Data in Brief (Oct 2018)
Data on items of AKUSSI in Alkaptonuria collected over three years from the United Kingdom National Alkaptonuria Centre and the impact of nitisinone
- R. Griffin,
- E.E. Psarelli,
- T.F. Cox,
- M. Khedr,
- A.M. Milan,
- A.S. Davison,
- A.T. Hughes,
- J.L. Usher,
- S. Taylor,
- N. Loftus,
- A. Daroszewska,
- E. West,
- A. Jones,
- M. Briggs,
- M. Fisher,
- M. McCormick,
- S. Judd,
- S. Vinjamuri,
- N. Sireau,
- J.P. Dillon,
- J.M. Devine,
- G. Hughes,
- J. Harrold,
- G.J. Barton,
- J.C. Jarvis,
- J.A. Gallagher,
- L.R. Ranganath
Affiliations
- R. Griffin
- Liverpool Cancer Trials Unit, University of Liverpool, Block C, Waterhouse Building, Liverpool L69 3GL, UK
- E.E. Psarelli
- Liverpool Cancer Trials Unit, University of Liverpool, Block C, Waterhouse Building, Liverpool L69 3GL, UK
- T.F. Cox
- Liverpool Cancer Trials Unit, University of Liverpool, Block C, Waterhouse Building, Liverpool L69 3GL, UK
- M. Khedr
- Department of Clinical Biochemistry and Metabolic Medicine, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK
- A.M. Milan
- AKU Society, 66 Devonshire Road, Cambridge, UK
- A.S. Davison
- Department of Clinical Biochemistry and Metabolic Medicine, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK
- A.T. Hughes
- Liverpool Cancer Trials Unit, University of Liverpool, Block C, Waterhouse Building, Liverpool L69 3GL, UK
- J.L. Usher
- Department of Clinical Biochemistry and Metabolic Medicine, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK
- S. Taylor
- Department of Physiotherapy, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK
- N. Loftus
- Department of Physiotherapy, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK
- A. Daroszewska
- Department of Rheumatology, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK; Department of Musculoskeletal Biology, University of Liverpool, L69 7ZX, UK
- E. West
- Department of Dermatology, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK
- A. Jones
- Department of Anaesthesia, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK
- M. Briggs
- Department of Ophthalmology, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK
- M. Fisher
- Department of Cardiology, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK
- M. McCormick
- Department of ENT, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK
- S. Judd
- Department of Dietetics, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK
- S. Vinjamuri
- Department of Nuclear Medicine, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK
- N. Sireau
- AKU Society, 66 Devonshire Road, Cambridge, UK
- J.P. Dillon
- Department of Musculoskeletal Biology, University of Liverpool, L69 7ZX, UK
- J.M. Devine
- Department of Musculoskeletal Biology, University of Liverpool, L69 7ZX, UK
- G. Hughes
- Department of Psychological Sciences, University of Liverpool, L69 7ZX, UK
- J. Harrold
- Department of Psychological Sciences, University of Liverpool, L69 7ZX, UK
- G.J. Barton
- School of Sport and Exercise Science, Liverpool John Moores University, Liverpool, UK
- J.C. Jarvis
- School of Sport and Exercise Science, Liverpool John Moores University, Liverpool, UK
- J.A. Gallagher
- Department of Musculoskeletal Biology, University of Liverpool, L69 7ZX, UK
- L.R. Ranganath
- Department of Clinical Biochemistry and Metabolic Medicine, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK; Corresponding author.
- Journal volume & issue
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Vol. 20
pp. 1620 – 1628
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.
