Background: Hemolytic uremic syndrome (HUS) is the most common cause of acute renal failure durin... more Background: Hemolytic uremic syndrome (HUS) is the most common cause of acute renal failure during infancy. Many symptoms and clinical features have been proposed as prognostic factors for HUS in the short and long term, while the results of different studies have often been controversial. The aim of this study was to evaluate short-term and long-term outcomes of HUS in Iranian children. Methods: Medical records of all 92 children suffering from HUS admitted to the pediatrics nephrology ward at Ali-Asghar Children Hospital in Tehran, Iran, from 1990 to 2004, were retrospectively reviewed. Results: Out of 92 children, mortality was observed in 18 patients (19.6%) during the acute phase of the disease. Significant correlation between mortality and seizures, coma and hypertension in the acute phase was found (p<0.05). No association was observed between type of treatment and mortality (p>0.05). In the long-term, the presence of hypertension in the acute phase of the disease (p=0.023; relative risk [RR] = 3.89; 95% confidence interval [95% CI], 1.01-13.76), hypertension at discharge time (p<0.001; RR=10; 95% CI, 2.44-40.91) and need for dialysis (p=0.021; RR=1.38; 95% CI, 1.13-1.70) were shown to be significant risk factors for future hypertension in HUS patients. Conclusion: Central nervous system involvement is associated with mortality in the acute phase of HUS, whereas the severity of disease in the admission phase is related to occurrence of hypertension in future.
Glomerular filtration rate is low in fetal and neonatal life. It increases after birth and reache... more Glomerular filtration rate is low in fetal and neonatal life. It increases after birth and reaches approximately 20 mL/min/1.73 m2 at 1 month of age in term and preterm neonates. Various methods have been used to measure glomerular filtration rate in neonates such as inulin clearance, creatinine clearance, and serum cystatin C. Serum creatinine concentrations are influenced by many factors. It is suggested to use other markers which are stable over time and are not affected by muscle mass or tubular reabsorption and secretion. Cystatin C incorporates these characteristics; however, there are some other limitations in the use of cystatin C as a marker of kidney function in neonates. Additionally, the numbers of studies focused on the use of cystatin C in neonates is limited. There is a need for further studies to determine cystatin C's normal range levels and investigate whether cystatin C can replace other tests such as serum creatinine as marker of kidney function in newborn ba...
NGAL is a member of the lipocalin protein family that has diverse function but similar structure.... more NGAL is a member of the lipocalin protein family that has diverse function but similar structure. The functions of NGAL are not clear, but it appears to be expressed in stress conditions and in tissues undergoing involution. Varied studies have shown increased levels of plasma or urinary NGAL in diverse renal damages. The aim of this study was the serial measurement of serum and urinary NGAL within the first week after renal transplantation in children to predict immediate and short-term graft function. A total of 27 patients were assessed. These patients were classified into those with rapid reduction in serum creatinine (more than 50% reduction in serum creatinine in the first day after transplantation) and patients with slow reduction in serum creatinine (&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;50% reduction in serum creatinine). We also assessed the absolute reduction in serum creatinine before and after transplantation. Serum and urinary NGAL on the first day post-transplantation were higher in recipients with slow reduction in serum creatinine (urinary NGAL at the first day: 197 ± 153 [s.e.m.] vs. 22.54 ± 8.5 [s.e.m.], p = 0.04; serum NGAL at the first day: 199 vs. 69.8, p = 0.003). The cutoff point of serum NGAL at the first day after transplantation for prediction of slow creatinine reduction was 174 ng/mL with a sensitivity of 100% and specificity of 95.5%. However, we did not find association between the absolute reduction in serum creatinine before and after transplantation with the amount of serum and urinary NGAL post-transplant. Additionally, we did not find any effect of high serum and urine NGAL concentration on the graft function at the first year post-transplant. Although it is supposed that high serum and urine NGAL may predict ischemia of graft in early phases; however, it appears that this mild ischemic injury to graft without DGF or SGF cannot affect the graft function in short-term period. Further studies are needed using larger transplant recipients in pediatric age group. It is also needed to determine the effects of mild ischemic injuries on the graft function in long-term period in future studies.
Recently, the role of B cells in the pathogenesis of nephrotic syndrome is explained by some rese... more Recently, the role of B cells in the pathogenesis of nephrotic syndrome is explained by some researchers. It has also been shown that the anti-CD20 antibody, rituximab, may be an option in the treatment of these patients. In this systematic review, we performed extensive search and identified studies on rituximab use in children with nephrotic syndrome. There are some case reports as well as larger series in this regard. The majority of these case reports and series have demonstrated the success of rituximab in the treatment of nephrotic syndrome, especially in pediatric patients with steroid-dependent and frequent-relapsing nephrotic syndrome. Nevertheless, the treatment strategies before and after rituximab infusion are not clear to date. On the other hand, it is believed that positive results on rituximab use in nephrotic syndrome are much more reported by researchers than the negative results and this is an important bias. Although most reports on rituximab use in pediatric patients have not recognized significant side effects, the long-term adverse events of rituximab are not known. Thus, controlled long-term studies are required to be done to assess the risk-benefit profile of rituximab in childhood nephrotic syndrome.
Saudi Journal of Kidney Diseases and Transplantation, May 1, 2011
Vesicoureteric reflux (VUR) is found in 1% of all children and in 30%-50% of those with urinary t... more Vesicoureteric reflux (VUR) is found in 1% of all children and in 30%-50% of those with urinary tract infection. Furthermore, VUR in childhood is the main reason for pyelonephritis, hypertension and chronic kidney disease. Recently, a variety of procedures with low radiation have been recommended for diagnosis of VUR. Therefore, in this study, voiding urosonography (VUS) or cystosonography was performed for evaluation of VUR and for comparing it with radio nucleotide cystography (RNC). We studied 25 children admitted with initial diagnosis of VUR in our center in the year 2007. Simultaneously, RNC and VUS were performed for all the patients. VUR was detected in eight patients with the VUS procedure and in nine children with RNC. Another patient was diagnosed only by RNC, and two other patients by only VUS. The two methods were concordant in detection and exclusion of urinary reflux in 87% (P: 0.000, r: 0.728). Furthermore, the diagnosis of various grades of reflux by these two schemes were comparable (P: 0.0000, r: 0.724). Sensitivity and specifity of VUS was determined as 87% and 88%, respectively, with a 94% positive predictive value and a 77% negative predictive value. We conclude that VUS is a highly accurate, safe and inexpensive tool for the screening, diagnosis and follow-up of VUR.
The outcome of pediatric renal transplantation was previously reported by a single-center study a... more The outcome of pediatric renal transplantation was previously reported by a single-center study at the year 2006. Therefore, we aimed to evaluate and report the characteristics and outcome of renal pediatric renal transplantation in a multi-center nationwide study. In this nationwide report, medical records of 907 children (≤18yr) with renal transplantation in eight major pediatric transplant centers of Iran were recorded. These 907 patients received a total of 922 transplants. All children who failed to follow-up were excluded. Rather than baseline characteristics, graft and patient outcomes were considered for survival analysis. For further analysis, they were divided into two groups: patients who had graft survival time more than 10yr (n=91) and the ones with graft survival time of equal or less than 10yr (n=831). Of 922 recipients, 515 (55.8%) were boys and 407 (44.2%) were girls with the mean age of 13.10 (s.d.=3.54) yr. DGF and AR were occurred in 10% and 39.5% of the transplanted children, respectively. Transplantation year, dialyzing status before transplantation, DGF, and AR were significant enough to predict graft survival in cox regression model (overall model: p&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;0.001). Nowadays, there is a successful live donor pediatric renal transplantation in Iran. Graft survival has improved in our recipients and now the graft survival rates are near to international standards.
Procalcitonin is a reliable and specific marker of bacterial infections such as urinary tract inf... more Procalcitonin is a reliable and specific marker of bacterial infections such as urinary tract infection. Some authors suggest measurement of serum procalcitonin as a predictor of vesicoureteral reflux (VUR). We investigated this association in children admitted because of acute pyelonephritis. Forty-eight children with the first febrile urinary tract infection were included. Twelve patients had low-grade VUR, 9 patients had high-grade VUR, and 27 patients did not have any VUR in their imaging assessment. There was a significant association between high-grade VUR and higher levels of procalcitonin (P = .04). The sensitivity of a procalcitonin level of 0.31 ng/mL or greater was 90% and the specificity was 32% for diagnosis of high-grade VUR. We concluded that serum procalcitonin concentration is a sensitive and promising predictor of high-grade VUR.
Congenital nephrotic syndrome (CNS), an uncommon form of kidney disease, presents during the firs... more Congenital nephrotic syndrome (CNS), an uncommon form of kidney disease, presents during the first year of life and is a diagnostic and therapeutic challenge for the pediatricians as well as pediatric nephrologists. Our study is the first study of Iranian children with CNS in two pediatric nephrology centers in Tehran, Iran. We reviewed medical charts of 30 infants diagnosed with CNS from 1990 to 2005. There were 15 boys and 15 girls with CNS (mean age, 1.7 months). The presentation of the disease was nephrotic syndrome in 96.6% of the patients. Eighty percent of the patients presented within 3 months of life and 16 in the neonatal period. The Finnish type of CNS was seen in 43.3% and diffuse mesangial sclerosis in 50%. Preterm labor and low birth weight was seen in 20%. A family history of nephrotic syndrome in infancy was noted for 8 children (26.7%). Numerous complications of nephrotic syndrome occurred in 73.3%. Seventy percent of the patients had 27 episodes of infections. Sepsis was seen in 43.3% of the children, of which 61.5% were caused by gram-negative bacteria and 38.6% were caused by Staphylococcus aureus. Thrombotic complications and hypertension developed in 6.6% and 23.3% of the patients, respectively. The mortality rate of patients was 86.6%. Diffuse mesangial sclerosis is an important cause of CNS. The outcome of our patients was poor and most of our patients died before reaching the age of 5 years old.
Some evidence suggest an increase in the prevalence of focal segmental glomerular sclerosis (FSGS... more Some evidence suggest an increase in the prevalence of focal segmental glomerular sclerosis (FSGS) in children. To date, there has been no study of the outcome in children with FSGS and its frequency over several decades in Iran. We aimed to report the changing trend of FSGS incidence and its outcome in a sample of Iranian children. Between 1982 and 2008, all 716 kidney biopsies performed in children referred to Ali Asghar Children Hospital were recorded and confirmed cases with FSGS lesions were further evaluated. Baseline and clinical characteristics of all FSGS patients were assessed and the therapies and outcomes were reviewed. The incidence rate of FSGS was 10.1% between 1982 and 1990, which was significantly increased to as high as 20.5% after the year 2000 (P = .001). Among 64 children with FSGS, 20 progressed to end-stage renal disease with a mean survival time of 11.45 years (standard error of mean, 1.34 years). Kidney survival rates were 90.4%, 69%, and 47% at 1, 5 and 10 years of follow-up. Our study demonstrates an increasing trend in FSGS incidence in Iranian children. However, kidney survival rates of our patients were similar to those reported by others in different countries.
Objective: Takayasu arteritis (TA) is an inflammatory arteritis involving large vessels, predomin... more Objective: Takayasu arteritis (TA) is an inflammatory arteritis involving large vessels, predominantly the aorta and its main branches. Angina pectoris or myocardial infarction may occur in 3-5% of patients due to coronary artery ostial narrowing from aortitis or coronary arteritis.Case presentation: We describe the case of an 11-year boy presented with hypertension and severe abdominal pain. After treatment he developed extensive myocardial infarction and died.Conclusion: Takayasu's disease should figure prominently amongst the causes of coronary artery disease in children and coronary arteriography should be undertaken more often during investigation of the arterial lesions of these patients.
Saudi journal of kidney diseases and transplantation : an official publication of the Saudi Center for Organ Transplantation, Saudi Arabia, 2011
Vesicoureteric reflux (VUR) is found in 1% of all children and in 30%-50% of those with urinary t... more Vesicoureteric reflux (VUR) is found in 1% of all children and in 30%-50% of those with urinary tract infection. Furthermore, VUR in childhood is the main reason for pyelonephritis, hypertension and chronic kidney disease. Recently, a variety of procedures with low radiation have been recommended for diagnosis of VUR. Therefore, in this study, voiding urosonography (VUS) or cystosonography was performed for evaluation of VUR and for comparing it with radio nucleotide cystography (RNC). We studied 25 children admitted with initial diagnosis of VUR in our center in the year 2007. Simultaneously, RNC and VUS were performed for all the patients. VUR was detected in eight patients with the VUS procedure and in nine children with RNC. Another patient was diagnosed only by RNC, and two other patients by only VUS. The two methods were concordant in detection and exclusion of urinary reflux in 87% (P: 0.000, r: 0.728). Furthermore, the diagnosis of various grades of reflux by these two schem...
Congenital nephrotic syndrome may be caused by mutations in NPHS1 and NPHS2, which encode nephrin... more Congenital nephrotic syndrome may be caused by mutations in NPHS1 and NPHS2, which encode nephrin and podocin, respectively. Since the identification of the NPHS2 gene, various investigators have demonstrated that its mutation is an important cause of steroid-resistant nephrotic syndrome. We aimed to evaluate frequency and spectrum of podocin mutations in the Iranian children with steroid-resistant nephritic syndrome. We examined 20 children with steroid-resistant nephritic syndrome referred to Ali Asghar Children's Hospital, in Tehran, Iran. Mutations in the 5th and 7th exons of NPHS2 were assessed. The mutational analysis of NPHS2 was performed by DNA sequencing. The mean age at the onset of proteinuria was 6.4 +/- 3.6 years. None of the children had mutations in the exons 5 or 7. Our study suggests that NPHS2 mutations in exons 5 and 7 are not seen in our children. Therefore, we cannot recommend NPHS2 (exons 5 and 7) mutation for screening in Iranian children with steroid-res...
Procalcitonin is a reliable and specific marker of bacterial infections such as urinary tract inf... more Procalcitonin is a reliable and specific marker of bacterial infections such as urinary tract infection. Some authors suggest measurement of serum procalcitonin as a predictor of vesicoureteral reflux (VUR). We investigated this association in children admitted because of acute pyelonephritis. Forty-eight children with the first febrile urinary tract infection were included. Twelve patients had low-grade VUR, 9 patients had high-grade VUR, and 27 patients did not have any VUR in their imaging assessment. There was a significant association between high-grade VUR and higher levels of procalcitonin (P = .04). The sensitivity of a procalcitonin level of 0.31 ng/mL or greater was 90% and the specificity was 32% for diagnosis of high-grade VUR. We concluded that serum procalcitonin concentration is a sensitive and promising predictor of high-grade VUR.
Background: Hemolytic uremic syndrome (HUS) is the most common cause of acute renal failure durin... more Background: Hemolytic uremic syndrome (HUS) is the most common cause of acute renal failure during infancy. Many symptoms and clinical features have been proposed as prognostic factors for HUS in the short and long term, while the results of different studies have often been controversial. The aim of this study was to evaluate short-term and long-term outcomes of HUS in Iranian children. Methods: Medical records of all 92 children suffering from HUS admitted to the pediatrics nephrology ward at Ali-Asghar Children Hospital in Tehran, Iran, from 1990 to 2004, were retrospectively reviewed. Results: Out of 92 children, mortality was observed in 18 patients (19.6%) during the acute phase of the disease. Significant correlation between mortality and seizures, coma and hypertension in the acute phase was found (p<0.05). No association was observed between type of treatment and mortality (p>0.05). In the long-term, the presence of hypertension in the acute phase of the disease (p=0.023; relative risk [RR] = 3.89; 95% confidence interval [95% CI], 1.01-13.76), hypertension at discharge time (p<0.001; RR=10; 95% CI, 2.44-40.91) and need for dialysis (p=0.021; RR=1.38; 95% CI, 1.13-1.70) were shown to be significant risk factors for future hypertension in HUS patients. Conclusion: Central nervous system involvement is associated with mortality in the acute phase of HUS, whereas the severity of disease in the admission phase is related to occurrence of hypertension in future.
Glomerular filtration rate is low in fetal and neonatal life. It increases after birth and reache... more Glomerular filtration rate is low in fetal and neonatal life. It increases after birth and reaches approximately 20 mL/min/1.73 m2 at 1 month of age in term and preterm neonates. Various methods have been used to measure glomerular filtration rate in neonates such as inulin clearance, creatinine clearance, and serum cystatin C. Serum creatinine concentrations are influenced by many factors. It is suggested to use other markers which are stable over time and are not affected by muscle mass or tubular reabsorption and secretion. Cystatin C incorporates these characteristics; however, there are some other limitations in the use of cystatin C as a marker of kidney function in neonates. Additionally, the numbers of studies focused on the use of cystatin C in neonates is limited. There is a need for further studies to determine cystatin C's normal range levels and investigate whether cystatin C can replace other tests such as serum creatinine as marker of kidney function in newborn ba...
NGAL is a member of the lipocalin protein family that has diverse function but similar structure.... more NGAL is a member of the lipocalin protein family that has diverse function but similar structure. The functions of NGAL are not clear, but it appears to be expressed in stress conditions and in tissues undergoing involution. Varied studies have shown increased levels of plasma or urinary NGAL in diverse renal damages. The aim of this study was the serial measurement of serum and urinary NGAL within the first week after renal transplantation in children to predict immediate and short-term graft function. A total of 27 patients were assessed. These patients were classified into those with rapid reduction in serum creatinine (more than 50% reduction in serum creatinine in the first day after transplantation) and patients with slow reduction in serum creatinine (&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;50% reduction in serum creatinine). We also assessed the absolute reduction in serum creatinine before and after transplantation. Serum and urinary NGAL on the first day post-transplantation were higher in recipients with slow reduction in serum creatinine (urinary NGAL at the first day: 197 ± 153 [s.e.m.] vs. 22.54 ± 8.5 [s.e.m.], p = 0.04; serum NGAL at the first day: 199 vs. 69.8, p = 0.003). The cutoff point of serum NGAL at the first day after transplantation for prediction of slow creatinine reduction was 174 ng/mL with a sensitivity of 100% and specificity of 95.5%. However, we did not find association between the absolute reduction in serum creatinine before and after transplantation with the amount of serum and urinary NGAL post-transplant. Additionally, we did not find any effect of high serum and urine NGAL concentration on the graft function at the first year post-transplant. Although it is supposed that high serum and urine NGAL may predict ischemia of graft in early phases; however, it appears that this mild ischemic injury to graft without DGF or SGF cannot affect the graft function in short-term period. Further studies are needed using larger transplant recipients in pediatric age group. It is also needed to determine the effects of mild ischemic injuries on the graft function in long-term period in future studies.
Recently, the role of B cells in the pathogenesis of nephrotic syndrome is explained by some rese... more Recently, the role of B cells in the pathogenesis of nephrotic syndrome is explained by some researchers. It has also been shown that the anti-CD20 antibody, rituximab, may be an option in the treatment of these patients. In this systematic review, we performed extensive search and identified studies on rituximab use in children with nephrotic syndrome. There are some case reports as well as larger series in this regard. The majority of these case reports and series have demonstrated the success of rituximab in the treatment of nephrotic syndrome, especially in pediatric patients with steroid-dependent and frequent-relapsing nephrotic syndrome. Nevertheless, the treatment strategies before and after rituximab infusion are not clear to date. On the other hand, it is believed that positive results on rituximab use in nephrotic syndrome are much more reported by researchers than the negative results and this is an important bias. Although most reports on rituximab use in pediatric patients have not recognized significant side effects, the long-term adverse events of rituximab are not known. Thus, controlled long-term studies are required to be done to assess the risk-benefit profile of rituximab in childhood nephrotic syndrome.
Saudi Journal of Kidney Diseases and Transplantation, May 1, 2011
Vesicoureteric reflux (VUR) is found in 1% of all children and in 30%-50% of those with urinary t... more Vesicoureteric reflux (VUR) is found in 1% of all children and in 30%-50% of those with urinary tract infection. Furthermore, VUR in childhood is the main reason for pyelonephritis, hypertension and chronic kidney disease. Recently, a variety of procedures with low radiation have been recommended for diagnosis of VUR. Therefore, in this study, voiding urosonography (VUS) or cystosonography was performed for evaluation of VUR and for comparing it with radio nucleotide cystography (RNC). We studied 25 children admitted with initial diagnosis of VUR in our center in the year 2007. Simultaneously, RNC and VUS were performed for all the patients. VUR was detected in eight patients with the VUS procedure and in nine children with RNC. Another patient was diagnosed only by RNC, and two other patients by only VUS. The two methods were concordant in detection and exclusion of urinary reflux in 87% (P: 0.000, r: 0.728). Furthermore, the diagnosis of various grades of reflux by these two schemes were comparable (P: 0.0000, r: 0.724). Sensitivity and specifity of VUS was determined as 87% and 88%, respectively, with a 94% positive predictive value and a 77% negative predictive value. We conclude that VUS is a highly accurate, safe and inexpensive tool for the screening, diagnosis and follow-up of VUR.
The outcome of pediatric renal transplantation was previously reported by a single-center study a... more The outcome of pediatric renal transplantation was previously reported by a single-center study at the year 2006. Therefore, we aimed to evaluate and report the characteristics and outcome of renal pediatric renal transplantation in a multi-center nationwide study. In this nationwide report, medical records of 907 children (≤18yr) with renal transplantation in eight major pediatric transplant centers of Iran were recorded. These 907 patients received a total of 922 transplants. All children who failed to follow-up were excluded. Rather than baseline characteristics, graft and patient outcomes were considered for survival analysis. For further analysis, they were divided into two groups: patients who had graft survival time more than 10yr (n=91) and the ones with graft survival time of equal or less than 10yr (n=831). Of 922 recipients, 515 (55.8%) were boys and 407 (44.2%) were girls with the mean age of 13.10 (s.d.=3.54) yr. DGF and AR were occurred in 10% and 39.5% of the transplanted children, respectively. Transplantation year, dialyzing status before transplantation, DGF, and AR were significant enough to predict graft survival in cox regression model (overall model: p&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;0.001). Nowadays, there is a successful live donor pediatric renal transplantation in Iran. Graft survival has improved in our recipients and now the graft survival rates are near to international standards.
Procalcitonin is a reliable and specific marker of bacterial infections such as urinary tract inf... more Procalcitonin is a reliable and specific marker of bacterial infections such as urinary tract infection. Some authors suggest measurement of serum procalcitonin as a predictor of vesicoureteral reflux (VUR). We investigated this association in children admitted because of acute pyelonephritis. Forty-eight children with the first febrile urinary tract infection were included. Twelve patients had low-grade VUR, 9 patients had high-grade VUR, and 27 patients did not have any VUR in their imaging assessment. There was a significant association between high-grade VUR and higher levels of procalcitonin (P = .04). The sensitivity of a procalcitonin level of 0.31 ng/mL or greater was 90% and the specificity was 32% for diagnosis of high-grade VUR. We concluded that serum procalcitonin concentration is a sensitive and promising predictor of high-grade VUR.
Congenital nephrotic syndrome (CNS), an uncommon form of kidney disease, presents during the firs... more Congenital nephrotic syndrome (CNS), an uncommon form of kidney disease, presents during the first year of life and is a diagnostic and therapeutic challenge for the pediatricians as well as pediatric nephrologists. Our study is the first study of Iranian children with CNS in two pediatric nephrology centers in Tehran, Iran. We reviewed medical charts of 30 infants diagnosed with CNS from 1990 to 2005. There were 15 boys and 15 girls with CNS (mean age, 1.7 months). The presentation of the disease was nephrotic syndrome in 96.6% of the patients. Eighty percent of the patients presented within 3 months of life and 16 in the neonatal period. The Finnish type of CNS was seen in 43.3% and diffuse mesangial sclerosis in 50%. Preterm labor and low birth weight was seen in 20%. A family history of nephrotic syndrome in infancy was noted for 8 children (26.7%). Numerous complications of nephrotic syndrome occurred in 73.3%. Seventy percent of the patients had 27 episodes of infections. Sepsis was seen in 43.3% of the children, of which 61.5% were caused by gram-negative bacteria and 38.6% were caused by Staphylococcus aureus. Thrombotic complications and hypertension developed in 6.6% and 23.3% of the patients, respectively. The mortality rate of patients was 86.6%. Diffuse mesangial sclerosis is an important cause of CNS. The outcome of our patients was poor and most of our patients died before reaching the age of 5 years old.
Some evidence suggest an increase in the prevalence of focal segmental glomerular sclerosis (FSGS... more Some evidence suggest an increase in the prevalence of focal segmental glomerular sclerosis (FSGS) in children. To date, there has been no study of the outcome in children with FSGS and its frequency over several decades in Iran. We aimed to report the changing trend of FSGS incidence and its outcome in a sample of Iranian children. Between 1982 and 2008, all 716 kidney biopsies performed in children referred to Ali Asghar Children Hospital were recorded and confirmed cases with FSGS lesions were further evaluated. Baseline and clinical characteristics of all FSGS patients were assessed and the therapies and outcomes were reviewed. The incidence rate of FSGS was 10.1% between 1982 and 1990, which was significantly increased to as high as 20.5% after the year 2000 (P = .001). Among 64 children with FSGS, 20 progressed to end-stage renal disease with a mean survival time of 11.45 years (standard error of mean, 1.34 years). Kidney survival rates were 90.4%, 69%, and 47% at 1, 5 and 10 years of follow-up. Our study demonstrates an increasing trend in FSGS incidence in Iranian children. However, kidney survival rates of our patients were similar to those reported by others in different countries.
Objective: Takayasu arteritis (TA) is an inflammatory arteritis involving large vessels, predomin... more Objective: Takayasu arteritis (TA) is an inflammatory arteritis involving large vessels, predominantly the aorta and its main branches. Angina pectoris or myocardial infarction may occur in 3-5% of patients due to coronary artery ostial narrowing from aortitis or coronary arteritis.Case presentation: We describe the case of an 11-year boy presented with hypertension and severe abdominal pain. After treatment he developed extensive myocardial infarction and died.Conclusion: Takayasu's disease should figure prominently amongst the causes of coronary artery disease in children and coronary arteriography should be undertaken more often during investigation of the arterial lesions of these patients.
Saudi journal of kidney diseases and transplantation : an official publication of the Saudi Center for Organ Transplantation, Saudi Arabia, 2011
Vesicoureteric reflux (VUR) is found in 1% of all children and in 30%-50% of those with urinary t... more Vesicoureteric reflux (VUR) is found in 1% of all children and in 30%-50% of those with urinary tract infection. Furthermore, VUR in childhood is the main reason for pyelonephritis, hypertension and chronic kidney disease. Recently, a variety of procedures with low radiation have been recommended for diagnosis of VUR. Therefore, in this study, voiding urosonography (VUS) or cystosonography was performed for evaluation of VUR and for comparing it with radio nucleotide cystography (RNC). We studied 25 children admitted with initial diagnosis of VUR in our center in the year 2007. Simultaneously, RNC and VUS were performed for all the patients. VUR was detected in eight patients with the VUS procedure and in nine children with RNC. Another patient was diagnosed only by RNC, and two other patients by only VUS. The two methods were concordant in detection and exclusion of urinary reflux in 87% (P: 0.000, r: 0.728). Furthermore, the diagnosis of various grades of reflux by these two schem...
Congenital nephrotic syndrome may be caused by mutations in NPHS1 and NPHS2, which encode nephrin... more Congenital nephrotic syndrome may be caused by mutations in NPHS1 and NPHS2, which encode nephrin and podocin, respectively. Since the identification of the NPHS2 gene, various investigators have demonstrated that its mutation is an important cause of steroid-resistant nephrotic syndrome. We aimed to evaluate frequency and spectrum of podocin mutations in the Iranian children with steroid-resistant nephritic syndrome. We examined 20 children with steroid-resistant nephritic syndrome referred to Ali Asghar Children's Hospital, in Tehran, Iran. Mutations in the 5th and 7th exons of NPHS2 were assessed. The mutational analysis of NPHS2 was performed by DNA sequencing. The mean age at the onset of proteinuria was 6.4 +/- 3.6 years. None of the children had mutations in the exons 5 or 7. Our study suggests that NPHS2 mutations in exons 5 and 7 are not seen in our children. Therefore, we cannot recommend NPHS2 (exons 5 and 7) mutation for screening in Iranian children with steroid-res...
Procalcitonin is a reliable and specific marker of bacterial infections such as urinary tract inf... more Procalcitonin is a reliable and specific marker of bacterial infections such as urinary tract infection. Some authors suggest measurement of serum procalcitonin as a predictor of vesicoureteral reflux (VUR). We investigated this association in children admitted because of acute pyelonephritis. Forty-eight children with the first febrile urinary tract infection were included. Twelve patients had low-grade VUR, 9 patients had high-grade VUR, and 27 patients did not have any VUR in their imaging assessment. There was a significant association between high-grade VUR and higher levels of procalcitonin (P = .04). The sensitivity of a procalcitonin level of 0.31 ng/mL or greater was 90% and the specificity was 32% for diagnosis of high-grade VUR. We concluded that serum procalcitonin concentration is a sensitive and promising predictor of high-grade VUR.
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