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.

Evaluation of proximal tubule functions in children with COVID-19: a prospective analytical study.

BACKGROUND: There are limited numbers of studies focusing on renal effects of coronavirus disease 2019 (COVID-19) infection and proximal tubular dysfunction in children with COVID-19 infections. The purpose of this study was to evaluate the functions of the proximal tubule in hospitalized children with confirmed acute COVID-19. METHODS: The children who were hospitalized for confirmed COVID-19 were included in this prospective descriptive analysis. The presence of at least two of the following four abnormalities was used to diagnose proximal tubule injury: abnormal tubular reabsorption of phosphate, normoglycemic glycosuria, hyperuricosuria, and proteinuria. RESULTS: A total of 115 patients were included in the study. About a third of the individuals had elevated blood creatinine levels or proteinuria. In addition, abnormal renal tubular phosphate loss measured by renal tubular phosphate loss was found in 10 (8.7%) patients, as was hyperuricosuria in 28.6%. As a result, total proximal tubular dysfunction was found in 24 (20.9%) patients. CONCLUSIONS: One in every five children with acute COVID-19 infections had proximal tubular dysfunction, according to our data. Although, the rate of proximal tubular dysfunction was lower than in adults, it should be noted. The recovery of proximal tubular function in children with COVID-19 should be followed.

Ultrasonographic evaluation of the children with candiduria for the fungal ball: Is It necessary?

INTRODUCTION: Imaging of the urinary tract by ultrasonography (USG) or computerized tomography scanning is recommended for detecting structural abnormalities, hydronephrosis, abscesses, emphysematous pyelonephritis, or fungus ball formation. Limited studies on the epidemiology and the imaging results of candiduria were present in the children. AIM: This study aimed to evaluate the results of renal ultrasonography imaging in hospitalized pediatric patients with candiduria. STUDY DESIGN: In this descriptive study, we reviewed our ultrasonography findings with hospitalized children and infants who with candiduria. The study included the period between January 2012 and December 2019. Demographic data, the previous medical history, the clinical features of the patients, ultrasonographic findings of the urinary tract system, presence of an indwelling urinary catheter, type of urinary samplings, type of candida species were retrospectively recorded. The study was approved by Institutional Review Board with the registration number 2019/366. RESULTS: During the study period, 220 children with nosocomial candiduria were evaluated. The most common isolated candida species was Candida albicans (68.2%) and followed by C. tropicalis (9.1%). Among all patients, 2 (0.9%) had renal fungal balls associated with C. Albicans. Twenty-five patients (11.4%) had findings including internal echogenicity in the bladder (n = 12), uroepithelial thickening of the kidney (n = 10), and sediments in the renal pelvis (n = 3). DISCUSSION: Candida albicans was the most prominent candida isolated from the patients. The fungal ball is an uncommon infection especially in children and predominantly caused by Candida species. Fungal ball in the kidney was reported as case reports especially in neonates, in immunosupressed patients, and in patients who had undergone surgical procedures. In our study, none of the patients with the fungal ball were in the neonatal period, however, the patients with fungus ball had underlying disease or condition. Our study has several limitations including a retrospective study, and the USG were not performed by a single radiologist. Besides these limitations, our findings are important to give information about the place of USG for diagnosis of the renal fungal ball in children adding valuable information to a topic in which data came from mainly case reports. CONCLUSIONS: Despite the low incidence of fungal balls reported, considering the high consequences of missing a fungal ball and elimination of it, a non-invasive method such as the renal bladder USG is still necessary for detection of fungal ball especially. More prospective studies are required for high risk groups to establish the diagnostic value of renal USG.

Effects of hyperuricemia on renal function in pediatric renal transplant recipients.

OBJECTIVES: Hyperuricemia is common in pediatric renal transplant recipients, and it is associated with poor allograft outcomes. Hyperuricemia occurs early after transplant and is associated with use of diuretics, cyclosporine therapy, a history of hyperuricemia, and decreased glomerular filtration rate. We aimed to investigate causes and effects of hyperuricemia on allograft outcomes in our patients. MATERIALS AND METHODS: There were 81 pediatric transplant patients (41 female and 40 male) included in the study. Demographic characteristics and laboratory parameters were recorded. Risk factors for hyperuricemia and the effects of plasma uric acid levels at 3, 6, 12, and 36 months on allograft outcomes were evaluated. RESULTS: Mean age was 16.9 ± 5.6 years. Mean follow-up after transplant was 3.5 ± 0.47 years. Hyperuricemia was detected in 17.6% patients. A significant negative correlation was observed between 6-month uric acid level and 36-month glomerular filtration rate (r = -0.33, P = .04; r = -0.33, P = .017). A significant positive correlation between 3- and 6-month uric acid levels and 36-month plasma creatinine level was observed (r = -0.44, P = .01; r = -0.51, P = .001). There was no significant correlation between plasma uric acid level and body mass index, plasma lipid levels, use of diuretics, or immunosuppressive treatment (tacrolimus or cyclosporine). CONCLUSIONS: Uric acid levels may have predictive value in the long-term assessment of renal function. Posttransplant hyperuricemia can be used as a long-term prognostic marker of poor renal outcome. Patients with hyperuricemia should be monitored closely for renal function.

Success of eculizumab in the treatment of atypical hemolytic uremic syndrome.

BACKGROUND: Disorders of complement regulation are the most important etiology of atypical hemolytic uremic syndrome (aHUS). Recent studies demonstrate that eculizumab is beneficial in long-term aHUS treatment. We present a series of children with aHUS resistant to/dependent on plasma exchange (PE) who were treated with eculizumab. METHODS: This was a retrospective study in which data were retrieved from the medical files of children who had received PE as treatment for aHUS. The data retrieved included age, sex, presenting symptoms, presence of diarrhea/vomiting, hospitalization duration, laboratory data on admission and follow-up, need for transfusion or dialysis, response to PE, response to eculizumab and outcome. RESULTS: Of the 15 children diagnosed with aHUS in 2011 and 2012 in our departments, ten were resistant to, or dependent on, plasma therapy and treated with eculizumab; these children were enrolled in the study. Three patients had relapses, and seven had a new diagnosis. Nine children had oliguria or anuria, and eight required dialysis. Hypertension was observed in six patients. Neurologic involvement developed in six patients, with the symptoms including seizures, loss of balance, vision loss and severe confusion. Five and five patients were resistant to and dependent on plasma therapy, respectively. Following the start of eculizumab treatment, all patients achieved full recovery of renal function and hematologic parameters. CONCLUSIONS: In our ten pediatric patients with aHUS who did not respond to PE, eculizumab was a lifesaving therapy and improved their quality of life. Early eculizumab use was a rescue therapy for renal function. Our results show that eculizumab is an effective treatment for aHUS. However, more studies are needed on the long-term efficacy and safety of eculizumab in children with aHUS and to determine the optimal duration of treatment.

Experience with continuous venovenous hemodiafiltration in four newborns: A case series and review of the literature.

When conventional methods for treating complicated problems such as acute and chronic renal failure or metabolic diseases fail, the therapy of choice is peritoneal dialysis (PD) in neonatal period. However, in cases that involve technical difficulties, such as bulky lesions in the abdomen or complications from previous abdominal surgeries, it is not always possible to place a peritoneal catheter. In such situations, continuous venovenous hemodiafiltration (CVVHDF) can be effective. This case series presents our experience in 2013 with the administration of CVVHDF to four patients in our neonatal intensive care unit who could not undergo PD for various reasons.

The assessment of time-dependent myocardial changes in infants with perinatal hypoxia.

OBJECTIVE: The aim of the study was to assess myocardial damage in infants due to perinatal hypoxia. METHODS: The findings of 29 infants with perinatal hypoxia and 20 healthy infants were compared. Blood gas analysis, serum lactate, cardiac troponin I (cTnI), troponin T (cTnT), creatine kinase-MB (CK-MB) and B-type natriuretic peptide (BNP) were evaluated. Echocardiography together with tissue Doppler imaging was performed. RESULTS: cTnT, CK-MB and BNP were higher in patients at the first day. There were positive correlations between the left ventricular (LV) myocardial performance index (MPI) and cTnT at first day and also at first month. LV ejection fraction and fractional shortening were lower at first day and at first month in patients. Myocardial systolic (Sm) and diastolic (Em and Am) velocities at all segments were lower at first day, and interventricular septum Sm, LV Sm, LV Em, right ventricular Em and LV Am were still lower at first month in patients. Isovolumic relaxation time at all segments together with LV MPI was higher at first day, ejection time values were lower and MPI values were higher at all segments at first month in patients. CONCLUSIONS: These findings demonstrated that the signs of myocardial damage due to perinatal hypoxia still present at first month.

Hypomelanosis of Ito and Sturge-Weber syndrome without facial nevus: an association or a new syndrome?

Sturge-Weber syndrome without facial nevus is rare. Twenty-four cases were previously reported. Although hypomelanosis of Ito is a relatively common disorder, there was only one previous case in association with Sturge-Weber syndrome. We describe an 11-year-old boy with Sturge-Weber syndrome without facial nevus, coexistent with hypomelanosis of Ito.