Long-term health outcomes in patients with Prader–Willi Syndrome: a nationwide cohort study in Denmark

Autor: N C Kreher, M S Olsen, E Hedgeman, K P Malobisky, M M Braun, S Carter, S P Ulrichsen, J Fryzek
Rok vydání: 2017
Předmět:
Male
Pediatrics
Denmark
Endocrinology
Diabetes and Metabolism
Myocardial Infarction
Medicine (miscellaneous)
Comorbidity
Anxiety
0302 clinical medicine
OBSTRUCTIVE SLEEP-APNEA
Prevalence
030212 general & internal medicine
Young adult
Child
POPULATION
Sleep Apnea
Obstructive
Nutrition and Dietetics
Depression
Sleep apnea
Venous Thromboembolism
Middle Aged
PREVALENCE
OBESITY
Female
DATA QUALITY
Prader-Willi Syndrome
Cohort study
Adult
congenital
hereditary
and neonatal diseases and abnormalities
medicine.medical_specialty
Adolescent
GROWTH-HORMONE TREATMENT
PULMONARY-EMBOLISM
Hypertension
Pulmonary
MEDLINE
Hyperphagia
Young Adult
03 medical and health sciences
medicine
Humans
Obesity
Proportional Hazards Models
VENOUS THROMBOEMBOLISM
business.industry
Proportional hazards model
Infant
nutritional and metabolic diseases
Retrospective cohort study
medicine.disease
BIRTH INCIDENCE
nervous system diseases
Term (time)
Patient Outcome Assessment
RISK-FACTORS
business
030217 neurology & neurosurgery
Zdroj: Hedgeman, E, Ulrichsen, S P, Carter, S, Kreher, N C, Malobisky, K P, Braun, M M, Fryzek, J & Olsen, M S 2017, ' Long-term health outcomes in patients with Prader-Willi Syndrome : a nationwide cohort study in Denmark ', International Journal of Obesity, vol. 41, no. 10, pp. 1531-1538 . https://doi.org/10.1038/ijo.2017.139
ISSN: 1476-5497
0307-0565
DOI: 10.1038/ijo.2017.139
Popis: BACKGROUND: Prader-Willi syndrome (PWS) is a rare congenital disease that affects growth, sexual development, cognitive function and behavior. Individuals exhibit food preoccupation and hyperphagia, which may lead to obesity with premature morbidity and mortality. The aim of this work was to evaluate the risk of venous thromboembolisms (VTEs), myocardial infarction, pulmonary hypertension, sleep apnea, depression, anxiety and all-cause mortality among persons with PWS as compared with an age-and sex-matched general-population cohort.METHODS: All persons diagnosed with PWS (n = 155) were identified in the Danish Health Registries; an age-and sex-matched comparison group was selected from the general population of Denmark (n = 15 500); diseases of interest were identified through the health registry and cause of death register. Follow-up began on date of birth or first medical record availability through to first occurrence of an outcome of interest; follow-up ceased at emigration from Denmark or end of study. Incidence rates (IRs) were calculated and Cox's proportional hazards models were used to understand the relative risk (RR) of disease.RESULTS: The IRs for VTE among patients with PWS was 144 (60-347) per 100 000 person-years. Risks for VTE events and all-cause mortality were 9.4 times (95% confidence interval (CI): 3.7-23.5) and 11.0 times (95% CI: 5.7-21.1) higher, respectively, for patients with PWS versus the general population. Increased risks were also found individually for deep venous thromboses (DVTs) (RR: 9.1; 95% CI: 3.2-25.2), pulmonary embolisms (RR: 11.0; 95% CI: 1.4-86.9), myocardial infarction (RR: 7.2; 95% CI: 1.7-30.2) and anxiety (RR: 2.8; 95% CI: 1.0-7.5). No cases of pulmonary hypertension, sleep apnea or depressive disorders were identified within this PWS cohort.CONCLUSIONS: Multiple cardiovascular and behavioral illnesses are more likely to occur among patients with PWS than within the general population. These increased risks may provide an impetus for enhanced disease prevention, screening, diagnosis and treatment.
Databáze: OpenAIRE
Externí odkaz: