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
[41]   Central adrenal insufficiency in young adults with Prader-Willi Syndrome [J].
Grugni, Graziano ;
Beccaria, Luciano ;
Corrias, Andrea ;
Crino, Antonino ;
Cappa, Marco ;
De Medici, Clotilde ;
Di Candia, Stefania ;
Gargantini, Luigi ;
Ragusa, Letizia ;
Salvatoni, Alessandro ;
Sartorio, Alessandro ;
Spera, Sabrina ;
Andrulli, Simeone ;
Chiumello, Giuseppe ;
Mussa, Alessandro .
CLINICAL ENDOCRINOLOGY, 2013, 79 (03) :371-378
[42]  
Hartman WR., 2012, J CLI MED RES, V4
[43]  
HELBINGZWANENBURG B, 1993, J INTELL DISABIL RES, V37, P533
[44]  
Hered R W, 1988, J Pediatr Ophthalmol Strabismus, V25, P145
[45]   SLEEP AND BREATHING PATTERNS IN PATIENTS WITH PRADER-WILLI-SYNDROME (PWS) - EFFECTS OF AGE AND GENDER [J].
HERTZ, G ;
CATALETTO, M ;
FEINSILVER, SH ;
ANGULO, M .
SLEEP, 1993, 16 (04) :366-371
[46]   Primary Testicular Dysfunction Is a Major Contributor to Abnormal Pubertal Development in Males with Prader-Willi Syndrome [J].
Hirsch, Harry J. ;
Eldar-Geva, Talia ;
Benarroch, Fortu ;
Rubinstein, Orit ;
Gross-Tsur, Varda .
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM, 2009, 94 (07) :2262-2268
[47]   HAND AND FOOT LENGTH IN PRADER-WILLI SYNDROME [J].
HUDGINS, L ;
CASSIDY, SB .
AMERICAN JOURNAL OF MEDICAL GENETICS, 1991, 41 (01) :5-9
[48]   Thyroid function in patients with Prader-Willi syndrome: an Italian multicenter study of 339 patients [J].
Iughetti, Lorenzo ;
Vivi, Giulia ;
Balsamo, Antonio ;
Corrias, Andrea ;
Crino, Antonino ;
Delvecchio, Maurizio ;
Gargantini, Luigi ;
Greggio, Nella Augusta ;
Grugni, Graziano ;
Hladnik, Uros ;
Pilotta, Alba ;
Ragusa, Letizia ;
Salvatoni, Alessandro ;
Wasniewska, Malgorzata ;
Weber, Giovanna ;
Predieri, Barbara .
JOURNAL OF PEDIATRIC ENDOCRINOLOGY & METABOLISM, 2019, 32 (02) :159-165
[49]   Testosterone Replacement Therapy to Improve Secondary Sexual Characteristics and Body Composition Without Adverse Behavioral Problems in Adult Male Patients With Prader-Willi Syndrome: An Observational Study [J].
Kido, Yasuhiro ;
Sakazume, Satoru ;
Abe, Yoshiko ;
Oto, Yuji ;
Itabashi, Hisashi ;
Shiraishi, Masahisa ;
Yoshino, Atsunori ;
Tanaka, Yuriko ;
Obata, Kazuo ;
Murakami, Nobuyuki ;
Nagai, Toshiro .
AMERICAN JOURNAL OF MEDICAL GENETICS PART A, 2013, 161 (09) :2167-2173
[50]   Successful rapid weight reduction and the use of liraglutide for morbid obesity in adolescent Prader-Willi syndrome [J].
Kim, Yoo-Mi ;
Lee, Yeoun Joo ;
Kim, Soo Yeon ;
Cheon, Chong Kun ;
Lim, Han Hyuk .
ANNALS OF PEDIATRIC ENDOCRINOLOGY & METABOLISM, 2020, 25 (01) :52-56