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.

[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.

[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.

[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.

[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.