Iranian Journal of Kidney Diseases
Volume:6 Issue: 1, Jan 2012

Goodpasture syndrome, a rare human autoimmune disorder, is characterized by the presence of pathogenic autoantibodies that react with the components of the glomerular basement membrane. The clinical condition of the Goodpasture syndrome is characterized by an acute necrotizing glomerulonephritis, often with accompanying pulmonary hemorrhage. Notably, the Goodpasture antigen has been localized to the noncollagenous domain of the alpha3 chain of type IV collagen. Additionally, human leukocyte antigen-DR2, and to a lesser extent human leukocyte antigen-DR4, have been identified as important restriction elements. The role of T cells in Goodpasture syndrome is indicated by the highly restricted specificity of the antibody response and the strong major histocompatibility complex class II association. In this review article, we briefly describe the latest views on the molecular and cellular themes of Goodpasture syndrome

By advances in surgical techniques, success in prevention and treatment of transplant-related infections, and introduction of new immunosuppressive drugs, the patient and graft survival rates in solid organ transplant recipients has steadily and remarkably improved. It has been shown that the longer the transplant patients survival rate, the more saturation with cardiovascular risk factors and the greater risk of cardiovascular mortality. Currently, cardiovascular disease is the primary cause of death after kidney transplantation and is among the three most common causes of death after heart and liver transplantation. Over the past decades, because of risk factor reduction, mortality from coronary artery disease has substantially decreased in the general population. Recent studies suggest that risk factors reduction also significantly decreases cardiovascular events and deaths in solid organ transplant recipients.

Human herpesvirus 8 (HHV8) is a herpesvirus that is always associated with Kaposi sarcoma. An enzyme-linked immunoenzymatic assay was used to detect antibodies to the latent nuclear antigen of HHV8 in kidney transplant patients and kidney transplant candidates from Tunisia. A significantly high HHV8 seroprevalence was documented; 17% of kidney transplant patients and 23% in kidney transplant candidates were seropositive. This is a first report of HHV8 in Tunisia

Introduction. Some evidence suggest an increase in the prevalence of focal segmental glomerular sclerosis (FSGS) in children. To date, there has been no study of the outcome in children with FSGS and its frequency over several decades in Iran. We aimed to report the changing trend of FSGS incidence and its outcome in a sample of Iranian children.Materials and Methods. Between 1982 and 2008, all 716 kidney biopsies performed in children referred to Ali Asghar Children Hospital were recorded and confirmed cases with FSGS lesions were further evaluated. Baseline and clinical characteristics of all FSGS patients were assessed and the therapies and outcomes were reviewed.Results. The incidence rate of FSGS was 10.1% between 1982 and 1990, which was significantly increased to as high as 20.5% after the year 2000 (P =. 001). Among 64 children with FSGS, 20 progressed to end-stage renal disease with a mean survival time of 11.45 years (standard error of mean, 1.34 years). Kidney survival rates were 90.4%, 69%, and 47% at 1, 5 and 10 years of follow-up.Conclusions. Our study demonstrates an increasing trend in FSGS incidence in Iranian children. However, kidney survival rates of our patients were similar to those reported by others in different countries.

Introduction. Gentamicin sulphate nephrotoxicity seems to be attributed to the generation of reactive oxygen species. Olive leaf extract (OLE) has been demonstrated to have antioxidant and anti-inflammatory effects. The aim of this study was to evaluate the inhibitory effect of OLE on gentamicin-induced nephrotoxicity in rats.Materials and Methods. Thirty-five Sprague-dawley rats were divided into 5 groups to receive saline; gentamicin, 100 mg/kg/d; and gentamicin plus OLE in 3 different doses (25 mg/kg/d, 50 mg/kg/d, and 100 mg/kg/d, once daily for 12 days. Serum and renal malondialdehyde were assessed, and tubular necrosis was studied semiquantitatively. Glomerular volume and volume density of the proximal convoluted tubules were estimated stereologically from paraffin sections. Serum creatinine and renal antioxidant enzymes activity were measured.Results. Gentamicin significantly increased serum creatinine, malondialdehyde, and tubular necrosis, and decreased creatinine clearance, volume density of the proximal convoluted tubules, renal glutathione, glutathione peroxidase, catalase, and superoxide dismutase compared with the control group. Cotreatment of gentamicin and OLE significantly decreased serum creatinine, malondialdehyde, tubular necrosis, and renal malondialdehyde, and increased renal glutathione, catalase, superoxide dismutase, volume density of proximal convoluted tubules, and creatinine clearance in comparison with gentamicin-only treated group. Serum malondialdehyde, serum creatinine, tubular necrosis, and volume density of proximal convoluted tubules were maintained at the same level as that of the control group by cotreatment of gentamicin and OLE.Conclusions. Olive leaf extract ameliorates gentamicin nephrotoxicity via antioxidant activity, increase of renal glutathione content, and increase of renal antioxidant enzymes activity, except for glutathione peroxidase.

Introduction. Even though dehydration could aggravate formation of urinary calculi, the effects of fluid and food restriction on calculus formation is not thoroughly defined. The purpose of this study is to evaluate the effects of fluid and food restriction in Ramadan fasting on urinary factors in kidney and urinary calculus formation.Materials and Methods. Fifty-seven men aged 30 to 55 years old, including 37 recurrent calcium calculus formers and 20 with no history of kidney calculi were evaluated for blood tests, ultrasonography investigations, urinalysis, urine culture, and also 24-hour urine collection test. Metabolites including calcium, oxalate, citrate, uric acid, magnesium, phosphate, potassium, sodium, and creatinine were measured before and during Ramadan fasting. The values of calculus-precipitating solutes as well as inhibitory factors were documented thoroughly. Results. Total excretion of calcium, phosphate, and magnesium in 24-hour urine and also urine volume during fasting were significantly lower than those in the nonfasting period. Urine concentration of calcium during fasting was significantly lower than nonfasting (P <. 001). Urine concentrations of uric acid, citrate, phosphate, sodium, and potassium during fasting were significantly higher than nonfasting. Uric acid supersaturation was accentuated, and calcium phosphate supersaturation was decreased significantly during fasting. There was no significant increase in calcium oxalate supersaturation during the fasting period.Conclusions. Fasting during Ramadan has different effects on total excretion and concentrations of urinary precipitate and inhibitory factors contributing to calculus formation. We did not find enough evidence in favor of increased risks of calculus formation during Ramadan fasting.

Introduction. Proteinuria and albuminuria are established risk factors for progressive renal damage. Albuminuria can be effectively controlled by antihypertensive drugs that interrupt the renin-angiotensin-aldosterone system. However, the efficiency of N-acetyl cysteine (NAC) in preventing diabetic nephropathy is uncertain. Renoprotective effects of angiotensin receptor blockers and NAC in preventing or reducing of proteinuria in patients with diabetic nephropathy was studied.Materials and Methods. In a randomized controlled trial, 70 patients with type 2 diabetic nephropathy (proteinuria and renal insufficiency) were studied. The patients were randomly divided into two groups and were treated with losartan, 25 mg, twice per day, with and without NAC, 600 mg twice daily (study and control groups, respectively; 35 patients in each group). Urine protein was checked before treatment and after 2 months of treatment.Results. The two groups were comparable regarding gender, age, serum creatinine, and urine protein excretion levels. Proteinuria improved in both groups. The mean proteinuria level decreased more in patients with losartan and NAC; however, comparison of proteinuria between the two groups showed no significant difference after 2 months.Conclusions. Angiotensin receptor blockers reduced proteinuria due to diabetic nephropathy, and this study failed to detect additional effect when NAC was combined with these medications.

Introduction. Erythropoietin is administered for treatment of anemia in chronic kidney disease and kidney transplantation. Erythropoietin improves oxygenation of organs and prevents them against apoptosis. The aim of this study was evaluation of erythropoietin's effect on graft survival in the early phase after transplantation.Materials and Methods. Forty kidney transplant candidates with a hemoglobin level of 8 g/dL to 10 g/dL were randomized to receive either erythropoietin (PD-Poietin) or placebo for the first posttransplant week. They were followed up for 6 months and serum creatinine levels, glomerular filtration rate (GFR), allograft rejection episodes, and graft loss were compared between the two groups.Results. The mean creatinine level and GFR were 1.16 ± 0.03 mg/mL and 85.1 ± 18.3 mL/min in the erythropoietin group and 1.2 ± 0.2 mg/dL and 83.3 ± 21.1 mL/min in the control group at baseline. After 6 months of follow-up, the mean of creatinine level and GFR reached to 1.11 ± 0.23 mg/dL and 86.6 ± 10.3 mL/min in the erythropoietin group and 1.31 ± 0.35 mg/dL and 79.7 ± 12.5 mL/min in the control group, respectively (P =. 04 and P =. 02). None of the patients lost their grafts and no death was reported. There were no adverse effects in the erythropoietin group.Conclusions. Our findings suggest that erythropoietin may have beneficial effects on graft function if administered early after transplantation. Erythropoietin can be used for all kidney transplant recipients for protecting the allograft due to its effects on tissue oxygenation.

Introduction. Metabolic complications are common after kidney transplantation and can cause considerable morbidity and even threaten graft function. This study aimed to investigate the prevalence of hyperlipidemia and its impact on graft function in 10 years of follow-up of patients undergoing kidney transplantation. Materials and Methods. This prospective study was conducted on 73 patients who underwent kidney transplantation between April 1996 and April 1998 to evaluate their lipid profile and graft function as well as the effect of hyperlipidemia in long-term kidney allograft function. Kidney allograft dysfunction was defined as a serum creatinine level greater than 1.8 mg/dL.Results. The mean serum triglyceride level was higher at 1, 3, 5, and 10 years, but not 7 years, among patients with graft dysfunction in comparison with patients with normal graft function. However, these differences were not significant. The mean serum total cholesterol level was significantly higher in patients with graft dysfunction at 1 year (P =. 03). Of the patients with graft dysfunction, 94.7% developed hypercholesterolemia at the first year visit, as compared to 70.4% of patients with normal graft function (P =. 03). The frequency of hypercholesterolemia was higher among those with a serum creatinine greater than 1.8 mg/dL at all other visits, but without significant difference.Conclusions. Hyperlipidemia is common after kidney transplantation, especially in the first year after transplantation. Higher serum total cholesterol levels might be related to graft dysfunction

Introduction. Chemokines and chemokine receptors have a pivotal role in immunity and inflammation. We aimed to evaluate their role in kidney transplant rejection. Materials and Methods. The association of chemokine (C-C motif) receptor 2 (CCR2)-V64I and CCR5-Delta32 gene polymorphisms with acute rejection (AR) and delayed graft function (DGF) were examined in 100 donor-recipient pairs. The CCR2-V64I and CCR5-Delta32 alleles were determined using polymerase chain reaction and polymerase chain reaction-restriction fragment length polymorphism, respectively.Results. No associations were found between donors or recipient's CCR2-V64I and CCR5-Delta32 gene polymorphisms and AR or DGF. Of the characteristics of the donors, recipients, and transplantation, glomerulonephritis as a cause of kidney failure in the recipients was weakly associated with AR (relative risk, 6.1; 95% confidence interval, 0.8 to 46.0; P =. 07). Transplantation of kidney from females to males was weakly associated with DGF (relative risk, 5.5; 95% confidence interval, 0.9 to 33.0; P =. 06). There was a significant association between AR, but not DGF, and graft loss in the patients (relative risk, 28.6; 95% confidence interval, 1.7 to 487.0; P =. 03).Conclusions. Our study failed to suggest CCR2-V64I or CCR5-Delta32 gene polymorphisms as risk factors for AR and DGF in kidney transplantation. Sex-matching between donors and recipients should be considered for living donor kidney transplantation.

Introduction. Living donor transplantation allows a priori scheduling and the recipient can receive immunosuppressive prophylaxis several days before surgery, which is preoperative induction therapy with oral agents. We evaluated the impact of preoperative mycophenolate mofetil on the outcomes of living donor kidney transplantations.Materials and Methods. In a randomized controlled trial was from November 2008 to November 2010, 99 patients receiving their first living donor kidney transplantation were divided into the mycophenolate mofetil (500 mg) and placebo groups, and received 2 tablets per day for 5 days before transplantation.Results. Forty-nine patients received mycophenolate mofetil and 48 received placebo. The mean serum creatinine on discharge day and hospitalization period were significantly less with mycophenolate mofetil compared to placebo (1.62 ± 1.00 mg/dL versus 1.22 ± 0.24 mg/dL, P = 0.03 and 20.8 ± 11.2 days versus 13.5 ± 4.4 days, P <. 001, respectively). No delayed graft function was observed. Slow graft function was 2-fold higher in the placebo group (14.6% versus 8.2%, P =. 32). Acute rejection was seen in 12.2% of the patients with mycophenolate mofetil and in 29.2% of the controls (P =. 04). Serum creatinine levels at discharge were significantly lower in the mycophenolate mofetil group compared with that in the placebo group (P =. 03).Conclusions. Prophylactic administration of mycophenolate mofetil before living donor kidney transplantation reduced hospitalization period, improved early graft function, and decreased the risk of acute rejection in the first month posttransplant.

Multiple myeloma is uncommon in individuals younger than 40 years. Renal involvement is common in this disease, but acute tubulointerstitial nephritis is very rare. In 20% of patients, only the light chain is produced and serum protein electrophoresis is normal; however, in urine protein electrophoresis of these patients, the M spike is present. We reported a case of multiple myeloma in a 39-year-old man with acute tubulointerstitial nephritis. Serum protein electrophoresis was normal and there was no bone lytic lesion. Remission of multiple myeloma was achieved after treatment with thalidomide and dexamethasone; however, kidney failure was not improved and the patient was maintained on hemodialysis.

Autosomal dominant polycystic kidney disease (ADPKD) with nephrotic syndrome is a rare coincidence. Among 19 reported cases since 1972, focal glomerulosclerosis is the dominant reported pathology. Here, we report the 6th case of focal segmental glomerulosclerosis with ADPKD. A 29-year-old man with a history of APCDK presented with massive proteinuria. He had a history of concurrent leptospirosis and brucellosis, and trace proteinuria and mild hypertension had been diagnosed 4 years earlier. Urine study showed proteinuria (21 g/d) and hematuria. Kidney biopsy report was compatible with focal and segmental sclerosis. The patient received prednisolone and cyclosporine. After 4 months, proteinuria decreased to 600 mg/d. Patients with ADPKD who show massive proteinuria should undergo kidney biopsy. It is possible that different mutations in these patients could clarify the nature of this coincidence.