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.

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.).

Comparison of parathyroid scintigraphy findings in pediatric and adult patients with secondary hyperparathyroidism.

OBJECTIVES: Secondary hyperparathyroidism (sHPT) is a compensatory complication of chronic kidney disease. The aim of this study was to compare PS findings in pediatric and adult patients with sHPT. METHODS: This study included 50 pediatric and 50 adult patients with sHPT. Parathyroid scintigraphy was performed with Tc-99m sestamibi. After radiopharmaceutical injection, early-phase (15 min) and late-phase (60-90 min) images were acquired. Planar images were interpreted visually for the presence / number of active foci compatible with a parathyroid lesion, the presence and degree of uptake in skeletal structures, and the degree of thyroid sestamibi uptake. Parathyroid surgery was performed in 21 pediatric and 28 adult patients. RESULTS: Serum PTH and ALP values were significantly higher in pediatric than in adult patients ( P < 0.05 for each). In operated patients, on a lesion-based analysis, the sensitivity of PS in pediatric and adult patients were 40% and 71%, respectively. A nonlocalizing scan was observed in 24% of pediatric patients. Pediatric patients had a higher incidence of reduced thyroid sestamibi uptake (42% versus 2%). Skeletal sestamibi uptake was detected in 40% of pediatric and 30% of adult patients and the degree of uptake was higher in pediatric patients. CONCLUSIONS: The results revealed more significant changes in the biochemical profile of pediatric compared with adult patients with sHPT. The sensitivity of PS was lower, and the likelihood of a nonlocalizing scan was higher in pediatric patients. The results may also suggest more severe skeletal findings in pediatric patients. Reduced thyroid sestamibi uptake in children needs further evaluation.

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.

The Clinical and Mutational Spectrum of 69 Turkish Children with Autosomal Recessive or Autosomal Dominant Polycystic Kidney Disease: A Multicenter Retrospective Cohort Study.

INTRODUCTION: Autosomal recessive polycystic kidney disease (ARPKD) is associated with pathogenic variants in the PKHD1 gene. Autosomal dominant polycystic kidney disease (ADPKD) is mainly associated with pathogenic variants in PKD1 or PKD2. The present study aimed to identify the clinical and genetic features of Turkish pediatric ARPKD and ADPKD patients. METHODS: This multicenter, retrospective cohort study included 21 genetically confirmed ARPKD and 48 genetically confirmed ADPKD patients from 7 pediatric nephrology centers. Demographic features, clinical, and laboratory findings at presentation and during 12-month intervals were recorded. RESULTS: The median age of the ARPKD patients at diagnosis was lower than the median age of ADPKD patients (10.5 months [range: 0-15 years] vs. 5.2 years [range: 0.1-16 years], respectively, [p = 0.014]). At the time of diagnosis, the median eGFR in the ARPKD patients was lower compared to that of ADPKD patients (81.6 [IQR: 28.7-110.5] mL/min/1.73 m2 and 118 [IQR: 91.2-139.8] mL/min/1.73 m2, respectively, [p = 0.0001]). In total, 11 (52.4%) ARPKD patients had malnutrition; 7 (33.3%) patients had growth retardation at presentation; and 4 (19%) patients had both malnutrition and growth retardation. At diagnosis, 8 (16.7%) of the ADPKD patients had malnutrition, and 5 (10.4%) patients had growth retardation. The malnutrition, growth retardation, and hypertension rates at diagnosis were higher in the ARPKD patients than the ADPKD patients (p = 0.002, p = 0.02, and p = 0.0001, respectively). ARPKD patients with malnutrition and growth retardation had worse renal survival compared to the patients without (p = 0.03 and p = 0.01). Similarly, ADPKD patients with malnutrition had worse renal survival compared to the patients without (p = 0.002). ARPKD patients with truncating variants had poorer 3- and 6-year renal outcome than those carrying non-truncating variants (p = 0.017). CONCLUSION: Based on renal survival analysis, type of genetic variant, growth retardation, and/or malnutrition at presentation were observed to be factors associated with progression to chronic kidney disease (CKD). Differentiation of ARPKD and ADPKD, and identification of the predictors of the development of CKD are vital for optimal management of patients with ARPKD or ADPKD.

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.

Relation of Anxiety, Depression, and Behavioral Problems With Time Allocated to Television, Computer, and Smartphone in Children Receiving Renal Replacement Therapy.

OBJECTIVES: Children and adolescents with chronic diseases have more screen exposure time compared with their healthy peers. In this study, we investigated screen exposure time of children who received renal replacement therapy, which included kidney transplant and dialysis treatment, versus a healthy control group. MATERIALS AND METHODS: Our study included 55 children and adolescents between the ages of 8 and 18 years. Although 28 participants did not have any chronic disease, 27 had chronic diseases and received renal replacement therapy. Among these patients, 17 had kidney transplant and 10 were receiving dialysis. A sociodemographic information form and the Conners Short-Form Parent Rating Scale were given to parents. Pediatric and adolescent patients completed the Children's Depression Inventory and Spielberger State-Trait Anxiety Scale-2. We analyzed differences between the groups with and without renal replacement therapy and examined relations between continuous variables. RESULTS: Duration of television screen time was significantly higher in children and adolescents receiving renal replacement therapy. Patients in the renal replacement therapy group showed a positive correlation between the Conners Short-Form Parent Rating Scale anxiety subscores and duration of smartphone use. In the kidney transplant recipient group, smartphone and computer durations were positively correlated and television duration was negatively correlated with the Conners Short-Form Parent Rating Scale behavioral problems subscores. CONCLUSIONS: Children on renal replacement therapy may be at risk in terms of excessive television exposure. Children who are on dialysis and have had a kidney transplant may be more prone to the negative effects of screen exposure than healthy peers who do not have chronic illnesses. These children and adolescents should be closely monitored to avoid the negative effects of excessive screen exposure.

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.

Evaluation of acute kidney injury after surgery for congenital heart disease in neonates: a tertiary hospital experience.

PURPOSE OF THE ARTICLE: Acute kidney injury (AKI) after cardiac surgery in children with congenital heart disease (CHD) is a serious complication closely associated with high morbidity and mortality. Despite numerous studies on AKI in children, most studies have excluded neonates. We sought to characterize AKI associated with cardiac surgery in neonates, determine its incidence, perioperative and postoperative risk factors, and short-term results. MATERIALS AND METHODS: This retrospective study included 177 neonates who were operated on for CHD in our hospital between January 2015 and December 2019. Data of the patients were analyzed according to nKDIGO (neonatal Kidney Disease Improving Global Outcomes) and nRIFLE (neonatal Risk, Injury, Failure, Loss of function, End-stage kidney disease) criteria for evaluating AKI retrospectively. Data of groups with and without AKI were analyzed. RESULTS: The average age of 177 neonates were 8.2 ± 6.1 (1-28) days. Twenty-two (12.4%) neonates had CS-AKI defined according to nKDIGO criteria. Four (2.3%) neonates reached nKDIGO stage I, 1 (0.6%) reached stage II, 17 (9.6%) reached stage III. Thirty-eight (21.5%) neonates had CS-AKI defined according to nRIFLE criteria. Twenty-four (13.6%) neonates reached nRIFLE stage risk(R), 6 (3.4%) reached stage injury(I), 8 (4.5%) reached stage failure (F). The incidence of cardiac surgery-associated acute kidney injury (CS-AKI) in neonates was 12.5% and 21.5% for nKDIGO and nRIFLE, respectively. The percentage difference between nKDIGO and nRIFLE for AKI assessment was due to the criteria for nRIFLE stage risk(R) urine output < 1.5 mL/kg/h for 24 h. In both classifications, the duration of cardiopulmonary bypass, operation, inotropic treatment, and mechanical ventilation, length of intensive care unit (ICU), and hospital stay were significantly higher in the AKI group than those without AKI group (p˂.05). The mortality rate in the groups with AKI was found to be significantly higher (p˂.05) than in the groups without AKI. In Kappa analysis, when two classifications were compared according to AKI stages, a significant agreement was found between nKDIGO and nRIFLE classifications (p˂.05) (Kappa: 0.299). CONCLUSION: AKI and mortality rates were similar between groups according to the nKDIGO and nRIFLE criteria. For early prediction of AKI and adverse outcomes, diagnostic reference intervals might be specified in more detail in neonates undergoing cardiac surgery for CHD.

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.