Likely Recurrence of C3 Glomerulonephritis in Kidney Transplantation: An Entity to Bear in Mind: Case Report.

BACKGROUND: C3 glomerulonephritis (C3GN) is an unusual entity that is caused by dysregulation and hyperactivity of the alternative complement pathway. Renal biopsy immunofluorescence study shows C3 deposits with absence of immunoglobulins and markers of the classical complement pathway. More than 50% of cases develop end-stage renal disease. Less well-known is the course of C3GN after kidney transplantation. CASE REPORT: We present the case of a 60-year-old woman with chronic kidney disease secondary to chronic glomerulonephritis of unknown origin who received a kidney transplant. Two years later, she presented worsening renal function with non-nephrotic proteinuria and microhematuria. Complement testing revealed low serum levels of C3. Kidney biopsy showed alterations compatible with C3GN that we interpreted as a relapse of the underlying disease.

Perfil de consumo de antiepilépticos en los pacientes epilépticos del Área Oeste de Málaga / Consumption patterns of antiepileptic drugs among epileptic patients in the Western Málaga district

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Síndrome de Sjögren y fracaso renal agudo tras cirugía oral / Sjögren’s syndrome and acute renal failure following oral surgery

El síndrome de Sjögren primario es una enfermedad inflamatoria multisistémica que suele cursar con lesiones de las glándulas exocrinas originando síntomas de sequedad oral y ocular. La expresión clínica también incluye manifestaciones generales, afección extraglandular y desarrollo de linfoma. La asociación de enfermedad renales frecuente. Habitualmente, la lesión observada corresponde a nefritis túbulo-intersticial. En cambio, es rara la afectación glomerular, así como los casos de fracaso renal agudo severo. Presentamos el caso de una mujer con síndrome de Sjögren primario que desarrolla un cuadro de insuficiencia renal aguda grave por glomerulonefritis crioglobulinémica con respuesta favorablea la terapia con esteroides, ciclofosfamida, plasmaféresisy rituximab (AU)

Primary Sjögren´s syndrome is a multisystemic inflammatory disorder that mainly affects the exocrine glands and usually presents as dryness of the mouth and eyes. The wide clinical pectrum of the disease includes general symptoms, extraglandular manifestations and lymphoma. The renalinvolvement is frequent. Interstitial nephritis is the most common renal manifestation, but glomerular involvement and acute renal failure may rarely occur. We describe acase of a female patient with primary Sjögren´s syndrome complicated by severe acute renal failure due to cryoglobulinaemic glomerulonephritis. Treatment with steroids, cyclophosphamide, plasma exchange and rituxim absuccessfully lead to recovery of acute renal failure (AU)

[Sjögren's syndrome and acute renal failure after oral surgery]. / Síndrome de Sjögren y fracaso renal agudo tras cirugía oral.

Primary Sjögren s syndrome is a multisystemic inflammatory disorder that mainly affects the exocrine glands and usually presents as dryness of the mouth and eyes. The wide clinical spectrum of the disease also includes general symptoms, extraglandular manifestations and lymphoma. It is frequently associated with renal diseases. Interstitial nephritis is the most common renal manifestation, but glomerular involvement and acute renal failure may also (rarely) occur. We describe a case of a female patient with primary Sjögren s syndrome complicated by severe acute renal failure due to cryoglobulinemic glomerulonephritis. Treatment with steroids, cyclophosphamide, plasma exchange and rituximab successfully led to recovery from acute renal failure.

[Rhabdomyolysis due to muscle enzyme deficiencies]. / Rabdomiolisis por déficits enzimáticos musculares.

Rhabdomyolysis is a syndrome characterized by injure of skeletal muscle with the release of intracellular constituents into the circulation. Acute renal failure is a common complication and is the leading cause of morbidity and mortality in these patients. The most common aetiology is traumatisms, muscle compressions and extreme exertions. Most commonly, the cause of rhabdomyolysis is evident from the careful clinical history. Nevertheless, when the precipitant is not obvious the diagnosis is difficult and a raised clinical suspicion is required. We should investigate used medication or drugs, infections, electrolyte abnormalities and a number of inherited enzyme deficiencies, in which cases the muscle is unable to use available energy. We report two clinical cases of acute renal failure due to rhabdomyolysis by metabolic myopathies due to a carnitine palmitoyltransferase deficiency on the one hand and by myophosphorylase deficiency on the other. We describe their clinical features and progress.

Acidosis tubular renal inducida portopiramato. A propósito de un caso / No disponible

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Monitorización de las concentraciones plasmáticas de vancomicina en pacientes en hemodiálisis / Monitorins of vancomycin plasma levels in patients undergoing hemodialysis

Objetivo: El objetivo del estudio es confirmar que los pacientesen hemodiálisis y en tratamiento con vancomicina, recibiendodosis de 1 g semanal o 500 mg/48 horas, alcanzan niveles plasmáticosóptimos (10-25 mcg/ml).Método: Se incluyeron 32 pacientes sobre un total de 34 episodiosde infección. La dosis más utilizada fue la de 1 g/semana(67,6% de los episodios de infección), frente a la de 500 mg/48horas (32,4%). Las membranas más frecuentemente empleadasfueron las de baja ultrafiltración (64,7%) frente a las de alta ultrafiltración(35,3%).La administración de vancomicina se realizó en infusión de unahora, durante la última hora de la sesión de hemodiálisis y lasmuestras plasmáticas se extrajeron antes del inicio de la sesión.Las muestras fueron analizadas por inmunoanálisis de polarizaciónde fluorescencia.Resultados: Los resultados de concentración promedio parala población total no alcanzaron en ningún momento los valoresóptimos (10-25 mg/ml). En el grupo que recibió 500 mg/48horas los valores promedio óptimos no se alcanzaron en ningunade las determinaciones y en el grupo de 1 g/semanal sólo sealcanzaron dichos valores en la cuarta determinación. Los nivelesplasmáticos promedio del antibiótico están por debajo de los valoresóptimos independientemente del tipo de membrana, baja oalta ultrafiltración, empleada.Conclusiones: A la vista de los resultados recomendamosmodificar las dosis de vancomicina en este grupo de pacientes yadministrar dosis de carga 20 mg/kg durante la última hora de lasesión de hemodiálisis y posteriormente dosis de mantenimientode 7 mg/kg después de cada sesión, así como monitorizar losniveles del fármaco en este grupo de pacientes

Objective: The aim of this study was to confirm whetherpatients undergoing dialysis and treated with vancomycin 1g/week or 500 mg/48 hours reach optimum plasma levels (10-25 mcg/ml).Method: Thirty two patients were included with a total numberof 34 infectious events. The most commonly used dose was 1g/week (67.6% of infectious events), versus 500 mg/48 hours(32.4%). Low ultrafiltration membranes were used more frequently(64.7%) than high ultrafiltration membranes (35.3%).Vancomycin was infused over an one-hour period during thelast hour of the hemodialysis session and plasma samples weredrawn prior to the beginning of the session. Samples were analyzedusing fluorescent polarization immunoassays.Results: Mean concentration results for the total populationdid not reach optimum values (10-25 mg/ml). In the group receiving500 mg/48 hours, optimum mean values were not reachedin any of the determinations, whereas in the group receiving 1g/week, optimum values were reached in the fourth determination.Mean plasma levels of the antibiotic were bellow optimumvalues, regardless the type of membrane used, either low or highultrafiltration.Conclusions: Based on the above results, we recommendtitration of the vancomycin dose in this group of patients and theadministration of a loading dose of 20 mg/kg during the last hourof the hemodialysis session, followed by a maintenance dose of 7mg/kg after each session, as well as the monitoring of the druglevels in this group of patients

[Monitoring of vancomycin plasma levels in patients undergoing hemodialysis]. / Monitorización de las concentraciones plasmáticas de vancomicina en pacientes en hemodiálisis.

OBJECTIVE: The aim of this study was to confirm whether patients undergoing dialysis and treated with vancomycin 1 g/week or 500 mg/48 hours reach optimum plasma levels (10-25 mcg/ml). METHOD: Thirty two patients were included with a total number of 34 infectious events. The most commonly used dose was 1 g/week (67.6% of infectious events), versus 500 mg/48 hours (32.4%). Low ultrafiltration membranes were used more frequently (64.7%) than high ultrafiltration membranes (35.3%).Vancomycin was infused over an one-hour period during the last hour of the hemodialysis session and plasma samples were drawn prior to the beginning of the session. Samples were analyzed using fluorescent polarization immunoassays. RESULTS: Mean concentration results for the total population did not reach optimum values (10-25 mg/ml). In the group receiving 500 mg/48 hours, optimum mean values were not reached in any of the determinations, whereas in the group receiving 1 g/week, optimum values were reached in the fourth determination. Mean plasma levels of the antibiotic were below optimum values, regardless the type of membrane used, either low or high ultrafiltration. CONCLUSIONS: Based on the above results, we recommend titration of the vancomycin dose in this group of patients and the administration of a loading dose of 20 mg/kg during the last hour of the hemodialysis session, followed by a maintenance dose of 7 mg/kg after each session, as well as the monitoring of the drug levels in this group of patients.

El acceso vascular y la anemia en hemodiálisis

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[Survival of myeloma patients treated with dialysis]. / Supervivencia de pacientes con mieloma tratados con diálisis.

BACKGROUND: Renal failure is a common complication of myeloma. Renal replacement therapy in these patients is controversial due to poor survival outcomes and low tolerance to treatment. We reviewed our experience on patients with myeloma undergoing dialysis therapy at one centre. PATIENTS AND METHODS: Between 1980 and 2000, 28 patients (21 men and 7 women) with myeloma were admitted to chronic dialysis programme and the following variables were analysed: sex, age when starting dialysis, lapse of time between diagnosis of myeloma and admission to dialysis (TD), disease stage, comorbity, mode of presentation, calcium, creatinine at diagnostic, albumin and Hb at the beginning of dialysis, and cause of death. We studied survival among these patients (Kaplan-Meier), identified predictors of survival outcome (Cox's regression) and compared survival between the two decades studied. RESULTS: Mean age was 65 years, median TD was 0.4 months, and modes of presentation were: end-stage renal failure (18 patients), acute renal failure (8), amyloldosis (2). Eleven patients (39%) had myeloma IgG, four (14%) IgA and thirteen (46%) had light chains. Kappa light chain was the most frequent one. In 75% of patients myeloma was at IIIb stage. Cause of death were: Cardiovascular disease (5 patients), infections (4), suspension of treatment (4), tumours (4), and others causes (2). Median survival for all patients was 16.8 months (range 0.4-78) and 25% survived over 39 months. Hb level was the only significant predictor in the multivariant analysis (p = 0.02). In the 80's median survival was 6.17 months versus 17 months in the 90's but this difference was not significant with long-rank test. CONCLUSION: Although survival of patients with myeloma treated with dialysis is still short, 25 percent survive over 3 years, being Hb level the only predictive factor. Moreover, we observed an improvement of survival in recent years.

Supervivencia de pacientes con mieloma tratados con diálisis / Survival in patients with myeloma treated with dialysis

Introducción: La insuficiencia renal es una complicación frecuente del mieloma (MM). La indicación de tratamiento sustitutivo de la función renal en estos pacientes es controvertida, debido a mala supervivencia y tolerancia. Se revisa la experiencia de nuestro centro con todos los pacientes diagnosticados de MM durante los últimos 20 años y admitidos a programa de diálisis crónica. Pacientes y métodos: Se estudian 28 pacientes (21 hombres y 7 mujeres) con MM y que iniciaron tratamiento con diálisis entre los años 1980 y 2000 en los que se evaluaron variables clínicas y analíticas: sexo, edad al inicio de diálisis, tiempo entre el diagnóstico y la entrada en diálisis (TD), estadio de la enfermedad, presencia de comorbilidad, forma de presentación, calcio sérico, creatinina en el momento del diagnóstico, albúmina, hemoglobina al inicio de la diálisis (Hb) y causa de muerte. Se estudia también la supervivencia (método de Kaplan-Meier) y se trató de identificar variables predictoras con el análisis de regresión de Cox. Para valorar la respuesta al tratamiento se dividieron los casos aparecidos en dos períodos de diez años. Resultados: Con una edad media de 65 años, la mediana para el intervalo diagnóstico inicio diálisis (TD) fue de 0,4 meses. En 18 casos la afectación renal se presentó como insuficiencia renal crónica, fracaso renal agudo en 8 y amiloidosis en 2. Once pacientes (39%) fueron MM IgG, 4 (14%) IgA, y 13 (46%) con cadenas ligeras. El 75% presentaban un estadio IIIb. Las causas de muerte fueron: cardiovasculares en 5 casos, infecciones en 4, cese del tratamiento en 4, tumoral en 4 y otras en 2. La mediana de la supervivencia fue de 16,8 meses para todo el grupo (rango 0,4-78), y en el análisis multivariante sólo las concentraciones de hemoglobina resultaron predictivas (p = 0,02). El 25% de los pacientes sobrevivió 39 meses. En la década de los ochenta la mediana fue de 6,17 meses y para los noventa de 17 meses. Esta diferencia no alcanzó significación estadística con el log-rank test (p = 0,12). Conclusión: Aunque la supervivencia de los pacientes con MM en diálisis sigue siendo baja, una proporción estimable sobrevive más de tres años y además se evidencia una tendencia hacia una mayor supervivencia en la última década. La Hb fue la única variable predictora encontrada

Background: Renal failure is a common complication of myeloma. Renal replacement therapy in these patients is controversial due to poor survival outcomes and low tolerance to treatment. We reviewed our experience on patients with myeloma undergoing dialysis therapy at one centre. Patients and methods: Between 1980 and 2000, 28 patients (21 men and 7 women) with myeloma were admitted to chronic dialysis programme and the following variables were analysed: sex, age when starting dialysis, lapse of time between diagnosis of myeloma and admission to dialysis (TD), disease stage, comorbidity, mode of presentation, calcium, creatinine at diagnostic, albumin and Hb at the beginning of dialysis, and cause of death. We studied survival among these patients (Kaplan-Meier), identified predictors of survival outcome (Cox’s regression) and compared survival between the two decades studied. Results: Mean age was 65 years, median TD was 0.4 months, and modes of presentation were: end-stage renal failure (18 patients), acute renal failure (8), amyloldosis (2). Eleven patients (39%) had myeloma IgG, four (14%) lgA and thirteen (46%) had light chains. Kappa light chain was the most frequent one. In 75% of patients myeloma was at Illb stage. Cause of death were: Cardiovascular disease (5 patients), infections (4), suspension of treatment (4), tumours (4), and others causes (2). Median survival for all patients was 16.8 months (range 0.4-78) and 25% survived over 39 months. Hb level was the only significant predictor in the multivariant analysis (p = 0.02). In the 80’s median survival was 6.17 months versus 17 months in the 90's but this difference was not significant with long- rank test. Conclusion: Although survival of patients with myeloma treated with dialysis is still short, 25 percent survives over 3 years, being Hb level the only predictive factor. Moreover, we observed an improvement of survival in recent years

[Intracytoplasmic crystals and Fanconi syndrome in a patient with IgA kappa myeloma]. / Cristales intracitoplasmáticos y síndrome de Fanconi en un paciente con mieloma IgA kappa.

A 45 year old man with IgA-Kappa myeloma had adult Fanconi Syndrome. Examination of renal biopsy revealed lesions in proximal tubules without glomerular lesions and without intratubular casts. By electron microscopy cytoplasmic crystalline inclusions were observed in renal proximal tubular epithelium. Increased plasma cells (28%) in bone marrow aspiration also contained crystalline inclusions. The treatment of myeloma produced partial remission of proliferative disease and Fanconi syndrome. We discuss the pathogenesis of Fanconi syndrome induced by light chains as well as the composition of crystalline deposits and the effects of treatment on Fanconi Syndrome.

Cristales intracitoplasmáticos y síndrome de Fanconi en un paciente con mieloma igA kappa / A patient with Fanconi's syndrome, myeloma IgA Kappa and cytoplasmic crystals

Se presenta un paciente de 45 años con Mieloma IgA Kappa y que se acompañaba de Síndrome de Fanconi. En la biopsia renal practicada, el estudio ultraestructural revelaba características inclusiones cristalinas intracitoplasmáticas a nivel del epitelio tubular proximal; no se evidenciaron cilindros mielomatosos ni lesiones glomerulares. El aspirado de médula ósea presentaba un 28 por ciento de células plasmáticas que contenían en su citoplasma el mismo tipo de inclusiones cristalinas. El paciente fue tratado de su enfermedad proliferativa obteniéndose remisión de la misma y disminución de las manifestaciones del Síndrome de Fanconi, pero no su desaparición. Se discute la patogenia del Síndrome de Fanconi asociado a Mieloma y la composición del depósito cristalino, su relación con las cadenas ligeras, así como las consecuencias del tratamiento del Mieloma sobre el Síndrome. (AU)