Factores genéticos e inmunológicos en la diabetes mellitus tipo I (insulino-dependiente) : estudio de marcadores predictores de la enfermedad

Tesis inéditas de la Universidad Complutense de Madrid, Facultad de Medicina, 1992.Fac. de MedicinaTRUEProQuestpu

Delayed Leukoencephalopathy: Three Case Reports and a Literature Review

Background: Delayed leukoencephalopathy (DL) is a rare entity associated with cerebral hypoxia and heroin consumption. We describe the clinical course of three cases of DL due to non-heroin drug use. Material and methods: We describe the cases of three DL patients admitted to our hospital in 2012. Discussion: These cases contribute to the aetiological spectrum of DL since multifactorial causes could account for the clinical symptoms

Endocarditis infecciosa aislada de la válvula tricúspide en paciente no adicto a drogas y sin cardiopatía previa predisponente

La endocarditis derecha es excepcional en pacientes no adictos a drogas y sin cardiopatía predisponente previa. En la literatura médica son pocos los casos descritos y su diagnóstico, en ocasiones, supone un reto clínico importante. Presentamos el caso de un paciente de 57 años, sin antecedente de venopunción ni uso de catéteres intravasculares, diagnosticado en nuestro centro de endocarditis de la válvula tricúspide, que era morfológicamente normal, cuya clínica se inició como síndrome febril agudo, púrpura petequial en piernas y oligoartritis. Esta entidad, que generalmente implica un buen pronóstico con buena respuesta al tratamiento médico, presenta ciertas características clínicas comunes (fiebre persistente, lesiones pulmonares, anemia y microhematuria) que pueden ayudar a sospechar el diagnóstico, que, no obstante, se basa en los estudios microbiológicos (Staphylococcus aureus es el germen más frecuente) y el ecocardiograma

Comorbidity negatively influences prognosis in patientswith extrahepatic cholangiocarcinoma

AIM: To study the outcome and prognostic factors in a series of patients with extrahepatic cholangiocarcinoma and determine the impact of comorbidity on survival. METHODS: A retrospective analysis of 68 patients with extrahepatic cholangiocarcinoma (perihilar, n = 37; distal, n = 31) seen at a single tertiary-care institution during the period 1999-2003 was performed. Data on presentation, management, and outcome were assessed by chart review. Pathologic confirmation was obtained in 37 cases (54.4%). Comorbidity was evaluated by using the Charlson comorbidity index (CCI). RESULTS: Mean age at diagnosis was 73.4 ± 11.5 years. Jaundice was the most common symptom presented (86.8%). Median CCI score was 1 (range, 0 to 4). Nineteen patients (27.9%) underwent tumor resection. Palliative biliary drainage was performed in 39 patients (57.4%), and 6 patients (8.8%) received only best supportive care. Tumor-free margin status (R0) was achieved in 15 cases (78.9% of resection group). Baseline serum carbohydrate antigen 19-9 (CA 19-9) level was revealed to be an independent predictor of surgical treatment (P = 0.026). Overall median survival was 3.1 ± 0.9 mo, with 1- and 2-year survival rates of 21% and 7%, respectively. In the univariate analysis, tumor resection, CCI score, and serum CA 19-9 levels correlated significantly with outcome. In the multivariate analysis, only resection (HR 0.10; 95% CI, 0.02-0.51, P = 0.005) and a CCI score ≥ 2 (HR 3.36; 95% CI, 1.0-10.9, P = 0.045) were found to independently predict survival. CONCLUSION: Tumor resection and comorbidity emerged as significant prognostic variables in extrahepatic cholangiocarcinoma. Comorbidity evaluation instruments should be applied in the clinical management of such patients

Cerebral neuroschistosomiasis: a rare clinical presentation and review of the literature

The symptomatic presentation of cerebral schistosomiasis is uncommon. The case of a 25-year-old woman from Equatorial Guinea with headache and seizures secondary to cerebral neuroschistosomiasis, as confirmed by histopathological examination and microbiological study, is presented. A review of the literature on this subject is also provided

Phenotypic and functional evaluation of CD3+CD4-CD8- T cells in human CD8 immunodeficiency

Derechos reservados de Haematologica. Ferrari Storti Foundation http://www.haematologica.org/Background: Human CD8 immunodeficiency is characterized by undetectable CD8(+) lymphocytes and an increased population of CD4(-)CD8(-) (double negative) T lymphocytes. Design and methods: We hypothesized that the double negative subset corresponds to the cellular population that should express CD8 and is committed to the cytotoxic T lymphocyte lineage. To assess this, we determined the phenotype and function of peripheral blood mononuclear cells and/or magnetically isolated double negative T lymphocytes from two CD8-deficient patients. To analyze the expression and co-localization with different organelles, 293T cells were transfected with plasmids bearing wild-type or mutated CD8α. Results: CD8α mutated protein was retained in the cytoplasm of transfected cells. The percentages of double negative cells in patients were lower than the percentages of CD8(+) T cells in healthy controls. Double negative cells mostly had an effector or effector memory phenotype whereas naïve T cells were under-represented. A low concentration of T-cell receptor excision circles together with a skewed T-cell receptor-V repertoire were observed in the double negative population. These data suggest that, in the absence of CD8 co-receptor, the thymic positive selection functions suboptimally and a limited number of mature T-cell clones would emerge from the thymus. In vitro, the double negative cells showed a mild defect in cytotoxic function and decreased proliferative capacity. Conclusions: It is possible that the double negative cells are major histocompatibility complex class-I restricted T cells with cytolytic function. These results show for the first time in humans that the presence of the CD8 co-receptor is dispensable for cytotoxic ability, but that it affects the generation of thymic precursors committed to the cytotoxic T lymphocyte lineage and the proliferation of mature cytotoxic T cells.Ministerio de Educación y CienciaMinisterio de Sanidad y ConsumoDepto. de Inmunología, Oftalmología y ORLFac. de MedicinaTRUEpu