title: Medical management for adults with Prader-Willi syndrome.
abstract: BACKGROUND
  Prader-Willi syndrome (PWS) is a genetic disorder characterized by morbid obesity resulting from insatiable appetite. Complications like diabetes mellitus or other cardiovascular diseases typically occur during lifetime. Lifestyle advice and education as well as limited caloric intake are the major concepts in assistance of PWS patients.
  
  
  METHODS
  A 12.9-month follow-up study, which included 8 formerly genetically diagnosed PWS patients, was conducted. Physical and laboratory examinations were done at the beginning and at the end of the follow-up. Improved medical care and lifestyle advice were given. The participants lived in an assisted living environment with a fixed daily schedule and limited caloric intake.
  
  
  RESULTS
  The group of patients consisted of manifest diabetics (n=6) as well as nondiabetics (n=2). Therapeutic concepts were simplified in 2 patients by switching from insulin to oral antidiabetics. The reduction in body weight was moderate, but attributable to a loss of fat mass. A significant reduction in blood cholesterol and triglyceride content was achieved along the observational period as well as a significant reduction of glycosylated hemoglobin (HbA1c) in the group of diabetic PWS patients. Systolic blood pressure improved during the study.
  
  
  CONCLUSIONS
  A combination of an assisted living environment following conservative treatments as well as consistent patient care improved the daily and medical situation of PWS patients. Weight loss and, as a consequence, improvements in terms of metabolic condition and blood pressure were observed. A combination of medical and daily routine management should be established in PWS patients to improve their quality of life.
authors.affiliation: 1 Heart and Diabetes Center NRW, Ruhr University Bochum , Bad Oeynhausen, Germany .
authors.forename: M
authors.initials: M
authors.lastname: Hauber
authors.forename: B
authors.initials: B
authors.lastname: Stratmann
authors.forename: N
authors.initials: N
authors.lastname: Hoedebeck-Stuntebeck
authors.forename: D
authors.initials: D
authors.lastname: Tschoepe
mesh_terms: D000328:Adult; D001794:Blood Pressure; D015992:Body Mass Index; D001835:Body Weight; D059168:Carotid Intima-Media Thickness; D002784:Cholesterol / Q000097:blood; D048909:Diabetes Complications / Q000097:blood / Q000628:therapy; D005260:Female; D005500:Follow-Up Studies; D005951:Glucose Tolerance Test; D006442:Glycated Hemoglobin / Q000378:metabolism; D006801:Humans; D007004:Hypoglycemic Agents / Q000627:therapeutic use; D008019:Life Style; D008297:Male; D009767:Obesity, Morbid / Q000097:blood / Q000628:therapy; D011218:Prader-Willi Syndrome / Q000097:blood / Q000628:therapy*; D011788:Quality of Life; D013599:Systole; D014280:Triglycerides / Q000097:blood; D015431:Weight Loss; D055815:Young Adult
chemical_list: D002784:Cholesterol; D006442:Glycated Hemoglobin; D007004:Hypoglycemic Agents; D014280:Triglycerides; C517652:hemoglobin A1c protein, human