Iranian Journal of Kidney Diseases
Volume:3 Issue: 4, Oct 2009

During the past decades, the number of altruistic living unrelated kidney donations has substantially increased in developed countries. However, the altruistic supply of transplantable kidneys has remained much less than the demand. As a result, severe kidney shortage has been associated with increasing number of patient deaths and increasing number of commercial transplants and transplant tourism. Studies have shown that there is still a need for living kidney donation because even all potential brain-dead donors cannot supply the escalating need for kidneys. The use of living unrelated kidney donors should be morally and ethically justified and should be compatible with ethical principles. Many experts believe that increasing number of patient deaths and commercial transplants will continue to happen if kidney donation system remains merely altruistic. While some transplant professionals support a paid and regulated system to eliminate kidney shortage, others argue that it will be destructive. Iran has a 20-year experience with a compensated and regulated living unrelated kidney donation program. This transplantation model was adopted in 1988, and successfully eliminated kidney transplant waiting list by the end of 1999. Currently, more than 50% of patients with end-stage kidney disease in Iran are living with a functioning graft. This Iranian transplantation model has many ethical successes. However, because it has not been well regulated by transplant ethicists, some ethical shortcomings have remained. Unfortunately, due to lack of interest and expertise in health authorities, the number of serious ethical failures is also increasing in this transplantation model.

The rapid increase in the prevalence of end-stage renal disease (ESRD) necessitates putting into practice some strategies to prevent its development and progression, especially in the developing world. Detailed chronological changes in the incidence of ESRD may sharpen the focus on its prevention. We, therefore, determined the detailed epidemiological features of ESRD in Iran. Data of the national registry of Iran’s ESRD provided by the Ministry of Health were used to retrieve the ESRD figures between 1997 and 2006. A total of 35 859 patients who initiated renal replacement therapy (20 633 men and 15 226 women) were registered during the study period from 1997 to 2006. The annual number of patients with ESRD beginning maintenance treatment in Iran increased 130% between 2000 and 2006. During 1997 to 2006, the proportion of new cases of ESRD attributed to diabetes mellitus increased 2-fold from 16% in 1997 to 31% in 2006. The mean age of newly registered men and women increased from 47.0 years and 49.0 years to 52.5 years and 53.0 years, respectively. As for all and major causes of ESRD, age-adjusted incidence rates for men generally were higher than those for women. Male-female ratio was 1.3:1, with no significant changes during this period. We strongly recommend considering chronic kidney disease prevention with initial focusing on strategies and treatment modalities that slow ESRD progression in order to postpone the need for renal replacement therapy.

We assessed whether cosupplementation of vitamins C and E has additive beneficial effects on reducing the kidney damage and attenuation of the arterial pressure elevation compared to administration of either vitamin C or vitamin E alone in deoxycorticosterone acetate-salt-induced hypertension. Forty rats were divided into 4 study groups and 1 sham-operated group. Unilateral nephrectomy was carried out in the study groups and hypertension was induced by deoxycorticosterone injection and 1% sodium chloride and 0.2% potassium chloride added to the drinking water. Vitamins C and E (200 mg/kg/day) or combination of them were administered with DOCA-salt for 4 weeks in 3 study groups. The effects of DOCA and salt and treatment with vitamins were compared in terms of blood pressure, urinary protein excretion, antioxidant activity of the kidneys, and renal histological changes. Four weeks of supplementations of vitamins C, vitamin E, and both in the DOCA-salt-treated rats had comparable significant effects in decreasing systolic blood pressure. Urinary protein excretion and histological damage did not significantly change with the combination therapy of vitamins C and E compared to the vitamin C or E alone. The renal levels of glutathione and ferric reducing/antioxidant power in combination therapy group were similar to the two other treatment groups and were significantly higher than non-treated group. Co-administration of vitamin C and E does not have an additive beneficial effect on reducing the kidney damage and hypertension compared to either vitamin C or E alone in DOCA-salt-induced hypertension.

Instruction: We investigated the correlation between atherosclerosis and tissue and serum levels of endothelin-1 in patients with chronic kidney disease (CKD). Arterial samples were obtained from 35 patients with CKD during arteriovenous fistula placement. Thirty-one patients with cardiovascular disease who underwent coronary artery bypass graft (CABG) were selected as the atherosclerotic group, and a piece of their aorta punched during CABG was obtained. Also, a small piece of the renal artery was dissected during donation in 24 kidney donors (control group). Tissue endothelin-1 level was measured and atherosclerosis grading was determined by pathologic examination. Serum levels of endothelin-1 were also measured in the three groups. The mean tissue endothelin-1 levels were 10.73±7.57 pg/mL, 12.16 ± 3.95 pg/mL, and 0.93 ± 1.06 pg/mL in the patients with CKD, those with CABG, and donors, respectively (P <. 001). The mean serum endothelin-1 level was 25.23 ± 15.15 pg/mL in the patients with CKD, 21.13 ± 17.22 pg/mL in the patients with CABG, and 2.66 ± 1.51 pg/mL in the donors (P <. 001). Atherosclerosis grades correlated with tissue endothelin-1 level (r = 0.823, P <. 001) and serum endothelin-1 level (r = 0.608, P <. 001) in the patients with CKD. Multiple regression analysis showed tissue endothelin-1 level as the main predicting factor of atherosclerosis (P <. 001). Tissue endothelin-1 concentration is more important than serum endothelin-1 or lipids levels in prediction of atherosclerosis. Thus, blockade of tissue endothelin-1 receptors with its antagonists may prevent atherosclerosis progression.

Steroid-resistant nephrotic syndrome (SRNS) is uncommon in children, but often leads to ESRD. We report our experience with SRNS and its treatments and outcomes. We assessed 73 children with SRNS admitted to Ali Asghar Children Hospital in Tehran, Iran. Their clinical presentations, treatment, and disease courses were reviewed. The mean follow-up duration was 6.0 ± 4.2 years. Moreover, survival times were calculated and the Cox regression method was used to determine variables able to predict survival of the kidneys. Age at the onset of the disease, sex, and hematuria were not predictive of the response to treatment with immunosuppressive drugs in the children with SRNS. The type of resistance (early or late) was associated with the responsiveness to immunosuppressives. Response to any of the immunosuppressive drugs determined the responsiveness to other immunosuppressive drugs. Cyclosporine was more effective than cyclophosphamide as initial therapy. The mean kidney survival time was 11.62 years. Kidney survival rates were 94.6%, 70.0%, 56.0%, and 34.0% at 1, 5, 10, and 15 years, respectively, in patients with initial resistance to steroid, while these were 100%, 100%, 83.0%, and 83.0% in those with late resistance, respectively (P =. 03).We showed that patients with late steroid resistance had better response to immunosuprressive drugs than patients with early resistance. We also showed that resistance to immunosuppressive therapies increased the risk of resistance to other immunosuppressive drugs. Achievement of complete or partial remission with any therapy reduced the risk of ESRD.

Cystinosis is an autosomal recessive disorder which is characterized by both renal and extrarenal symptoms. Gastrointestinal dysfunction has been reported in adolescent with cystinosis, and it is rarely considered in the infants. The present case series reviewed gastrointestinal manifestations of these patients. Gastrointestinal signs and symptoms of 23 children aged 5.99 ± 0.50 years (range, 1.0 to 12.5 years) on average with cystinosis, admitted to our department of nephrology between 1996 and 2005, were retrospectively reviewed. The inclusion criteria were the presence of the crystals of cystine in bone marrow aspiration and corneal deposition detected by slit lamp examination. Gastrointestinal signs and symptoms were as follows: vomiting in 16 patients (69.6%), hepatomegaly in 8 (34.8%), diarrhea in 6 (26.1%), splenomegaly in 5 (21.7%), constipation in 4 (17.4%), anorexia in 4 (17.4%), abdominal pain in 3 (13.0%), nausea in 2 (8.7%), and ascites in 2 (8.7%). Height below the 3rd percentile in was seen in 16 patients (69.6%) and weight below the 3rd percentile, in 17 (73.9%). Fifteen patients (65.2%) had both low weight and low height. Esophagogastroduodenoscopy had been performed in 6 cases and chronic inactive gastritis with H pylori infection was detected in 2 patients (8.7%). Our study revealed a wide spectrum of gastrointestinal disturbances in young patients with cystinosis. Such findings should lead to greater awareness of the presence of gastrointestinal dysfunction in these children, encourage prompt gastrointestinal evaluation, and encourage treatment of more severely affected patients.

Recent studies have suggested theophylline for prevention of kidney dysfunction in asphyxia. This study was designed to determine whether theophylline could prevent or ameliorate kidney dysfunction in term neonates with perinatal asphyxia. We assigned 36 severely asphyxiated term infants (Apgar score? 5) into 2 groups to receive intravenously a single dose of either theophylline (5mg/kg; n =17) or placebo (n =19) during their first 60 minutes of life. The 24-hour fluid intake and the urine volumes were recorded during the 1st, 3rd, and 5th days of life. Severe kidney dysfunction was defined as a serum creatinine level elevated up to more than 1.50 mg/dL for at least 2 consecutive days after a fluid challenge, or 0.3-mg/dL/d rising levels of serum creatinine.On the 1st day, the 24-hour fluid balance was more positive in infants receiving placebo compared to infants receiving theophylline. Over the next few days, the change in fluid balance favored the theophylline group. Significantly higher serum creatinine values were recorded in the placebo group on the 3rd day. Severe kidney dysfunction was present in 2 infants of the theophylline group (11.7. %) and in 8 (42.1%) of the controls. The glomerular filtration rate was markedly increased in the theophylline group. There was no difference in the severity of the asphyxia between the infants of the theophylline and control groups. Prophylactic theophylline, given early after birth, has beneficial effects on reducing kidney dysfunction in neonates with asphyxia.

We compared the effect of higher and lower doses of folic acid compared to our routine daily dose on plasma homocysteine levels, in our hemodialysis patients. Eighty patients on hemodialysis receiving oral folic acid, 10 mg/d, were randomized to receive folic acid at either doses of 5 mg/d (group 1) or 15 mg/d (group 2) for 2 months. Plasma levels of total homocysteine were measured before and after the study period.Hyperhomocysteinemia was seen in 75 patients (93.8%) before, and in 37 patients of group 1 (92.5%) and 39 of group 2 (97.5%) after the study period. In group 1, a nonsignificant decrease occurred in plasma homocysteine level (29.67 ± 12.26? mol/L to 27.78 ± 9.94? mol/L, P =. 30), while in group 2, there was a significant decrease in homocysteine level (32.40 ± 9.76? mol/L to 29.58 ± 9.62? mol/L, P =. 01). Changes in homocysteine level correlated with its baseline level (r = -0.42, P <. 001). In both groups, significant reductions in homocysteine level were seen mostly in those patients with high baseline homocysteines. Routine folic acid supplementation of 10 mg/d could not normalize plasma homocysteine levels in most of our patients. Increasing folic acid dose made a statistically significant but clinically trivial decrease in homocysteine levels, and could not normalize homocysteine level in most patients. Patients with a higher baseline homocysteine level achieved a greater reduction, which may be explained by primary noncompliance of some patient. Further investigation of folic acid dosage is suggested.

Serum cystatin C is more sensitive for glomerular filtration rate (GFR) measurement, but it is not available for clinical use in all laboratories. Regarding the importance of accurate estimation of GFR in kidney transplant recipients, we compared cystatin C-based equations with creatinine-based formulas to estimate GFR as precisely and simply as possible in kidney transplant recipients.Materials and Methods Seventy living donor kidney transplant recipients with stable kidney function were enrolled in our study. The patients’ GFRs were estimated by 3 creatinine-based equations (the modification of diet in renal disease [MDRD], abbreviated MDRD, and Cockcroft-Gault) and 5 cystatin C-based equations (Filler, Le Bricon, Rule, Hoek, and Larsson), and the results were analyzed. The mean age of the recipients was 38.7 ± 13.4 years. The mean GFRs were 67.1 ± 25.9 mL/min/1.73 m2, by the Cockcroft-Gault; 61.0 ± 17.7 mL/min/1.73 m2, by the abbreviated MDRD; and 60.0 ±18.6 mL/min/1.73 m2, by the MDRD formulas. Cystatin C-based GFR estimations were 43.6 ± 16.2 mL/min/1.73 m2, 44.0 ± 13.2 mL/min/1.73 m2, 33.8 ± 14.1 mL/min/1.73 m2, 35.6 ± 13.7 mL/min/1.73 m2, and 36.9 ± 13.6 mL/min/1.73 m2 by the Filler, Le Bricon, Larsson, Rule, and Hoek equations, respectively. The estimates by creatinine-based and cystatin C-based equations were significantly different and the MDRD estimate was the closest to the cystatin C-based GFRs. Our findings revealed the MDRD equation could be provide a closer estimate of GFR to the cystatin C-based equations than other creatinine-based GFR calculations in kidney transplant recipients.

We reviewed records of patients with ESRD in all dialysis and kidney transplant centers of Guilan province to determine the incidence and modalities for treatment of ESRD from 2005 to 2007. Records of 759 patients were reviewed during the 3-year period of the study. The male-female ratio was 1.34:1 and the men age at the time of diagnosis was 56.3 ± 16.8 years. The most frequent age group was 60 years and older (45.8%). The incidence ESRD during 2005, 2006, and 2007 were 100.36 pmp, 100.60 pmp, and 110.66 pmp, respectively. The most common causes of ESRD were hypertension (29.4%), unknown (26.6%), and diabetes mellitus (17.9%). The most common modality at initiation of renal replacement therapy was hemodialysis (96.0%). The entrance rate of hemodialysis in 2005, 2006, and 2007 were 29.6%, 35.0%, and 35.4%, and the discontinuing rate of hemodialysis in these years were 29.5%, 32.1%, and 38.4%, respectively.

Although the revised dyadic adjustment scale (RDAS) has been widely used as an indicator of the quality of marital relationship, no report is available on the reliability of this measure in patients on hemodialysis. We examined the internal consistency of the RDAS in a group of Iranian patients undergoing maintenance hemodialysis. A translated Persian version of the RDAS was self-administered to 135 patients. The internal consistency of the RDAS was tested using the Chronbach? coefficient which was 0.898, 0.683, 0.779, 0.827, and 0.836 for the RDAS total score and the dyadic consensus, affective expression, dyadic satisfaction, and dyadic cohesion subdomains, respectively. All of the Chronbach? scores were higher in patients with higher income and education level. Using the RDAS to examine marital relationship quality in patients on hemodialysis, the total score and almost all subscores except for dyadic consensus had adequate internal consistency

Autosomal dominant polycystic kidney disease is associated with cysts in many organs including the liver, pancreas, lungs, spleen, ovaries, testes, thyroid, and uterus. However, there is no report, to our knowledge, of cystic changes of the breast along with this disease. We describe 3 members of a family with multiple bilateral breast cysts in association with autosomal dominant polycystic kidney disease