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Lambda light chain-induced monoclonal gammopathy of renal significance, manifesting with Fanconi Syndrome and osteomalacia

Sat, 01 Jan 2022 00:00:00 GMT

Título: Lambda light chain-induced monoclonal gammopathy of renal significance, manifesting with Fanconi Syndrome and osteomalacia Autor(es): Gutiérrez-Peredo, Gabriel Brayan; Oliveira Filho, José César Batista; Montaño-Castellón, Iris; Gutiérrez-Peredo, Andrea Jimena; Crusoé, Edvan de Queiroz; Gusmao-Flores, Dimitri Abstract: Background: Monoclonal gammopathy of renal significance (MGRS) encompasses a heterogeneous group of kidney diseases in which a monoclonal immunoglobulin secreted by a clone of B cells or plasma cells causes kidney damage without meeting the hematological criteria for malignancy. Among the various forms of involvement, MGRS can manifest as a proximal tubule disorder, such as Fanconi syndrome (FS), characterized by urinary loss of phosphate, glucose, amino acids, uric acid and bicarbonate. Few cases of MGRS have been described in the literature, manifesting as FS and monoclonal production of lambda light chains, almost all of which are secondary to the production of kappa light chains. Case presentation: Here we report a clinical case of a 45-year-old Brazilian male, African descent, with proximal weakness of the lower limbs, whose initial assessment showed a urine summary with the presence of proteinuria and glycosuria without hyperglycemia, associated with mild worsening of renal function, hypouricemia, hypocalcemia and phosphaturia. Evolution was characterized by a MGRS manifesting as FS and osteomalacia. Conclusion: The diagnosis of MGRS is not always easy, it requires knowledge of the clinical characteristics, diagnostic criteria and prognosis of each case. Therefore, all possible efforts should be made for multidisciplinary diagnosis. Editora / Evento / Instituição: Programa de Pós-Graduação em Medicina e Saúde (PPgMS), Universidade Federal da Bahia (UFBA) Tipo: Artigo de Periódico

The urine protein/creatinine ratio as a reliable indicator of 24-h urine protein excretion across different levels of renal function and proteinuria: the TUNARI prospective study

Mon, 01 Jan 2024 00:00:00 GMT

Título: The urine protein/creatinine ratio as a reliable indicator of 24-h urine protein excretion across different levels of renal function and proteinuria: the TUNARI prospective study Autor(es): Gutiérrez-Peredo, Gabriel Brayan; Montaño-Castellón, Iris; Gutiérrez-Peredo, Andrea Jimena; Lopes, Marcelo Barreto; Tapioca, Fernanda Pinheiro Martin; Guimaraes, Maria Gabriela Motta; Montaño-Castellón, Sony; Guedes, Sammara Azevedo; Costa, Fernanda Pita Mendes da; Mattoso, Ricardo José Costa; Oliveira Filho, José César Batista; Norris, Keith C.; Almeida, Antonio Raimundo Pinto de; Lopes, Antonio Alberto Abstract: Introdução A excreção urinária de proteína em 24 horas (24-hUP) é o padrão-ouro para a avaliação da proteinúria. Este estudo teve como objetivo avaliar a eficácia diagnóstica da razão proteína/creatinina (PCR) para estimar a 24-hUP em diferentes níveis de função renal e de proteinúria. Métodos Estudo transversal realizado entre dezembro de 2021 e dezembro de 2023, em Salvador, Bahia – Brasil, como uma extensão de dados previamente publicados do estudo TUNARI. Foram incluídas 217 amostras de 152 pacientes com diferentes níveis de função renal e proteinúria. A PCR em amostras isoladas e a 24-hUP foram determinadas por métodos convencionais dentro de um intervalo de 24 horas. Os pacientes foram classificados em três grupos de acordo com o nível de função renal (Grupo 1 = 10 a <30 mL/min; Grupo 2 = 30–60 mL/min; e Grupo 3 = >60 mL/min) e de acordo com o nível de proteinúria (<0,3 g/dia; 0,3–3,5 g/dia; e >3,5 g/dia). Os dados foram analisados utilizando a correlação de Spearman (rs), o coeficiente de determinação (r²), gráficos de Bland–Altman e curva ROC (receiver operating characteristic). Razões de verossimilhança positiva (LR+) e negativa (LR-) foram derivadas da sensibilidade e especificidade da PCR. Resultados A média de idade foi de 41,5 ± 15,7 anos; 61,8% eram mulheres, 36,8% negros e 52% pardos. Glomerulopatias representaram 80,3%, sendo 46,1% com nefrite lúpica. Das amostras totais de urina, observou-se alta correlação entre a PCR e a 24-hUP (rs = 0,86; p < 0,001) em todos os níveis de função renal. No entanto, a concordância entre PCR e 24-hUP foi reduzida nos níveis mais elevados de proteinúria. A análise ROC mostrou uma AUC de 0,95 (IC 95% = 0,92–0,98), com sensibilidade de 91% e especificidade de 86,5% (LR+ 6,7; LR– 0,1), com ponto de corte ótimo de 0,77. Esses resultados foram semelhantes entre os diferentes níveis de função renal. Para proteinúria ≤0,3 g/dia, a sensibilidade foi de 83,3% e a especificidade de 90%, com AUC de 0,85 (IC 95% = 0,71–0,94). Na faixa de 24-hUP >0,3–3,5 g/dia, a sensibilidade foi de 64,1%, especificidade de 84,6% e AUC de 0,76 (IC 95% = 0,67–0,84). A PCR identificou todos os casos com proteinúria >3,5 g/dia. Conclusões A PCR é uma medida adequada para ser usada como indicador da 24-hUP em diferentes níveis de função renal, embora possa apresentar limitações em níveis elevados de proteinúria. A análise da PCR por faixa de proteinúria mostrou que a concordância e a sensibilidade diminuem nos níveis mais altos, mas a especificidade se mantém boa, sendo capaz de identificar proteinúria na faixa nefrótica. Editora / Evento / Instituição: Programa de Pós-Graduação em Medicina e Saúde (PPgMS), Universidade Federal da Bahia (UFBA) Tipo: Artigo de Periódico

CHA2DS2-VASc score as a predictor of cardiovascular and all-cause mortality in a prospective cohort of hemodialysis patients of predominantly African ancestry: The PROHEMO

Wed, 01 Jan 2025 00:00:00 GMT

Título: CHA2DS2-VASc score as a predictor of cardiovascular and all-cause mortality in a prospective cohort of hemodialysis patients of predominantly African ancestry: The PROHEMO Autor(es): Gutiérrez-Peredo, Gabriel Brayan; Gutiérrez-Peredo, Andrea Jimena; Montaño-Castellón, Iris; Silva Neto, Marinho Marques da; Silva, Fernanda Albuquerque da; Martins, Marcia Tereza Silva; Matos, Cacia Mendes; Monteiro, Jean Michell Correia; Silva, Pedro Guimarães; Lopes, Gildete Barreto; Lopes, Marcelo Barreto; Correia, Luis Claudio; Pecoits-Filho, Roberto; Norris, Keith C.; Lopes, Antonio Alberto Abstract: Background: Patients with chronic kidney disease undergoing maintenance hemodialysis (MHD) have an increased mortality. The CHA2DS2-VASc score, initially used for stroke prediction in atrial fibrillation, is relevant for various cardiovascular conditions. This study evaluates its effectiveness in predicting cardiovascular and all-cause mortality in MHD patients. Methods: Data are from the "Prospective Study of the Prognosis of Patients on Chronic Hemodialysis" (PROHEMO) in Salvador, Brazil. Patients were divided by CHA2DS2-VASc scores: ≤2 and >2. Cox regression estimated hazard ratios (HR) for death, both unadjusted and adjusted for confounders. We assessed the distribution of each score variable and its association with mortality. A modified CHA2DS2-VASc score was created due to the low percentage of patients over 75 (1.3%) and normotensive (4.6%). Results: A total of 237 patients (mean age 51.6 years; 57.0% male) were included in the study. There were 55 deaths, 21 from cardiovascular causes. For patients with a CHA2DS2-VASc score >2, the unadjusted hazard of all-cause mortality was doubled (HR = 2.05; 95% CI: 1.20, 3.49) compared to those with a score ≤2, and the risk for cardiovascular deaths was more than threefold (HR = 3.53; 95% CI: 1.46, 8.54). These ratios remained consistent after adjusting for covariates. In the most comprehensive Cox model, the HR for all-cause mortality was 2.43 (95% CI: 1.38, 4.23) and for cardiovascular mortality was 3.52 (95% CI: 1.40, 8.84), similar to results from the modified CHA2DS2-VASc score. Conclusions: The results support the CHA2DS2-VASc score as a practical tool for identifying MHD patients at higher risk of mortality, especially from cardiovascular causes. Editora / Evento / Instituição: Programa de Pós-Graduação em Medicina e Saúde (PPgMS), Universidade Federal da Bahia (UFBA) Tipo: Artigo de Periódico

Fatigue by the Chalder Questionnaire and post-hemodialysis recovery in a population of predominantly African descent: The PROHEMO

Mon, 01 Jan 2024 00:00:00 GMT

Título: Fatigue by the Chalder Questionnaire and post-hemodialysis recovery in a population of predominantly African descent: The PROHEMO Autor(es): Gutiérrez-Peredo, Gabriel Brayan; Martins, Márcia Tereza Silva; Silva, Fernanda Albuquerque da; Lopes, Marcelo Barreto; Lopes, Gildete Barreto; James, Sherman A; Norris, Keith C; Lopes, Antonio Alberto Abstract: Background/objective: A high prevalence of fatigue and a positive association between fatigue and post-hemodialysis recovery have been reported in predominantly white populations of maintenance hemodialysis (MHD) patients. The present study evaluates associations between self-reported fatigue by the 11-item Chalder Fatigue Questionnaire (CFQ-11) and the need for post-hemodialysis recovery in a predominantly African-descent MHD population. Methods: A total of 233 patients (94% Black or Mixed-Race) participating in the "Prospective Study of the Prognosis of Patients on Maintenance Hemodialysis" (PROHEMO), Salvador, Brazil were recruited for this cross-sectional study. The CFQ-11 was used to measure fatigue: <4 for absent or mild, ⩾4 for moderate to severe. Patients were also asked if they needed some time to recover after the hemodialysis. Logistic regression was used to estimate odds ratio (OR) of the association with adjustments for age, sex, race, educational level, economic class level, diabetes, hearth failure, and hemoglobin. Results: Mean age was 51.5 ± 12.5 years. Moderate to severe fatigue (⩾4 points) was observed in 70.8% (165/233), and absent or mild fatigue (<4 points) in 29.2% (68/233). Compared to patients with fatigue scores <4 (20.6%), the need for post-hemodialysis recovery was 2.5 times greater in patients with fatigue scores ⩾4 (52.7%). The covariate-adjusted logistic regression OR was 4.60, 95% CI: 2.27, 9.21. Conclusion: This study in MHD patients of predominantly African descent supports self-reported fatigue assessed by the CFQ-11 as a relevant predictor of the need for post-hemodialysis recovery. The results offer a rationale for investigating whether interventions to prevent fatigue reduce the need of post-hemodialysis recovery. Editora / Evento / Instituição: Programa de Pós-Graduação em Medicina e Saúde (PPgMS), Universidade Federal da Bahia (UFBA) Tipo: Artigo de Periódico