Splenic marginal lymphoma and glomerulonephritis: case report and review of the literature.

Malignant lymphomas can affect kidneys in several ways. They may precipitate acute renal failure by causing ureteral or renal vascular obstruction, or by direct renal parenchymal infiltration. Furthermore, they may insult renal function via paraneoplastic mechanisms such as hypercalcemia. Lymphomas only rarely can cause glomerulonephritis (GN). We report a case of a 72-year-old male who presented with mild renal function impairment, proteinuria, and microscopic hematuria, suggesting active glomerulonephritis, and pancytopenia of immune origin. A bone marrow biopsy led to a diagnosis of splenic marginal zone lymphoma. Although a kidney biopsy was not performed, glomerulonephritis was attributed to the lymphoma and splenic marginal zone lymphoma-related glomerulonephritis was the final diagnosis. The course of splenic marginal zone lymphoma is extremely indolent. The first manifestation in some patients can be immune cytopenia or other autoimmune phenomena. These patients may respond well to corticosteroids. Therefore, our patient was started on prednisolone resulting in a good hematologic response. Renal function also improved and proteinuria and hematuria disappeared, suggesting a lymphoma-related origin of the GN. Two years after full steroids withdrawal, the patient remained stable with a good renal function and daily protein excretion less than 300 mg. Lymphomas rarely are the cause of secondary glomerulonephritis; however, with a lack of an apparent cause, the clinician should be aware of them, particularly in the elderly with autoimmune manifestations.

Total parathyroidectomy without autotransplantation in dialysis patients and renal transplant recipients, long-term follow-up evaluation.

BACKGROUND: Persistent secondary hyperparathyroidism not responding to medication is treated successfully with surgical excision of parathyroid glands (total parathyroidectomy [PTX]). PTX without autotransplantation of parathyroid glands excludes the risk for recurrence of hyperparathyroidism. METHODS: During the years 2002 to 2005, 36 total parathyroidectomies were performed in 33 patients: 21 dialysis patients because of end-stage renal disease and 12 renal transplant recipients. RESULTS: PTX without autotransplantation was performed successfully in 33 patients, whereas 3 patients were reoperated for remaining parathyroid glands. Immediate improvement of clinical symptoms and a decrease of serum calcium and parathormone levels were observed after surgical procedures. Oral replacement treatment with vitamin D (1a-calcidiol) and calcium was commenced and long-term follow-up evaluation (23.5 +/- 7.6 mo) showed that calcium homeostasis was controlled adequately. CONCLUSIONS: PTX without autotransplantation is a safe and effective surgical procedure for the treatment of resistant secondary hyperparathyroidism with immediate response of clinical symptoms. Replacement treatment with vitamin D and calcium provides satisfactory coverage of individual needs.

COL4A3/COL4A4 mutations producing focal segmental glomerulosclerosis and renal failure in thin basement membrane nephropathy.

Mutations in the COL4A3/COL4A4 genes of type IV collagen have been found in approximately 40% of cases of thin basement membrane nephropathy, which is characterized by microscopic hematuria and is classically thought to cause proteinuria and chronic renal failure rarely. Here we report our observations of 116 subjects from 13 Cypriot families clinically affected with thin basement membrane nephropathy. These families first came to our attention because they segregated microscopic hematuria, mild proteinuria, and variable degrees of renal impairment, but a dual diagnosis of focal segmental glomerulosclerosis (FSGS) and thin basement membrane nephropathy was made in 20 biopsied cases. Molecular studies identified founder mutations in both COL4A3 and COL4A4 genes in 10 families. None of 82 heterozygous patients had any extrarenal manifestations, supporting the diagnosis of thin basement membrane nephropathy. During follow-up of up to three decades, 31 of these 82 patients (37.8%) developed chronic renal failure and 16 (19.5%) reached end-stage renal disease. Mutations G1334E and G871C were detected in seven and three families, respectively, and were probably introduced by founders. We conclude that these particular COL4A3/COL4A4 mutations either predispose some patients to FSGS and chronic renal failure, or that thin basement membrane nephropathy sometimes coexists with another genetic modifier that is responsible for FSGS and progressive renal failure. The findings presented here do not justify the labelling of thin basement membrane nephropathy as a benign condition with excellent prognosis.

Reduction in glucocorticoid receptors in renal biopsies of patients with lupus nephritis.

OBJECTIVES: The first-line treatment for lupus nephritis is the administration of glucocorticoids (GC) that mediate their effects via the glucocorticoid receptor (GR). The aim of this study was to investigate the expression of GR protein in the cortical area of renal parenchyma of normal and diseased renal biopsies from treated and untreated patients. DESIGN AND METHODS: The immunohistochemical EnVision/HRP technique was performed on renal tissue to detect GR protein. Statistical analysis was performed by SAS (2001). RESULTS: The antigen was mainly detected in glomerular podocytes and in tubules. The number of GR-positive podocytes of the controls was significantly higher than in the untreated patients, which was accordingly higher than in patients who were under medication. CONCLUSIONS: The lower number of GR-positive cells in the diseased kidney compared to controls is possibly linked to tissue-specific GC resistance, whereas the decreased GR expression in podocytes of treated compared to untreated patients may be due to a down-regulation effect after GCs' administration.

A fatal case of paraquat poisoning following minimal dermal exposure.

Paraquat is a pesticide widely used in agriculture. Numerous cases of paraquat intoxication have been reported either accidentally or intentionally as a suicidal attempt. The most severe cases of paraquat poisoning refer to oral ingestion. Complications include respiratory, hepatic, and renal failure, and are usually fatal. Dermal exposure is less frequent and rarely fatal. This article reports a case of an 81-year-old man with minimal skin burn after accidental paraquat exposure. The patient developed acute renal and respiratory failure and, despite aggressive treatment with hemodialysis, hemoperfusion, and mechanical ventilation, died two days later.

Human herpesvirus 8 infection in hemodialysis patients.

BACKGROUND: The aim of the present study was to evaluate human herpesvirus 8 (HHV-8) seroprevalence in Greek hemodialysis patients. Patterns of change in HHV-8 serostatus (seroconversions and seroreversions) over time were also evaluated. METHODS: Serum samples obtained from a cohort of 485 Greek hemodialysis patients were tested for antibodies to HHV-8 by whole virus lysate enzyme-linked immunosorbent assay, and reactive samples were confirmed by means of the orf-73 enzyme-linked immunosorbent assay. HHV-8 seroprevalence at study entry and the incidence of seroreversions and seroconversions per 100 person-years were estimated. RESULTS: The prevalence of HHV-8 antibodies in Greek hemodialysis patients at enrollment was 7.2%. No univariate associations were established between HHV-8 serostatus and patients' characteristics. Incidences of seroreversions and seroconversions were 16.4/100 person-years (95% confidence interval, 7.1 to 32.3) and 0.28/100 person-years (95% confidence interval, 0.03 to 1.02), respectively. Patients 50 years and younger had an increased probability for seroreversion to HHV-8 antibodies than patients older than 50 years (log-rank test, P = 0.018). CONCLUSION: We observed a fair number of seroreversions and a low incidence of seroconversion to HHV-8 infection in hemodialysis patients in Greece. Our data provide indirect evidence that HHV-8 transmission in the hemodialysis setting is uncommon.

Incidence and patterns of hepatitis C virus seroconversion in a cohort of hemodialysis patients.

BACKGROUND: The aim of this multicenter hemodialysis (HD) cohort study is to prospectively investigate the incidence of hepatitis C virus (HCV) infection in Greece from 1993 to 1995 and delineate early virological and serological events associated with HCV seroconversion in the HD setting. METHODS: Sequential serum samples collected weekly from 562 patients were tested biochemically and serologically by means of a second- (EIA-2) and third-generation enzyme immunoassay (EIA-3). All patients with positive antibody to HCV test results (anti-HCV + ) and sequential samples from seroconverting patients were tested for HCV RNA. RESULTS: Anti-HCV prevalence at study entry was 29% (163 of 562 patients), and viremia was detectable in 110 of 163 anti-HCV + patients (67.5%). HCV incidence was 6.2 cases/100 person-years. Seroconversions could not be attributed to transfusions after study entry (only 1 patient had been administered transfusion), and HD unit was associated with increased hazard for seroconversion ( P = 0.002), even after adjusting for potential differences among their patients. According to Kaplan-Meier estimation, the median interval by which the HCV RNA assay detected HCV infection earlier than anti-HCV testing was 246 and 154 days for EIA-2 and EIA-3, respectively. Detectable HCV RNA and at least 2 consecutive abnormal alanine aminotransferase levels in the preseroconversion period were observed in 29 of 30 (97%) and 14 of 32 patients (44%), respectively. Reductions in HCV RNA levels immediately after seroconversion were transient or did not occur. CONCLUSION: On the grounds of apparent nosocomial transmission, the wide window period of HCV infection in HD patients emphasizes the need for strict adherence to specific infection-control measures in this setting.

Carnitine action on red blood cell osmotic resistance in hemodialysis patients.

Low red blood cell osmotic resistance (RBCOR) in dialysis patients aggravates anemia and raises EPO needs. We studied RBCOR in 30 stable patients (22 M, 8 F), dialyzed for more than one year, with no systemic illness, blood transfusions, recent infection or treatment by ACE inhibitors. Nineteen were on EPO. Cuprophane dialysis membranes were used in 21, synthetic in the remaining nine. RBCOR was low in 13 patients and these patients received L-carnitine, 20 mg/kg IV, post dialysis, for one year (A); 17 with normal RBCOR served as untreated controls (B). We investigated the relations between RBCOR and membrane material, time on HD (THD), weekly dialysis duration (WHDT), serum total carnitine (TC), acyl carnitine (AC), free carnitine (FC) and AC/FC in all patients, before and after carnitine supplementation. RBCOR changes under carnitine treatment were evaluated. Age, sex, primary renal disease, THD, EPO dose, hematology and biochemistry were similar in treated patients and controls. Patients in group A (dialysed with cuprophane) had lower RBCOR than group B, (dialysed with synthetic and cuprophane membranes) (0.473 +/- 0.02 vs. 0.420 +/- 0.02, P < 0.001). RBCOR remained stable under carnitine in group A, but became abnormally low in controls (especially in 10/17 patients). RBCOR was significantly higher than the pretreatment values in 5/13 group A patients (month 0-M0: 0.48 +/- 0.02, M12: 0.45, +/- 0.02, P < 0.05). Carnitine levels, similar in both groups before treatment, remained stable in group A, but dropped in group B (TC: 52.1 +/- 9.6/31.1 +/- 21.2, FC: 33.1 +/- 8.3/15.6 +/- 19.2 micromol/L, P < 0.002). Patients with shorter WHDT (< or = 12 vs. > 12 h) had higher FC levels (36 +/- 6.9 vs. 30 +/- 6 micromol/L, P < 0.03). Low RBCOR is frequent in stable dialyzed patients. It is related to dialysis membranes and is aggravated by time on hemodialysis. Serum carnitine levels depend on weekly dialysis time and on carnitine supplementation, that normalizes osmotic resistance in some dialysis patients.