دورية أكاديمية
Adrenal insufficiency in patients with Prader-Willi syndrome.
| العنوان: | Adrenal insufficiency in patients with Prader-Willi syndrome. |
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| المؤلفون: | Kusz MJ; Department of Pediatrics and Pediatric Endocrinology, Medical University of Silesia, Katowice, Poland.; The Faculty of Medical Sciences, The Doctoral School of the Medical University of Silesia, Katowice, Poland., Gawlik AM; Department of Pediatrics and Pediatric Endocrinology, Medical University of Silesia, Katowice, Poland. |
| المصدر: | Frontiers in endocrinology [Front Endocrinol (Lausanne)] 2022 Nov 17; Vol. 13, pp. 1021704. Date of Electronic Publication: 2022 Nov 17 (Print Publication: 2022). |
| نوع المنشور: | Journal Article; Review |
| اللغة: | English |
| بيانات الدورية: | Publisher: Frontiers Research Foundation] Country of Publication: Switzerland NLM ID: 101555782 Publication Model: eCollection Cited Medium: Print ISSN: 1664-2392 (Print) Linking ISSN: 16642392 NLM ISO Abbreviation: Front Endocrinol (Lausanne) Subsets: MEDLINE |
| أسماء مطبوعة: | Original Publication: [Lausanne : Frontiers Research Foundation] |
| مواضيع طبية MeSH: | Prader-Willi Syndrome*/complications , Prader-Willi Syndrome*/drug therapy , Human Growth Hormone*/therapeutic use , Adrenal Insufficiency*/complications , Adrenal Insufficiency*/drug therapy, Child ; Humans ; Hypothalamo-Hypophyseal System ; Pituitary-Adrenal System ; Hydrocortisone |
| مستخلص: | The generalized dysfunction of the hypothalamic-pituitary axis in patients with Prader-Willi syndrome (PWS) is the most likely cause of hypogonadism, inadequate growth hormone secretion, excessive appetite and associated obesity, impaired body temperature regulation, and hypothyroidism. The syndrome is also related to an increased risk of central adrenal insufficiency, although its prevalence remains unknown. The results of the studies in which different methods of pharmacological stimulation were used do not provide conclusive outcomes. As a result, there are no clear guidelines with regard to diagnosis, prevention, or long-term care when adrenal insufficiency is suspected in patients with PWS. Currently, most patients with PWS are treated with recombinant human growth hormone (rhGH). It has been confirmed that rhGH therapy has a positive effect on growth, body composition, body mass index (BMI), and potentially on psychomotor development in children with PWS. Additionally, rhGH may reduce the conversion of cortisone to cortisol through inhibition of 11β-hydroxysteroid dehydrogenase type 1. However, its influence on basal adrenal function and adrenal stress response remains unexplained in children with PWS. This paper reviews the literature related to the hypothalamic-pituitary-adrenal axis dysfunction in the PWS patient population with a focus on children. Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. (Copyright © 2022 Kusz and Gawlik.) |
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| فهرسة مساهمة: | Keywords: LDSST; PWS; Prader-Willi syndrome; Synacthen; adrenal insufficiency |
| المشرفين على المادة: | WI4X0X7BPJ (Hydrocortisone) 12629-01-5 (Human Growth Hormone) |
| تواريخ الأحداث: | Date Created: 20221205 Date Completed: 20221206 Latest Revision: 20221213 |
| رمز التحديث: | 20221214 |
| مُعرف محوري في PubMed: | PMC9714690 |
| DOI: | 10.3389/fendo.2022.1021704 |
| PMID: | 36465638 |
| قاعدة البيانات: | MEDLINE |
Full Text Finder | تدمد: | 1664-2392 |
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| DOI: | 10.3389/fendo.2022.1021704 |