Predicting Future Renal Function Decline in Patients with Autosomal Dominant Polycystic Kidney Disease Using Mayo Clinic Classification.

INTRODUCTION: Mayo clinic classification (MCC) has been proposed in patients with autosomal dominant polycystic kidney disease (ADPKD) to identify who may experience a rapid decline of renal function. Our aim was to validate this predictive model in a population from southern Spain. METHODS: ADPKD patients with measurements of height-adjusted total kidney volume (HtTKV) and baseline estimated glomerular filtration rate (eGFR) >30 mL/min/1.73 m2 were selected. Last eGFR was estimated with Mayo Clinic (MC) equation and bias and accuracy were studied. We also analyzed predictive capacity of MCC classes using survival analysis and Cox regression models. RESULTS: We included 134 patients with a mean follow-up of 82 months. While baseline eGFR was not different between classes, last eGFR decreased significantly with them. eGFR variation rate was different according to the MCC class with a more rapid decline in 1C, 1D, and 1E classes. Final eGFR predicted was not significantly different from the real one, with an absolute bias of 0.6 ± 17.0 mL/min/1.73 m2. P10 accuracy was low ranging from 37.5 to 59.5% in classes 1C, 1D, and 1E. Using MC equation, the rate of eGFR decline was underestimated in 1C, 1D, and 1E classes. Cox regression analysis showed that MCC class is a predictor of renal survival after adjusting with baseline eGFR, age, sex, and HtTKV, with 1D and 1E classes having the worst prognosis. CONCLUSION: MCC classification is able to identify patients who will undergo a more rapid decline of renal function in a Spanish population. Prediction of future eGFR with MC equation is acceptable as a group, although it shows a loss of accuracy considering individual values. The rate of eGFR decline calculated using MC equation can underestimate the real rate presented by patients of 1C, 1D, and 1E classes.

Hypocitraturia is present when renal function is impaired in diverse nephropathies and is not related with serum bicarbonate levels.

BACKGROUND: In autosomal dominant polycystic kidney disease (ADPKD) it is frequently found a reduction in urinary citrate of unknown origin. It has been suggested that it could be a marker of acid retention in chronic kidney disease. Our aim was to compare urinary citrate in ADPKD with other nephropathies and to show its relation with serum bicarbonate. METHODS: We determined urinary citrate in patients with several nephropathies and varied renal function. We included 291 patients, 119 with glomerular diseases, 116 with ADPKD, 21 with other nephropathies, and 35 patients with normal renal function. RESULTS: Urinary citrate was higher in women and in patients with normal renal function. ADPKD patients showed similar values of urinary citrate to patients with glomerular diseases and with other nephropathies. We observed a progressive reduction in urinary citrate with renal impairment, in a comparable way among patients with ADPKD and glomerular diseases. We did not observe a relationship with serum bicarbonate. Serum uric acid levels were significantly higher in patients with glomerular diseases than in ADPKD patients, even after correction with the degree of renal function. CONCLUSIONS: Hypocitraturia is not specific of ADPKD but it is also present in all tested nephropathies and is related with renal impairment and not with serum bicarbonate. It could be interesting to study urinary citrate as a marker of renal function and as a prognostic factor.

Urinary citrate as a marker of renal function in patients with autosomal dominant polycystic kidney disease.

INTRODUCTION: Autosomal dominant polycystic kidney disease (ADPKD) is frequent to find low urinary citrate levels. Recently, it has been suggested that urinary citrate could be a marker of covert metabolic acidosis in chronic kidney disease. OBJECTIVE: Our aim was to analyze relationship between urinary citrate levels, renal function, and serum bicarbonate in ADPKD patients. METHODS: We determined citrate in 24-h collected urine from ADPKD patients and correlated with glomerular filtration rate (CKD-EPI equation) and serum bicarbonate concentration. RESULTS: We included 120 patients, 60% men, eGFR was 71 ± 32 mL/min/1.73 m2. Urinary citrate/creatinine ratio was 195 ± 152 mg/gCr (range 1.2-689) with levels significantly higher in females. Urinary citrate lower than 300 mg/gCr was present in 75% of patients and when considering chronic kidney stages (CKD), we observed reduced levels in 48.8% in CKD1 stage, in 79.4% in CKD2 stage, in 96.2% in CKD3 stage, and in 94.7% of patients in CKD4 stage. Urinary citrate was correlated with serum creatinine (r = - 0.61, p < 0.001) and eGFR (r = 0.55, p < 0.001) in both gender. We did not find any correlation with serum bicarbonate. Using a general linear modeling analysis, we found as predictors of urinary citrate/creatinine ratio to glomerular filtration rate, gender, and age. Lower levels of urinary citrate were accompanied by a decline in urinary osmolality and in renal excretion of calcium and uric acid. In a subgroup of patients, we measured total kidney volume and we found an inverse correlation with urinary citrate levels that disappeared when it was corrected with glomerular filtration rate. CONCLUSIONS: Urinary citrate is very frequently reduced in ADPKD patients being present from very early CKD stages. Their levels in urine are inversely correlated with glomerular filtration rate and it is not related with serum bicarbonate concentration. We think that it would be interesting to study urinary citrate as a marker of chronic kidney disease in ADPKD patients.

Infiltración renal linfomatosa en paciente con síndrome nefrótico / Renal lymphomatous infiltration in patient with nefrotic syndrome

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Consecuencias renales del uso de esteroides anabolizantes y práctica de culturismo / Kidney damage due to the use of anabolic androgenic steroides and practice of bodybuilding

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Treatment by long haemodialysis sessions with high cut-off filters in myeloma cast nephropathy: our experience.

UNLABELLED: Multiple myeloma (MM) is the uncontrolled proliferation of plasma cells with variable amounts of production of immunoglobulins or their chains. Acute renal failure can be a symptom of MM, and it is sometimes its form of presentation. Circulating free light chains (FLC) could lead to renal failure due to their intratubular precipitation, causing a cast nephropathy. The treatment of myeloma, adequate hydration and the removal of FLC by apheresis techniques are currently the treatments that are accepted for this disease. Several apheresis techniques have been attempted for the removal of FLC, with long haemodialysis sessions with filters for the removal of these light chains (high cut-off filters) being proposed as the most effective treatment for myeloma nephropathy. METHODS: We report 5 cases of myeloma nephropathy: three had cast nephropathy (CN) diagnosed by renal biopsy and the other two had a high probability of CN (FLC levels >500 mg/l). They were treated with long haemodialysis sessions with a high cut-off membrane. All patients had suffered acute renal failure; four required renal replacement therapy and one patient had advanced renal failure. In all patients, FLC levels were very high. They received specific treatment for myeloma in addition to high cut-off haemodialysis until they achieved FLC levels of <500 mg/l. RESULTS: Four of the five patients recovered renal function, and became independent of dialysis. The progression time for myeloma from the time the first symptoms appeared varied (1-6 months). The number of treatment sessions ranged from 8-16. The patient with the longest progression time required more sessions and did not recover renal function. CONCLUSIONS: Long haemodialysis sessions with high cut-off filters in addition to specific myeloma chemotherapy seems to be an effective treatment for acute renal failure due to myeloma nephropathy. The early initiation of treatment could be a determining factor for the response.

Nefropatía IgA: efectos a corto plazo del tratamiento con prednisona sobre la proteinuria, función renal y relación con la clasificación de Oxford / IgA nephropathy: Short term effects of prednisone treatment on proteinuria, renal function and relation with Oxford classification

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El índice de resistencia vascular renal no tiene implicaciones pronósticas en el trasplante renal / Vascular renal resistance index is not related with prognosis in kidney transplantation

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Osmolalidad urinaria en pacientes con poliquistosis renal: ¿medida o calculada? / Urinary osmolality in patients with polycystic kidney disease: Measured or calculated?

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Valoración del flujo de la fístula arteriovenosa en pacientes en tratamiento con quelantes con calcio / Evaluation of arteriovenous fistula blood flow in patients with calcium chelation therapy

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Tratamiento con hemodiálisis larga con filtros de alto cut-off en la nefropatía por cilindros del mieloma: nuestra experiencia / Treatment by long haemodialysis sessions with high cut-off filters in myeloma cast nephropathy: our experience

El mieloma múltiple (MM) consiste en la proliferación incontrolada de células plasmáticas con producción de cantidades variables de inmunoglobulinas o sus cadenas. La insuficiencia renal aguda puede ser un síntoma del MM, y a veces su forma de presentación. Las cadenas ligeras libres circulantes (CLL) pueden dar lugar al fallo renal por la precipitación intratubular de ellas, causando una nefropatía por cilindros. El tratamiento del mieloma, una adecuada hidratación y la eliminación de CLL mediante técnicas de aféresis son los tratamientos admitidos actualmente para esta entidad. Se han intentado diversas técnicas de aféresis para intentar eliminar las CLL, siendo la hemodiálisis de larga duración con filtros para eliminar dichas cadenas ligeras (alto cut-off) la que se postula como el tratamiento más eficaz para la nefropatía del mieloma. MÉTODOS: Presentamos cinco casos de nefropatía de mieloma: tres con nefropatía por cilindros (NC) diagnosticada por biopsia renal y dos con alta probabilidad de NC (niveles de CLL > 500 mg/l) tratados con hemodiálisis larga con membrana de alto cut-off. Todos presentaban insuficiencia renal aguda, en cuatro de ellos con necesidad de terapia sustitutiva y uno en situación de insuficiencia renal avanzada. En todos ellos los niveles de CLL fueron muy elevados. Recibieron tratamiento específico para el mieloma más hemodiálisis de alto cut-off hasta alcanzar niveles de CLL < 500 mg/l. RESULTADOS: Cuatro de los cinco pacientes recuperaron función renal, quedando independientes de diálisis. El tiempo de evolución del mieloma desde el inicio de la clínica fue variable (1-6 m). El número de sesiones varió entre 8-16. El paciente de más tiempo de evolución precisó más sesiones y no recuperó función renal. CONCLUSIONES: La hemodiálisis larga con filtros de alto cut-off más tratamiento con quimioterapia del mieloma parece ser un tratamiento eficaz en la insuficiencia renal aguda debida a nefropatía del mieloma. La precocidad en el inicio del tratamiento puede ser un factor determinante de la respuesta

Multiple myeloma (MM) is the uncontrolled proliferation of plasma cells with variable amounts of production of immunoglobulin or their chains. Acute renal failure can be a symptom of MM, and it is sometimes their presentation form. Circulating free light chains (FLC) could led to renal failure by intratubular precipitation of themselves causing a cast nephropathy. Myeloma's treatment, an adequate hydration and FLC's elimination by aphaeresis treatments are currently eligible therapy for this entity. Several aphaeresis techniques have been tried to eliminate the FLC being long-term hemodialysis with filters to remove these light chains (High Cut-Off filters). This treatment is postulated as the most effective treatment for myeloma nephropathy. METHODS: We report 5 cases of myeloma nephropathy: three of them with cast nephropathy (CN) diagnosed by renal biopsy and another two with high probability of NC (FLC levels >500mg/L). All of them were treated by hemodialysis with membrane high Cut-Off. The five patients had had an acute renal failure; in four of them need replacement renal therapy. The fifth patient only had an advanced renal failure. In all patients, FLC levels were very high. All patients received specific treatment for myeloma in addiction on hemodialysis high Cut-Off until the FLC levels were <500mg/ L. RESULTS: Four of the five patients recovered renal function, being independent of dialysis. The evolution time of myeloma since the first symptoms appeared was variable (1-6 months). The number of treatment sessions ranged from 8-16. The patient whose evolution time was the longest one required more sessions and did not recovered the renal function. CONCLUSIONS: Length hemodialysis with filters high cut-off plus specific myeloma chemotherapy seems to be an effective treatment in acute renal failure due to cast myeloma. The early initiation of treatment could be an important factor for the response