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ABSTRACT: El síndrome de hipoventilación alveolar central congénita (SHACC) es un raro trastorno respiratorio del dormir, aunque cada vez más frecuentemente diagnosticado en clínicas de sueño y servicios de neumología pediátrica. Si bien se desconoce su epidemiología, en la literatura médica existen cerca de 300 casos reportados, y su incidencia es de 1 caso por cada 200,000 recién nacidos vivos. Se caracteriza por hipoventilación alveolar que se presenta o empeora durante el sueño. Es secundario a la disminución/ausencia de la respuesta ventilatoria a la hipercapnia o hipoxemia, y en el 90% de los casos es debido a una mutación tipo PARM del gen PHOX2B. Su tratamiento incluye ventilación mecánica y marcapasos diafragmático. Si la terapéutica no se inicia en forma temprana, el paciente desarrollará insuficiencia respiratoria crónica, hipertensión arterial pulmonar, cor pulmonale y la muerte.
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ABSTRACT: Objective: The World Health Organization endorsed the Xpert MTB/RIF assay for the diagnosis of pulmonary tuberculosis, but there is limited information about the utility of this assay for the diagnosis of tuberculous lymphadenitis. Therefore, the objective of this study was to assess the diagnostic accuracy of Xpert MTB/RIF assay in HIV-infected patients with palpable cervical lymph nodes. Study Design: Prospective, diagnostic test study. Methods: Consecutive patients with cervical lymphadenopathy were prospectively enrolled between January 2011 and March 2013. Lymph node specimens were obtained through fine-needle aspiration or excisional biopsy. Mycobacterial culture was considered as the gold standard. Results: Mycobacterium tuberculosis was cultured from 15 of 68 specimens (22.05%), and 53 specimens had negative cultures (77.94%). The sensitivity of Xpert MTB/RIF was 100% (95% CI, 74.65-100%); and the specificity was 100% (95% CI, 91.58-100%). Smear microscopy had a lower diagnostic performance. Conclusion: Although based on a limited sample size, our study indicates that Xpert MTB/RIF is a useful method for the diagnosis of cervical tuberculous lymphadenitis in HIV-infected patients, regardless of the bacillary load in smear positive samples or the CD4 T cell count. The sensitivity, specificity, positive predictive value and negative predictive value were similar to gold-standard culture.
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ABSTRACT: Results supporting the use and the effectiveness of positive expiratory, pressure devices in chronic obstructive pulmonary disease (COPD) patients are still controversial, We have tested the hypothesis that adding TPEP or IPPB to standard pharmacological therapy may provide additional clinical benefit over, pharmacological therapy only in patients with severe COPD. Fourty-five patients were randomized in three groups: a group was treated; with IPPB,a group was treated with TPEP and a group with pharmacological; therapy alone (control group). Primary outcome measures included the measurement of scale or, questionnaire concerning dyspnea (MRC scale),dyspnea,cough, and, sputum (BCSS) and quality of life (COPD assessment test) (CAT). Secondary, outcome measures were respiratory function testing,arterial blood gas,analysis,and hematological examinations. Both patients in the IPPB group and in the TPEP group showed a significant, improvement in two of three tests (MRC,CAT) compared to the control, group.However,in the group comparison analysis for, the same variables between IPPB group and TPEP group we observed a, significant improvement in the IPPB group (P≤.05 for MRC and P≤.01 for, CAT). The difference of action of the two techniques are evident in the results of, pulmonary function testing: IPPB increases FVC, FEV1, and MIP; this reflects, its capacity to increase lung volume. Also TPEP increases FVC and FEV1 (less, than IPPB), but increases MEP, while decreasing total lung capacity and, residual volume. The two techniques (IPPB and TPEP) improves significantly dyspnea; quality of; life tools and lung function in patients with severe COPD. IPPB demonstrated a greater effectiveness to improve dyspnea and quality of life tools (MRC, CAT) than TPEP.
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