Pediatric kidney care experience after the 2023 Turkey/Syria earthquake.

BACKGROUND/AIMS: Two earthquakes on February 6th, 2023 destroyed ten cities in Türkiye. We report our experience with pediatric victims during these catastrophes, with a focus on crush syndrome related-acute kidney injury (Crush-AKI) and death. METHOD: A web-based software was prepared. Patient demographics, time under rubble (TUR), admission laboratory data, dialysis, and kidney and overall outcomes were asked. RESULTS: 903 injured children (median age: 11.62 years) were evaluated. Mean TUR was 13 h (Interquartile range-IQR: 32.5), max 240 h). 31 of 32 patients with a TUR of >120 h survived. The patient who rescued after ten days survived.Two-thirds of the patients were given 50 mEq/L sodium-bicarbonate in 0.45% sodium-chloride solution on admission day. 58% of patients were given intravenous fluid (IVF) at a volume of 2000-3000 mL/m2 body surface area (BSA), 40% of 3000-4000 mL/m2 BSA, and only 2% of >4000 mL/m2 BSA. 425 patients had surgeries, 48 suffered from major bleeding. Amputations were recorded in 96 patients. Eighty-two and 66 patients required ventilator and inotropic support, respectively.Crush-AKI developed in 314 patients (36% of all patients). 189 patients were dialyzed. Age > 15 years, creatine phosphokinase (CK)≥20 950 U/L, TUR≥10 h, and the first-day IVF volume < 3000-4000 mL/m2 BSA were associated with Crush-AKI development. 22 deaths were recorded, 20 of 22 occurred in patients with Crush-AKI and within the first 4 days of admission. All patients admitted after 7 days survived. CONCLUSIONS: This is the most extensive pediatric kidney disaster data after an earthquake. Serum CK level was significantly associated with Crush-AKI at the levels of >20 950 U/L, but not with death. Adolescent age and initial IVF of less than 3000-4000 mL/m2 BSA were also asscoiated with Crush-AKI. Given that mildly injured victims can survive longer periods in the disaster field, we suggest uninterrupted rescue activity for at least 10 days.

Immunity Rates of Live Viral Vaccines in Pediatric Renal Transplant Candidates: A Single-Center Experience.

OBJECTIVES: Solid-organ transplant recipients are at an increased risk of severe infections due to their immunosuppressed state. Despite the recommendation of routine screening and vaccination before transplant to mitigate this danger, vaccination rates in these patients are still below desirable levels. We aimed to investigate the prevalence of positive antibody rates for measles, mumps, rubella, and varicella among children who are candidates for renal transplant. MATERIALS AND METHODS: This retrospective study was conducted at a single center and included 144 pediatric kidney transplant patients for the past 7 years. We reviewed the medical records of all participants to evaluate their serologic status for measles, mumps, rubella, and varicella viruses before kidney transplant. RESULTS: In this study, 144 pediatric kidney transplant candidates (mean age 11.5 years, 56.9% male) were enrolled, and the most frequent causes of the chronic renal disease were congenital anomalies of the kidney and urinary tract and glomerular diseases (32.6%). Seropositivity rates for measles, mumps, rubella, and varicella were 59.0%, 31.9%, 46.5%, and 43.6%, respectively, and all patients who tested negative for antibodies were vaccinated before transplant. Younger age at transplant (OR = 0.909, 95% CI = 0.840-0.923; P = .017) and congenital anomalies of the kidney and urinary tract (OR = 3.46, 95% CI = 1.1548-7.735; P = .002) were significantly associated with increased measles seropositivity, although no significant associations were observed for the other viruses. CONCLUSIONS: We observed lower seropositivity rates for measles, mumps, rubella, and varicella in pediatric kidney transplant patients versus healthy children and other previous studies. It is essential to address these suboptimal rates to protect the health of these vulnerable patients. Future research should focus on targeted interventions to improve vaccination rates and outcomes in this population.

Characteristics and predictors of chronic kidney disease in children with myelomeningocele: a nationwide cohort study.

BACKGROUND: Myelomeningocele (MMC) is highly prevalent in developing countries, and MMC-related neurogenic bladder is an important cause of childhood chronic kidney disease (CKD). This nationwide study aimed to evaluate demographic and clinical features of pediatric patients with MMC in Turkey and risk factors associated with CKD stage 5. METHODS: Data from children aged 0-19 years old, living with MMC in 2022, were retrospectively collected from 27 pediatric nephrology centers. Patients > 1 year of age without pre-existing kidney abnormalities were divided into five groups according to eGFR; CKD stages 1-5. Patients on dialysis, kidney transplant recipients, and those with eGFR < 15 ml/min/1.73 m2 but not on kidney replacement therapy at time of study constituted the CKD stage 5 group. RESULTS: A total of 911 (57.8% female) patients were enrolled, most of whom were expectantly managed. Stages 1-4 CKD were found in 34.3%, 4.2%, 4.1%, and 2.4%, respectively. CKD stage 5 was observed in 5.3% of patients at median 13 years old (range 2-18 years). Current age, age at first abnormal DMSA scan, moderate-to-severe trabeculated bladder on US and/or VCUG, and VUR history were independent risk factors for development of CKD stage 5 (OR 0.752; 95%; CI 0.658-0.859; p < 0.001; OR 1.187; 95% CI 1.031-1.367; p = 0.017; OR 10.031; 95% CI 2.210-45.544; p = 0.003; OR 2.722; 95% CI 1.215-6.102; p = 0.015, respectively). Only eight CKD stage 5 patients underwent surgery related to a hostile bladder between 1 and 15 years old. CONCLUSION: MMC-related CKD is common in childhood in Turkey. A proactive approach to neurogenic bladder management and early protective surgery in selected cases where conservative treatment has failed should be implemented to prevent progressive kidney failure in the pediatric MMC population in our country.

Antibiotic Prophylaxis in Infants with Grade III, IV, or V Vesicoureteral Reflux.

BACKGROUND: The efficacy of continuous antibiotic prophylaxis in preventing urinary tract infection (UTI) in infants with grade III, IV, or V vesicoureteral reflux is controversial. METHODS: In this investigator-initiated, randomized, open-label trial performed in 39 European centers, we randomly assigned infants 1 to 5 months of age with grade III, IV, or V vesicoureteral reflux and no previous UTIs to receive continuous antibiotic prophylaxis (prophylaxis group) or no treatment (untreated group) for 24 months. The primary outcome was the occurrence of the first UTI during the trial period. Secondary outcomes included new kidney scarring and the estimated glomerular filtration rate (GFR) at 24 months. RESULTS: A total of 292 participants underwent randomization (146 per group). Approximately 75% of the participants were male; the median age was 3 months, and 235 participants (80.5%) had grade IV or V vesicoureteral reflux. In the intention-to-treat analysis, a first UTI occurred in 31 participants (21.2%) in the prophylaxis group and in 52 participants (35.6%) in the untreated group (hazard ratio, 0.55; 95% confidence interval [CI], 0.35 to 0.86; P = 0.008); the number needed to treat for 2 years to prevent one UTI was 7 children (95% CI, 4 to 29). Among untreated participants, 64.4% had no UTI during the trial. The incidence of new kidney scars and the estimated GFR at 24 months did not differ substantially between the two groups. Pseudomonas species, other non-Escherichia coli organisms, and antibiotic resistance were more common in UTI isolates obtained from participants in the prophylaxis group than in isolates obtained from those in the untreated group. Serious adverse events were similar in the two groups. CONCLUSIONS: In infants with grade III, IV, or V vesicoureteral reflux and no previous UTIs, continuous antibiotic prophylaxis provided a small but significant benefit in preventing a first UTI despite an increased occurrence of non-E. coli organisms and antibiotic resistance. (Funded by the Italian Ministry of Health and others; PREDICT ClinicalTrials.gov number, NCT02021006; EudraCT number, 2013-000309-21.).

Long-term effect of eplerenone treatment in children with chronic allograft nephropathy.

BACKGROUND: Literature supports the protective role of mineralocorticoid antagonist (MRA) against the renal injury induced by aldosterone in kidney transplant recipients. However, there is limited data available regarding the safety and efficacy of MRAs in pediatric renal transplant patients. Therefore, we aimed to investigate the effect of long-term eplerenone administration in children with chronic allograft nephropathy (CAN). METHODS: Twenty-six renal transplant children with biopsy-proven CAN, an estimated glomerular filtration rate (eGFR ) > 40 mL/min per 1.73 m2 and with a significant proteinuria were included. Selected patients were randomly divided into two groups as follows; Group 1 (n = 10) patients received 25 mg/day eplerenone and Group 2 (n = 16) patients did not receive eplerenone for 36 months. Patients were examined in the renal transplant outpatient clinic biweekly for the first month and once a month thereafter. The primary outcome of the patients was compared. RESULTS: Mean eGFR stayed stable in group 1 patients, but significantly decreased in group 2 at 36 months (57.53 ± 7.53 vs. 44.94 ± 8.04 mL/min per 1.73 m2 , p = .001). Similarly, spot protein-creatinine ratio was significantly lower in group 1 compared to group 2 patients at 36 months (1.02 ± 7.53 vs. 3.61 ± 0.53, p < .001). Eplerenone associated hyperkalemia was not observed in group 1 patients (4.6 ± 0.2 vs. 4.56 ± 0.3, p = .713). CONCLUSION: The long-term eplerenone administration blunted the chronic allograft nephropathy by maintaining a stable eGFR levels and decreasing urine protein-creatinine ratio. Eplerenone associated hyperkalemia was not observed in our study.

Can we identify "at-risk" children and adolescents for poor transplant outcomes in the psychosocial evaluation before solid organ transplantation? The reliability and validity study of Pediatric Transplant Rating Instrument (P-TRI) in Turkish pediatric renal transplant patients.

BACKGROUND: This study aims to translate the Pediatric Transplant Rating Instrument (P-TRI) to conduct a validity and reliability study on Turkish children and define a cutoff value of this scale. METHOD: A total of 151 pediatric kidney transplant patients were included in the study. The files of the patients were reviewed by two clinicians, and the scale was filled for inter-rater reliability. One of the clinicians filled the scale again after one month for intra-rater reliability. Glomerular filtration rate (GFR) and creatinine values were used for predictive validity. A GFR below <60 ml/min/1.73 m2 and creatinine up to 3.0 mg/dl was defined as risk factors. RESULTS: Correlation of P-TRI with GFR (r = .252, p = .003) and creatinine (r = -.249, p = .002) was performed, and the internal consistency of the scale items as measured by Cronbach's alpha coefficient was found to be 0.825. When the test was performed again, the intra-class correlation coefficient was found as .922 for intra-rater reliability and as .798 for inter-rater reliability. For both creatinine and GFR, the best cutoff point for the total score was found to be 66.5. CONCLUSIONS: Patients who received P-TRI above 66.5 could be at risk in the post-transplant period. Identification of these patients before transplantation and following these young people more closely will aid in the prevention of serious consequences. The reliability and validity scores are satisfactory for use in transplantation clinics for psychosocial evaluation and compliance in Turkish pediatric renal transplantation patients.

Impact of Matrix Metalloproteinases 2 and 9 and Tissue Inhibitor of Metalloproteinase 2 Gene Polymorphisms on Allograft Rejection in Pediatric Renal Transplant Recipients.

OBJECTIVES: Acute and chronic allograft rejection have been continuously an important obstacle in the follow-up of renal transplant recipients. During clinical management, several factors acting simultaneously result in acute rejection and chronic allograft nephropathy. Matrix metalloproteinases and tissue inhibitors of metalloproteinases are responsible for the organization of the extracellular matrix and play roles in cell proliferation and cellular invasion. Changes in matrix metalloproteinase expression levels have been reported to be associated with renal allograft rejection and interstitial fibrosis. In this study, we aimed to investigate functional polymorphisms of MMP2, MMP9, and TIMP2 genes in pediatric renal transplant recipients. MATERIALS AND METHODS: Our study included 68 kidney transplant recipients and 58 control patients. The kidney transplant recipient group was further divided into 2 subgroups: no graft rejection (n = 47) and graft rejection (n =21). MMP2 -735C >T (rs2285053), MMP2 -1306C >T (rs243865), MMP2 -1575G >A (rs243866), MMP9 c.-1562C >T (rs3918242), TIMP2 -418G >C (rs8179090), and TIMP2 303C > T (rs2277698) polymorphisms were analyzed with the use of polymerase chain reaction and restriction fragment-length polymorphism methods. Allele prevalence was compared with reference values of the control group, and Hardy-Weinberg equilibrium was tested. RESULTS: Mean ages were 16.7 ± 3.9 years for the study group and 14.8 ± 5.6 years for the control group. The mean follow-up time after transplant was 37.7 ± 7.9 months. We compared allele frequencies in the 2 groups and calculated a statistically significant difference in rs2285053, rs243865, rs243866, rs3918242, rs8179090, and rs2277698 polymorphism frequencies between the transplant recipients and control patients. When the transplant recipient group was compared in itself with regard to allograft rejection, all investigated polymorphisms except TIMP2 -418G >C (rs8179090) revealed a statistically significant difference between those with and without rejection (P < .05). CONCLUSIONS: Matrix metalloproteinases and their tissue inhibitors could be important predictive biological markers for the follow-up of kidney transplant recipients.

Left renal vein entrapment in postural proteinuria: the diagnostic utility of the aortomesenteric angle.

Nutcracker syndrome related to the left kidney vein compression is a cause of orthostatic proteinuria during childhood. Some studies have shown that the ratios between maximum velocities and anterior-posterior diameters of hilar and aortomesenteric segments of the left kidney vein between upright and supine positions must be more than 4 in order to make a Nutcracker syndrome diagnosis. Our aim was to investigate whether the use of a decrease in aortomesenteric angle between upright and supine positions in the presence of isolated orthostatic proteinuria can be a criterion for the diagnosis of Nutcracker syndrome. Relevant patient information, which included demographic data, clinical examination findings, laboratory data, urinary system ultrasound, and kidney color flow Doppler ultrasound results, were prospectively collected. Thirty-nine pediatric patients with orthostatic proteinuria were included in the study. Left kidney vein compression findings were demonstrated in 31 patients. The ratio of maximum velocities of hilar and aortomesenteric segments of the left kidney vein between upright and supine positions was above 4 in only 7 of our patients. Ratio of aortomesenteric angle between upright and supine positions was significantly decreased for patients with left kidney vein compression findings.  Conclusion: The use of a decrease in the ratio of aortomesenteric angle between upright and supine positions in the presence of orthostatic proteinuria, instead of the ratios for maximum velocities and anterior-posterior diameters of hilar and aortomesenteric segments, can be more helpful for the diagnosis of Nutcracker syndrome in the differential diagnosis of orthostatic proteinuria. What is Known: • Proteinuria may be a sign of an impending kidney disease • Nutcracker syndrome is a cause of orthostatic proteinuria. What is New: • Ratio of aortomesenteric angle between upright and supine positions > 0.6 can be used for Nutcracker syndrome diagnosis.

BK Polyomavirus Infection and Risk Factors in Pediatric Patients Undergoing Kidney Transplant.

OBJECTIVES: BK polyomavirus infection is a critical complication affecting graft survival after kidney transplant. We aimed to determine the frequency, the effect on graft function, and the risk factors of BK polyomavirus infection in pediatric kidney transplant patients. MATERIALS AND METHODS: We retrospectively reviewed data of 144 pediatric patients (female/male: 67/77; 0-18 years of age) who received kidney transplants in the past 10 years at our center. Demographic/ laboratory data, kidney failure etiologies, donor types, and immunosuppressive treatments were recorded. Patients were grouped as those with and without BKV infection, with groups compared in terms of transplant age, sex, kidney failure etiology, donor type, immunosuppressive treatments, presence of ureteral stents, acute rejection episodes, accompanying viral infections, glomerular filtration rate, and graft loss rate. RESULTS: Twelve patients (8.3%) had BK polyomavirus infection. All 12 patients had viruria (8.3%), 8 (5.5%) had viremia, and 4 (2.8%) had BK polyomavirus nephropathy. Two patients (1.4%) had graft loss because of BK polyomavirus nephropathy. When patients with and without infection were compared, no significant differences were found in terms of sex, transplant age, donor type, presence of a ureteral stent, acute rejection, graft loss, or immunosuppressive treatment (P > .05). Rates of congenital anomalies of the kidney and urinary tract were 30.3% and 66.6% in those without and with BK polyomavirus infection, respectively (P < .05). The group positive for BK polyomavirus had a significantly higher incidence of cytomegalovirus infection versus the group without infection (P < .05). Glomerular filtration rate values at years 1 and 3 were similar between groups (P > .05). CONCLUSIONS: Frequency of BK polyomavirus nephropathy in pediatric patients undergoing kidney transplant in our center was consistent with data from other centers. Graft loss can be prevented by early detection and treatment through close periodic control and adequate evaluation of risk factors.

The Role of Platelet-Lymphocyte Ratio and Neutrophil-Lymphocyte Ratio in Predicting the Delayed Graft Function in Pediatric Renal Transplant Patients.

OBJECTIVES: Delayed graft function is a common adverse outcome after renal transplant. Attempts for early prediction and prevention of delayed graft function are often challenging and misleading. Herein, we investigated for the first time the correlation between delayed graft function and preoperative noninvasive hematologic parameters to predict the possible adverse outcomes for renal transplant in pediatric patients. MATERIALS AND METHODS: In this study, preoperative hematologic parameters of 51 pediatric renal transplant recipients followed between 2015 and 2021 were analyzed retrospectively. The selected 16 renal transplant patients with delayed graft function and 35 patients without delayed graft function had no concomitant comorbidities. The cutoff values for platelet-to-lymphocyte ratio of <5 and neutrophilto- lymphocyte ratio of <175 were considered low. RESULTS: We retrospectively evaluated a total of 51 (male/female, 33/18) pediatric kidney transplant recipients with a median age of 12 (interquartile range, 8-18) years. Median level of circulating lymphocytes was significantly higher in patients with delayed graft function compared with patients without delayed graft function (2 vs 1, P = .040). The preoperative low values for platelet-to-lymphocyte ratio and neutrophil-to-lymphocyte ratio were more prevalent in recipients who developed delayed graft function versus those who did not develop delayed graft function (68.8% vs 31.4% [P = .014] and 68.8% vs 34.3% [P = .023], respectively). CONCLUSIONS: Pretransplant low platelet-to-lymphocyte ratio and neutrophil-to-lymphocyte were associated with increased number of delayed graft dysfunction. These novels and noninvasive inflammatory biomarkers may contribute to an early prediction of delayed graft function in pediatric kidney transplant recipients.

Long-Term Outcomes of Kidney Transplant Recipients With Juvenile Nephronophthisis.

OBJECTIVES: Nephronophthisis is the most common genetic cause of kidney failure in childhood. Treatment for nephronophthisis is symptomatic, and kidney transplant is a good treatment option when kidney failure has developed. We reported the outcomes of kidney transplant recipients with primary diagnosis of juvenile nephronophthisis who were followed-up in our center. MATERIALS AND METHODS: We retrospectively examined medical records of 17 kidney transplant patients with a primary diagnosis of juvenile nephronophthisis. We compared this group of 17 patients with kidney transplant recipients who had other etiologies of kidney failure in terms of transplant age, donor type, immunosuppressive treatment, acute rejection, graft loss rates, and glomerular filtration rates at 1 and 5 years posttransplant (N = 180 total analyzed). RESULTS: Among 180 kidney transplant recipients, the 17 patients (9.4%) with nephronophthisis had a mean age of 12.6 ± 4.3 years and mean follow-up time posttransplant of 79.5 ± 41.9 months. Five of 17 patients received a kidney transplant from a deceased donor (29.4%), and the remaining 12 patients (70.6%) received transplants from living related donors. Preemptive kidney transplant was performed in 4 patients (23.5%). There was a statistically significant difference (P < .05) in terms of acute rejection between patients with nephronophthisis (17.6%) versus patients with other primary diagnoses (34%). However, the patients with nephronophthisis versus those with other primary diagnoses were similar (P > .05) in terms of transplant age (12.6 ± 4.3 vs 13.8 ± 6.7 years, respectively) and follow-up time (79.5 ± 41.9 vs 59.1 ± 38.8 months, respectively). Donor type, immunosuppressive treatment, and 1-year (96.7 ± 23.2 vs 97.6 ± 28.4 mL/min/1.73 m2) and 5-year (84.7 ± 31.1 vs 86.7 ± 21.7 mL/min/1.73 m2) glomerular filtration rates were also similar (P > .05) between groups. CONCLUSIONS: Posttransplant prognosis was good among kidney transplant recipients with juvenile nephronophthisis.

Association Between Vitamin D Deficiency and Anemia in Pediatric Kidney Transplant Recipients.

OBJECTIVES: The association between vitamin D deficiency and anemia is known. Vitamin D deficiency and anemia are common in kidney transplant recipients. We examined the relationship between vitamin D levels and anemia in pediatric kidney transplant recipients. MATERIALS AND METHODS: We reviewed retrospectively the data of 75 pediatric kidney transplant recipients (0-18 years of age). Patients were evaluated in 3 groups according to serum 25-hydroxyvitamin D levels (<20, 20-30, and >30 ng/mL) in the first year posttransplant: group 1 was the vitamin D deficiency group, group 2 was the vitamin D insufficiency group, and group 3 was normal vitamin D level group, respectively. Groups were compared in terms of anemia parameters, calcium, phosphorus, alkaline phosphatase, and parathyroid hormone levels, as well as infection, rejection, and graft loss status. All patients included in the study were grouped as those with anemia and without anemia, and the 2 groups were compared in terms of vitamin D levels, serum parathyroid hormone values, estimated glomerular filtration rate, and infection, rejection, and graft loss status. RESULTS: There were 41 patients (54.7%) in group 1, 24 patients (32%) in group 2, and 10 patients (13%) in group 3. There were 65 patients (86.7%) with vitamin D deficiency/insufficiency. When groups were compared, the hematocrit level was found to be lower in groups 1 and 2 (P < .05) and ferritin level was found to be lower in group 1 (P < .05). Anemia was present in 20 patients (26.6%): 61% of patients with anemia had vitamin D deficiency and 33% had vitamin D insufficiency (P > .05). In total, 94% of patients with anemia had vitamin D deficiency/insufficiency. CONCLUSIONS: Vitamin D deficiency/insufficiency is common in pediatric kidney transplant recipients. Vitamin D levels should be measured, especially in all kidney transplant recipients with persistent anemia. Thus, risk factors associated anemia can be reduced by treating the deficiency/insufficiency.

Use of Eculizumab in Pediatric Patients with Late Antibody-Mediated Rejection After Kidney Transplantation.

Late antibody-mediated rejection triggered by donor-specific antibodies is a leading cause of kidney allograft failure. Effective treatment options for late antibody-mediated rejection are limited in renal transplant recipients. Here, we report 2 pediatric cases of severe late antibody-mediated rejection resistant to conventional immunosuppressive therapy who were successfully treated with eculizumab. Two patients who fulfilled the late antibody-mediated rejection diagnostic criteria (positive donor-specific antibodies, elevated mean fluorescence index, acute and/or chronic morphological lesions in the microvasculature, and abnormal kidney function test) were included in this study. Both patients were previously unsensitized with negative panel-reactive antibody. Case 1 was a 12-year-old male patient with kidney failure secondary to vesicoureteral reflux who underwent related-living donor kidney transplantation 2 years ago. Eleven months later, he was diagnosed with late antibody-mediated rejection. Despite an aggressive conventional immunosuppressive regimen, signs of rejection persisted. After the patient was treated with 2 doses of eculizumab, his mean fluorescence index dropped and serum creatinine decreased from 3.8 to 1.5 mg/dL. Case 2 was an unsensitized 16-year-old male patient with kidney failure secondary posterior urethral valve who underwent related-living donor kidney transplantation 4 years ago. Two years later, he was diagnosed with late antibody-mediated rejection. Despite an aggressive conventional immunosuppressive regimen, signs of rejection persisted. After treatment with 2 doses of eculizumab, his mean fluorescence index dropped and serum creatinine decreased from 2.1 to 1.01 mg/dL. In both patients, eculizumab therapy effectively reduced the markers of late antibody-mediated rejection and improved the kidney function.

Primary Focal Segmental Glomerulosclerosis Recurrence After Pediatric Renal Transplantation.

OBJECTIVES: Focal segmental glomerulosclerosis recurrence after renal transplant occurs frequently in pediatric patients and is associated with poor graft survival when patients reach adulthood. We investigated recurrence rates, recurrence risk factors, management strategies, and long-term graft function among pediatric renal transplant recipients with focal segmental glomerulosclerosis as primary disease. MATERIALS AND METHODS: We retrospectively evaluated medical records of 34 pediatric patients with primary focal segmental glomerulosclerosis who had undergone renal transplant between 2004 and 2019 at our center. Focal segmental glomerulosclerosis recurrence was diagnosed by the presence of nephrotic range proteinuria after transplant and confirmed by graft biopsy. Preoperative prophylactic plasma exchange was administered to pediatric renal transplant recipients with primary focal segmental glomerulosclerosis. Plasma exchange was also used to treat focal segmental glomerulosclerosis recurrence, with rituximab added if the patient did not respond to plasma exchange. RESULTS: All patients (male-to-female ratio of 19:15) in our group underwent renal transplant. Mean patient age at the time of transplant was 12.72 ± 5.46 years. Twenty-nine patients received living- related donor allografts (85.3%) and 5 received organs from deceased donors (14.7%). We identified focal segmental glomerulosclerosis recurrence in 5 recipients (14.7%). Time from focal segmental glomerulosclerosis diagnosis to end-stage renal disease and duration of dialysis were shorter in the recurrence group than in the nonrecurrence group (48.4 months [range, 2-90 mo] vs 65.1 months [range, 8-123 mo] and 1.41 ± 0.82 vs 3.18 ± 1.88 years, respectively; P < .05). Donor type and transplant age were similar in both groups. Of those with recurrence who had received plasma exchange and rituximab, 3 patients (75%) had complete remission and 1 patient (25%) had partial remission. CONCLUSIONS: Prophylactic plasma exchange and the combined plasma exchange-rituximab regimen for treatment of focal segmental glomerulosclerosis recurrence resulted in low recurrence and good remission rates in our pediatric cohort.

COVID-19 Infections in Pediatric Renal Transplant Recipients.

OBJECTIVES: The new coronavirus SARS-CoV-2 (COVID-19) first appeared in Turkey in March 2020, spread rapidly, and caused many deaths. Although COVID-19 is mostly a respiratory disease, it can cause kidney and multiorgan failure in some cases. We believe that by sharing information about the course and effects of COVID-19 infection in kidney transplant recipients receiving long-term immunosuppressive therapy our understanding will improve. MATERIALS AND METHODS: Between March 2020 and October 2021, COVID-19 was researched in kidney transplant recipients under the age of 20 years who were followed at the Baskent University Transplantation Center. We documented the clinical characteristics and prognosis of pediatric kidney transplant recipients with COVID-19 disease. RESULTS: Our study group included 23 patients with COVID-19 infection from 215 pediatric kidney transplant recipients. The mean age of the patients was 14.6 ± 4.7 years; there were 9 female patients. The mean follow-up time posttransplant was 62.3 ± 43.2 months. In 13 patients (56.5%), fever was the most frequent symptom. Most patients (n = 18, 78%) had minor symptoms and recovered completely after receiving supportive treatment. Four patients (17%) required hospitalization. One was diagnosed with COVID-19 infection 1 week after being treated with rituximab for acute antibody-mediated rejection. That patient died because of significant lung disease and multiorgan failure. CONCLUSIONS: Despite the fact that most of our pediatric transplant recipients had mild symptoms of COVID-19, we believe that particular caution should be observed in patients who have recently received intensive immunosuppressive medications. As a result of potential new vaccines, national immunization programs, and the emergence of novel virus strains, the clinical picture may change in the future. We believe that, as information sharing increases, we will learn more about COVID-19 in renal transplant recipients.

Eculizumab treatment and discontinuation in pediatric patients with atypical hemolytic uremic syndrome: a multicentric retrospective study.

INTRODUCTION: Eculizumab is effective treatment of pediatric atypical hemolytic uremic syndrome (aHUS). However, the optimal duration of treatment is not clearly defined. The aim of this study was to retrospectively analyze the outcome of pediatric patients with aHUS, who started eculizumab treatment but discontinued it during the follow-up period. METHODS: The clinical and laboratory findings of the pediatric patients with aHUS were recorded on a web-based, national registry system, known as the Turkish aHUS Registry. The study included 63 patients who had to have received more than four doses of eculizumab during the acute phase of the disease. RESULTS: The median age at diagnosis was 3.62 (IQR: 1.29-6.14) years. During the follow-up period, 39 patients continued to receive standard eculizumab treatment (standard treatment group, treatment every 2 weeks) while 24 received an extended dose of eculizumab at three-four-week intervals (non-standard treatment group). There was no significant difference between both groups in terms of clinical and laboratory parameters. Eculizumab treatment was discontinued in 18 patients (30.7%, F/M:11/7), and the median age of these patients at diagnosis and their median follow-up duration were 4.0 (IQR:2.7-10.2) and 4.2 (IQR:2.2-7) years respectively. The median eGFR at the last visit was 110 (84.7-146.1)ml/min/1.73 m2. Fourteen patients remained in remission without any sign of the disease. Recurrence occurred in four (22.2%) patients, in which eculizumab was immediately started again and complete remission was achieved. CONCLUSION: Eculizumab is a successful treatment option in pediatric patients with aHUS and it can be safely discontinued with close monitoring in a selected group of patients. In case of recurrence, eculizumab should be restarted immediately to achieve complete remission.

Comparison of diagnostic criteria for children with familial Mediterranean fever.

Familial Mediterranean fever (FMF) is an autoinflammatory disease characterized by recurrent attacks of fever and serositis. Diagnosis is made according to clinical findings and supported by genetic analysis. The most commonly used adult diagnostic criteria are the Tel-Hashomer criteria. Pediatric criteria for FMF diagnosis were described in 2009, but their reliability should be supported by additional reports. In this study, we aimed to compare the pediatric criteria and the Tel-Hashomer and 2019 Eurofever/PRINTO classification criteria using our FMF cohort. A total of 113 patients diagnosed with FMF were included. Demographic features and laboratory findings were retrospectively collected from the patients' files. The patients were evaluated with the Tel-Hashomer, pediatric and Eurofever/PRINTO classification criteria. At least two of five new pediatric criteria were as sensitive (89%) and specific (85%) as the Tel-Hashomer criteria (sensitivity 70%, specificity 96%). We also evaluated the Eurofever/PRINTO classification criteria using our cohort and found a sensitivity of 94% and specificity of 91%.   Conclusion: Using pediatric criteria for the diagnosis of FMF in children is a feasible and simple approach that can diagnose the disease based on at least two criteria. Therefore, our study supports the use of pediatric criteria in FMF diagnosis of children. Our results also confirm that the Eurofever/PRINTO classification criteria can be successfully applied for the diagnosis of FMF due to their high sensitivity (94%) and specificity (91%). What is Known: • The FMF diagnosis is made according clinical findings and supported by genetic analysis. • The use of adult diagnostic criteria in pediatric FMF patients is controversial since classical clinical presentation is often absent in children. What is New: • Our study supports both the use of pediatric criteria and Eurofever/PRINTO classification criteria in clinical practice.

COVID-19 in pediatric nephrology centers in Turkey.

BACKGROUND: There is limited data on COVID-19 disease in children with kidney disease. We aimed to investigate the characteristics and prognosis of COVID-19 in pediatric nephrology patients in Turkey. METHODS: This was a national, multicenter, retrospective cohort study based on an online survey evaluating the data between 11th March 2020 and 11th March 2021 as an initial step of a detailed pediatric nephrology COVID-19 registry. RESULTS: Two hundred and three patients (89 girls and 114 boys) were diagnosed with COVID-19. One-third of these patients (36.9%) were between 10-15 years old. Half of the patients were on kidney replacement therapy: kidney transplant (KTx) recipients (n = 56, 27.5%), patients receiving chronic hemodialysis (n = 33, 16.3%) and those on peritoneal dialysis (PD) (n = 18, 8.9%). Fifty-four (26.6%) children were asymptomatic. Eighty-two (40.3%) patients were hospitalized and 23 (28%) needed intensive care unit admission. Fifty-five percent of the patients were not treated, while the remaining was given favipiravir (20.7%), steroid (16.3%), and hydroxychloroquine (11.3%). Acute kidney injury developed in 19.5% of hospitalized patients. Five (2.4%) had MIS-C. Eighty-three percent of the patients were discharged without any apparent sequelae, while 7 (3.4%) died. One hundred and eight health care staff were infected during the study period. DISCUSSION: COVID-19 was most commonly seen in patients who underwent KTx and received HD. The combined immunosuppressive therapy and frequent exposure to the hospital setting may increase these patients' susceptibility. Staff infections before vaccination era were alarming, various precautions should be taken for infection control, particularly optimal vaccination coverage.