Growth hormone secretion decreases with age in paediatric <scp>P</scp>rader‐<scp>W</scp>illi syndrome

Cohen, Michal; Harrington, Jennifer; Narang, Indra; Hamilton, Jill

doi:10.1111/cen.12694

Cited by 15 publications

(14 citation statements)

References 23 publications

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“…It is likely that there is a combination of subnormal GH reserve and IGF-1 generation in individuals with PWS leading to subnormal growth response. Pituitary GH reserve may progressively decline with age in PWS children and GH levels are noted to be relatively lower in obese PWS patients than those who are non-obese (7). Additionally, one study has shown genotypic differences in GH secretary responses on GH stimulation testing, with higher GH response in PWS patients with paternal deletion of 15q11-q13 than in those with maternal UPD15 (8).…”

Section: Growth Hormone (Gh)mentioning

confidence: 99%

Review of Prader-Willi syndrome: the endocrine approach

et al. 2017

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Prader-Willi syndrome (PWS) is a complex genetic disorder with implications on the endocrine and neurologic systems, metabolism, and behavior. Early in life, PWS is characterized by hypotonia and failure to thrive, followed by obesity and hyperphagia. Patients with PWS develop hypothalamic dysfunction which may lead growth hormone deficiency (GHD), hypogonadism, hypothyroidism, adrenal insufficiency, and poor bone mineral density (BMD). In addition to hypothalamic dysfunction, individuals with PWS have increased risk for obesity which may be complicated by metabolic syndrome and type 2 diabetes mellitus (T2DM). In this paper, we will review the current literature pertaining to the endocrine concerns of PWS and current recommendations for screening and management of these conditions.

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Section: Growth Hormone (Gh)mentioning

confidence: 99%

Review of Prader-Willi syndrome: the endocrine approach

et al. 2017

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“…One of these studies in 27 children with PWS also performed a combined test (GHRH‐arginine or GHRH‐pyridostigmine test), which showed a much lower prevalence of GHD (15%) than the clonidine and arginine tests . As studies in adults with PWS have described a higher prevalence of GHD after combined stimulation tests, the authors of both studies suggested that GHD in PWS might be due to an evolving process, with pituitary GH reserve decreasing with age . Although we did not perform GH stimulation tests during childhood before starting GH treatment, our data show that, of the 18 children with an IGF‐I <−2 SDS at start of GH treatment, all but two patients had normal serum IGF‐I levels (>−2 SDS) after cessation of GH at adult height attainment.…”

Section: Discussionmentioning

confidence: 74%

“…Two studies in young children with PWS described a low GH peak after clonidine or arginine in 68%‐85% . One of these studies in 27 children with PWS also performed a combined test (GHRH‐arginine or GHRH‐pyridostigmine test), which showed a much lower prevalence of GHD (15%) than the clonidine and arginine tests .…”

Section: Discussionmentioning

confidence: 99%

“…Reduced serum insulin‐like growth factor (IGF‐I) levels and a reduced GH response to provocative stimuli were found in a varying percentage of children and adults with PWS . Most studies in adults with PWS have investigated GHD in adults who were not treated with GH during childhood and the prevalence of GHD varied dependent on the diagnostic test and the chosen cut‐off points, which were or were not corrected for BMI.…”

Section: Introductionmentioning

confidence: 99%

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Prevalence of growth hormone (GH) deficiency in previously GH‐treated young adults with Prader‐Willi syndrome

Donze

Damen

Velden³

et al. 2019

Clinical Endocrinology

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ObjectiveSome features of subjects with Prader‐Willi syndrome (PWS) resemble those seen in growth hormone deficiency (GHD). Children with PWS are treated with growth hormone (GH), which has substantially changed their phenotype. Currently, young adults with PWS must discontinue GH after attainment of adult height when they do not fulfil the criteria of adult GHD. Limited information is available about the prevalence of GHD in adults with PWS. This study aimed to investigate the GH/insulin‐like growth factor (IGF‐I) axis and the prevalence of GHD in previously GH‐treated young adults with PWS.DesignCross‐sectional study in 60 young adults with PWS.MeasurementsSerum IGF‐I and IGFBP‐3 levels, GH peak during combined growth hormone‐releasing hormone (GHRH)‐arginine stimulation test.ResultsSerum IGF‐I was <−2 standard deviation scores (SDS) in 2 (3%) patients, and IGFBP‐3 was within the normal range in all but one patient. Median (IQR) GH peak was 17.8 μg/L (12.2; 29.7) [~53.4 mU/L] and below 9 μg/L in 9 (15%) patients. Not one patient fulfilled the criteria for adult GHD (GH peak < 9 μg/L and IGF‐I < −2 SDS), also when BMI‐dependent criteria were used. A higher BMI and a higher fat mass percentage were significantly associated with a lower GH peak. There was no significant difference in GH peak between patients with a deletion or a maternal uniparental disomy (mUPD).ConclusionsIn a large group of previously GH‐treated young adults with PWS, approximately 1 in 7 exhibited a GH peak <9 μg/L during a GHRH‐arginine test. However, none of the patients fulfilled the consensus criteria for adult GHD.

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“… 15 These findings seem to support the hypothesis that GH pituitary reserve, which is still preserved in young patients, gradually declines with age, suggesting that GHD in PWS may be due to an evolving process. 16 Another critical point is related to the concomitant presence of obesity, which is characterized per se by reduced GH secretion. A higher prevalence of GHD is detected in obese PWS children compared with nonobese subjects.…”

Section: Gh and Childhoodmentioning

confidence: 99%

Growth hormone therapy for Prader–Willi syndrome: challenges and solutions

Grugni¹,

Sartório²,

Crinò³

2016

TCRM

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Prader–Willi syndrome (PWS) is characterized by a dysregulation of growth hormone (GH)/insulin-like growth factor I axis, as the consequence of a complex hypothalamic involvement. PWS’ clinical picture seems to resemble the classic non-PWS GH deficiency (GHD), including short stature, excessive body fat, decreased muscle mass, and impaired quality of life. GH therapy is able to ameliorate the phenotypic appearance of the syndrome, as well as to improve body composition, physical strength, and cognitive level. In this regard, however, some pathophysiologic and clinical questions still remain, representing a challenge to give the most appropriate care to PWS patients. Data about the prevalence of GHD in PWS children are not unequivocal, ranging from 40% to 100%. In this context, to establish whether the presence (or not) of GHD may have a different effect on clinical course during GH therapy may be helpful. In addition, the comparison of GH effects in PWS children diagnosed as small for gestational age with those obtained in subjects born appropriate for gestational age is of potential interest for future trials. Emerging information seems to demonstrate the maintenance of beneficial effects of GH therapy in PWS subjects after adolescent years. Thus, GH retesting after achievement of final height should be taken into consideration for all PWS patients. However, it is noteworthy that GH administration exerts positive effects both in PWS adults with and without GHD. Another critical issue is to clarify whether the genotype–phenotype correlations may be relevant to specific outcome measures related to GH therapy. Moreover, progress of our understanding of the role of GH replacement and concomitant therapies on bone characteristics of PWS is required. Finally, a long-term surveillance of benefits and risks of GH therapy is strongly recommended for PWS population, since most of the current studies are uncontrolled and of short duration.

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Growth hormone secretion decreases with age in paediatric Prader‐Willi syndrome

Abstract: The utility of performing GH stimulation tests as an indication of GH status under 18 months of age in Prader-Willi syndrome is questionable. If performed, results should be carefully interpreted in the context of age.

Cited by 15 publications

References 23 publications

Review of Prader-Willi syndrome: the endocrine approach

Review of Prader-Willi syndrome: the endocrine approach

Prevalence of growth hormone (GH) deficiency in previously GH‐treated young adults with Prader‐Willi syndrome

Growth hormone therapy for Prader–Willi syndrome: challenges and solutions

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Growth hormone secretion decreases with age in paediatric Prader‐Willi syndrome

Abstract: The utility of performing GH stimulation tests as an indication of GH status under 18 months of age in Prader-Willi syndrome is questionable. If performed, results should be carefully interpreted in the context of age.

Cited by 15 publications

References 23 publications

Review of Prader-Willi syndrome: the endocrine approach

Review of Prader-Willi syndrome: the endocrine approach

Prevalence of growth hormone (GH) deficiency in previously GH‐treated young adults with Prader‐Willi syndrome

Growth hormone therapy for Prader&ndash;Willi syndrome: challenges and solutions

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Growth hormone therapy for Prader–Willi syndrome: challenges and solutions