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1.
J Ren Nutr ; 33(1): 140-146, 2023 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-35367358

RESUMO

OBJECTIVES: The aims of this study are to analyze the prevalence of malnutrition in hemodialysis (HD) patients in Spain, and to assess the association of malnutrition in these patients with sociodemographic characteristics, comorbidity, and parameters related to HD. DESIGN AND METHODS: A multicenter, retrospective, cross-sectional study in HD patients from centers all over Spain was conducted. Nutritional status of patients was assessed using Malnutrition Inflammation Score (MIS), and was stratified according to MIS values into 5 categories: ≤2, normal nutrition; >2 to ≤5, mild malnutrition or risk of malnutrition; >5 to ≤7, moderate malnutrition; >7 to ≤10, severe malnutrition, and >10, extreme malnutrition. RESULTS: A total of 52 Spanish HD Units participated in the study enrolling 2,748 patients. Mean age of patients was 68.20 ± 14.24 years, 1,811 (65.9%) were men. Mean time on HD was 55.63 ± 63.25 months. Using an MIS cut-off point of 2 for malnutrition, 89% of patients were malnourished (MIS > 2). However, with a cut-off point of 5, more commonly described in the literature, the percentage of patients with malnutrition was reduced to 51.7%. Using this cut-off, we observed significant differences between patients with malnutrition and normo-nourished patients in biochemical parameters, age, Charlson Index, HD residual renal function, scheme, and vascular access (permanent catheter vs arteriovenous fistula). A multivariate regression analysis showed that age, sex, HD scheme, vascular access, residual renal function, and comorbidity index were predictive factors for malnutrition. We found that a high percentage of HD patients with malnutrition did not receive oral supplementation. CONCLUSIONS: The prevalence of malnutrition in HD patients in Spain, assessed using the MIS scale, was high. Higher malnutrition was associated with the use of catheter versus fistula, and standard HD versus online hemodiafiltration, and with the absence of residual renal function, older age, greater comorbidity, and male sex. Malnourished patients had a low rate of oral supplementation.


Assuntos
Desnutrição , Humanos , Masculino , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Feminino , Estudos Transversais , Estudos Retrospectivos , Desnutrição/epidemiologia , Estado Nutricional , Inflamação/epidemiologia , Diálise Renal
2.
J Clin Immunol ; 40(6): 851-860, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32594341

RESUMO

Although recurrent infections in Rubinstein-Taybi syndrome (RSTS) are common, and probably multifactorial, immunological abnormalities have not been extensively described with only isolated cases or small case series of immune deficiency and dysregulation having been reported. The objective of this study was to investigate primary immunodeficiency (PID) and immune dysregulation in an international cohort of patients with RSTS. All published cases of RSTS were identified. The corresponding authors and researchers involved in the diagnosis of inborn errors of immunity or genetic syndromes were contacted to obtain up-to-date clinical and immunological information. Ninety-seven RSTS patients were identified. For 45 patients, we retrieved data from the published reports while for 52 patients, a clinical update was provided. Recurrent or severe infections, autoimmune/autoinflammatory complications, and lymphoproliferation were observed in 72.1%, 12.3%, and 8.2% of patients. Syndromic immunodeficiency was diagnosed in 46.4% of individuals. Despite the broad heterogeneity of immunodeficiency disorders, antibody defects were observed in 11.3% of subjects. In particular, these patients presented hypogammaglobulinemia associated with low B cell counts and reduction of switched memory B cell numbers. Immunoglobulin replacement therapy, antibiotic prophylaxis, and immunosuppressive treatment were employed in 16.4%, 8.2%, and 9.8% of patients, respectively. Manifestations of immune dysfunctions, affecting mostly B cells, are more common than previously recognized in patients with RSTS. Full immunological assessment is warranted in these patients, who may require detailed investigation and specific supportive treatment. Graphical Abstract.


Assuntos
Doenças do Sistema Imunitário/epidemiologia , Doenças do Sistema Imunitário/etiologia , Síndrome de Rubinstein-Taybi/complicações , Síndrome de Rubinstein-Taybi/epidemiologia , Adolescente , Adulto , Autoimunidade , Linfócitos B/imunologia , Linfócitos B/metabolismo , Biomarcadores , Criança , Pré-Escolar , Estudos de Coortes , Suscetibilidade a Doenças/imunologia , Feminino , Estudos de Associação Genética , Humanos , Doenças do Sistema Imunitário/diagnóstico , Isotipos de Imunoglobulinas/sangue , Isotipos de Imunoglobulinas/imunologia , Incidência , Lactente , Masculino , Pessoa de Meia-Idade , Mutação , Fenótipo , Prevalência , Linfócitos T/imunologia , Linfócitos T/metabolismo , Adulto Jovem
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