title: Duration of Diabetes Predicts Aortic Pulse Wave Velocity and Vascular Events in Alström Syndrome.
abstract: BACKGROUND
  Alström syndrome is characterized by increased risk of cardiovascular disease from childhood.
  
  
  OBJECTIVE
  To explore the association between risk factors for cardiovascular disease, aortic pulse wave velocity, and vascular events in Alström syndrome.
  
  
  METHODS
  Cross-sectional analyses with 5-year follow-up.
  
  
  METHODS
  The UK NHS nationally commissioned specialist clinics for Alström syndrome.
  
  
  METHODS
  Thirty-one Alström patients undertook vascular risk assessment, cardiac studies, and aortic pulse wave velocity measurement. Subsequent clinical outcomes were recorded.
  
  
  METHODS
  Insulin resistance was treated with lifestyle intervention and metformin, and diabetes with the addition of glitazones, glucagon-like peptide 1 agonists, and/or insulin. Thyroid and T deficiencies were corrected. Dyslipidemia was treated with statins and nicotinic acid derivatives. Cardiomyopathy was treated with standard therapy as required.
  
  
  METHODS
  The associations of age, gender, and risk factors for cardiovascular disease with aortic pulse wave velocity were assessed and correlated with the effects of reduction in left ventricular function. Vascular events were monitored for 5 years.
  
  
  RESULTS
  Aortic pulse wave velocity was positively associated with the duration of diabetes (P = .001) and inversely with left ventricular ejection fraction (P = .036). Five of the cohort with cardiovascular events had higher aortic pulse wave velocity (P = .0247), and all had long duration of diabetes.
  
  
  CONCLUSIONS
  Duration of diabetes predicted aortic pulse wave velocity in Alström syndrome, which in turn predicted cardiovascular events. This offers hope of secondary prevention because type 2 diabetes can be delayed or reversed by lifestyle interventions.
authors.affiliation: Diabetes Research Unit (R.B.P., J.S., C.C., S.B.), Horizon Centre, Torbay Hospital NHS Foundation Trust, Lawes Bridge, Torquay, Devon TQ2 7AA, United Kingdom; School of Clinical and Experimental Medicine (T.B.), College of Medicine and Dentistry, University of Birmingham, Edgbaston, Birmingham B15 2TT, United Kingdom; Leeds Children's Hospital (F.C.), Leeds, West Yorkshire LS1 3EX, United Kingdom; Internal Medicine 3 (P.M.), Department of Medicine, University Hospital of Padua, 35122 Padua, Italy; The Jackson Laboratory (J.D.M.), Bar Harbor, Maine 04609; University of Nottingham Medical School (C.P.), Nottingham NG7 2UH, United Kingdom; and Department of Cardiology (R.P.S., N.C.E.), and Department of Endocrinology (T.G.), University Hospitals Birmingham NHS Foundation Trust, Queen Elizabeth Hospital, Queen Elizabeth Medical Centre, Birmingham B15 2TH, United Kingdom.
authors.forename: Richard B
authors.initials: RB
authors.lastname: Paisey
authors.affiliation: Diabetes Research Unit (R.B.P., J.S., C.C., S.B.), Horizon Centre, Torbay Hospital NHS Foundation Trust, Lawes Bridge, Torquay, Devon TQ2 7AA, United Kingdom; School of Clinical and Experimental Medicine (T.B.), College of Medicine and Dentistry, University of Birmingham, Edgbaston, Birmingham B15 2TT, United Kingdom; Leeds Children's Hospital (F.C.), Leeds, West Yorkshire LS1 3EX, United Kingdom; Internal Medicine 3 (P.M.), Department of Medicine, University Hospital of Padua, 35122 Padua, Italy; The Jackson Laboratory (J.D.M.), Bar Harbor, Maine 04609; University of Nottingham Medical School (C.P.), Nottingham NG7 2UH, United Kingdom; and Department of Cardiology (R.P.S., N.C.E.), and Department of Endocrinology (T.G.), University Hospitals Birmingham NHS Foundation Trust, Queen Elizabeth Hospital, Queen Elizabeth Medical Centre, Birmingham B15 2TH, United Kingdom.
authors.forename: Jamie
authors.initials: J
authors.lastname: Smith
authors.affiliation: Diabetes Research Unit (R.B.P., J.S., C.C., S.B.), Horizon Centre, Torbay Hospital NHS Foundation Trust, Lawes Bridge, Torquay, Devon TQ2 7AA, United Kingdom; School of Clinical and Experimental Medicine (T.B.), College of Medicine and Dentistry, University of Birmingham, Edgbaston, Birmingham B15 2TT, United Kingdom; Leeds Children's Hospital (F.C.), Leeds, West Yorkshire LS1 3EX, United Kingdom; Internal Medicine 3 (P.M.), Department of Medicine, University Hospital of Padua, 35122 Padua, Italy; The Jackson Laboratory (J.D.M.), Bar Harbor, Maine 04609; University of Nottingham Medical School (C.P.), Nottingham NG7 2UH, United Kingdom; and Department of Cardiology (R.P.S., N.C.E.), and Department of Endocrinology (T.G.), University Hospitals Birmingham NHS Foundation Trust, Queen Elizabeth Hospital, Queen Elizabeth Medical Centre, Birmingham B15 2TH, United Kingdom.
authors.forename: Catherine
authors.initials: C
authors.lastname: Carey
authors.affiliation: Diabetes Research Unit (R.B.P., J.S., C.C., S.B.), Horizon Centre, Torbay Hospital NHS Foundation Trust, Lawes Bridge, Torquay, Devon TQ2 7AA, United Kingdom; School of Clinical and Experimental Medicine (T.B.), College of Medicine and Dentistry, University of Birmingham, Edgbaston, Birmingham B15 2TT, United Kingdom; Leeds Children's Hospital (F.C.), Leeds, West Yorkshire LS1 3EX, United Kingdom; Internal Medicine 3 (P.M.), Department of Medicine, University Hospital of Padua, 35122 Padua, Italy; The Jackson Laboratory (J.D.M.), Bar Harbor, Maine 04609; University of Nottingham Medical School (C.P.), Nottingham NG7 2UH, United Kingdom; and Department of Cardiology (R.P.S., N.C.E.), and Department of Endocrinology (T.G.), University Hospitals Birmingham NHS Foundation Trust, Queen Elizabeth Hospital, Queen Elizabeth Medical Centre, Birmingham B15 2TH, United Kingdom.
authors.forename: Timothy
authors.initials: T
authors.lastname: Barrett
authors.affiliation: Diabetes Research Unit (R.B.P., J.S., C.C., S.B.), Horizon Centre, Torbay Hospital NHS Foundation Trust, Lawes Bridge, Torquay, Devon TQ2 7AA, United Kingdom; School of Clinical and Experimental Medicine (T.B.), College of Medicine and Dentistry, University of Birmingham, Edgbaston, Birmingham B15 2TT, United Kingdom; Leeds Children's Hospital (F.C.), Leeds, West Yorkshire LS1 3EX, United Kingdom; Internal Medicine 3 (P.M.), Department of Medicine, University Hospital of Padua, 35122 Padua, Italy; The Jackson Laboratory (J.D.M.), Bar Harbor, Maine 04609; University of Nottingham Medical School (C.P.), Nottingham NG7 2UH, United Kingdom; and Department of Cardiology (R.P.S., N.C.E.), and Department of Endocrinology (T.G.), University Hospitals Birmingham NHS Foundation Trust, Queen Elizabeth Hospital, Queen Elizabeth Medical Centre, Birmingham B15 2TH, United Kingdom.
authors.forename: Fiona
authors.initials: F
authors.lastname: Campbell
authors.affiliation: Diabetes Research Unit (R.B.P., J.S., C.C., S.B.), Horizon Centre, Torbay Hospital NHS Foundation Trust, Lawes Bridge, Torquay, Devon TQ2 7AA, United Kingdom; School of Clinical and Experimental Medicine (T.B.), College of Medicine and Dentistry, University of Birmingham, Edgbaston, Birmingham B15 2TT, United Kingdom; Leeds Children's Hospital (F.C.), Leeds, West Yorkshire LS1 3EX, United Kingdom; Internal Medicine 3 (P.M.), Department of Medicine, University Hospital of Padua, 35122 Padua, Italy; The Jackson Laboratory (J.D.M.), Bar Harbor, Maine 04609; University of Nottingham Medical School (C.P.), Nottingham NG7 2UH, United Kingdom; and Department of Cardiology (R.P.S., N.C.E.), and Department of Endocrinology (T.G.), University Hospitals Birmingham NHS Foundation Trust, Queen Elizabeth Hospital, Queen Elizabeth Medical Centre, Birmingham B15 2TH, United Kingdom.
authors.forename: Pietro
authors.initials: P
authors.lastname: Maffei
authors.affiliation: Diabetes Research Unit (R.B.P., J.S., C.C., S.B.), Horizon Centre, Torbay Hospital NHS Foundation Trust, Lawes Bridge, Torquay, Devon TQ2 7AA, United Kingdom; School of Clinical and Experimental Medicine (T.B.), College of Medicine and Dentistry, University of Birmingham, Edgbaston, Birmingham B15 2TT, United Kingdom; Leeds Children's Hospital (F.C.), Leeds, West Yorkshire LS1 3EX, United Kingdom; Internal Medicine 3 (P.M.), Department of Medicine, University Hospital of Padua, 35122 Padua, Italy; The Jackson Laboratory (J.D.M.), Bar Harbor, Maine 04609; University of Nottingham Medical School (C.P.), Nottingham NG7 2UH, United Kingdom; and Department of Cardiology (R.P.S., N.C.E.), and Department of Endocrinology (T.G.), University Hospitals Birmingham NHS Foundation Trust, Queen Elizabeth Hospital, Queen Elizabeth Medical Centre, Birmingham B15 2TH, United Kingdom.
authors.forename: Jan D
authors.initials: JD
authors.lastname: Marshall
authors.affiliation: Diabetes Research Unit (R.B.P., J.S., C.C., S.B.), Horizon Centre, Torbay Hospital NHS Foundation Trust, Lawes Bridge, Torquay, Devon TQ2 7AA, United Kingdom; School of Clinical and Experimental Medicine (T.B.), College of Medicine and Dentistry, University of Birmingham, Edgbaston, Birmingham B15 2TT, United Kingdom; Leeds Children's Hospital (F.C.), Leeds, West Yorkshire LS1 3EX, United Kingdom; Internal Medicine 3 (P.M.), Department of Medicine, University Hospital of Padua, 35122 Padua, Italy; The Jackson Laboratory (J.D.M.), Bar Harbor, Maine 04609; University of Nottingham Medical School (C.P.), Nottingham NG7 2UH, United Kingdom; and Department of Cardiology (R.P.S., N.C.E.), and Department of Endocrinology (T.G.), University Hospitals Birmingham NHS Foundation Trust, Queen Elizabeth Hospital, Queen Elizabeth Medical Centre, Birmingham B15 2TH, United Kingdom.
authors.forename: Christopher
authors.initials: C
authors.lastname: Paisey
authors.affiliation: Diabetes Research Unit (R.B.P., J.S., C.C., S.B.), Horizon Centre, Torbay Hospital NHS Foundation Trust, Lawes Bridge, Torquay, Devon TQ2 7AA, United Kingdom; School of Clinical and Experimental Medicine (T.B.), College of Medicine and Dentistry, University of Birmingham, Edgbaston, Birmingham B15 2TT, United Kingdom; Leeds Children's Hospital (F.C.), Leeds, West Yorkshire LS1 3EX, United Kingdom; Internal Medicine 3 (P.M.), Department of Medicine, University Hospital of Padua, 35122 Padua, Italy; The Jackson Laboratory (J.D.M.), Bar Harbor, Maine 04609; University of Nottingham Medical School (C.P.), Nottingham NG7 2UH, United Kingdom; and Department of Cardiology (R.P.S., N.C.E.), and Department of Endocrinology (T.G.), University Hospitals Birmingham NHS Foundation Trust, Queen Elizabeth Hospital, Queen Elizabeth Medical Centre, Birmingham B15 2TH, United Kingdom.
authors.forename: Richard P
authors.initials: RP
authors.lastname: Steeds
authors.affiliation: Diabetes Research Unit (R.B.P., J.S., C.C., S.B.), Horizon Centre, Torbay Hospital NHS Foundation Trust, Lawes Bridge, Torquay, Devon TQ2 7AA, United Kingdom; School of Clinical and Experimental Medicine (T.B.), College of Medicine and Dentistry, University of Birmingham, Edgbaston, Birmingham B15 2TT, United Kingdom; Leeds Children's Hospital (F.C.), Leeds, West Yorkshire LS1 3EX, United Kingdom; Internal Medicine 3 (P.M.), Department of Medicine, University Hospital of Padua, 35122 Padua, Italy; The Jackson Laboratory (J.D.M.), Bar Harbor, Maine 04609; University of Nottingham Medical School (C.P.), Nottingham NG7 2UH, United Kingdom; and Department of Cardiology (R.P.S., N.C.E.), and Department of Endocrinology (T.G.), University Hospitals Birmingham NHS Foundation Trust, Queen Elizabeth Hospital, Queen Elizabeth Medical Centre, Birmingham B15 2TH, United Kingdom.
authors.forename: Nicola C
authors.initials: NC
authors.lastname: Edwards
authors.affiliation: Diabetes Research Unit (R.B.P., J.S., C.C., S.B.), Horizon Centre, Torbay Hospital NHS Foundation Trust, Lawes Bridge, Torquay, Devon TQ2 7AA, United Kingdom; School of Clinical and Experimental Medicine (T.B.), College of Medicine and Dentistry, University of Birmingham, Edgbaston, Birmingham B15 2TT, United Kingdom; Leeds Children's Hospital (F.C.), Leeds, West Yorkshire LS1 3EX, United Kingdom; Internal Medicine 3 (P.M.), Department of Medicine, University Hospital of Padua, 35122 Padua, Italy; The Jackson Laboratory (J.D.M.), Bar Harbor, Maine 04609; University of Nottingham Medical School (C.P.), Nottingham NG7 2UH, United Kingdom; and Department of Cardiology (R.P.S., N.C.E.), and Department of Endocrinology (T.G.), University Hospitals Birmingham NHS Foundation Trust, Queen Elizabeth Hospital, Queen Elizabeth Medical Centre, Birmingham B15 2TH, United Kingdom.
authors.forename: Susan
authors.initials: S
authors.lastname: Bunce
authors.affiliation: Diabetes Research Unit (R.B.P., J.S., C.C., S.B.), Horizon Centre, Torbay Hospital NHS Foundation Trust, Lawes Bridge, Torquay, Devon TQ2 7AA, United Kingdom; School of Clinical and Experimental Medicine (T.B.), College of Medicine and Dentistry, University of Birmingham, Edgbaston, Birmingham B15 2TT, United Kingdom; Leeds Children's Hospital (F.C.), Leeds, West Yorkshire LS1 3EX, United Kingdom; Internal Medicine 3 (P.M.), Department of Medicine, University Hospital of Padua, 35122 Padua, Italy; The Jackson Laboratory (J.D.M.), Bar Harbor, Maine 04609; University of Nottingham Medical School (C.P.), Nottingham NG7 2UH, United Kingdom; and Department of Cardiology (R.P.S., N.C.E.), and Department of Endocrinology (T.G.), University Hospitals Birmingham NHS Foundation Trust, Queen Elizabeth Hospital, Queen Elizabeth Medical Centre, Birmingham B15 2TH, United Kingdom.
authors.forename: Tarekegn
authors.initials: T
authors.lastname: Geberhiwot
mesh_terms: D000293:Adolescent; D000328:Adult; D056769:Alstrom Syndrome / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology*; D002318:Cardiovascular Diseases / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D002648:Child; D003430:Cross-Sectional Studies; D003924:Diabetes Mellitus, Type 2 / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D011379:Prognosis; D063177:Pulse Wave Analysis; D013997:Time Factors; D055815:Young Adult