[Care continuity for patients with Prader-Willi syndrome during transition from childhood to adulthood].
Prader-Willi syndrome(PWS) is a complex multisystem genetic disorder, of which characteristic phenotypes include neonatal hypotonia, hyperphagia resulting in obesity, mental retardation, hypogonadism, and behavioral and psychiatric problems. The diagnosis can be obtained as early as during neonatal period thanks to development of genetic testing. Clinical features of PWS will change depending on age, although core phenotypes of hyperphagia, obesity and psychiatric issues stay for lifetime. Therefore, integrated multidisciplinary approach starting from neonatal period is mandatory to ensure optimal management to improve lifelong quality of life. For successful transition from childhood to adulthood, multidisciplinary team need to share clinical information, and should keep the same policy about food, environment and psychiatric issues.
Saitoh
Shinji
S
Department of Pediatrics, Hokkaido University Graduate School of Medicine.
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003266:Continuity of Patient Care; D005260:Female; D005820:Genetic Testing; D006801:Humans; D006963:Hyperphagia; D007223:Infant; D007231:Infant, Newborn; D033183:Interdisciplinary Communication; D008297:Male; D009123:Muscle Hypotonia; D009765:Obesity; D010348:Patient Care Team; D011218:Prader-Willi Syndrome / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology / Q000628:therapy*; D012017:Referral and Consultation; D055815:Young Adult