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Wilson's disease in southern Brazil: a 40-year follow-up study

Overview of attention for article published in Clinics, January 2011
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Title
Wilson's disease in southern Brazil: a 40-year follow-up study
Published in
Clinics, January 2011
DOI 10.1590/s1807-59322011000300008
Pubmed ID
Authors

Ricardo Schmitt de, Dominique Araujo Muzzillo, Marta Mitiko Deguti, Egberto Reis Barbosa, Lineu César Werneck, Hélio Afonso Ghizoni Teive

Abstract

Long-term data on the clinical follow-up and the treatment effectiveness of Wilson's disease are limited because of the low disease frequency. This study evaluated a retrospective cohort of Wilson's disease patients from southern Brazil during a 40-year follow-up period. Thirty-six Wilson's disease patients, diagnosed from 1971 to 2010, were retrospectively evaluated according to their clinical presentation, epidemiological and social features, response to therapy and outcome. Examining the patients' continental origins showed that 74.5% had a European ancestor. The mean age at the initial symptom presentation was 23.3 ± 9.3 years, with a delay of 27.5 ± 41.9 months until definitive diagnosis. At presentation, hepatic symptoms were predominant (38.9%), followed by mixed symptoms (hepatic and neuropsychiatric) (30.6%) and neuropsychiatric symptoms (25%). Kayser-Fleischer rings were identified in 55.6% of patients, with a higher frequency among those patients with neuropsychiatric symptoms (77.8%). Eighteen patients developed neuropsychiatric features, most commonly cerebellar syndrome. Neuroradiological imaging abnormalities were observed in 72.2% of these patients. Chronic liver disease was detected in 68% of the patients with hepatic symptoms. 94.2% of all the patients were treated with D-penicillamine for a mean time of 129.9 ± 108.3 months. Other treatments included zinc salts, combined therapy and liver transplantation. After initiating therapy, 78.8% of the patients had a stable or improved outcome, and the overall survival rate was 90.1%. This study is the first retrospective description of a population of Wilson's disease patients of mainly European continental origin who live in southern Brazil. Wilson's disease is treatable if correctly diagnosed, and an adequate quality of life can be achieved, resulting in a long overall survival.

Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 52 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Brazil 2 4%
Unknown 50 96%

Demographic breakdown

Readers by professional status Count As %
Student > Master 9 17%
Student > Postgraduate 6 12%
Student > Ph. D. Student 6 12%
Student > Bachelor 4 8%
Other 4 8%
Other 9 17%
Unknown 14 27%
Readers by discipline Count As %
Medicine and Dentistry 20 38%
Neuroscience 5 10%
Nursing and Health Professions 3 6%
Agricultural and Biological Sciences 2 4%
Pharmacology, Toxicology and Pharmaceutical Science 2 4%
Other 5 10%
Unknown 15 29%
Attention Score in Context

Attention Score in Context

This research output has an Altmetric Attention Score of 1. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 02 October 2016.
All research outputs
#20,655,488
of 25,371,288 outputs
Outputs from Clinics
#861
of 1,215 outputs
Outputs of similar age
#170,819
of 190,474 outputs
Outputs of similar age from Clinics
#60
of 77 outputs
Altmetric has tracked 25,371,288 research outputs across all sources so far. This one is in the 10th percentile – i.e., 10% of other outputs scored the same or lower than it.
So far Altmetric has tracked 1,215 research outputs from this source. They typically receive a little more attention than average, with a mean Attention Score of 7.2. This one is in the 17th percentile – i.e., 17% of its peers scored the same or lower than it.
Older research outputs will score higher simply because they've had more time to accumulate mentions. To account for age we can compare this Altmetric Attention Score to the 190,474 tracked outputs that were published within six weeks on either side of this one in any source. This one is in the 5th percentile – i.e., 5% of its contemporaries scored the same or lower than it.
We're also able to compare this research output to 77 others from the same source and published within six weeks on either side of this one. This one is in the 12th percentile – i.e., 12% of its contemporaries scored the same or lower than it.