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

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.

Functional dependence and the mental dimension of quality of life in Hemodialysis patients: the PROHEMO study.

BACKGROUND: Functional dependence is highly prevalent in maintenance hemodialysis (MHD) settings. Also, poor health-related quality of life (HRQoL) and high levels of depressive symptoms have been reported by MHD patients. We investigated associations between functional status and mental aspects of quality of life in Brazilian MHD patients. METHODS: Cross sectional study of 235 patients enrolled in two of the four participating MHD clinics of the Prospective Study of the Prognosis of Chronic Hemodialysis Patients (PROHEMO) in Salvador, BA, Brazil. Data were collected from September 2016 to August 2017. The Katz's questionnaire was used for basic activities of daily living (ADL) and the Lawton-Brody's questionnaire for instrumental activities of daily living (IADL). ADL and IADL scores were combined to create 3 functional status groups: highly dependent (n = 47), moderately dependent (n = 109) and independent (n = 82). The validated Brazilian version of the Kidney Disease Quality of Life Short Form (KDQOL-SF) was used for scores of two distinct HRQoL measures, i.e., the mental component summary (MCS) and the 5-item mental health inventory (MHI-5). We used linear regression to estimate differences in scores with adjustment for possible confounders: months of dialysis, age, gender, other sociodemographic variables, body mass index, type of vascular access, dialysis dose by Kt/V, laboratory variables (albumin, blood hemoglobin, calcium, phosphorus, urea, creatinine and parathyroid hormone) and nine comorbid conditions. RESULTS: Mean age was 51.2 ± 12.4 yr (median age = 51.0 yr), 59.1% were male, 93.2% were non-White. The prevalence of self-reported functional status differed by age: 54.4% for age < 45 yr, 67.8% for age 45-60 yr and 73.9% for age ≥ 60 yr. Using functionally independent as reference, lower scores were observed for highly dependent patients in MCS (difference: -4.69, 95% CI: -8.09, -0.29) and MHI-5 (difference: -5.97, 95% CI: -8.09, -1.29) patients. These differences changed slightly with extensive adjustments for covariates. CONCLUSIONS: Our results call attention to a high prevalence of functional dependence in younger and older MHD patients. The results suggest that the lower self-reported mental quality of life in functionally dependent MHD patients cannot be explained by differences in age and comorbidities.

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

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.

Self-reported fatigue by the Chalder fatigue questionnaire and mortality in Brazilian hemodialysis patients: The PROHEMO.

BACKGROUND: The existing data support the Chalder Fatigue Questionnaire (CFQ-11) as a valid instrument to assess fatigue in maintenance hemodialysis (MHD) patients. The objective of this work was to investigate whether self-reported fatigue can serve as an independent prognostic indicator for mortality in MHD patients. METHODS: The data are from 233 adult patients enrolled in the cohort "The Prospective Study of the Prognosis of Chronic Hemodialysis Patients" (PROHEMO) developed in Salvador, BA, Brazil. The Brazilian version of the validated CFQ-11 was used to calculate self-reported fatigue. The CFQ-11 scores may range from 0 to 33; higher scores represent more fatigue. Fatigue categories were created based on proposed cut point: absence or mild degree if CFQ-11 scores <4 and moderate to severe if scores ≥ 4. Cox models were used to estimate the hazard ratios (HR) and 95% confidence intervals (CI) of associations between fatigue and mortality with adjustments for sociodemographic factors, time on dialysis, education, economic class, hemoglobin concentration, diabetes, heart failure, depression, and other psychiatric disorders. RESULTS: The mean age was 51.5±2.5 years, 58% were male and 30% diabetics. Self-reported moderate to severe fatigue was reported by 71% of patients. The mortality rate was 8.6 cases/100 person-years. Patients with moderate to severe fatigue had a more than threefold mortality rate (HR = 3.07, 95% CI: 1.19, 7.93) compared to patients with absent or mild fatigue, after extensive adjustments for covariates. CONCLUSION: The study provides evidence that self-reported fatigue can help identify MHD patients at higher risk of earlier death.

Mortality, health-related quality of life, and depression symptoms in younger and older men and women undergoing hemodialysis.

BACKGROUND AND OBJECTIVE: Some studies on maintenance hemodialysis (MHD) patients report a longer survival, albeit with poorer health-related quality of life (HRQoL), and more depression symptoms in women than in men. Whether these gender differences vary with age is uncertain. We tested the associations of gender with mortality, depression symptoms, and HRQoL in MHD patients of different age groups. METHODS: We used data from 1504 adult MHD patients enrolled in the PROHEMO, a prospective cohort in Salvador, Brazil. The KDQOL-SF was used for the component summaries of the mental (MCS) and physical (PCS) HRQoL scales. Depression symptoms were assessed by the complete version of the Center for Epidemiological Studies Depression Screening Index (CES-D). To test for gender differences, extensively adjusted linear models were used for depression and HRQoL scores, and Cox models for death hazard ratio (HR). RESULTS: Women reported worse HRQoL than men, particularly for ages ⩾60 years. In the age group ⩾60 years, the adjusted difference (AD) in score was -3.45; 95% CI: -6.81, -0.70 for MCS -3.16; -5.72, -0.60 for PCS. Older (⩾60 years) women also had more depression symptoms (AD 4.98; 2.33, 7.64). Mortality was slightly lower in women than in men with an adjusted HR of 0.89 (0.71, 1.11) and consistent across age categories. CONCLUSIONS: In a sample of Brazilian MHD patients, women had a slightly lower mortality, albeit with more depression symptoms and poorer HRQoL than men, particularly among older patients. This study highlights the need to investigate gender inequalities for MHD patients across different cultures and populations.

Comparison of Urinary Protein/Creatinine Ratio as an Alternative to 24-h Proteinuria in Lupus Nephritis: TUNARI Study.

BACKGROUND: Lupus nephritis (LN) occurs in approximately 50% of people with systemic lupus erythematosus (SLE). The 24-h proteinuria (gold standard) is measured among other tests for the control and monitoring of LN activity. This study investigates the use of the protein/creatinine ratio (PCR) as an alternative for the detection of proteinuria and its accuracy compared to the gold standard in a predominantly non-white population. METHODS: This was a prospective study conducted in Salvador, Brazil, between December 2021 and May 2022. We invited adult patients diagnosed with SLE and LN, regardless of their disease activity. The estimation of the PCR and 24-h proteinuria was performed using conventional methods. The analysis used was Spearman's r correlation coefficient (rs), coefficient of determination (r2), and concordance by the Bland-Altman method. A specific sensitivity was measured by the ROC curve with its respective cut-off by the Youden Index. RESULTS: We compared 112 samples of 75 patients with LN, with a mean age of 34.5 ± 11.8 years. Of these patients, 85% were women, 87.9% were non-white. A high degree of correlation was observed between PCR with 24-h proteinuria (rs = 0.77 and r2 = 0.59). The ROC analysis shows an area under the curve of 0.92 and the cut-off point calculated by the Youden Index was 0.78 with a sensitivity of 90.0% and specificity of 82%. However, the Bland-Altman graph indicated decreasing concordance as the degree of proteinuria increased, despite showing concordance at high levels of proteinuria. CONCLUSION: The PCR shows high sensitivity to follow-up patients with LN when compared with 24-h proteinuria. Our findings suggest that PCR is a useful parameter for the evaluating and monitoring patients in complete remission. However, in cases of partial remission, the utility of PCR is limited.