In loving memory Professor Mohammad Ismaeili

In loving memory Professor Mohammad Ismaeil

How accurate is ultrasonography for the detection of renal cortical defects

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Acute Kidney Injury in Iranian Children -What Do We Know About It? - Part 2

Acute kidney injury (AKI) is reversible deterioration of renal function in which waste products accumulate and fluid imbalance occurs. The epidemiology of AKI has been changing over years. The aim of this study was to evaluate the epidemiology of AKI in hospitalized children in Iran. A literature search from March 2000 to March 2014 was conducted through MEDLINE, EMBASE, Scholar.google, IranMedex, MagIran, SID, and manual reference search of identified articles. Retrospective and prospective cross-sectional studies with a clear definition of acute kidney injury or failure were included. Seven out of twenty three articles which were found met the criteria. The incidence of AKI declined from 36% (2006-2008) to 15.4% (2010-2011) in the PICU setting of three referral teaching hospitals in Tehran. According to the classification, 10% had pre-renal failure, 86% had intrinsic renal failure, and 4% had post-obstructive uropathy. Follow-up was limited to the days of hospitalization. The overall reported mortality rate was 18% in pediatric departments. Acute glomerulonephritis including hemolytic uremic syndrome was the most common underlying disease (46.5%). Acute tubular necrosis was reported in 33% of the cases. One third of the cases of acute renal failure occurred in children less than two years. The real incidence of acute kidney injury might be higher considering a unified standard definition. Acute glomerulonephritis and acute tubular necrosis comprised the majority of the etiologies. Keywords: Acute Kidney Injury; Middle East; Iran; Etiology; Child; Incidence; Review Systematic

History of Pediatric Nephrology in Iran

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Genetic Study of Nephrotic Syndrome in Iranian Children- Systematic Review

Idiopathic nephrotic syndrome is a heterogeneous disease with a spectrum of age at presentation, phenotype, renal pathology, and response to treatment. Many mutations are recognized to be implicated in sporadic or hereditary forms. The aim of this review was to summarize the results of the genetic studies which have already been carried out in Iran considering their limitations.A literature search was conducted from March 1970 to September 2015 through MEDLINE, EMBASE, Google Scholar, Google, Iran Medex, Magiran, and SID. Eleven studies were relevant. Three articles were excluded due to insufficient data, duplicated case, and a syndromic nephrotic case without genetic studies. Our results showed that in the southwest of Iran, 80% of the patients had mutations in NPHS1 while in Fars Province, one third showed mutations in NPHS2 when all exons were assessed. In two different studies conducted in one center in Tehran, no mutation was detected in exon 5 but when all exons were studied, more than 65% had hot spot mutation in exon 8 of NPHS2. Interestingly, none of adolescents with FSGS showed mutation in p.R229Q (NPHS2, exon 5). This review revealed that both NPHS1 and NPHS2 were prevalent in Iranian children with SRNS. No mutation of p.R229Q was reported in Iranian adolescent with SRNS.Keywords: Nephrin; NPHS2 protein; Nephrotic Syndrome; Glomerulosclerosis; Focal Segmental

Evaluation and Treatment of Growth Failure in Children with CKD- Letter to the editor

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Evaluation of Children with Steroid-resistant Nephrotic Syndrome Showing Pathologic Findings of Focal Segmental Glomerulosclerosis (FSGS) after Renal Transplantation in Iranian Educational Medical Centers from 1998 to 2018

Introduction: Steroid resistant nephrotic syndrome due to idiopathic focal segmental glomerulosclerosis (FSGS) or genetic mutations is one of the most common causes of end-stage renal disease (ESRD) that leads to renal transplantation. The relapse of the disease in the transplanted kidney, despite proper therapeutic management pre and post transplantation, may result in graft loss. Lack of accurate data about the status of transplanted patients due to FSGS encouraged us to obtain data from all pediatric nephrologists in Iran to achieve better pre and post transplantation therapeutic management.Material and Methods: The personal data of the pediatric nephrologist as well as the data of surgical and medical management prior to transplantation, relapse in the allograft kidney, and the therapeutic response rate after relapse were collected via a questionnaire.Results: Of 82 cases of FSGS from 1998 to 2018, 23 had relapse, mostly within 1 year after transplantation. When relapse occurred, nearly all centers used plasmapheresis and rituximab and some used angiotensin converting enzyme inhibitor (ACEI) or angiotensin receptor blocker (ARB) in addition to immunosuppressive medications and methyl prednisolone pulse therapy. Genetic studies were done in only two centers and there was no difference in immunosuppressive medications between these two centers and the idiopathic group. Pre-transplant plasmapheresis and rituximab were administered in four centers, while two centers used IVIG and one center only used plasmapheresis. Delayed graft function (DGF) was negative in 7 and positive in 9 centers. In most centers, immunosuppressive therapy consisted of a corticosteroid, mycophenolate mofetil, and tacrolimus. Relapse and recovery rates varied from less than 10% to more than 50% in all centers. Seven centers had no response to any medication. The lowest relapse rates were seen in two centers that had deceased donors and used rituximab and plasmapheresis prior to transplantation.Conclusion: It can be concluded that with regard to the possibility of relapse after transplantation and variable therapeutic management modalities before and after transplantation, it is reasonable that genetic analysis of mutations, identification of idiopathic and high-risk cases, and use of appropriate therapeutic protocols should be considered to decrease the relapse rate.Keywords: FSGS; Renal transplantation; Nephrotic syndrome; Child

Prevalence of Failure to Thrive in Iranian Children with Chronic Kidney Disease

Introduction: Malnutrition and inflammation are considered risk factors of morbidity, hospitalization, and mortality in chronic kidney disease (CKD) children. The aim of this study was to determine the prevalence and severity of failure to thrive (FTT) in children with moderate to severe CKD.Materials and Methods: This cross-sectional study was conducted in 84 children with CKD (30 female, 54 males) aged 2-16 years old from June 2014 to June 2015.The inclusion criteria were eGFR less than 90 ml/min/1.73m2, being healthy in the month before the visit, and lack other chronic diseases except CKD. Anthropometric data including the body mass index, height, weight, and mid upper arm circumference were collected. Protein wasting energy was scored and the severity of failure to thrive was estimated using Gomez and Jelliffe classifications. P-values less than 0.05 were considered significantResults: Glomerulopathy and hereditary tubulopathy were the main causes of underlying disease. About 79% of CKD children had FTT and the rate increased with a decline in the renal function (p-value< 0.05). Using modified PWE, 65.5% were identified to score ≥2, which was more frequent in eGFR less than 30 (P>0.05). A quarter of the patients with FTT were classified as no PWE and vice versa.Conclusion: The majority of the children with moderate to severe chronic kidney disease had failure to thrive and protein wasting energy. There was no correlation between inflammatory markers and the severity of CKD or the presence of failure to thrive.Keywords: Failure to Thrive; Renal Insufficiency; Chronic; Child