Benefits of multidisciplinary care in Prader-Willi syndrome

被引:8
作者
Salvatoni, Alessandro [1 ]
Nosetti, Luana [1 ]
Salvatore, Silvia [1 ]
Agosti, Massimo [1 ]
机构
[1] Insubria Univ, Dept Pediat, Varese, Italy
关键词
Prader-Willi; multidisciplinary; obesity; hypotonia; growth hormone; osas; CENTRAL ADRENAL INSUFFICIENCY; BODY-COMPOSITION; THYROID-FUNCTION; HIP-DYSPLASIA; CHILDREN; PREVALENCE; ADOLESCENTS; CONTRIBUTES; DYSFUNCTION; MANAGEMENT;
D O I
10.1080/17446651.2021.1898375
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Introduction: Prader-Willi syndrome (PWS) is the most well-known condition of genetic obesity. Over the past 20 years, advances have been achieved in the diagnosis and treatment of PWS with a significant improvement in prognosis. Areas covered: This review focuses on the benefits of multidisciplinary approach in children and adolescents with PWS. In particular, the neonatologist and geneticist play a key role in early diagnosis and the clinical follow-up of the PWS patient must be guaranteed by a team including pediatric endocrinologist, psychologist, nutritionist/dietician, neurologist/neuropsychiatrist, sleep specialist, ears, nose and throat specialist (END, lung specialist, dentist, orthopedist and ophthalmologist and, eventually, gastroenterologist. We searched PubMed and critically summarized what has been reported in the last 10 years on PWS. Expert opinion: The multidisciplinary care in association with an early diagnosis and GH treatment postpones overweight development and decreases prevalence of obesity in individuals with PWS. Further prognostic improvements are expected through the selection of teams particularly experienced in the management of individuals with PWS and the discovery of new drugs.
引用
收藏
页码:63 / 71
页数:9
相关论文
共 94 条
[21]   Effect of topiramate on eating behaviours in Prader-Willi syndrome: TOPRADER double-blind randomised placebo-controlled study [J].
Consoli, Angele ;
Berthoumieu, Sophie Cabal ;
Raffin, Marie ;
Thuilleaux, Denise ;
Poitou, Christine ;
Coupaye, Muriel ;
Pinto, Graziella ;
Lebbah, Said ;
Zahr, Noel ;
Tauber, Maithe ;
Cohen, David ;
Bonnot, Olivier .
TRANSLATIONAL PSYCHIATRY, 2019, 9 (1)
[22]   Safety and effectiveness of growth hormone therapy in infants with Prader-Willi syndrome younger than 2 years: a prospective study [J].
Corripio, Raquel ;
Tubau, Carla ;
Calvo, Laura ;
Brun, Carme ;
Capdevila, Nuria ;
Larramona, Helena ;
Gabau, Elisabeth .
JOURNAL OF PEDIATRIC ENDOCRINOLOGY & METABOLISM, 2019, 32 (08) :879-884
[23]   Hypogonadism and pubertal development in Prader-Willi syndrome [J].
Crinò, A ;
Schiaffini, R ;
Ciampalini, P ;
Spera, S ;
Beccaria, L ;
Benzi, F ;
Bosio, L ;
Corrias, A ;
Gargantini, L ;
Salvatoni, A ;
Tonini, G ;
Trifirò, G ;
Livieri, C .
EUROPEAN JOURNAL OF PEDIATRICS, 2003, 162 (05) :327-333
[24]   Liraglutide therapy in Prader-Willi syndrome [J].
Cyganek, K. ;
Koblik, T. ;
Kozek, E. ;
Wojcik, M. ;
Starzyk, J. ;
Malecki, M. T. .
DIABETIC MEDICINE, 2011, 28 (06) :755-756
[25]   Calorie seeking, but not hedonic response, contributes to hyperphagia in a mouse model for Prader-Willi syndrome [J].
Davies, Jennifer R. ;
Humby, Trevor ;
Dwyer, Dominic M. ;
Garfield, Alastair S. ;
Furby, Hannah ;
Wilkinson, Lawrence S. ;
Wells, Timothy ;
Isles, Anthony R. .
EUROPEAN JOURNAL OF NEUROSCIENCE, 2015, 42 (04) :2105-2113
[26]   Growth Hormone Research Society Workshop Summary: Consensus Guidelines for Recombinant Human Growth Hormone Therapy in Prader-Willi Syndrome [J].
Deal, Cheri L. ;
Tony, Michele ;
Hoybye, Charlotte ;
Allen, David B. ;
Tauber, Maithe ;
Christiansen, Jens Sandahl .
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM, 2013, 98 (06) :E1072-E1087
[27]   Bone mineral density in young adults with Prader-Willi syndrome: A randomized, placebo-controlled, crossover GH trial [J].
Donze, Stephany H. ;
Kuppens, Renske J. ;
Bakker, Nienke E. ;
van Alfen-van der Velden, Janielle A. E. M. ;
Hokken-Koelega, Anita C. S. .
CLINICAL ENDOCRINOLOGY, 2018, 88 (06) :806-812
[28]  
Driscoll DJ., 1998, PRADER WILLI SYNDROM, P1
[29]  
Driscoll DJ, 1993, GENEREVIEWS
[30]   A multidisciplinary approach to the clinical management of Prader-Willi syndrome [J].
Duis, Jessica ;
van Wattum, Pieter J. ;
Scheimann, Ann ;
Salehi, Parisa ;
Brokamp, Elly ;
Fairbrother, Laura ;
Childers, Anna ;
Shelton, Althea Robinson ;
Bingham, Nathan C. ;
Shoemaker, Ashley H. ;
Miller, Jennifer L. .
MOLECULAR GENETICS & GENOMIC MEDICINE, 2019, 7 (03)