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Data on items of AKUSSI in Alkaptonuria collected over three years from the United Kingdom National Alkaptonuria Centre and the impact of nitisinone.
Griffin, R; Psarelli, E E; Cox, T F; Khedr, M; Milan, A M; Davison, A S; Hughes, A T; Usher, J L; Taylor, S; Loftus, N; Daroszewska, A; West, E; Jones, A; Briggs, M; Fisher, M; McCormick, M; Judd, S; Vinjamuri, S; Sireau, N; Dillon, J P; Devine, J M; Hughes, G; Harrold, J; Barton, G J; Jarvis, J C; Gallagher, J A; Ranganath, L R.
Afiliação
  • Griffin R; Liverpool Cancer Trials Unit, University of Liverpool, Block C, Waterhouse Building, Liverpool L69 3GL, UK.
  • Psarelli EE; Liverpool Cancer Trials Unit, University of Liverpool, Block C, Waterhouse Building, Liverpool L69 3GL, UK.
  • Cox TF; Liverpool Cancer Trials Unit, University of Liverpool, Block C, Waterhouse Building, Liverpool L69 3GL, UK.
  • Khedr M; Department of Clinical Biochemistry and Metabolic Medicine, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK.
  • Milan AM; AKU Society, 66 Devonshire Road, Cambridge, UK.
  • Davison AS; Department of Clinical Biochemistry and Metabolic Medicine, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK.
  • Hughes AT; Liverpool Cancer Trials Unit, University of Liverpool, Block C, Waterhouse Building, Liverpool L69 3GL, UK.
  • Usher JL; Department of Clinical Biochemistry and Metabolic Medicine, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK.
  • Taylor S; Department of Physiotherapy, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK.
  • Loftus N; Department of Physiotherapy, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK.
  • Daroszewska A; Department of Rheumatology, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK.
  • West E; Department of Musculoskeletal Biology, University of Liverpool, L69 7ZX, UK.
  • Jones A; Department of Dermatology, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK.
  • Briggs M; Department of Anaesthesia, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK.
  • Fisher M; Department of Ophthalmology, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK.
  • McCormick M; Department of Cardiology, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK.
  • Judd S; Department of ENT, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK.
  • Vinjamuri S; Department of Dietetics, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK.
  • Sireau N; Department of Nuclear Medicine, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK.
  • Dillon JP; AKU Society, 66 Devonshire Road, Cambridge, UK.
  • Devine JM; Department of Musculoskeletal Biology, University of Liverpool, L69 7ZX, UK.
  • Hughes G; Department of Musculoskeletal Biology, University of Liverpool, L69 7ZX, UK.
  • Harrold J; Department of Psychological Sciences, University of Liverpool, L69 7ZX, UK.
  • Barton GJ; Department of Psychological Sciences, University of Liverpool, L69 7ZX, UK.
  • Jarvis JC; School of Sport and Exercise Science, Liverpool John Moores University, Liverpool, UK.
  • Gallagher JA; School of Sport and Exercise Science, Liverpool John Moores University, Liverpool, UK.
  • Ranganath LR; Department of Musculoskeletal Biology, University of Liverpool, L69 7ZX, UK.
Data Brief ; 20: 1620-1628, 2018 Oct.
Article em En | MEDLINE | ID: mdl-30263914
ABSTRACT
Alkaptonuria is a rare genetic disorder characterized by a high level of circulating (and urine) homogentisic acid (HGA), which contributes to ochronosis when it is deposited in connective tissue as a pigmented polymer. In an observational study carried out by National AKU Centre (NAC) in Liverpool, a total of thirty-nine AKU patients attended yearly visits in varying numbers. At each visit a mixture of clinical, joint and spinal assessments were carried out and the results calculated to yield an AKUSSI (Alkaptonuria Severity Score Index), see "Nitisinone arrests ochronosis and decreases rate of progression of Alkaptonuria evaluation of the effect of nitisinone in the United Kingdom National Alkaptonuria Centre" (Ranganath at el., 2018). The aim of this data article is to produce visual representation of the change in the components of AKUSSI over 3 years, through radar charts. The metabolic effect of nitisinone is shown through box plots.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Tipo de estudo: Observational_studies Idioma: En Revista: Data Brief Ano de publicação: 2018 Tipo de documento: Article País de afiliação: Reino Unido
Texto completo
Imprimir
XML
PubMed Links
Buscar no Google

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Tipo de estudo: Observational_studies Idioma: En Revista: Data Brief Ano de publicação: 2018 Tipo de documento: Article País de afiliação: Reino Unido