Background and Aims Response to calcineurin inhibitors (CNI) is associated with a significant imp... more Background and Aims Response to calcineurin inhibitors (CNI) is associated with a significant improvement in long-term kidney survival of children with non-genetic steroid resistant nephrotic syndrome (SRNS). On the contrary, these agents are considered non-efficacious in monogenic SRNS and are contraindicated according to the latest International Pediatric Nephrology Association (IPNA) Clinical Practice Recommendations for SRNS. However, there is evidence suggesting that remission with CNI therapy in this subgroup of children with SRNS is possible, but no studies to date have assessed this question in a systematic way. We aimed to study the incidence of response to CNI in children with monogenic SRNS, factors predictive of remission and the effect of treatment on kidney survival. Method Retrospective cohort study of children 0–18 years with genetically confirmed SRNS treated with a CNI for at least 3 months. Demographic, clinical, genetic, biochemical, histopathologic and treatment...
Background C3 glomerulopathy(C3G) is a complement-mediated disease caused by abnormalities in the... more Background C3 glomerulopathy(C3G) is a complement-mediated disease caused by abnormalities in the alternative complement pathway. Although genetic studies are not required for diagnosis, they are valuable for treatment planning and prognosis prediction. The aim of this study is to investigate the clinical phenotypes, kidney survival, and response to MMF treatment in pediatric C3G patients with and without mutations in complement related genes.Methods Sixty pediatric C3G patients were included, divided into two groups based on complement related gene mutations. Demographic and clinical-pathological findings, treatment modalities, and outcome data were compared, and Kaplan-Meier analysis was performed for kidney survival.Results Out of the 60 patients, 17 had mutations, with the most common mutation in the CH gene (47%). The mean age at diagnosis was significantly higher in the group with mutation (12.9 ± 3.6 vs 11.2 ± 4.1 years p = 0.039). While the patients without mutation were mos...
Background Dent's disease type 1 (DD1) is a rare X-linked nephropathy caused by CLCN5 mutatio... more Background Dent's disease type 1 (DD1) is a rare X-linked nephropathy caused by CLCN5 mutations, characterized by proximal tubule dysfunction, including low molecular weight proteinuria (LMWP), hypercalciuria, nephrolithiasis–nephrocalcinosis, progressive chronic kidney disease (CKD) and kidney failure (KF). Current management is symptomatic and does not prevent disease progression. Here we describe the contemporary DD1 picture across Europe to highlight its unmet needs. Methods A physician-based anonymous international e-survey supported by several European nephrology networks/societies was conducted. Questions focused on DD1 clinical features, diagnostic procedure and mutation spectra. Results A total of 207 DD1 male patients were reported; clinical data were available for 163 with confirmed CLCN5 mutations. Proteinuria was the most common manifestation (49.1%). During follow-up, all patients showed LMWP, 66.4% nephrocalcinosis, 44.4% hypercalciuria and 26.4% nephrolithiasis. ...
Objective: This study aimed to evaluate patients with renal transplantation in terms of clinical ... more Objective: This study aimed to evaluate patients with renal transplantation in terms of clinical and laboratory. parameters. Material and Methods: This study was performed retrospectively with records of 48 patients who underwent renal transplantation before 18 years of age, between June 2008 and July 2019. Results: Congenital malformations of the urinary tract were the most common underlying causes of chronic kidney disease stage 5. Surgical complications occurred in 33.4% of the patients and BK viremia was the most common opportunistic viral infection during the follow-up. At the last clinic visit, 57.4% of our patients had CKD stage 1, hypertension and nephrotic range proteinuria were seen in eight and two patients, respectively. Conclusion: Although renal transplantation is the most ideal renal replacement therapy, patients may experience various complications during the follow-up. Therefore, they should be monitored regularly
Eryavuz et al.: The role of oxidative stress in determining prognosis in children with FMF and th... more Eryavuz et al.: The role of oxidative stress in determining prognosis in children with FMF and the relationship between markers of oxidative stress and gene mutation. Pediatric Rheumatology 2015 13(Suppl 1):P107.
A 4-year-old girl presented with complaints of swelling around the eyes and reduced urine output ... more A 4-year-old girl presented with complaints of swelling around the eyes and reduced urine output for 3 days. On admission, her body weight was 14 kg (10–25th percentile), and height was 88 cm (< 3rd percentile). Her fever was 36°C, heart rate was 108/min, respiratory rate was 24/ min, and blood pressure was 110/50 mmHg. In her physical exam, there were ascites in her abdomen and four positive pitting pretibial edemas. The rest of the physical examination was normal. Her past medical history was unremarkable. Her parents were first-degree cousins, and her parents’ cousins suffered from chronic kidney disease (CKD) of unknown etiology. In the laboratory study, hemoglobin was 12.9 g/dl, white blood cell count was 9800 mm, and platelet count was 281,000 mm. The other laboratory parameters were as follows: blood urea nitrogen 23 mg/dl, serum creatinine 0.7 mg/ dl, uric acid 5 mg/dl, sodium 135 mmol/L, potassium 3.7 mmol/L, chlorine 105 mmol/L, total protein 3.5 g/dl, albumin 1.52 g/dl, triglyceride 647 mg/dl, and cholesterol 424 mg/dl. The C3 was within the normal range (119 mg/dl). There was no evidence of hematuria in urine analysis; only proteinuria was detected. Microprotein to creatinine ratio was 3:4. The 24-h urine proteinuria was 45 mg/m/h, and GFR was 77 ml/min/1.73 m. Her abdominal ultrasonography revealed an increase in bilateral kidney echogenicity. The patient was given prednisolone for 4 weeks. However, she was resistant to steroid treatment. The patient’s 6-year-old brother, given the family history of kidney failure, was screened for nephrotic syndrome and was also found to have hypoalbuminemia and non-nephrotic range proteinuria without hematuria. Kidney biopsy was performed and demonstrated mild mesangial and endocapillary cell proliferation and basement membrane thickening without any immunofluorescence staining for IgG, IgM, IgA, C3, C1q, kappa, or lambda light chains (Figs. 1 and 2). The genetic testing for nephrin (NPHS1), podocin (NPHS2), and Wilms Tumor 1 (WT1) genes were unremarkable. The patient was given multiple immunosuppressive medications including cyclosporine, tacrolimus, MMF, and rituximab. However, she did not respond to the treatment and reached stage 5 CKD (CKD 5) at the 5th year of follow-up. Her brother was followed up with steroid and cyclosporine treatment.
An 11-year-old girl was admitted with hemolytic anemia and polyarthralgia that had persisted for ... more An 11-year-old girl was admitted with hemolytic anemia and polyarthralgia that had persisted for 6 months. Her family history was remarkable for Sjogren's syndrome. On admission, she had a pallor appearance and arthralgia on the ankles and knees. Laboratory findings were as follows: hemoglobin 4.7 g/dL, white blood cell count 8000/mm 3 , platelets 210.000/mm 3 , reticulocyte ratio 7.9%, BUN 16 mg/dL, creatinine 0.41 mg/dl, LDH 576 U/L, ANA 1:1000, anti-ds-DNA negative, ACA positive, anti-beta2 glycoprotein antibody positive, serum C3 and 4 normal, direct globulin positive, lupus anticoagulant ratio 1.27 (1+), p-ANCA and c-ANCA negative. A 24-h urine collection showed nephrotic range proteinuria of 1632 mg/m 2 /day. Kidney ultrasound revealed increased renal parenchymal echogenicity.
Vesicoureteral reflux (VUR) is considered the most important predisposing factor for urinary trac... more Vesicoureteral reflux (VUR) is considered the most important predisposing factor for urinary tract infection (UTI). Renal damage due to VUR, subsequently renal scarring and the possibility of reflux nephropathy warrant early detection of VUR. Our aim was to evaluate the value of positioned instillation of contrast (PIC) cystography in the detection of VUR in children with recurrent UTI and a normal voiding cystourethrography (VCUG). Materials and Methods: PIC cystography was performed in each child with the indication of recurrent UTI with a normal VCUG between June 2015 and November 2017. Results: Thirty-four children (32 girls, 2 boys) aged 7 to 17 years (median, 10 years), were examined. Twenty (58.8%) patients had normal ultrasound and 12 patients (35.3%) had bilateral scars detected using 99mTc-dimercaptosuccinic acid (DMSA). Thirty-one (91.2%) patients were shown to have VUR on PIC cystography. Nine (29%) patients had no renal scar with positive PIC and 3 (12%) patients had scars with negative PIC. Scars were detected in 13 (72.2%) patients with grade I-II VUR, and 4 (30.8%) with grade III-IV VUR had no scars on DMSA. There was no significant difference between the results of PIC and DMSA renal scan. Twenty-six patients (76.5%) with PIC-VUR underwent simultaneous endoscopic injections. During the postoperative follow-up with an average of 12 months, 27 patients showed no evidence of febrile UTIs. Conclusion: Based on our results, PIC cystogram seems to be a good alternative to invasive voiding cystourethrogram in screening children for VUR.
Having a low nephron number is a well-known risk factor for hypertension. There is an inverse rel... more Having a low nephron number is a well-known risk factor for hypertension. There is an inverse relationship between the filtration surface area and systemic hypertension. A significant percentage of masked hypertension can be detected in children with nephron loss by ambulatory blood pressure monitoring (ABPM). We prospectively investigated ABPM results of children having reduced kidney mass with normal office blood pressures (BPs) and kidney function. Forty-three children with congenital solitary kidney (group 1), 11 children with acquired solitary kidney (group 2), and 76 children with hypofunctioning kidney (group 3) were compared with age, gender, and BMI-matched healthy control group (group 4). The dietary salt intake of 76 patients was evaluated as salt equivalent (g/day). The primary endpoint was change from baseline in mean 24-h ABPM variables and the proportion of patients with masked hypertension when assessed by ABPM. The masked hypertension ratio of all patients was 12.3% when assessed with ABPM. Night hypertension was significantly higher in all patient groups than in the control group (p = 0.01). Diastolic BP loads of groups 1 and 3 were higher than in controls (p = 0.024). Systolic BP loads were higher only in group 1 than in the control group (p = 0.003). The dietary salt equivalent of patients in group 1 correlated positively with 24-h SBP and mean arterial pressure (MAP) values. Patients with excessive dietary salt intake in group 1 had a significantly higher diastolic BP load than those without excessive salt intake in group 1 (p = 0.002). Masked hypertension can be seen in children with a solitary kidney or when one of the kidneys is hypofunctioning. Systolic BP loads are higher in children with congenital solitary kidney, and salt intake correlates with systolic BP profiles especially in those. Our results suggest that being born with a congenital solitary kidney increases predisposition to hypertension and salt sensitivity.
Background Familial Mediterranean fever (FMF) is an inherited inflammatory disorder characterized... more Background Familial Mediterranean fever (FMF) is an inherited inflammatory disorder characterized by attacks of fever with polyserositis. Objective The purpose of this study was to evaluate pediatric patients with FMF who had central nervous system (CNS) findings. Materials and Methods Our medical records database for 2003 to 2014 was screened retrospectively. In total, 104 patients with FMF were identified, 22 of whom had undergone neurological examination for CNS symptoms. Results Neurological findings included headache in 16 patients (72.7%), epilepsy in 6 patients (27.3%), pseudotumor cerebri in 2 patients (9.1%), multiple sclerosis in 1 patient (4.5%), and tremor in 1 patient (4.5%). The most common MEFV gene mutation was homozygous M694V (40.9%). Conclusions Patients with FMF can present with various CNS manifestations. Further studies that include large populations are needed to elucidate the neurological manifestations of FMF.
To early detect extra-articular organ involvement such as kidney, heart and lung in rheumatic dis... more To early detect extra-articular organ involvement such as kidney, heart and lung in rheumatic disorders is very important for prognosis. Renal involvement in children with rheumatic diseases results from underlying disease or drugs used to treat rheumatic diseases. Here, we will review the pathogenesis, clinical and laboratory findings of renal disorders associated with childhood rheumatic diseases other than vasculitis, auto-inflammatory diseases and systemic lupus erythematosus, and will summarize management of renal involvement in rheumatic diseases.
Objective: In order to contribute the empirical treatment of urinary tract infections in children... more Objective: In order to contribute the empirical treatment of urinary tract infections in children, the antibiotic resistance patterns of uropathogens isolated in one center over a year, and clinical risk factors were evaluated in this study. Methods: In this single-center study, the positive urine cultures and their antibiotic susceptibilities in a Child Nephrology Outpatient Clinic between March 2018 and March 2019 were evaluated retrospectively. Patient records, demographic data, obtaining method of urine and accompanying urinary tract problems were noted. Antibiotic susceptibility testing results of patients with significant bacteriuria were evaluated. Multidrug resistant (MDR) organisms were described as having resistance to at least three different groups of antimicrobials. Independent clinical risk factors were investigated by logistic regression analysis in infections due to MDR organisms. Results: The most common two organisms were Escherichia coli (74.3%) and Klebsiella spp. (10.6%). Resistance rates to cefuroxime and trimethoprim-sulfamethoxazole (TMP-SXT) was 40.6% and 44% for E. coli, and 60.8% and 63% for Klebsiella spp. respectively (p<0.05). Extended spectrum β-lactamase (ESBL) positivity rate was 25.6% among all bacterial isolates. MDR isolates were detected in 63.8% of ESBL-positive organisms whereas it was 36.2% in ESBL-negative ones. Male gender rate was 31.9% in infections due to MDR pathogens, and 20.1% in infections with susceptible ones (p<0.05). Neurogenic bladder and male gender were determined as independent risk factors for isolation of MDR pathogens.
Background and Aims Response to calcineurin inhibitors (CNI) is associated with a significant imp... more Background and Aims Response to calcineurin inhibitors (CNI) is associated with a significant improvement in long-term kidney survival of children with non-genetic steroid resistant nephrotic syndrome (SRNS). On the contrary, these agents are considered non-efficacious in monogenic SRNS and are contraindicated according to the latest International Pediatric Nephrology Association (IPNA) Clinical Practice Recommendations for SRNS. However, there is evidence suggesting that remission with CNI therapy in this subgroup of children with SRNS is possible, but no studies to date have assessed this question in a systematic way. We aimed to study the incidence of response to CNI in children with monogenic SRNS, factors predictive of remission and the effect of treatment on kidney survival. Method Retrospective cohort study of children 0–18 years with genetically confirmed SRNS treated with a CNI for at least 3 months. Demographic, clinical, genetic, biochemical, histopathologic and treatment...
Background C3 glomerulopathy(C3G) is a complement-mediated disease caused by abnormalities in the... more Background C3 glomerulopathy(C3G) is a complement-mediated disease caused by abnormalities in the alternative complement pathway. Although genetic studies are not required for diagnosis, they are valuable for treatment planning and prognosis prediction. The aim of this study is to investigate the clinical phenotypes, kidney survival, and response to MMF treatment in pediatric C3G patients with and without mutations in complement related genes.Methods Sixty pediatric C3G patients were included, divided into two groups based on complement related gene mutations. Demographic and clinical-pathological findings, treatment modalities, and outcome data were compared, and Kaplan-Meier analysis was performed for kidney survival.Results Out of the 60 patients, 17 had mutations, with the most common mutation in the CH gene (47%). The mean age at diagnosis was significantly higher in the group with mutation (12.9 ± 3.6 vs 11.2 ± 4.1 years p = 0.039). While the patients without mutation were mos...
Background Dent's disease type 1 (DD1) is a rare X-linked nephropathy caused by CLCN5 mutatio... more Background Dent's disease type 1 (DD1) is a rare X-linked nephropathy caused by CLCN5 mutations, characterized by proximal tubule dysfunction, including low molecular weight proteinuria (LMWP), hypercalciuria, nephrolithiasis–nephrocalcinosis, progressive chronic kidney disease (CKD) and kidney failure (KF). Current management is symptomatic and does not prevent disease progression. Here we describe the contemporary DD1 picture across Europe to highlight its unmet needs. Methods A physician-based anonymous international e-survey supported by several European nephrology networks/societies was conducted. Questions focused on DD1 clinical features, diagnostic procedure and mutation spectra. Results A total of 207 DD1 male patients were reported; clinical data were available for 163 with confirmed CLCN5 mutations. Proteinuria was the most common manifestation (49.1%). During follow-up, all patients showed LMWP, 66.4% nephrocalcinosis, 44.4% hypercalciuria and 26.4% nephrolithiasis. ...
Objective: This study aimed to evaluate patients with renal transplantation in terms of clinical ... more Objective: This study aimed to evaluate patients with renal transplantation in terms of clinical and laboratory. parameters. Material and Methods: This study was performed retrospectively with records of 48 patients who underwent renal transplantation before 18 years of age, between June 2008 and July 2019. Results: Congenital malformations of the urinary tract were the most common underlying causes of chronic kidney disease stage 5. Surgical complications occurred in 33.4% of the patients and BK viremia was the most common opportunistic viral infection during the follow-up. At the last clinic visit, 57.4% of our patients had CKD stage 1, hypertension and nephrotic range proteinuria were seen in eight and two patients, respectively. Conclusion: Although renal transplantation is the most ideal renal replacement therapy, patients may experience various complications during the follow-up. Therefore, they should be monitored regularly
Eryavuz et al.: The role of oxidative stress in determining prognosis in children with FMF and th... more Eryavuz et al.: The role of oxidative stress in determining prognosis in children with FMF and the relationship between markers of oxidative stress and gene mutation. Pediatric Rheumatology 2015 13(Suppl 1):P107.
A 4-year-old girl presented with complaints of swelling around the eyes and reduced urine output ... more A 4-year-old girl presented with complaints of swelling around the eyes and reduced urine output for 3 days. On admission, her body weight was 14 kg (10–25th percentile), and height was 88 cm (< 3rd percentile). Her fever was 36°C, heart rate was 108/min, respiratory rate was 24/ min, and blood pressure was 110/50 mmHg. In her physical exam, there were ascites in her abdomen and four positive pitting pretibial edemas. The rest of the physical examination was normal. Her past medical history was unremarkable. Her parents were first-degree cousins, and her parents’ cousins suffered from chronic kidney disease (CKD) of unknown etiology. In the laboratory study, hemoglobin was 12.9 g/dl, white blood cell count was 9800 mm, and platelet count was 281,000 mm. The other laboratory parameters were as follows: blood urea nitrogen 23 mg/dl, serum creatinine 0.7 mg/ dl, uric acid 5 mg/dl, sodium 135 mmol/L, potassium 3.7 mmol/L, chlorine 105 mmol/L, total protein 3.5 g/dl, albumin 1.52 g/dl, triglyceride 647 mg/dl, and cholesterol 424 mg/dl. The C3 was within the normal range (119 mg/dl). There was no evidence of hematuria in urine analysis; only proteinuria was detected. Microprotein to creatinine ratio was 3:4. The 24-h urine proteinuria was 45 mg/m/h, and GFR was 77 ml/min/1.73 m. Her abdominal ultrasonography revealed an increase in bilateral kidney echogenicity. The patient was given prednisolone for 4 weeks. However, she was resistant to steroid treatment. The patient’s 6-year-old brother, given the family history of kidney failure, was screened for nephrotic syndrome and was also found to have hypoalbuminemia and non-nephrotic range proteinuria without hematuria. Kidney biopsy was performed and demonstrated mild mesangial and endocapillary cell proliferation and basement membrane thickening without any immunofluorescence staining for IgG, IgM, IgA, C3, C1q, kappa, or lambda light chains (Figs. 1 and 2). The genetic testing for nephrin (NPHS1), podocin (NPHS2), and Wilms Tumor 1 (WT1) genes were unremarkable. The patient was given multiple immunosuppressive medications including cyclosporine, tacrolimus, MMF, and rituximab. However, she did not respond to the treatment and reached stage 5 CKD (CKD 5) at the 5th year of follow-up. Her brother was followed up with steroid and cyclosporine treatment.
An 11-year-old girl was admitted with hemolytic anemia and polyarthralgia that had persisted for ... more An 11-year-old girl was admitted with hemolytic anemia and polyarthralgia that had persisted for 6 months. Her family history was remarkable for Sjogren's syndrome. On admission, she had a pallor appearance and arthralgia on the ankles and knees. Laboratory findings were as follows: hemoglobin 4.7 g/dL, white blood cell count 8000/mm 3 , platelets 210.000/mm 3 , reticulocyte ratio 7.9%, BUN 16 mg/dL, creatinine 0.41 mg/dl, LDH 576 U/L, ANA 1:1000, anti-ds-DNA negative, ACA positive, anti-beta2 glycoprotein antibody positive, serum C3 and 4 normal, direct globulin positive, lupus anticoagulant ratio 1.27 (1+), p-ANCA and c-ANCA negative. A 24-h urine collection showed nephrotic range proteinuria of 1632 mg/m 2 /day. Kidney ultrasound revealed increased renal parenchymal echogenicity.
Vesicoureteral reflux (VUR) is considered the most important predisposing factor for urinary trac... more Vesicoureteral reflux (VUR) is considered the most important predisposing factor for urinary tract infection (UTI). Renal damage due to VUR, subsequently renal scarring and the possibility of reflux nephropathy warrant early detection of VUR. Our aim was to evaluate the value of positioned instillation of contrast (PIC) cystography in the detection of VUR in children with recurrent UTI and a normal voiding cystourethrography (VCUG). Materials and Methods: PIC cystography was performed in each child with the indication of recurrent UTI with a normal VCUG between June 2015 and November 2017. Results: Thirty-four children (32 girls, 2 boys) aged 7 to 17 years (median, 10 years), were examined. Twenty (58.8%) patients had normal ultrasound and 12 patients (35.3%) had bilateral scars detected using 99mTc-dimercaptosuccinic acid (DMSA). Thirty-one (91.2%) patients were shown to have VUR on PIC cystography. Nine (29%) patients had no renal scar with positive PIC and 3 (12%) patients had scars with negative PIC. Scars were detected in 13 (72.2%) patients with grade I-II VUR, and 4 (30.8%) with grade III-IV VUR had no scars on DMSA. There was no significant difference between the results of PIC and DMSA renal scan. Twenty-six patients (76.5%) with PIC-VUR underwent simultaneous endoscopic injections. During the postoperative follow-up with an average of 12 months, 27 patients showed no evidence of febrile UTIs. Conclusion: Based on our results, PIC cystogram seems to be a good alternative to invasive voiding cystourethrogram in screening children for VUR.
Having a low nephron number is a well-known risk factor for hypertension. There is an inverse rel... more Having a low nephron number is a well-known risk factor for hypertension. There is an inverse relationship between the filtration surface area and systemic hypertension. A significant percentage of masked hypertension can be detected in children with nephron loss by ambulatory blood pressure monitoring (ABPM). We prospectively investigated ABPM results of children having reduced kidney mass with normal office blood pressures (BPs) and kidney function. Forty-three children with congenital solitary kidney (group 1), 11 children with acquired solitary kidney (group 2), and 76 children with hypofunctioning kidney (group 3) were compared with age, gender, and BMI-matched healthy control group (group 4). The dietary salt intake of 76 patients was evaluated as salt equivalent (g/day). The primary endpoint was change from baseline in mean 24-h ABPM variables and the proportion of patients with masked hypertension when assessed by ABPM. The masked hypertension ratio of all patients was 12.3% when assessed with ABPM. Night hypertension was significantly higher in all patient groups than in the control group (p = 0.01). Diastolic BP loads of groups 1 and 3 were higher than in controls (p = 0.024). Systolic BP loads were higher only in group 1 than in the control group (p = 0.003). The dietary salt equivalent of patients in group 1 correlated positively with 24-h SBP and mean arterial pressure (MAP) values. Patients with excessive dietary salt intake in group 1 had a significantly higher diastolic BP load than those without excessive salt intake in group 1 (p = 0.002). Masked hypertension can be seen in children with a solitary kidney or when one of the kidneys is hypofunctioning. Systolic BP loads are higher in children with congenital solitary kidney, and salt intake correlates with systolic BP profiles especially in those. Our results suggest that being born with a congenital solitary kidney increases predisposition to hypertension and salt sensitivity.
Background Familial Mediterranean fever (FMF) is an inherited inflammatory disorder characterized... more Background Familial Mediterranean fever (FMF) is an inherited inflammatory disorder characterized by attacks of fever with polyserositis. Objective The purpose of this study was to evaluate pediatric patients with FMF who had central nervous system (CNS) findings. Materials and Methods Our medical records database for 2003 to 2014 was screened retrospectively. In total, 104 patients with FMF were identified, 22 of whom had undergone neurological examination for CNS symptoms. Results Neurological findings included headache in 16 patients (72.7%), epilepsy in 6 patients (27.3%), pseudotumor cerebri in 2 patients (9.1%), multiple sclerosis in 1 patient (4.5%), and tremor in 1 patient (4.5%). The most common MEFV gene mutation was homozygous M694V (40.9%). Conclusions Patients with FMF can present with various CNS manifestations. Further studies that include large populations are needed to elucidate the neurological manifestations of FMF.
To early detect extra-articular organ involvement such as kidney, heart and lung in rheumatic dis... more To early detect extra-articular organ involvement such as kidney, heart and lung in rheumatic disorders is very important for prognosis. Renal involvement in children with rheumatic diseases results from underlying disease or drugs used to treat rheumatic diseases. Here, we will review the pathogenesis, clinical and laboratory findings of renal disorders associated with childhood rheumatic diseases other than vasculitis, auto-inflammatory diseases and systemic lupus erythematosus, and will summarize management of renal involvement in rheumatic diseases.
Objective: In order to contribute the empirical treatment of urinary tract infections in children... more Objective: In order to contribute the empirical treatment of urinary tract infections in children, the antibiotic resistance patterns of uropathogens isolated in one center over a year, and clinical risk factors were evaluated in this study. Methods: In this single-center study, the positive urine cultures and their antibiotic susceptibilities in a Child Nephrology Outpatient Clinic between March 2018 and March 2019 were evaluated retrospectively. Patient records, demographic data, obtaining method of urine and accompanying urinary tract problems were noted. Antibiotic susceptibility testing results of patients with significant bacteriuria were evaluated. Multidrug resistant (MDR) organisms were described as having resistance to at least three different groups of antimicrobials. Independent clinical risk factors were investigated by logistic regression analysis in infections due to MDR organisms. Results: The most common two organisms were Escherichia coli (74.3%) and Klebsiella spp. (10.6%). Resistance rates to cefuroxime and trimethoprim-sulfamethoxazole (TMP-SXT) was 40.6% and 44% for E. coli, and 60.8% and 63% for Klebsiella spp. respectively (p<0.05). Extended spectrum β-lactamase (ESBL) positivity rate was 25.6% among all bacterial isolates. MDR isolates were detected in 63.8% of ESBL-positive organisms whereas it was 36.2% in ESBL-negative ones. Male gender rate was 31.9% in infections due to MDR pathogens, and 20.1% in infections with susceptible ones (p<0.05). Neurogenic bladder and male gender were determined as independent risk factors for isolation of MDR pathogens.
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