Trabalho de evento-resumo periodico

A new scoring system for scaling patient with LHON: getting away from the carrier/affected paradigm (2007)

• Authors:
  • Sadun, A. A
  • Carelli, V
  • Barboni, P
  • Ramos, C. d. V. F - Universidade Federal de São Paulo (UNIFESP)
  • Ventura, Dora Selma Fix
  • Sadun, F
  • Ross-Cisneros, F.N
  • Sherman, J
  • Berezovsky, A - Universidade Federal de São Paulo (UNIFESP)
  • Salomão, S. R - Universidade Federal de São Paulo (UNIFESP)
• Autor USP: VENTURA, DORA SELMA FIX - IP
• Unidade: IP
• Subjects: NERVO ÓPTICO; NEUROFTALMOLOGIA; MITOCÔNDRIAS; OFTALMOPATIAS
• Language: Inglês
• Abstract: Purpose:Recently, we described subclinical visual manifestations in asymptomatic Carriers with Leber’s Hereditary Optic Neuropathy (LHON) found in a giant pedigree from Brazil. What is required is a scoring system that provides a sliding scale of signs and psychophysical measures for all individuals with this type of mtDNA mutation.Methods:We have prospectively studied a pedigree of 332 patients with 11778 LHON (J-haplogroup) seen regularly over the last 6 years with field investigations in Brazil and quantitated several signs and psychophysical features in asymptomatic Carriers. In particular, we have characterized abnormalities regarding examinations of the fundus, visual fields (HVF), color and contrast sensitivity functions, OCT, GDx and a serological marker, neuron specific enolase (NSE).Results:Subclinical or occult abnormalities were found in structural and functional measures of the optic nerve and retina. We designed a 1 to 10 point scoring system to be used on LHON Carriers and Affected patients. This scale was developed by assigning points to each of seven measures (0-3 points for OCT, 0-2 for fundus changes and 0-1 for all other signs and tests). Asymptomatic LHON subjects may show minimal to nil evidence of their disease (a score of 0-1). However, most LHON Carriers demonstrated mild manifestations (2-4), or a pre-conversion accumulation of anomalies (5-7). LHON Affected patients also had a sliding scale of abnormalities (8-10). Conclusions:LHONpatients may show subtle variable expressions of their disease that can be quantitated in contrast to the conventional description of Affected vs. Carrier states. This new paradigm allows for the use of a 0-10 sliding scale, which may prove useful as an outcome measure for both retrospective analysis and clinical trials
• Imprenta:
  • Publisher place: New York
  • Date published: 2007
• Source:
  • Título do periódico: Investigative Ophthalmology & Visual Neuroscience
  • Volume/Número/Paginação/Ano: v. 48, p. 2472-B262, 2007


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How to cite

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• ABNT

  SADUN, A. A et al. A new scoring system for scaling patient with LHON: getting away from the carrier/affected paradigm. Investigative Ophthalmology & Visual Neuroscience. New York: Instituto de Psicologia, Universidade de São Paulo. Disponível em: http://abstracts.iovs.org/cgi/content/abstract/48/5/2472?maxtoshow=&HITS. Acesso em: 09 maio 2024. , 2007

• APA

  Sadun, A. A., Carelli, V., Barboni, P., Ramos, C. d. V. F., Ventura, D. S. F., Sadun, F., et al. (2007). A new scoring system for scaling patient with LHON: getting away from the carrier/affected paradigm. Investigative Ophthalmology & Visual Neuroscience. New York: Instituto de Psicologia, Universidade de São Paulo. Recuperado de http://abstracts.iovs.org/cgi/content/abstract/48/5/2472?maxtoshow=&HITS

• NLM

  Sadun AA, Carelli V, Barboni P, Ramos C d. VF, Ventura DSF, Sadun F, Ross-Cisneros FN, Sherman J, Berezovsky A, Salomão SR. A new scoring system for scaling patient with LHON: getting away from the carrier/affected paradigm [Internet]. Investigative Ophthalmology & Visual Neuroscience. 2007 ; 48 2472-B262.[citado 2024 maio 09 ] Available from: http://abstracts.iovs.org/cgi/content/abstract/48/5/2472?maxtoshow=&HITS

• Vancouver

  Sadun AA, Carelli V, Barboni P, Ramos C d. VF, Ventura DSF, Sadun F, Ross-Cisneros FN, Sherman J, Berezovsky A, Salomão SR. A new scoring system for scaling patient with LHON: getting away from the carrier/affected paradigm [Internet]. Investigative Ophthalmology & Visual Neuroscience. 2007 ; 48 2472-B262.[citado 2024 maio 09 ] Available from: http://abstracts.iovs.org/cgi/content/abstract/48/5/2472?maxtoshow=&HITS

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