Recent clinical reports have demonstrated that the progression and prognosis of renal diseases ar... more Recent clinical reports have demonstrated that the progression and prognosis of renal diseases are possibly influenced by apolipoprotein E (apoE) genotypes and alleles. In this study we investigated whether apoE genotypes and alleles can be a prognostic criterion for the steroid responsiveness in childhood nephrotic syndrome. One hundred and seven pediatric patients with primary idiopathic nephrotic syndrome and 83 healthy volunteers were enrolled in the study. Eighty-seven of the patients had steroid-sensitive nephrotic syndrome (SSNS) and 20 had steroid-resistant nephrotic syndrome (SRNS). The varepsilon2 allele frequency and varepsilon2/3 genotype frequency of the SNRS group were statistically higher when compared with SSNS and control groups ( P<0.05). The higher frequency of the varepsilon2 allele in steroid resistant nephrotic patients suggests that the varepsilon2 allele gives a possible genetic predisposition to steroid resistance in our population, but further studies are needed to clarify this subject.
Few data on the renal effects of thalassemia syndrome are available in the literature. Recent cli... more Few data on the renal effects of thalassemia syndrome are available in the literature. Recent clinical studies identified proximal tubular damage and glomerular filtration abnormalities in thalassemia. Iron-chelating agents might be nephrotoxic as well, but proven glomerular injury, either due to anemia or chelating therapy, has not previously been demonstrated in thalassemia patients. Here, we report the first thalassemia patient presenting with nephrotic syndrome to be diagnosed with membranous nephropathy in the literature.
The efficacy and side effects of the oral administration of cyclosporin A (Cyc-A) were investigat... more The efficacy and side effects of the oral administration of cyclosporin A (Cyc-A) were investigated in 13 children with the nephrotic syndrome. Two of these children were steroid-dependent, 9 were resistant to steroids, and 2 displayed frequent relapses. The diagnosis of 6 patients was mesangioproliferative glomerulonephritis (MezPGN), of another 6 mesangiocapillary glomerulonephritis, and of 1 minimal change disease. Cyc-A was administered for 6 months at a dosage of 7 mg/kg/day with continual monitoring of the serum levels. At the end of the treatment period, 7 of the patients (53.8%) had a total remission, 5 (38.4%) a partial remission, and 1 (7.8%) did not respond to treatment. While total remission was achieved in 4 patients who had responded well to steroids, this was true for only 3 (33.3%) of the 9 patients who had not benefited from steroids. The effects and side effects of Cyc-A on histopathological morphology were also investigated by means of posttreatment biopsies. While histopathological improvement was seen in 3 patients with MezPGN, there was evidence of deterioration in 1 patient with MLH.
Since 1990, the role of angiotensin converting enzyme (ACE) gene polymorphism in various renal an... more Since 1990, the role of angiotensin converting enzyme (ACE) gene polymorphism in various renal and cardiac diseases is still debated. This study comprised 71 pediatric patients with nephrotic syndrome, 47 males (66%) and 24 females (34%) with a mean age of 57.4 +/- 37.6 months, and a control group of 83 healthy males (59%) and 57 healthy females (41%) with a mean age of 505 +/- 160.5 months. The distribution of the ACE genotype in the control group was II, 11%; ID, 53%; and DD, 36%, and the nephrotic syndrome was II, 4%; ID, 78%; and DD, 18%. Angiotensin-converting enzyme genotypes were significantly different between patients and control groups (p<0.05). The study groups consisted of 52 (73%) with steroid-sensitive nephrotic syndrome (SNSS) and 19 (27%) with steroid-resistant nephrotic syndrome (SRNS). The distribution of the ACE genotype was II, 6%; ID, 75%; and DD, 19% in the SSNS population and ID, 84% and DD, 16% in the SRNS population. No statistically significant difference was found between steroid sensitivity and ACE genotypes (p=0.5). The results show that ACE I/D polymorphism does not contribute to the steroid resistance, even though this study indicates that the presence of the I/D genotype has a much higher risk--approximately 2.8 times--of having nephrotic syndrome. Further studies with a larger number of patients are needed.
Congenital solitary functioning kidney (CSFK) is a relatively common renal malformation and in ch... more Congenital solitary functioning kidney (CSFK) is a relatively common renal malformation and in children is frequently complicated by anomalies of the ipsilateral genital organs and occasionally by anomalies of other organs. The aim of this study was to determine the incidence of associated urological, cardiac, gastrointestinal, hematological, neurological, skeletal, and other congenital malformations in children with CSFK. We retrospectively reviewed
Annexin V has a molecular weight of 32–35 kDa and has been reported to possess anticoagulant acti... more Annexin V has a molecular weight of 32–35 kDa and has been reported to possess anticoagulant activity, inhibition of phospholipase A2, regulation of membrane transport, proliferation and signal transduction. It is reported that urinary annexin V concentration may be an indicator of apoptosis and acute renal injury related to the urinary protein level. The aim of this study was to define
The role of endothelial nitric oxide synthase gene intron 4 a/b (eNOS4a/b) variable number of tan... more The role of endothelial nitric oxide synthase gene intron 4 a/b (eNOS4a/b) variable number of tandem repeats (VNTR) polymorphism in various renal diseases was investigated. We investigated whether the eNOS4a/b VNTR polymorphism is associated with susceptibility to acute poststreptococcal glomerulonephritis (APSGN) and its clinical features. Endothelial NOS4a/b VNTR polymorphism is determined by the polymerase chain reaction in 60 children with
Clinical journal of the American Society of Nephrology : CJASN, Jan 29, 2015
Steroid-resistant nephrotic syndrome is a rare kidney disease involving either immune-mediated or... more Steroid-resistant nephrotic syndrome is a rare kidney disease involving either immune-mediated or genetic alterations of podocyte structure and function. The rare nature, heterogeneity, and slow evolution of the disorder are major obstacles to systematic genotype-phenotype, intervention, and outcome studies, hampering the development of evidence-based diagnostic and therapeutic concepts. To overcome these limitations, the PodoNet Consortium has created an international registry for congenital nephrotic syndrome and childhood-onset steroid-resistant nephrotic syndrome. Since August of 2009, clinical, biochemical, genetic, and histopathologic information was collected both retrospectively and prospectively from 1655 patients with childhood-onset steroid-resistant nephrotic syndrome, congenital nephrotic syndrome, or persistent subnephrotic proteinuria of likely genetic origin at 67 centers in 21 countries through an online portal. Steroid-resistant nephrotic syndrome manifested in the...
In patients with β-thalassemia major, the most important cause of mortality and morbidity is orga... more In patients with β-thalassemia major, the most important cause of mortality and morbidity is organ failure due to deposits of iron.. In this study, the nature of the kidney injury and possible pathogenetic factors were investigated. Seventy children with β-thalassemia major and 14 age and sex-matched healthy children were involved in the study. Blood and timed urine samples were obtained
It has been suggested that urinary glycosaminoglycans (GAGs) form a natural defense mechanism aga... more It has been suggested that urinary glycosaminoglycans (GAGs) form a natural defense mechanism against urinary tract infections (UTIs). This study investigated whether urinary GAGs play a role in pediatric UTIs, and whether urinary GAG level can be used to differentiate upper UTI from lower UTI. Forty-one children with UTIs (33 girls and eight boys; mean age 5.4±3.7 years) and 46 age-
We evaluated the clinical usefulness of 99Tcm-N,N-ethylenedicysteine (99Tcm-EC), a new renal tubu... more We evaluated the clinical usefulness of 99Tcm-N,N-ethylenedicysteine (99Tcm-EC), a new renal tubular agent, in normal children and in children with various renal disorders and compared it with 99Tcm-mercaptoacetyltriglycine (99Tcm-MAG3). In a first group of children (Group 1), which included 15 patients and 4 normal children, both 99Tcm-EC and 99Tcm-MAG3 renal scintigraphy were performed within a period of 1-5 days. In a second group (Group 2), consisting of 12 patients, only 99Tcm-EC scintigraphy was performed. In the normal children, renograms, differential renal function (DRF) ratios and semi-quantitative parameters of the two agents were similar. For 99Tcm-EC and 99Tcm-MAG3, the mean (+/- S.D.) time to peak activity (Tmax) values were 3.2 +/- 0.5 and 3.1 +/- 0.4 min respectively, and the mean time from peak activity to 50% activity (T1/2) values were 6.3 +/- 0.5 and 6.4 +/- 0.4 min respectively. The mean parenchymal transit time index (PTTI) and mean whole-kidney transit time index (WKTTI) for 99Tcm-EC were 1.7 +/- 0.3 and 2.8 +/- 0.4 respectively, and for 99Tcm-MAG3 they were 1.8 +/- 0.2 and 3.0 +/- 0.3 min respectively. There were also no significant differences between these parameters for the two agents in children with various renal disorders, and the correlation coefficients (r) for DRF, Tmax, T1/2, PTTI and WKTTI were 0.99, 0.98, 0.94, 0.77 and 0.63 respectively. We conclude that 99Tcm-EC has excellent imaging characteristics in children, and even has some advantages over 99Tcm-MAG3. We suggest this agent can be used routinely with children.
The osmolality and osmolal gap (OG) of physiological fluids tend to be dominated by small molecul... more The osmolality and osmolal gap (OG) of physiological fluids tend to be dominated by small molecules which are present in high concentrations [1]. The OG is an indication of unmeasured solute in the blood; it is defined as the difference between the measured serum osmolality and the calculated serum osmolality [2, 3]. An OG value greater than 10 mosm/kg is considered a critical value, and it is clinically important in two conditions: (a) assessment of serum water content, especially in hyponatremia accompanied by hyperlipemia or hyperproteinemia; (b) evaluation of exogenous low-molecular-weight substances, such as ethanol, methanol and acetone.
Recent clinical reports have demonstrated that the progression and prognosis of renal diseases ar... more Recent clinical reports have demonstrated that the progression and prognosis of renal diseases are possibly influenced by apolipoprotein E (apoE) genotypes and alleles. In this study we investigated whether apoE genotypes and alleles can be a prognostic criterion for the steroid responsiveness in childhood nephrotic syndrome. One hundred and seven pediatric patients with primary idiopathic nephrotic syndrome and 83 healthy volunteers were enrolled in the study. Eighty-seven of the patients had steroid-sensitive nephrotic syndrome (SSNS) and 20 had steroid-resistant nephrotic syndrome (SRNS). The varepsilon2 allele frequency and varepsilon2/3 genotype frequency of the SNRS group were statistically higher when compared with SSNS and control groups ( P<0.05). The higher frequency of the varepsilon2 allele in steroid resistant nephrotic patients suggests that the varepsilon2 allele gives a possible genetic predisposition to steroid resistance in our population, but further studies are needed to clarify this subject.
Few data on the renal effects of thalassemia syndrome are available in the literature. Recent cli... more Few data on the renal effects of thalassemia syndrome are available in the literature. Recent clinical studies identified proximal tubular damage and glomerular filtration abnormalities in thalassemia. Iron-chelating agents might be nephrotoxic as well, but proven glomerular injury, either due to anemia or chelating therapy, has not previously been demonstrated in thalassemia patients. Here, we report the first thalassemia patient presenting with nephrotic syndrome to be diagnosed with membranous nephropathy in the literature.
The efficacy and side effects of the oral administration of cyclosporin A (Cyc-A) were investigat... more The efficacy and side effects of the oral administration of cyclosporin A (Cyc-A) were investigated in 13 children with the nephrotic syndrome. Two of these children were steroid-dependent, 9 were resistant to steroids, and 2 displayed frequent relapses. The diagnosis of 6 patients was mesangioproliferative glomerulonephritis (MezPGN), of another 6 mesangiocapillary glomerulonephritis, and of 1 minimal change disease. Cyc-A was administered for 6 months at a dosage of 7 mg/kg/day with continual monitoring of the serum levels. At the end of the treatment period, 7 of the patients (53.8%) had a total remission, 5 (38.4%) a partial remission, and 1 (7.8%) did not respond to treatment. While total remission was achieved in 4 patients who had responded well to steroids, this was true for only 3 (33.3%) of the 9 patients who had not benefited from steroids. The effects and side effects of Cyc-A on histopathological morphology were also investigated by means of posttreatment biopsies. While histopathological improvement was seen in 3 patients with MezPGN, there was evidence of deterioration in 1 patient with MLH.
Since 1990, the role of angiotensin converting enzyme (ACE) gene polymorphism in various renal an... more Since 1990, the role of angiotensin converting enzyme (ACE) gene polymorphism in various renal and cardiac diseases is still debated. This study comprised 71 pediatric patients with nephrotic syndrome, 47 males (66%) and 24 females (34%) with a mean age of 57.4 +/- 37.6 months, and a control group of 83 healthy males (59%) and 57 healthy females (41%) with a mean age of 505 +/- 160.5 months. The distribution of the ACE genotype in the control group was II, 11%; ID, 53%; and DD, 36%, and the nephrotic syndrome was II, 4%; ID, 78%; and DD, 18%. Angiotensin-converting enzyme genotypes were significantly different between patients and control groups (p<0.05). The study groups consisted of 52 (73%) with steroid-sensitive nephrotic syndrome (SNSS) and 19 (27%) with steroid-resistant nephrotic syndrome (SRNS). The distribution of the ACE genotype was II, 6%; ID, 75%; and DD, 19% in the SSNS population and ID, 84% and DD, 16% in the SRNS population. No statistically significant difference was found between steroid sensitivity and ACE genotypes (p=0.5). The results show that ACE I/D polymorphism does not contribute to the steroid resistance, even though this study indicates that the presence of the I/D genotype has a much higher risk--approximately 2.8 times--of having nephrotic syndrome. Further studies with a larger number of patients are needed.
Congenital solitary functioning kidney (CSFK) is a relatively common renal malformation and in ch... more Congenital solitary functioning kidney (CSFK) is a relatively common renal malformation and in children is frequently complicated by anomalies of the ipsilateral genital organs and occasionally by anomalies of other organs. The aim of this study was to determine the incidence of associated urological, cardiac, gastrointestinal, hematological, neurological, skeletal, and other congenital malformations in children with CSFK. We retrospectively reviewed
Annexin V has a molecular weight of 32–35 kDa and has been reported to possess anticoagulant acti... more Annexin V has a molecular weight of 32–35 kDa and has been reported to possess anticoagulant activity, inhibition of phospholipase A2, regulation of membrane transport, proliferation and signal transduction. It is reported that urinary annexin V concentration may be an indicator of apoptosis and acute renal injury related to the urinary protein level. The aim of this study was to define
The role of endothelial nitric oxide synthase gene intron 4 a/b (eNOS4a/b) variable number of tan... more The role of endothelial nitric oxide synthase gene intron 4 a/b (eNOS4a/b) variable number of tandem repeats (VNTR) polymorphism in various renal diseases was investigated. We investigated whether the eNOS4a/b VNTR polymorphism is associated with susceptibility to acute poststreptococcal glomerulonephritis (APSGN) and its clinical features. Endothelial NOS4a/b VNTR polymorphism is determined by the polymerase chain reaction in 60 children with
Clinical journal of the American Society of Nephrology : CJASN, Jan 29, 2015
Steroid-resistant nephrotic syndrome is a rare kidney disease involving either immune-mediated or... more Steroid-resistant nephrotic syndrome is a rare kidney disease involving either immune-mediated or genetic alterations of podocyte structure and function. The rare nature, heterogeneity, and slow evolution of the disorder are major obstacles to systematic genotype-phenotype, intervention, and outcome studies, hampering the development of evidence-based diagnostic and therapeutic concepts. To overcome these limitations, the PodoNet Consortium has created an international registry for congenital nephrotic syndrome and childhood-onset steroid-resistant nephrotic syndrome. Since August of 2009, clinical, biochemical, genetic, and histopathologic information was collected both retrospectively and prospectively from 1655 patients with childhood-onset steroid-resistant nephrotic syndrome, congenital nephrotic syndrome, or persistent subnephrotic proteinuria of likely genetic origin at 67 centers in 21 countries through an online portal. Steroid-resistant nephrotic syndrome manifested in the...
In patients with β-thalassemia major, the most important cause of mortality and morbidity is orga... more In patients with β-thalassemia major, the most important cause of mortality and morbidity is organ failure due to deposits of iron.. In this study, the nature of the kidney injury and possible pathogenetic factors were investigated. Seventy children with β-thalassemia major and 14 age and sex-matched healthy children were involved in the study. Blood and timed urine samples were obtained
It has been suggested that urinary glycosaminoglycans (GAGs) form a natural defense mechanism aga... more It has been suggested that urinary glycosaminoglycans (GAGs) form a natural defense mechanism against urinary tract infections (UTIs). This study investigated whether urinary GAGs play a role in pediatric UTIs, and whether urinary GAG level can be used to differentiate upper UTI from lower UTI. Forty-one children with UTIs (33 girls and eight boys; mean age 5.4±3.7 years) and 46 age-
We evaluated the clinical usefulness of 99Tcm-N,N-ethylenedicysteine (99Tcm-EC), a new renal tubu... more We evaluated the clinical usefulness of 99Tcm-N,N-ethylenedicysteine (99Tcm-EC), a new renal tubular agent, in normal children and in children with various renal disorders and compared it with 99Tcm-mercaptoacetyltriglycine (99Tcm-MAG3). In a first group of children (Group 1), which included 15 patients and 4 normal children, both 99Tcm-EC and 99Tcm-MAG3 renal scintigraphy were performed within a period of 1-5 days. In a second group (Group 2), consisting of 12 patients, only 99Tcm-EC scintigraphy was performed. In the normal children, renograms, differential renal function (DRF) ratios and semi-quantitative parameters of the two agents were similar. For 99Tcm-EC and 99Tcm-MAG3, the mean (+/- S.D.) time to peak activity (Tmax) values were 3.2 +/- 0.5 and 3.1 +/- 0.4 min respectively, and the mean time from peak activity to 50% activity (T1/2) values were 6.3 +/- 0.5 and 6.4 +/- 0.4 min respectively. The mean parenchymal transit time index (PTTI) and mean whole-kidney transit time index (WKTTI) for 99Tcm-EC were 1.7 +/- 0.3 and 2.8 +/- 0.4 respectively, and for 99Tcm-MAG3 they were 1.8 +/- 0.2 and 3.0 +/- 0.3 min respectively. There were also no significant differences between these parameters for the two agents in children with various renal disorders, and the correlation coefficients (r) for DRF, Tmax, T1/2, PTTI and WKTTI were 0.99, 0.98, 0.94, 0.77 and 0.63 respectively. We conclude that 99Tcm-EC has excellent imaging characteristics in children, and even has some advantages over 99Tcm-MAG3. We suggest this agent can be used routinely with children.
The osmolality and osmolal gap (OG) of physiological fluids tend to be dominated by small molecul... more The osmolality and osmolal gap (OG) of physiological fluids tend to be dominated by small molecules which are present in high concentrations [1]. The OG is an indication of unmeasured solute in the blood; it is defined as the difference between the measured serum osmolality and the calculated serum osmolality [2, 3]. An OG value greater than 10 mosm/kg is considered a critical value, and it is clinically important in two conditions: (a) assessment of serum water content, especially in hyponatremia accompanied by hyperlipemia or hyperproteinemia; (b) evaluation of exogenous low-molecular-weight substances, such as ethanol, methanol and acetone.
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Papers by Ali Anarat