on growth but also for future health in adulthood. In adults with reconfirmed GHD, particularly those with non-IGHD, early recommencement of GH should be considered.

scholarly journals Impact of the primary aetiology upon the clinical outcome of adults with childhood-onset GH deficiency

2007 ◽  
Vol 157 (5) ◽  
pp. 589-596 ◽  
Author(s):  
Charlotte Hoybye ◽  
Peter Jönsson ◽  
John P Monson ◽  
Maria Kołtowska-Häggström ◽  
Václav Hána ◽  
...  
Keyword(s):  
Brain Tumour ◽  
Gh Deficiency ◽  
Childhood Onset ◽  
Gh Treatment ◽  
Gh Replacement ◽  
Igf I ◽  
Tumour Group ◽  
Organic Disorder ◽  
The Impact ◽  
The Brain

AbstractObjectiveThe impact of the aetiology of childhood-onset GH deficiency (CO-GHD) on the clinical presentation during adulthood and the response to GH replacement has been poorly defined. Our study aims to characterize CO-GHD in adults due to different aetiologies and evaluate the effect of 2 years of GH replacement therapy.Design and methodsData from 353 adults with CO-GHD from Pfizer International Metabolic Database KIMS were retrospectively grouped according to GHD aetiology: non-organic disorder (n=147), organic pituitary disease (n=159), and brain tumour (n=47). Extent of pituitary dysfunction, IGF-I concentration, lipid concentrations and quality-of-life (QoL) were assessed at baseline and after 2 years of GH replacement.ResultsGHD was diagnosed at a later age in the organic pituitary group than in the other groups, resulting in a shorter duration of GH treatment during childhood. However, the final height was greater in the organic pituitary group. Panhypopituitarism was most common in the non-organic disorder and in the organic pituitary groups, while isolated GHD was more prominent in the brain tumour group. Serum IGF-I levels were the lowest in the non-organic group. QoL was the poorest in the brain tumour group. Lipid profile and QoL improved significantly during GH replacement.ConclusionThe adverse consequences of CO-GHD in adulthood vary between aetiologies, but improve similarly with GH treatment. It is, therefore, important to consider retesting all patients with CO-GHD in early adulthood and, if persistent severe GHD is confirmed, recommence GH replacement.

scholarly journals High Risk of Adrenal Insufficiency in Adults Previously Treated for Idiopathic Childhood Onset Growth Hormone Deficiency

2003 ◽  
Vol 88 (12) ◽  
pp. 5784-5789 ◽  
Author(s):  
Martin Lange ◽  
Ulla Feldt-Rasmussen ◽  
Ole Lander Svendsen ◽  
Knud William Kastrup ◽  
Anders Juul ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Gh Deficiency ◽  
Tolerance Test ◽  
Hormone Deficiency ◽  
Childhood Onset ◽  
Gh Treatment ◽  
Insulin Tolerance ◽  
Igf I ◽  
Synacthen Test ◽  
Previously Treated

Abstract The aim was to reevaluate a group of adults treated for idiopathic childhood onset GH deficiency (GHD) after 18 yr without GH treatment. Twenty-six (11 females) patients participated. All but two had isolated GHD. Childhood diagnosis was established by insulin tolerance test (ITT). The patients were retested with an ITT to evaluate adult GH status. In five patients, an arginine and a synacthen test were performed instead of an ITT. Eleven of 25 patients had a subnormal cortisol response to ITT or synacthen. Ten patients had a GH peak less than 3.0 μg/liter (0.5. ± 0.5 μg/liter), whereas 16 patients displayed a normal GH response (12.3 ± 10.6 μg/liter) after ITT. IGF-I values were decreased in the patients with a pathological retest as well as in patients with a normal GH response compared with controls (P < 0.005). In 26 idiopathic childhood onset GHD patients, 44% of the patients had developed adrenal insufficiency; 38.5% had persistent GHD in adulthood, using the same test in both childhood and adulthood. Patients having a normal GH test had decreased IGF-I levels, compared with controls, indicating impaired function of a seemingly normal GH axis. It is imperative that pituitary axes other than the GH axis are tested at regular intervals, even in the absence of GHD in adulthood.

scholarly journals Consensus guidelines for the diagnosis and treatment of adults with GH deficiency II: a statement of the GH Research Society in association with the European Society for Pediatric Endocrinology, Lawson Wilkins Society, European Society of Endocrinology, Japan Endocrine Society, and Endocrine Society of Australia

2007 ◽  
Vol 157 (6) ◽  
pp. 695-700 ◽  
Author(s):  
Ken K Y Ho ◽  
_ _
Keyword(s):  
European Society ◽  
De Novo ◽  
Gh Deficiency ◽  
Tolerance Test ◽  
Research Society ◽  
Cranial Irradiation ◽  
Pediatric Endocrinology ◽  
Endocrine Society ◽  
Consensus Statements ◽  
Igf I

ObjectiveThe GH Research Society held a Consensus Workshop in Sydney, Australia, 2007 to incorporate the important advances in the management of GH deficiency (GHD) in adults, which have taken place since the inaugural 1997 Consensus Workshop.MethodTwo commissioned review papers, previously published Consensus Statements of the Society and key questions were circulated before the Workshop, which comprised a rigorous structure of review with breakout discussion groups. A writing group transcribed the summary group reports for drafting in a plenary forum on the last day. All participants were sent a polished draft for additional comments and gave signed approval to the final revision.ConclusionTesting for GHD should be extended from hypothalamic–pituitary disease and cranial irradiation to include traumatic brain injury. Testing may indicate isolated GHD; however, idiopathic isolated GHD occurring de novo in the adult is not a recognized entity. The insulin tolerance test, combined administration of GHRH with arginine or growth hormone-releasing peptide, and glucagon are validated GH stimulation tests in the adult. A low IGF-I is a reliable diagnostic indicator of GHD in the presence of hypopituitarism, but a normal IGF-I does not rule out GHD. GH status should be reevaluated in the transition age for continued treatment to complete somatic development. Interaction of GH with other axes may influence thyroid, glucocorticoid, and sex hormone requirements. Response should be assessed clinically by monitoring biochemistry, body composition, and quality of life. There is no evidence that GH replacement increases the risk of tumor recurrence or de novo malignancy.

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