Phosphomannomutase deficiency (PMM2-CDG) causes a cerebellar syndrome that has been evaluated using the International Cooperative Ataxia Rating Scale (ICARS). However, no particular dysarthria tests have been used. Sp...
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Phosphomannomutase deficiency (PMM2-CDG) causes a cerebellar syndrome that has been evaluated using the International Cooperative Ataxia Rating Scale (ICARS). However, no particular dysarthria tests have been used. Speech ICARS subscore subjectively assesses fluency and clarity of speech with two items. Repetition of syllables, traditionally used for characterization of ataxic speech, was validated in earlyonset ataxia conditions. We assess the validity of the PATA test (SCA Functional Index [SCAN]) in PMM2-CDG patients. PATA rates from 20 patients were compared with a control population were and correlated with ICARS and neuroimaging. There was a difference between the PATA rate in patients and controls. PATA rate increased with age in controls. In patients, the improvement of PATA rate with age was not significant. In patients, the PATA rate was negatively correlated with the total ICARS score and the Speech ICARS subscore. Regarding neuroimaging, midsaggital vermin relative diameter was positively correlated with PATA results. These last differences were also significant when the results are corrected by age. PATA rate provides an easy measure for a quantitative assessment of dysarthria that may help clinicians to monitor patients' evolution in a regular consultation. It could also be used in PMM2-CDG clinical trials implementing ICARS speech subscore information.
Breast cancer is a very important public health issue, due its high incidence and mortality. Neoadjuvant chemotherapy has proven to be a valid option in the treatment of operable breast cancer and a better aesthetic r...
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Breast cancer is a very important public health issue, due its high incidence and mortality. Neoadjuvant chemotherapy has proven to be a valid option in the treatment of operable breast cancer and a better aesthetic result if breast-tumour ratio is unfavourable. The administration of the therapy itself is complicated, but the difficulty lies mainly in the management of side effects or adverse effects of the treatment. The emergence of a new therapeutic applications need to be constantly reviewed, which combined with the high work loads in the oncology day hospital requires easily managed, standardised and updated methodologies. A method of choice would be the use of clinical pathways that are just emerging in Spain, but could be helpful in managing this disease. The aim of this publication is to share the design of a clinical path for this specific disease with the scientific community. The process of preparing this document has been work of reflection, literature review and unification of criteria that may be used to facilitate work within the Oncology Day Hospital, improving the records of the work involved, especially those related to nursing practice. Their evaluation would enable proposals to be defined for improving and facilitating evidence-based practice. (C) 2009 Elsevier Espana, S.L All rights reserved.
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