فهرست مطالب

Kidney Diseases - Volume:12 Issue: 6, Nov 2018

Iranian Journal of Kidney Diseases
Volume:12 Issue: 6, Nov 2018

  • تاریخ انتشار: 1397/08/12
  • تعداد عناوین: 9
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  • Seçil Arslansoyu Camlar *, Alper Soylu, Salih Kavukçu Pages 319-330
    Cyclosporine began to be used as one of the immunosuppressive agents in transplantation in the beginning of the 1980s, and in the treatment of nephrotic syndrome in the pediatric nephrology. Therapeutic area of cyclosporine is narrow and its side effects limit its usage. Preference for cyclosporine and tacrolimus as a calcineurin inhibitor is left for the choice of the department. There are still countries with preferred-cyclosporine use because of economic reasons. Cyclosporine is currently being used in the treatment of nephrotic syndrome, but due to its high relapse rates in a short-term use, and nephrotoxicity in long-term use, search for new drugs with fewer side effects keeps continuing. As long as its use is indispensable, it will be necessary to keep track of kidney function and blood level of this medication closely to protect the patients from toxicity.
    Keywords: cyclosporine, pediatric nephrology, sideeffects, transplantation, nephrotic syndrome
  • Doaa Mohammed Youssef *, Mohamed Abdelsalam Gomaa, Ahmed El, Akhras, Sabry Abdel Rahman Tolba, Ghada Mohamed Abd Allah, Osama Daoud, Sameh Saber Pages 331-340
    Introduction: Disturbances of lipid metabolism has been reported in nephrotic syndrome (NS) and may predispose to atherosclerosis. This study aimed to investigate the correlation between cardiovascular risk factors and carotid intima-media thickness (CIMT) and brachial artery flow-mediated dilatation in patients with idiopathic NS. Materials and methods: This case-control study included 31 patients with NS and 31 healthy individuals as the control group. All patients were subjected to full clinical examination; laboratory investigations in the form of lipid profile, kidney function tests, serum protein, serum albumin, C-reactive protein, and ferritin; carotid ultrasonography, and brachial artery flow-mediated dilatation. Results: Serum cholesterol, low-density lipoprotein cholesterol, and triglyceride levels was significantly higher in the case group than the control group. High-density lipoprotein cholesterol and albumin levels were significantly lower in the case group. The absolute change in brachial artery diameter was significantly lower in the case group than that of the control group. Proportionate change in brachial artery diameter was significantly lower in the case group than that of the control group. Common carotid artery CIMT in the case group was significantly higher than that of the controls. Lastly, there were significant increases in weight and body mass index in the relapse group than the remission group. Conclusions: Patients with NS are more prone to atherosclerosis and vascular changes; CIMT was thicker in nephrotic children compared to the controls. The significantly abnormal values of flow-mediated dilatation in children with NS suggests an ongoing process of endothelial dysfunction.
    Keywords: nephroticsyndrome, carotid intimamediathickness, brachialartery flow-mediated dilatation, atherosclerosis
  • Alaleh Gheissari, Rokhsareh Meamar, Majid Kheirollahi, Maedeh Rouigari, Moein Dehbashi, Leila Dehghani, Amin Abedini * Pages 341-349
    Introduction: Focal segmental glomerulosclerosis (FSGS) ranks among nephrotic syndromes. Research shows that FSGS is brought about by several genes including transient receptor potential cation channel subfamily c member 6 (TRPC6). This study aimed to investigate TRPC6 gene in Iranian FSGS children. Materials and Methods: Twenty-six FSGS patients were included. They were all under 16 years old. Polymerase chain reaction amplification and sequencing were performed to examine exons 2 and 13 of TRPC6 gene. Results: Sampling was performed when the patients had a mean age of 9.26 ± 3.19 years. Sixteen children were boys (61.5%); male-female ratio was 1.35:1. Four patients (15.4%) were diagnosed with TRPC6 variants. Three missense nonsynonymous mutations (C121S, D130V, and G162R) and 1 synonymous mutation (I111I) were detected. All variants were novel; in silico analysis predicted D130V and G162R as pathogenic. Patients with and without mutations were not different significantly regarding age at disease onset, sex, consanguinity, hypertension, hematuria, serum creatinine and albumin, rate of progression to kidney failure, response to steroids, and resistance to cyclosporine A and cyclophosphamide. Conclusions: This study examined exons 2 and 13 of TRPC6 gene in Iranian FSGS children. Four novel TRPC6 variants were detected; in silico analysis showed that 2 variants (D130V and G162R) could be pathogenic. It could be concluded that TRPC6 may be useful for genetic screening in Iranian FSGS children.
    Keywords: TRPC6, focalsegmental glomerulosclerosis, missense mutation, steroidresistantnephrotic syndrome
  • Fatemeh Bitarafan, Masoud Garshasbi * Pages 350-358
    Introduction: A wide variety of mutations are spread throughout the PKHD1 gene, which encodes a 4074-bp amino acid protein, namely fibrocystin/polyductin, and is responsible for all features of autosomal recessive polycystic kidney disease (ARPKD). Autosomal recessive polycystic kidney disease is a hereditary early-onset form of polycystic kidney disease characterized by fusiform dilation of collecting ducts and congenital hepatic fibrosis. The highest level of PKDH1 expression is in the kidneys of fetus and adults, suggesting the functionally importance of the gene in the mature kidney in addition to its role in kidney development. Materials and Methods: Mutational analysis of the PKHD1 gene was performed in 11 families with a history of 1 to 6 fetuses or children affected by ARPKD, which either were aborted or died shortly after birth. Analyses were done using the Next Generation sequencing and Sanger sequencing techniques. Results: Four novel mutations, including c.6469C>T, c.9218 G>A, c.10456T>C, and c.8863C>G, and 3 previously reported ones, including c.9524A>G, c.1095G>A, c.1123C>T, were identified. Conclusions: In view of high consanguineous marriages in Iranian population, the frequency of disease is expected to be higher than the world average.
    Keywords: autosomalrecessive polycystic kidneydisease, PKHD1, genemutations
  • Bahman Bashardoust, Anahita Zakeri, Nasrin Fouladi, Zeinab Izadi *, Fariba Hosseini Pages 359-363
    Introduction: Vitamin D deficiency is a common problem in end-stage renal disease patients under hemodialysis. Both active and nutritional vitamin D supplementation have been recommended for its treatment. In this study we aimed to evaluate the effects of treatment with ergocalciferol on bone metabolism indexes in hemodialysis patients. Materials and Methods: In a randomized controlled trial, 40 hemodialysis patients were randomly allocated to the intervention (n = 20) and placebo (n = 20) groups. During the study, 4 patients in the placebo and 1 in the intervention group were excluded. Patients received calcitriol, 0.25 mg/d, with ergocalciferol, 50 000 IU, or placebo weekly for 3 months. Serum levels of 25-hydroxyvitamin D, calcium, parathyroid hormone, and alkaline phosphatase were measured before and after treatment. Results: 25-hydroxyvitamin D levels were significantly improved in the intervention group (12.00 ± 4.90 ng/mL versus 29.89 ± 9.48 ng/mL, P < .001), but the placebo group had no improvement (14.23 ± 7.62 ng/mL versus 13.87 ± 8.04 ng/mL, P > .05). There was no significant changes in serum calcium, parathyroid hormone, or alkaline phosphatase levels in each group. Eight patients (42.1%) in the intervention compared to zero cases in the placebo group had normal 25-hydroxyvitamin D levels after treatment (P = .004). No cases of hypercalcemia were seen in the studied patients. Conclusions: Treatment with ergocalciferol could significantly improve vitamin D deficiency with no significant effects of serum calcium or parathyroid hormone levels.
    Keywords: end-stage renaldisease, hemodialysis, ergocalciferol, Vitamin D
  • Maryam Miri, Sepideh Hejazi, Forough Maghsoudlou, Mahnaz Ahmadi * Pages 364-368
    Introduction: Pulmonary artery hypertension is a serious comorbidityof dialysis in patients with end-stage renal disease. The prevalence of dialysis-induced pulmonary artery hypertension is still a subject of debate. The aim of this study was to determine the prevalence of pulmonary artery hypertension in patients undergoing hemodialysis and peritoneal dialysis. Materials and Methods: This cross-sectional study was conducted on patients undergoing either hemodialysis or peritoneal dialysis in Montaserieh Dialysis Center in Mashhad, Iran during 2015 and 2016. Pulmonary artery pressure, ejection fraction, and serum levels of calcium, phosphorus, creatinine, and parathyroid hormone were measured. Results: A total of 50 patients (25 on hemodialysis and 25 on peritoneal dialysis) participated in the study. The mean age of the participants was 34 ± 12 years. The mean pulmonary artery pressure was significantly higher in the hemodialysis group compared to the peritoneal dialysis group (P < .001). Serum calcium was significantly higher in the peritoneal dialysis group compared (P = .04). Pulmonary artery hypertension was observed in 11 patients (22%), all of whom were in the hemodialysis group. There was a significant negative relationship between serum calcium and pulmonary artery pressure (P< .01). Hemodialysis was significantly related to higher pulmonary artery pressure (P < .001). Conclusions: This study revealed a high prevalence of pulmonary artery hypertension among end-stage renal disease patients undergoing dialysis. This study also found a novel significant negative relationship between serum calcium level and pulmonary artery pressure, and hemodialysis was found to be significantly related to higher pulmonary artery pressure.
    Keywords: pulmonary arteryhypertension, hemodialysis, peritoneal dialysis
  • Mohsen Nafar, Shiva Samavat, Elham Shahraki * Pages 369-375
    Introduction: Chronic allograft nephropathy is characterized by interstitial fibrosis and tubular atrophy. The main players in the process of fibrosis are transforming growth factor-β (TGF-β) and miR-21 expression with a bidirectional interplay. This study aimed to evaluate the effects of angiotensin receptor type 1 antagonist, losartan, on peripheral blood and tissue expression of TGF-β and miR-21 and histologic findings in allograft biopsy in kidney transplant recipients. Materials and Methods: In a randomized controlled trial, 54 patients were enrolled and divided randomly into 2 groups. Group 1 was treated with a daily dose of 25 mg of losartan and group 2 was considered as control. Blood sampling was done at 48 hours posttransplantation and the 3rd and 6th months after transplantation for measurement of TGF-β RNA and miR-21. Protocol biopsy was performed at the 6th month posttransplantation for RNA extraction and histologic evaluation of interstitial fibrosis and tubular atrophy. Results: Although patients were not different initially, those who underwent treatment with losartan had lower miR-21 and TGF-β levels in circulating PBMCs, and there was a decreasing trend in peripheral blood TGF-β levels during the 6-month follow-up period. Tissue expression of miR-21 and TGF-β was also considerably lower among the losartan-treated patients at the time of tissue biopsy. Conclusions: Losartan treatment decreased the tissue expression of miR-21 and TGF-β and tissue fibrosis in kidney transplant patient, and it had a protective effect on allograft function and may delay chronic allograft dysfunction by reducing mediators of fibrosis.
    Keywords: kidneytransplantation_fibrosis_losartan_transforming growthfactor-?_miR21_angiotensinreceptor type 1 antagonist
  • Nooshin Dalili, Babak Behnam, Farzaneh Vali, Mahmoud Parvin, Peyman Torbati, Nakisa Rasaii, Fariba Samadian, Pedram Ahmadpoor * Pages 376-381
    Complement C3 glomerulopathy refers to a disease process in which abnormal control of complement activation or degradation results in predominant C3 fragment deposition within the glomerulus and causes glomerular damage. Abnormal control of the complement alternative pathway is a well-established risk factor for the occurrence of C3 glomerulonephritis. It is the first reported case in Iran with multiple mutations in complement factor H, with one of these mutations we have expected in hemolytic uremic syndrome rather than C3 glomerulopathy Genetic analysis showed that the molecular abnormalities of factor H led to complement factor H malfunction that were polymorphous and not restricted to the C-terminal domains of the protein.
    Keywords: complement C3glomerulopathy, complementpathway, complement factor H, gene mutations
  • Ahmed Yahia Elmowafy *, Hanzada Mohamed El Maghrabi, Mohamed Ahmed Zahab, Salwa Mahmoud Elwasif, Mohamed Adel Bakr Pages 382-384
    Direct antivirals showed dramatic response in hepatitis C virus (HCV) eradication, but their effect on extrahepatic manifestations is still unclear. A 49-year-old woman was referred to us suffering from lower limb edema and frothy urine. Renal biopsy was done and she was diagnosed with HCV-related membranoproliferative glomerulonephritis with cryoglobulinemia. Treatment with interferon plus ribavirin, steroid, and cyclophosphamide was tried but failed. After introduction of a sofosbuvir-based regimen to the treatment, sustained virologic response was achieved and nephrotic syndrome remission was induced successfully. We could conclude that HCV-related membranoproliferative glomerulonephritis with cryoglobulinemia could be treated successfully with immunosuppressive drugs plus sofosbuvir and dacalatasvir.
    Keywords: nephroticsyndrome, direct actingantivirals, vasculitis