Surgical complications of hemolytic uremic syndrome: Single center experiences

Purpose: To determine the prevalence, outcome and prognostic factors in children with hemolytic uremic syndrome (HUS) who underwent laparotomy. Materials and Methods: The medical records of 104 patients with HUS who presented to our center between 1986 and 2003 were reviewed retrospectively. Data were analyzed using Student�s t test for comparing means, Fisher�s exact test for frequencies and Pearson�s correlation for finding the correlations. Results: 78 of cases presented with vomiting and diarrhea. Seven out of 104 needed surgical exploration. The indications of surgery were acute abdomen, severe abdominal distention and the sign of peritonitis. The findings at laparotomy were intussusceptions, perforation (colon, ileum), gangrene of entire colon, rectosigmoidal tearing, duodenal obstruction and toxic megacolon. Pathological findings were transmural infarction in two cases in which staged surgical management was performed (cecostomy, resection, later anastomosis). Four out of seven patients died because of pulmonary failure, coma and multiple organ failure ( P 0.05) compared to those who did not need laparotomy. The patients requiring surgery were young ( 3 years), had high leukocyte count (20000 mm3 ) and low albumin level ( 3g/dl) ( P 0.05). Conclusion: Surgical complications of HUS are rare but are assorted with high mortality due to respiratory failure and multiple organ failure. Early decision of laparotomy associated with intensive care, including mechanical ventilation, adequate dialysis and ultrafiltration, are recommended

The frequency of thromboembolic complications in pediatric nephrotic syndrome

Background: Nephrotic syndrome (NS) is a common renal disease in the pediatric population, which can be complicated with venous thromboembolic events. Objectives: In the present study, the researchers evaluated the risk factors of venous thrombotic events in children with nephrotic syndrome. Methods: In this descriptive cross sectional study, the researchers evaluated 43 cases of NS including 35 uncomplicated and eight complicated patients with venous thrombotic events, who were admitted to the nephrology ward of Ali-Asghar Children Hospital from 2011 to 2015. Two groups were matched for age onset of NS. Data were recorded on age, gender, body mass index (BMI), histopathologic varieties of NS, and serum albumin level. Results: The mean age of cases with venous thrombotic events was 7.31 ± 4.1 years. There were six females. Of eight cases with venous thrombotic events, five cases suffered of pulmonary thromboembolism (PTE) and five cases of deep vein thrombosis (DVT). The mean serum albumin level in the thrombotic group was 1.87 ± 0.4 g/dL. The histopathologic results showed two cases of membranous proliferative glomerulonephritis (MPGN), three cases of minimal change disease (MCD) and one case of focal segmental glomerulosclerosis (FSGS). The researchers found significant differences between mean serum albumin level and histopathologic results in the case and control groups. Conclusions: Venous thrombosis and pulmonary emboli are important complications in pediatric NS. The risk of VTE increases with lower serum albumin level. The risk of VTE increases with lower serum albumin level. The researchers suggest the use of anti-thrombotic agents as prophylaxis in nephrotic patients with serum albumin level of less than 2 g/dL. Copyright © 2019, Journal of Comprehensive Pediatrics

Urine macrophage migration inhibitory factor in pediatric systemic lupus erythematosus

We reported a series of ten patients with lupus nephritis (five patients in the relapse phase and five in the remission phase) and measured the macrophage migration inhibitory factor (MIF), an important pro-inflammatory cytokine with probable role in the pathogenesis of many inflammatory diseases, in their urine samples. MIF/creatinine (Cr) ratio directly correlated with disease activity and it does not have any significant difference between inactive disease and normal ones. We found that the urine MIF/Cr ratio not only differentiates active disease from inactive disease and normal ones but also correlates with the activity indices of renal pathology. © Clinical Rheumatology 2007

Slow graft function after pediatric renal transplantation from volunteer live donors

Slow graft function (SGF) may occur during the early post-transplant period. In this paper, we present our findings regarding SGF after pediatric renal transplantation and its predictive variables. From 1985 to 2004, a total of 300 pediatric renal transplants were performed at our institution. A total of 10 cases with SGF and 50 controls that were operated by the same surgeons were enrolled in this study. The mean age of the recipients and donors was 11.4 (3-15 yr) and 28.05 yr (20-50 yr), respectively. All kidneys were retrieved from living donors. We compared patients with SGF with controls regarding four independent variables: age difference between donors and recipients, serum hemoglobin difference between donors and recipients, mean blood pressure (systolic blood pressure + 2 diastolic blood pressure/3) difference between donors and recipients, and weight difference between donors and recipients. The mean age of recipients was 10.5 ± 4.1 in SGF group and 11.6 ± 2.5 in control group (p = 0.4). The differences between donors and recipients regarding weight and mean blood pressure in subjects with SGF were not higher than other patients (42 kg vs. 37.4 kg, p = 0.4; -3 mmHg vs. -4.1 mmHg, p = 0.8). The mean hemoglobin difference between donors and recipients was 6.3 ± 2.1 g/dL in SGF group and 6.7 ± 2.3 g/dL in control group (p = 0.6). The differences between donors and recipients regarding age, weight, mean blood pressure and serum hemoglobin level are not predictive variables for SGF. © 2007 Blackwell Munksgaard

The value of serum uric acid as a mortality prediction in critically ill children

Objective: The role of initial serum uric acid on admission in critically ill patients is controversial; we presumed that uric acid level can predict the mortality of the admitted patients to intensive care unit as a simple test. Methods: Totally, 220 consecutively admitted children (96 girls, 124 boys) with mean age 3.5 years, who were at least 24 hours in pediatric intensive care unit (PICU), were enrolled in a prospective cohort study during January 2006 to December 2007. The subsequent PICU admission in the same hospitalization, those who were discharged from the hospital and then re-admitted to the PICU during the observation period, and the patients with chronic renal failure were excluded. Serum uric acid level was measured during the first day of PICU admission. Death or transfer from PICU was considered as final outcome. The statistical analysis was done by using linear regression analysis, ROC curve, Student t-test, and Chisquare. P value less than 0.05 was considered significant. Findings: From 44 patients who had serum uric acid level more than 8 mg/dl, 17 cases died showing with a higher relative risk of 1.88, higher mortality (P8 mg/dl and needed vasopressor was 1.04, and in those under mechanical ventilation 1.33. In patients who scored pediatric risk of mortality of >38 it was 1.4, and in septic cases 4 (P<0.05). Stepwise linear regression analysis showed that mainly the need for mechanical ventilation (P=0.001) and vasopressor had statistically significant correlation with the poor outcome (P=0.001). Conclusion: Uric acid level during the first day of intensive critical care admission is not an independent risk of mortality in PICU. Need for mechanical ventilation or inotropic agents was associated with poor outcome and only higher uric acid level in sepsis played an additive risk factor role. © 2010 by Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences

Exome sequencing utility in defining the genetic landscape of hearing loss and novel-gene discovery in Iran

Hearing loss (HL) is one of the most common sensory defects affecting more than 466 million individuals worldwide. It is clinically and genetically heterogeneous with over 120 genes causing non-syndromic HL identified to date. Here, we performed exome sequencing (ES) on a cohort of Iranian families with no disease-causing variants in known deafness-associated genes after screening with a targeted gene panel. We identified likely causal variants in 20 out of 71 families screened. Fifteen families segregated variants in known deafness-associated genes. Eight families segregated variants in novel candidate genes for HL: DBH, TOP3A, COX18, USP31, TCF19, SCP2, TENM1, and CARMIL1. In the three of these families, intrafamilial locus heterogeneity was observed with variants in both known and novel candidate genes. In aggregate, we were able to identify the underlying genetic cause of HL in nearly 30 of our study cohort using ES. This study corroborates the observation that high-throughput DNA sequencing in populations with high rates of consanguineous marriages represents a more appropriate strategy to elucidate the genetic etiology of heterogeneous conditions such as HL. © 2021 John Wiley & Sons A/S. Published by John Wiley & Sons Lt

Fungal peritonitis in Iranian children on continuous ambulatory peritoneal dialysis: a national experience.

INTRODUCTION. Fungal peritonitis (FP), causing catheter obstruction, dialysis failure, and peritoneal dysfunction, is a rare but serious complication of peritoneal dialysis. In this study, the frequency and risk factors of FP are evaluated in children who underwent peritoneal dialysis. MATERIALS AND METHODS. A retrospective multicenter study was performed at the 5 pediatric peritoneal dialysis centers in Iran from 1971 to 2006, and FP episodes among 93 children were reviewed. Risk ratios were calculated for the clinical and demographic variables to determine the risk factors of FP. RESULTS. Ninety-three children aged 39 months on average were included in study. Sixteen out of 155 episodes of peritonitis were fungi infections, all by Candida albicans. The risk of FP was higher in those with relapsing bacterial peritonitis (P = .009). Also, all of the patients had received antibiotics within the 1 month prior to the development of FP. Catheters were removed in all patients after 1 to 7 days of developing FP. Six out of 12 patients had catheter obstruction and peritoneal loss after the treatment and 5 died due to infection. CONCLUSIONS. Fungal peritonitis, accompanied by high morbidity and mortality in children should be reduced by prevention of bacterial peritonitis. Early removal of catheter after recognition of FP should be considered

Fungal peritonitis in Iranian children on continuous ambulatory peritoneal dialysis: a national experience.

INTRODUCTION. Fungal peritonitis (FP), causing catheter obstruction, dialysis failure, and peritoneal dysfunction, is a rare but serious complication of peritoneal dialysis. In this study, the frequency and risk factors of FP are evaluated in children who underwent peritoneal dialysis. MATERIALS AND METHODS. A retrospective multicenter study was performed at the 5 pediatric peritoneal dialysis centers in Iran from 1971 to 2006, and FP episodes among 93 children were reviewed. Risk ratios were calculated for the clinical and demographic variables to determine the risk factors of FP. RESULTS. Ninety-three children aged 39 months on average were included in study. Sixteen out of 155 episodes of peritonitis were fungi infections, all by Candida albicans. The risk of FP was higher in those with relapsing bacterial peritonitis (P = .009). Also, all of the patients had received antibiotics within the 1 month prior to the development of FP. Catheters were removed in all patients after 1 to 7 days of developing FP. Six out of 12 patients had catheter obstruction and peritoneal loss after the treatment and 5 died due to infection. CONCLUSIONS. Fungal peritonitis, accompanied by high morbidity and mortality in children should be reduced by prevention of bacterial peritonitis. Early removal of catheter after recognition of FP should be considered