RESUMO
Acquired angioedema due to C1-inhibitor (C1-INH) deficiency (AAE-C1-INH) is rare and is associated with underlying lymphoproliferative diseases. C1-INH deficiency may be due to neoplastic over-consumption of C1-INH and the generation of anti-C1-INH autoantibodies. Uncovering an occult malignancy can lead to earlier oncology referral and improvement of angioedema after treatment of the underlying lymphoproliferative disorder. We characterized seven patients with C1-INH-AAE that highlights the importance of recognizing the association between C1-INH-AAE and underlying malignancy. In acute attacks, patients may be resistant to C1-INH therapy due to the presence of anti-C1-INH autoantibodies or rapid complement consumption, and may respond better to icatibant or ecallantide, which directly affect bradykinin. Treatment of the underlying malignancy also improves AAE-C1-INH symptoms and supports the role of lymphoproliferative B cells in AAE-C1-INH pathophysiology. Monitoring levels of C4, C1-INH function and level, and C1q may be predictive of AAE-C1-INH control and be used as surrogates for treatment efficacy. With close monitoring, low-dose danazol can be effective for long-term prophylaxis. Annual evaluation in AAE-C1-INH is recommended if an underlying malignancy is not found, as angioedema may precede the development of malignancy by several years. Our single-center study has aided in standardization of comprehensive AAE-C1-INH diagnosis, treatment, and monitoring strategies towards future therapeutic clinical trials.
Assuntos
Angioedema/patologia , Proteína Inibidora do Complemento C1/genética , Angioedema Hereditário Tipos I e II/genética , Transtornos Linfoproliferativos/patologia , Idoso , Angioedema/genética , Anti-Inflamatórios não Esteroides/uso terapêutico , Autoanticorpos/imunologia , Bradicinina/análogos & derivados , Bradicinina/uso terapêutico , Antagonistas de Receptor B2 da Bradicinina/uso terapêutico , Proteína Inibidora do Complemento C1/imunologia , Complemento C1q/antagonistas & inibidores , Complemento C1q/metabolismo , Feminino , Humanos , Transtornos Linfoproliferativos/genética , Masculino , Pessoa de Meia-Idade , Peptídeos/uso terapêutico , Estudos RetrospectivosRESUMO
OBJECTIVE: Hospital palliative care has been shown to improve quality of life and optimize hospital utilization for seriously ill patients who need intensive care. The present review examined whether hospital palliative care in intensive care (ICU) and non-ICU settings will influence hospital length of stay and in-hospital mortality. METHOD: A systematic search of CINAHL/EBSCO, the Cochrane Library, Google Scholar, MEDLINE/Ovid, PubMed, and the Web of Science through 12 October 2016 identified 16 studies that examined the effects of hospital palliative care and reported on hospital length of stay and in-hospital death. Random-effects pooled odds ratios and mean differences with corresponding 95% confidence intervals were estimated. Heterogeneity was measured by the I 2 test. The Grading of Recommendations Assessment, Development, and Evaluation (GRADE) system was utilized to assess the overall quality of the evidence. RESULTS: Of the reviewed 932 articles found in our search, we reviewed the full text of 76 eligible articles and excluded 60 of those, which resulted in a final total of 16 studies for analysis. Five studies were duplicated with regard to outcomes. A total of 18,330 and 9,452 patients were analyzed for hospital length of stay and in-hospital mortality from 11 and 10 studies, respectively. Hospital palliative care increased mean hospital length of stay by 0.19 days (pooled mean difference = 0.19; 95% confidence interval [CI 95%] = -2.22-2.61 days; p = 0.87; I 2 = 95.88%) and reduced in-hospital mortality by 34% (pooled odds ratio = 0.66; CI 95% = 0.52-0.84; p < 0.01; I 2 = 48.82%). The overall quality of evidence for both hospital length of stay and in-hospital mortality was rated as very low and low, respectively. SIGNIFICANCE OF RESULTS: Hospital palliative care was associated with a 34% reduction of in-hospital mortality but had no correlation with hospital length of stay.
Assuntos
Mortalidade Hospitalar , Tempo de Internação/tendências , Cuidados Paliativos/normas , Humanos , Unidades de Terapia Intensiva/organização & administração , Cuidados Paliativos/tendênciasAssuntos
Meios de Contraste/efeitos adversos , Hipersensibilidade a Drogas/diagnóstico , Hipersensibilidade a Drogas/etiologia , Hipersensibilidade Imediata/diagnóstico , Hipersensibilidade Imediata/etiologia , Imunoglobulina E/imunologia , Compostos Radiofarmacêuticos/efeitos adversos , Hipersensibilidade a Drogas/prevenção & controle , Humanos , Hipersensibilidade Imediata/prevenção & controle , Pré-Medicação , Testes CutâneosRESUMO
Mpox, caused by infection with Monkeypox virus, usually presents as a mild, self-limited illness in immunocompetent persons that resolves within 2-4 weeks. Serious complications have been reported when mpox lesions involve vulnerable anatomic sites, such as the eye, and in those with substantial immunosuppression. We describe a patient with advanced human immunodeficiency virus infection and sustained viral shedding of mpox with ocular involvement, which resulted in vision loss.
RESUMO
Chronic rhinosinusitis (CRS) is common in adults. It is diagnosed based on a high index of suspicion alongside objective means of assessing sinus inflammation. Determining the impact of CRS on patient quality of life is an important starting point for discussions regarding treatment, and is critical for longitudinal assessment of response to specific treatments. CRS can be further categorized by the presence or absence of nasal polyps. Recent Joint Task Force on Practice Parameters Grading of Recommendations Assessment, Development, and Evaluation guidelines for the management of CRS with nasal polyps (CRSwNP) focused on 3 treatment options: intranasal corticosteroids with multiple delivery methods, biologics (monoclonal antibodies targeting type 2 inflammation), and aspirin therapy after desensitization, which only applies to the subset of patients with CRSwNP who experience acute respiratory reactions following nonsteroidal antiinflammatory drug ingestion. The authors of the guidelines made conditional recommendations in favor of each of these 3 treatment options, highlighting the importance of shared decisionmaking when choosing appropriate therapy for individuals with CRSwNP.