Iranian Journal of Kidney Diseases
Volume:5 Issue: 1, Jan 2011

Autosomal dominant polycystic kidney disease (ADPKD) is the most common life-threatening hereditary disease of the kidney. It presents with progressive enlargement of the kidneys with numerous cysts that distort the parenchyma and result in progressive decline in kidney function. Autosomal dominant polycystic kidney disease is genetically modified with the responsible genes localized to separate loci on chromosome 16 (PKD1 gene), accounting for the majority of ADPKD cases, and chromosome 4 (PKD2 gene), accounting for the remainder. This review discusses the current understanding of the pathogenesis of ADPKD, focusing on renal volume and its pivotal role on the manifestations of the disease. Specifically, activation of the renin-angiotensin-aldosterone system, hypertension, left ventricular hypertrophy, kidney function deterioration, pain, and hematuria are examined as consequences of renal volume increase. Recent developments on diagnostic modalities and criteria of the ADPKD are also discussed.

Introduction. Treatment modalities for end-stage renal disease affect quality of life (QOL) of the patients. This study was conducted to assess the QOL of patients on hemodialysis and compare it with caregivers of these patients. Cause of ESRD and dialysis-related factors affecting QOL were also examined.Materials and Methods. This cross-sectional study was conducted on patient on maintenance hemodialysis for more than 3 months at 3 dialysis centers of Lahore. Fifty healthy individuals were included as controls from among the patients’ caregivers. The QOL index was measured using the World Health Organization QOL questionnaire, with higher scores corresponding to better QOL of patients.Results. Eighty-nine patients (71.2%) were men, 99 (79.2%) were married, 75 (60.0%) were older than 45 years, and 77 (61.6%) were on dialysis for more than 8 months. Patients on hemodialysis had a poorer QOL as compared to their caregivers in all domains except for domain 4 (environment). There was no difference in the QOL between the three dialysis centers of the study, except for domain 3 (social relationship) of the patients at Mayo Hospital (a public hospital), which was significantly better. Nondiabetic patients had a better QOL in domain 1 (physical health) as compared to diabetic patients. Duration of dialysis had a reverse correlation with the overall QOL.Conclusions. We found that QOL of hemodialysis patients was poor as compared to caregivers of the patients, especially that of diabetics. Also, duration of dialysis had a reverse correlation with QOL.

Introduction. We searched for a pathophysiologically based feature of major water electrolytes, which may define water quality better than the water hardness, respecting urinary calculus formation.Materials and Methods. Utilizing a multistage stratified sampling, 2310 patients were diagnosed in the imaging centers of the provincial capitals in Iran between 2007 and 2008. These were composed of 1755 patients who were settled residents of 24 provincial capitals. Data on the regional drinking water composition, obtained from an accredited registry, and their relationships with the region’s incidence of urinary calculi were evaluated by metaregression models. The stone risk index (defined as the ratio of calcium to magnesium-bicarbonate product in drinking water) was used to assess the risk of calculus formation.Results. No correlation was found between the urinary calculus incidence and the amount of calcium, bicarbonate, or the total hardness of the drinking water. In contrast, water magnesium had a marginally significant nonlinear inverse relationship with the incidence of the disease in the capitals (R2 = 26%, P =. 05 for a power model). The stone risk index was associated nonlinearly with the calculus incidence (R2 = 28.4%, P =. 04).Conclusions. Urinary calculus incidence was inversely related with drinking water magnesium content. We introduced a new index constructed on the foundation of a pathophysiologically based formula; the stone risk index had a strong positive association with calculus incidence. This index can have therapeutic and preventive applications, yet to be confirmed by clinical trials.

Introduction. Hyperuricemia is an independent risk factor for kidney dysfunction in diabetic patients. On the other hand, albuminuria is considered as the proxy of early stages of diabetic nephropathy. We investigated the correlation between hyperuricemia and albuminuria in patients with diabetes mellitus.Materials and Methods. In a cross-sectional study of 1275 patients (555 men and 720 women) with type 2 diabetes mellitus, serum uric acid and urinary albumin-creatinine ratio were determined. Other metabolic parameters including lipid profile, hemoglobin A1c, glomerular filtration rate, body mass index, blood pressure, blood glucose were assessed, as well.Results. The mean age of the patients was 52.45 ± 10.11 years old. Serum uric acid levels for normoalbuminuric, microalbuminuric, and macroalbuminuric patients were 4.49 ± 1.22 mg/dL, 4.84 ± 1.52 mg/dL, and 6.15 ± 1.68 mg/dL, respectively. Among patients with clinical metabolic syndrome, 233 (27.5%) were in the forth upper quartile of uric acid level (> 5.3 mg/dL), but in diabetic patients without this syndrome, only 80 (18.7%) were in this group. There was a significant relationship between hyperuricemia and serum triglyceride, fasting blood glucose, hemoglobin A1c, glomerular filtration rate, and serum creatinine levels (P <. 001). No significant correlation was found between hyperuricemia and cholesterol levels, age, duration of diabetes mellitus, and body mass index. Serum uric acid level correlated positively with urinary albumin-creatinine ratio (P =. 04).Conclusions. We showed that higher serum uric acid concentrations were associated with a greater probability of albuminuria in patients with type 2 diabetes mellitus

Introduction. Fasciotomy may increase the morbidity and mortality in patients with crush-induced acute kidney injury (AKI), by creating an open wound, increasing the risk of bleeding, coagulopathy, and potentially fatal sepsis. This study evaluates the outcomes of fasciotomy in these patients after Bam earthquake in Iran.Materials and Methods. We reviewed medical records of victims of Bam earthquake complicated with crush-induced AKI. Demographic, biochemical, and clinical data of patients who underwent fasciotomy were evaluated and compared with other patients with AKI.Results. Fasciotomy was performed for 70 of 200 patients with crush-induced AKI (35.0%). There were no significant differences regarding sex, age, time under the rubble, and muscle enzymes level between these patients and those without fasciotomy. They did not experience higher rates of disseminated intravascular coagulopathy, sepsis, adult respiratory distress syndrome, amputation, and dialysis session. Neither did they have a longer hospitalization period or higher death rate.Conclusions. This study showed that fasciotomy did not have any deteriorating effect on morbidity and mortality of patients with crush-induced AKI after Bam earthquake.

Introduction. This study was conducted to determine the effect of statins on the serum levels of interleukin-6 (IL-6), low-density lipoprotein cholesterol (LDLC), and high-sensitivity C-reactive protein (HSCPR).Materials and Methods. This randomized clinical trial was carried out on 95 hemodialysis patients divided into three groups of atorvastatin, 10 mg; simvastatin, 20 mg; and lovastatin, 40 mg, daily, administered for 2 months. Levels of serum HSCRP, IL-6, and LDLC were all measured before and after the study period.Results. At baseline, 59% of the hemodialysis patients presented with elevated HSCRP, 46.3% them had increased IL-6, and 26.3% had an increased LDLC level. The three drugs were capable to lower the level of HSCRP, among which atorvastatin had the highest effect size (41.8% reduction, P =. 001). Lovastatin stood in the next (37.6% reduction, P =. 02), while HSCRP reduction was not significant in the simvastatin group (25% reduction, P =. 14). Neither of the drugs significantly reduced IL-6 levels. Effects of atorvastatin and simvastatin on the LDLC levels were significant, while lovastatin had a marginal effect.Conclusions. Use of statins resulted in CRP reduction in patients on hemodialysis. Atorvastatin was much more effective than lovastatin, while CRP reduction was not significant by simvastatin. However, simvastatin had the greatest impact on LDLC. None of these drugs could reduce IL-6 levels within 2 months.

Introduction. Arteriovenous fistulas and grafts are two common permanent access methods for hemodialysis. Selection of an appropriate access, appropriate surgical techniques, and treatment of complications are basic principles of long-term maintenance of vascular access. We aimed to assess the survival of arteriovenous fistulas and grafts in our dialysis center.Materials and Methods. A total of 200 cases of vascular access placement in 155 patients were reviewed. Data on the type of access and the duration of a functional access were collected for survival analysis.Results. The median duration of dialysis was 70.6 months. Among different methods of vascular access, elbow's wrinkle arteriovenous fistulas (57.0%) were the most frequently used vascular access. The longest mean survival period based on the anatomical location was seen in patients with the arteriovenous fistulas of the wrist (100.2 months); however, there were no significant relationships between anatomical location and durability of vascular access. The mean survival of vascular access was 96.4 months in men and 104.9 months in women. The mean vascular access survival in diabetic patients was 78.8 months as compared with 101.6 months in nondiabetics.Conclusions. Antecubital arteriovenous fistulas were the most common forms of vascular access among our hemodialysis patients; however, arteriovenous fistulas of the wrist provided a better survival. Monitoring of the arteriovenous access and early diagnosis of its insufficiency can guarantee timely intervention and a better survival time

Introduction. It has been shown that inflammation affects thyroid function. In patients with end-stage renal disease, low plasma triiodothyronine (T3) may be an unsuspected expression of the inflammatory state of these patients. This study evaluated the correlation between T3 and high-sensitivity C-reactive protein (HSCRP) levels in patients on peritoneal dialysis (PD) and hemodialysis.Materials and Methods. This is a cross-sectional study aiming at the correlation between T3 and HSCRP levels among 30 patients on PD, 30 patients on hemodialysis, and 20 healthy individuals. Serum levels of HSCRP, T3, thyroxine (T4), thyroid stimulating hormone, T3 resin uptake, and free T3 index (FT3I) and free T4 index (FT4I) were compared between the three groups.Results. There were no significant differences between hemodialysis and PD patients in respect to T3, T4, FT3I, and FT4I. In PD and hemodialysis patients, T3 and FT3I were lower than in controls (P <. 001), but there was no significant difference between PD and hemodialysis patients. T3 resin uptake and thyroid stimulating hormone differed significantly between PD and hemodialysis patients. There was a significant inverse correlation between HSCRP and T3 and FT3I among hemodialysis patients (P =. 04); however, there was no such correlations in PD patients.Conclusions. The relationship between T3 and HSCRP suggests that inflammation might be involved in the low T3 syndrome in hemodialysis patients, but we did not find a significant correlation between T3 and HSCRP levels in patients on peritoneal dialysis

Introduction. Hyperhomocysteinemia is common in patients with chronic kidney disease. There is a direct relationship between cardiovascular mortality and increase of blood homocysteine. Folic acid is used as common treatment in such patients. Folinic acid, a shortened form of folic acid, is not affected by inhibitors of dihydrofolate reductase enzyme such as methoterxate. This study was performed to evaluate the effect of oral folinic acid on the blood homocysteine level of hemodialysis patients, in comparison with folic acid.Materials and Methods. This clinical trial was performed on 60 hemodialysis patients. The participants were divided into 2 groups to receive either 15 mg of oral folic acid or 15 mg of oral folinic acid, daily. Blood homocysteine levels were measured before dialysis and after the study period.Results. Folic acid and folinic acid decreased the blood homocysteine levels by 33.0% and 28.7%, respectively (P <. 001). However, only 3 patients (6.5%) enjoyed a normalized homocysteine level.Conclusions. Our study showed that both folic and folinic acid decreased the blood homocysteine level and no meaningful difference was observed between them; therefore, we suggest they can be used interchangeably.

Introduction. This study was conducted to evaluate the sensitivity and specificity of pyuria detection in centrifuged urine samples of patients on hemodialysis, and its relationship with urinary tract infection.Materials and Methods. Clean-catch midstream urine samples of 90 hemodialysis patients (34 women and 56 men) were obtained and divided into two parts for examination of urine sediment and urine culture. Pyuria was defined as the presence of more than 10 leukocytes per high-power field of microscope.Results. Ninety patients with a mean age of 52.8 ± 14.2 and a mean period of dialysis of 3.3 ± 2.3 years were studied. Forty-five participants had pyuria and only 16 (35.5%) of them had a positive urine culture for infection. Pyuria and urinary tract infection were present in 52.9% and 29.4% of the women and 48.2% and 10.7% of the men, respectively. The sensitivity and specificity of pyuria screening for urinary tract infection was 100% and 61.8%, respectively. The positive and negative predictive values were 35.5% and 100%, respectively.Conclusions. In patients on hemodialysis, because of the low specificity and positive predictive values, samples with positive pyuria should be cultured to confirm urinary tract infections

Introduction. This study aimed to compare outcomes of kidney transplantation in patients with systemic lupus erythematosus (SLE) and a matched control group of non-SLE kidney recipients.Materials and Methods. In a case-control study, 33 patients with kidney transplantation due to end-stage renal disease caused by SLE were matched to a control group consisted of 33 non-SLE patients who had been transplanted during the same period of time in our center. The clinical characteristics, complications, and patient and graft survival were compared between the two groups.Results. In each group, 12 patients (36.4%) received a kidney from a deceased donor, 15 (45.4%) from a living unrelated donor, and 6 (18.2%) from a living related donor. There was no significant difference between the outcome in SLE patients and duration of dialysis before transplantation. The mean duration of hospital stay was 23.4 ± 18.1 days in the SLE group, while it was 13.0 ± 7.3 days in the controls (P =. 006). One-year graft survival was 79.0% in patients with SLE and 90.9% in non-SLE patients (P =. 17). One-year patient survival was 93.9% in patients with SLE versus 81.8% in the controls (P =. 26). Nine patients in the SLE group versus 11 patients in the control group developed posttransplant complications (P =. 59).Conclusions. Although hospital stay after transplantation was longer in the SLE kidney recipients than controls, safety of kidney transplantation was comparable. Graft failure in the SLE patients was not significantly different between patients with different sources of kidneys

Introduction. After kidney transplantation, patients appear to have vitamin D deficiency due to the use of immunosuppressive treatment and prevention of sunlight. This study was designed to determine vitamin D serum levels in kidney transplant patients in comparison with healthy individuals.Materials and Methods. Forty-six kidney transplant patients with a creatinine clearance greater than 60 mL/min and 46 healthy individuals with normal kidney function were tested for serum levels of calcium, phosphorus, 25-hydroxyvitamin D, and parathyroid hormone at the end of the summer.Results. Thirty-one participants were men and 15 were women in each group. The mean age was 41.0 ± 14.2 years in kidney transplant recipients and 41.4 ±13.7 years in the control group. Inadequate serum 25-hydroxyvitamin D was seen in 93.5% of the transplant patients and in 89.1% of the controls. There was a 58.7% vitamin D insufficiency (20 ng/mL to 30 ng/mL) and a 34.8% deficiency (lower than 20 ng/mL) in the patients, and these rates were 58.7% and 26.0% in the control group, respectively. There was no significant difference between the two groups.Conclusions. Vitamin D deficiency is prevalent in kidney transplant patients. Lack of a significant difference between our two groups may be attributable to the high prevalence of vitamin D deficiency in general population and the use of vitamin D supplementation in transplant patients. Indeed, adequate doses of vitamin D in these patients are undetermined. They may need higher doses for normalization of serum vitamin D and metabolic requirements.

Renal insufficiency occurs in a considerable proportion of patients with sickle cell disease. Common advanced glomerular lesions include focal segmental glomerulosclerosis and nonimmune membranoproliferative glomerulonephritis. Due to the paucity of data supporting an immune-mediated pathophysiology, anti-inflammatory and immunosuppressive therapies have not been successfully evaluated in such patients. We present a case of membranoproliferative glomerulonephritis in a postpartum patient with sickle cell disease, where treatment with steroids was helpful.

Tubulointerstitial nephritis and uveitis (TINU) is a rare syndrome with unknown pathogenesis. Data have shown a higher prevalence in female gender. We present a man with tubulointerstitial nephritis and uveitis syndrome and antitubular antibody. A 23-year-old man presented with a history of weight loss, nausea, and vomiting, and uveitis. His serum creatinine was 2.1mg/d with pyuria and proteinuria in urinalysis. Other laboratory and imaging studies were unremarkable. Kidney biopsy showed granulomatous interstitial nephritis. Normal renal tissue specimen treated with patient’s serum showed focal cytoplasmic staining in cortical tubular cells. The patient received prednisolone for 1 month. Interstitial nephritis and uveitis were well controlled. There was no recurrence in 1-year follow-up. We suggest that tubulointerstitial nephritis and uveitis syndrome should be considered in differential diagnosis of patients with interstitial nephritis and uveitis. Antitubular antibody may be used as a diagnosis marker for this syndrome.