Impact of transitional care on endocrine and anthropometric parameters in Prader–Willi syndrome
Context: The transition of patients with Prader–Willi syndrome (PWS) to adult life for medical care is challenging because of multiple comorbidities, including hormone deficiencies, obesity and cognitive and behavioral disabilities. Objective: To assess endocrine management, and metabolic and anthr...
| Main Authors: | , , , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Bioscientifica
2018-05-01
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| Series: | Endocrine Connections |
| Subjects: | |
| Online Access: | http://www.endocrineconnections.com/content/7/5/663.full |
| _version_ | 1818597605903433728 |
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| author | A C Paepegaey M Coupaye A Jaziri F Ménesguen B Dubern M Polak J M Oppert M Tauber G Pinto C Poitou |
| author_facet | A C Paepegaey M Coupaye A Jaziri F Ménesguen B Dubern M Polak J M Oppert M Tauber G Pinto C Poitou |
| author_sort | A C Paepegaey |
| collection | DOAJ |
| description | Context: The transition of patients with Prader–Willi syndrome (PWS) to adult life for medical care is challenging because of multiple comorbidities, including hormone deficiencies, obesity and cognitive and behavioral disabilities.
Objective: To assess endocrine management, and metabolic and anthropometric parameters of PWS adults who received (n = 31) or not (n = 64) transitional care, defined as specialized pediatric care followed by a structured care pathway to a multidisciplinary adult team.
Patients and study design: Hormonal and metabolic parameters were retrospectively recorded in 95 adults with PWS (mean ± s.d. age 24.7 ± 8.2 years, BMI: 39.8 ± 12.1 kg/m²) referred to our Reference Center and compared according to transition.
Results: Among the entire cohort, 35.8% received growth hormone (GH) during childhood and 16.8% had a GH stimulation test after completion of growth. In adulthood, 14.7% were treated with GH, 56.8% received sex-hormone therapy, whereas 91.1% were hypogonadic and 37.9% had undergone valid screening of the corticotropic axis. The main reason for suboptimal endocrine management was marked behavioral disorders. Patients receiving transitional care were more likely to have had a GH stimulation test and hormonal substitutions in childhood. They also had a lower BMI, percentage of fat mass, improved metabolic parameters and fewer antidepressant treatments. Transitional care remained significantly associated with these parameters in multivariate analysis when adjusted on GH treatment.
Conclusion: A coordinated care pathway with specialized pediatric care and transition to a multidisciplinary adult team accustomed to managing complex disability including psychiatric troubles are associated with a better health status in adults with PWS. |
| first_indexed | 2024-12-16T11:50:28Z |
| format | Article |
| id | doaj.art-d0eae4f67dbf4159b3f0748089702153 |
| institution | Directory Open Access Journal |
| issn | 2049-3614 2049-3614 |
| language | English |
| last_indexed | 2024-12-16T11:50:28Z |
| publishDate | 2018-05-01 |
| publisher | Bioscientifica |
| record_format | Article |
| series | Endocrine Connections |
| spelling | doaj.art-d0eae4f67dbf4159b3f07480897021532022-12-21T22:32:44ZengBioscientificaEndocrine Connections2049-36142049-36142018-05-0175663672https://doi.org/10.1530/EC-18-0089Impact of transitional care on endocrine and anthropometric parameters in Prader–Willi syndromeA C Paepegaey0M Coupaye1A Jaziri2F Ménesguen3B Dubern4M Polak5J M Oppert6M Tauber7G Pinto8C Poitou9Nutrition Department, Assistance-Publique Hôpitaux de Paris (AP-HP), Pitié-Salpêtrière Hospital, French Reference Center for Prader-Willi Syndrome, Sorbonne Université, Paris, FranceNutrition Department, Assistance-Publique Hôpitaux de Paris (AP-HP), Pitié-Salpêtrière Hospital, French Reference Center for Prader-Willi Syndrome, Sorbonne Université, Paris, FranceNutrition Department, Assistance-Publique Hôpitaux de Paris (AP-HP), Pitié-Salpêtrière Hospital, French Reference Center for Prader-Willi Syndrome, Sorbonne Université, Paris, FranceNutrition Department, Assistance-Publique Hôpitaux de Paris (AP-HP), Pitié-Salpêtrière Hospital, French Reference Center for Prader-Willi Syndrome, Sorbonne Université, Paris, FranceNutrition and Gastroenterology Department, Assistance-Publique Hôpitaux de Paris (AP-HP), Armand Trousseau Children’s Hospital, Paris, FrancePediatric Endocrinology, Diabetology and Gynecology Department, Assistance-Publique Hôpitaux de Paris (AP-HP), Necker Enfants Malades Hospital University Hospital, Paris, FranceNutrition Department, Assistance-Publique Hôpitaux de Paris (AP-HP), Pitié-Salpêtrière Hospital, French Reference Center for Prader-Willi Syndrome, Sorbonne Université, Paris, FrancePediatric Endocrinology Department, Children’s Hospital, French Reference Center for Prader-Willi Syndrome, Toulouse, FrancePediatric Endocrinology, Diabetology and Gynecology Department, Assistance-Publique Hôpitaux de Paris (AP-HP), Necker Enfants Malades Hospital University Hospital, Paris, FranceNutrition Department, Assistance-Publique Hôpitaux de Paris (AP-HP), Pitié-Salpêtrière Hospital, French Reference Center for Prader-Willi Syndrome, Sorbonne Université, Paris, France; INSERM, UMRS 1166, Nutriomic Group 6, Paris, France; Sorbonne Université, UMRS1166, Paris, FranceContext: The transition of patients with Prader–Willi syndrome (PWS) to adult life for medical care is challenging because of multiple comorbidities, including hormone deficiencies, obesity and cognitive and behavioral disabilities. Objective: To assess endocrine management, and metabolic and anthropometric parameters of PWS adults who received (n = 31) or not (n = 64) transitional care, defined as specialized pediatric care followed by a structured care pathway to a multidisciplinary adult team. Patients and study design: Hormonal and metabolic parameters were retrospectively recorded in 95 adults with PWS (mean ± s.d. age 24.7 ± 8.2 years, BMI: 39.8 ± 12.1 kg/m²) referred to our Reference Center and compared according to transition. Results: Among the entire cohort, 35.8% received growth hormone (GH) during childhood and 16.8% had a GH stimulation test after completion of growth. In adulthood, 14.7% were treated with GH, 56.8% received sex-hormone therapy, whereas 91.1% were hypogonadic and 37.9% had undergone valid screening of the corticotropic axis. The main reason for suboptimal endocrine management was marked behavioral disorders. Patients receiving transitional care were more likely to have had a GH stimulation test and hormonal substitutions in childhood. They also had a lower BMI, percentage of fat mass, improved metabolic parameters and fewer antidepressant treatments. Transitional care remained significantly associated with these parameters in multivariate analysis when adjusted on GH treatment. Conclusion: A coordinated care pathway with specialized pediatric care and transition to a multidisciplinary adult team accustomed to managing complex disability including psychiatric troubles are associated with a better health status in adults with PWS.http://www.endocrineconnections.com/content/7/5/663.fullPrader–Willi syndrometransitionGH treatmentobesity |
| spellingShingle | A C Paepegaey M Coupaye A Jaziri F Ménesguen B Dubern M Polak J M Oppert M Tauber G Pinto C Poitou Impact of transitional care on endocrine and anthropometric parameters in Prader–Willi syndrome Endocrine Connections Prader–Willi syndrome transition GH treatment obesity |
| title | Impact of transitional care on endocrine and anthropometric parameters in Prader–Willi syndrome |
| title_full | Impact of transitional care on endocrine and anthropometric parameters in Prader–Willi syndrome |
| title_fullStr | Impact of transitional care on endocrine and anthropometric parameters in Prader–Willi syndrome |
| title_full_unstemmed | Impact of transitional care on endocrine and anthropometric parameters in Prader–Willi syndrome |
| title_short | Impact of transitional care on endocrine and anthropometric parameters in Prader–Willi syndrome |
| title_sort | impact of transitional care on endocrine and anthropometric parameters in prader willi syndrome |
| topic | Prader–Willi syndrome transition GH treatment obesity |
| url | http://www.endocrineconnections.com/content/7/5/663.full |
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