Serum indoxyl sulfate concentrations associate with progression of chronic kidney disease in children

The uremic toxins indoxyl sulfate (IS) and p-cresyl sulfate (pCS) accumulate in patients with chronic kidney disease (CKD) as a consequence of altered gut microbiota metabolism and a decline in renal excretion. Despite of solid experimental evidence for nephrotoxic effects, the impact of uremic toxins on the progression of CKD has not been investigated in representative patient cohorts. In this analysis, IS and pCS serum concentrations were measured in 604 pediatric participants (mean eGFR of 27 ± 11 ml/min/1.73m2) at enrolment into the prospective Cardiovascular Comorbidity in Children with CKD study. Associations with progression of CKD were analyzed by Kaplan-Meier analyses and Cox proportional hazard models. During a median follow up time of 2.2 years (IQR 4.3-0.8 years), the composite renal survival endpoint, defined as 50% loss of eGFR, or eGFR <10ml/min/1.73m2 or start of renal replacement therapy, was reached by 360 patients (60%). Median survival time was shorter in patients with IS and pCS levels in the highest versus lowest quartile for both IS (1.5 years, 95%CI [1.1,2.0] versus 6.0 years, 95%CI [5.0,8.4]) and pCS (1.8 years, 95%CI [1.5,2.8] versus 4.4 years, 95%CI [3.4,6.0]). Multivariable Cox regression disclosed a significant association of IS, but not pCS, with renal survival, which was independent of other risk factors including baseline eGFR, proteinuria and blood pressure. In this exploratory analysis we provide the first data showing a significant association of IS, but not pCS serum concentrations with the progression of CKD in children, independent of other known risk factors. In the absence of comorbidities, which interfere with serum levels of uremic toxins, such as diabetes, obesity and metabolic syndrome, these results highlight the important role of uremic toxins and accentuate the unmet need of effective elimination strategies to lower the uremic toxin burden and abate progression of CKD

Parvovirus B19-induced acute bilateral carpal tunnel syndrome in twin girls

We describe 2 cases of 6-year-old twin girls presenting with acute carpal tunnel syndrome (CTS) associated with human parvovirus B19 (HPV-B19) infection, as evidenced by serological data and detection of HPV-B19 DNA in blood with use of polymerase chain reaction (PCR). To our knowledge, this is the first time that HPV-B19 infection has been suggested as the causal agent of simultaneous acute bilateral CTS in twins, thus presenting the possibility that similar immunologic responses can be observed in twins during viral infections

An Unusual Presentationof Herpes Simplex Virus Type 1Infection in a Child

We describe an 11-year-old girl presenting with lichen simplex chronicus (LSC) and acute bilateral carpal tunnel syndrome (CTS) following herpes simplex virus type 1 (HSV-1) infection as evidenced by serological data and by detection of HSV-1 DNA in the blood with the use of PCR. Based on the literature search, this case represents the first childhood case of LSC and acute bilateral CTS following HSV-1 infection. The experience with this patient emphasizes the importance of serological tests and PCR as well as the other laboratory techniques for the accurate diagnosis and management of the disease

Can We Differentiate Pyelonephritis and Cystitis without 99mTc-Dimercaptosuccinic Acid Scan in Children?

Purpose: Urinary tract infection is one of the most common infections in childhood. Because of the long term sequelae, differentiation of pyelonephritis from cystitis is important. The aim of this study is to determine the value of biomarkers such as C-reactive protein and procalcitonin and whether preferred to predict pyelonephritis in children without 99mTc-Dimercaptosuccinic Acid scan. Material and Methods: Fifty children aged 3 months to 16 years with a first urinary tract infection were included in this retrospective observational study. The medians, sensitivity, specificity, and cut-off values of serum C - reactive protein and procalcitonin to predict pyelonephritis were determined. Results: Thirty-two (64%) patients were diagnosed with pyelonephritis and 18 (36%) were diagnosed with cystitis. The cut-off value for C - reactive protein was 34 mg/L to predict pyelonephritis, with 69% sensitivity and 61% specificity. The cut-off value for procalcitonin was 0.23 ng/mL to predict pyehlonephritis, with 69% sensitivity and 66% specificity. In combination, these biomarkers were 63% sensitive and 78% specific to predict pyelonephritis. Conclusion: Using a combination of procalcitonin and C-Reactive Protein is preferred to predict pyelonephritis in children, instead of the 99mTc-Dimercaptosuccinic Acid scan. Because of its disadvantages, the 99mTc-Dimercaptosuccinic Acid scan should be avoided in children

Neurologic involvement in atypical hemolytic uremic syndrome and successful treatment with eculizumab

WOS: 000316571400019PubMed ID: 23389237Atypical hemolytic uremic syndrome (aHUS) is associated with defective regulation of the complement pathway. Neurological involvement is the most common extrarenal complication and represents a major cause of mortality and morbidity. Two girls aged 11 and 6 years, respectively, developed aHUS and were treated immediately with plasma exchange (PE) and fresh frozen plasma infusion (PI). Although initial improvement in renal function was seen in both cases, the first patient showed progressing thrombotic microangiopathy (TMA) despite daily PE, and neurological manifestations (seizures, vision loss, loss of balance, and confusion) developed after 1 month. The second patient developed cerebral TMA (seizures, vision loss, and nystagmus) 6 days after initial presentation and remained unresponsive to PE/PI. Neurological symptoms were similar in both patients, even though they had different complement protein mutations. Treatment with eculizumab achieved complete control of neurological symptoms within 24 h and gradually normalized hematological and renal parameters in both children. Based on our two cases, we conclude that eculizumab is a rapid-acting, effective, and life-saving treatment for pediatric patients with aHUS and severe neurological involvement, which works by inhibiting complement-mediated TMA in the kidney and other organs, such as the brain.Alexion PharmaceuticalsAssistance with writing this manuscript was provided by Bioscript Stirling Ltd, UK, and funded by Alexion Pharmaceuticals. We give special thanks to Dr. Reyhan Diz Kucukkaya in the Department of Internal Medicine, Division of Hematology at Istanbul Bilim University, Istanbul, Turkey

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