Search

Background Post-transplantation immunosuppressive therapy reduces the risk of graft rejection but raises the risk of infection and malignancy. A biomarker of the level of immunosuppression can be helpful in monitoring immunosuppressive therapy. Inverse correlation between Torque teno virus (TTV) from the Anelloviridae (AV) family load and immune competence was described in previous studies. The aim of this study was to analyze the association between AV family viruses' kinetics and the risk for graft rejection in the first year after kidney transplantation in children. Methods The titers of three genera (TTV, TTMDV, and TTMV) from the AV family were monitored by real-time PCR in consecutive samples from children before and after kidney transplantation. Results Twenty-one children who underwent kidney transplantation were enrolled. Five out of 21 patients experienced acute graft rejection within a year from transplantation. We found that in patients who experienced graft rejection, the median titers of TTV and total AV titers at 5-6 months post-transplantation were lower than in those who did not. Using a threshold determined by ROC analysis, significant differences in TTV and total AV load were found between patients who had or did not have graft rejection (p = 0.002 and 0.004, respectively). No association was found between the dominance of any AV genus titer and the likelihood of rejection. Conclusion This pilot study suggests that children after kidney transplantation with low TTV and total AV titers 5-6 months post-transplantation are at increased risk for graft rejection within a year after transplantation. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information, Author(s): Yifat Eldar-Yedidia [sup.1] , Efrat Ben-Shalom [sup.2] [sup.3] , Miriam Hillel [sup.1] , Ruth Belostotsky [sup.2] , Orli Megged [sup.3] , Yossi Freier-Dror [sup.4] , Yaacov Frishberg [sup.2] [sup.3] [...]

Background Systemic oxalosis is a severe complication seen in primary hyperoxaluria type I patients with kidney failure. Deposition of insoluble calcium oxalate crystals in multiple organs leads to significant morbidity and mortality. Methods We describe a retrospective cohort of 11 patients with systemic oxalosis treated at our dialysis unit from 1982 to 1998 (group 1) and 2007-2019 (group 2). Clinical and demographic data were collected from medical records. Imaging studies were only available for patients in group 2 (n = 5). Results Median age at dialysis initiation was 6.1 months (IQR 4-21.6), 64% were male. Dialysis modality was mostly peritoneal dialysis in group 1 and daily hemodialysis in group 2. Bone disease was the first manifestation of systemic oxalosis, starting with the appearance of sclerotic bands (mean 166 days, range 1-235), followed by pathological fractures in long bones (mean 200.4 days, range 173-235 days). Advanced disease was characterized by vertebral fractures with resulting kyphosis, worsening splenomegaly, and adynamic bone disease. Two patients developed pulmonary hypertension, 4 and 8 months prior to their death. Four of 11 patients developed hypothyroidism 0-60 months after dialysis initiation. Only one patient survived after a successful liver-kidney transplantation. Four patients died after liver or liver-kidney transplantation. Conclusions This is the first comprehensive description of the natural history of pediatric systemic oxalosis. We hope that our findings will provide basis for a quantitative severity score in future, larger studies. Graphical abstract, Author(s): Efrat Ben-Shalom [sup.1] , Ruth Cytter-Kuint [sup.2] , Choni Rinat [sup.1] [sup.3] , Rachel Becker-Cohen [sup.1] [sup.3] , Shimrit Tzvi-Behr [sup.1] , Jenny Goichberg [sup.1] , Vardit Peles [sup.1] [...]

Background Beta-2 microglobulin ([beta]2mG) is a low-molecular-weight protein that is almost exclusively eliminated through the kidneys. It is freely filtered in the glomeruli and almost completely reabsorbed and degraded in the proximal tubules. Normal urinary [beta]2mG levels are very low (between 0.04 and 0.22 mg/L). No reference values are known in infants and young children. Methods Urinary [beta]2mG levels were measured in 103 healthy term neonates during the first week of life by nephelometric technology. Results The average level of urinary [beta]2mG was 0.65 mg/L (95% confidence interval between 0 and 10.8 mg/L). There was a minor difference between male and female neonates but it did not reach statistical significance. There was no effect of the gestational week, birth weight, or weight loss in the first week of life, on urinary [beta]2mG levels. Conclusions First-week urinary [beta]2mG levels in healthy term infants were higher than adult levels. Incomplete maturation of kidney tubules in neonates could be a possible explanation. These can now be used in clinical practice and further studies that assess the degree of proximal tubular function in health and disease., Author(s): Shimrit Tzvi-Behr [sup.1] , Heftziba Ivgi [sup.2] , Yaacov Frishberg [sup.1] , Efrat Ben Shalom [sup.1] Author Affiliations: (1) grid.415593.f, 0000 0004 0470 7791, Division of Pediatric Nephrology, Shaare [...]

Background Infections are an important cause of morbidity and mortality in solid organ transplant recipients. Neutrophils play a crucial role in the initial host defense against bacterial pathogens. Neutropenia is not uncommon after renal transplantation in adults; however, there are scarce published data in children. We conducted a historical cohort study to evaluate the incidence, clinical course, and management of severe neutropenia after renal transplantation in children. Methods In a single-center study, we collected clinical and laboratory data on all children ( Results Of the 72 patients studied, 46 (64 %) had at least one episode of neutropenia [absolute neutrophil count (ANC) Summary Post-transplant neutropenia in children is common, and mostly occurs in the first few months. Its etiology is probably primarily a result of the combination of immunosuppressive agents and prophylactic treatment of infections in the early post-transplant period. Conclusions Decreasing immunosuppressive or antimicrobial medications carries the risk of acute rejection or infection. Off-label treatment with G-CSF may present a safe and effective alternative., Author(s): Rachel Becker-Cohen[sup.1] , Efrat Ben-Shalom[sup.1] , Choni Rinat[sup.1] , Sofia Feinstein[sup.1] , Michael Geylis[sup.1] , Yaacov Frishberg[sup.1] Author Affiliations: (1) Division of Pediatric Nephrology, Shaare Zedek Medical Center, Jerusalem, [...]

Primary hyperoxalurias (PH) comprise a group of three distinct metabolic diseases caused by derangement of glyoxylate metabolism in the liver. Recent years have seen advances in several aspects of PH research. This paper reviews current knowledge of the genetic and biochemical basis of PH, the specific epidemiology and clinical presentation of each type, and therapeutic approaches in different disease stages. Potential future specific therapies are discussed., Author(s): Efrat Ben-Shalom[sup.1] , Yaacov Frishberg[sup.1] [sup.2] Author Affiliations: (1) Division of Pediatric Nephrology, Shaare Zedek Medical Center, P.O.Box 3235, Jerusalem, Israel (2) The Hebrew University School of Medicine, Jerusalem, [...]

Background Central venous catheters are frequently used as access for hemodialysis (HD) in children. One of the known complications is central venous stenosis. Although this complication is not rare, it is often asymptomatic and therefore unacknowledged. Superior vena cava (SVC) stenosis is obviously suspected in the presence of upper body edema, but several other signs and symptoms are often unrecognized as being part of this syndrome. Case-diagnosis/treatment We describe four patients with various manifestations of central venous stenosis and SVC syndrome. These sometimes life- or organ-threatening conditions include obstructive sleep apnea, unresolving stridor, increased intracranial pressure, increased intraocular pressure, rightsided pleural effusion, protein-losing enteropathy and lymphadenopathy. The temporal relationship of these complications associated with the use of central venous catheters and documentation of venous stenosis, together with their resolution after alleviation of high venous pressure, points to a causal role. We suggest pathophysiological mechanisms for the formation of each of these complications. Conclusions In patients with occlusion of the SVC, various unexpected clinical entities can be caused by high central venous pressure. As often the etiology is not obvious, a high index of suspicion is needed as in some cases prompt alleviation of the high pressure is mandatory. Keywords Superior vena cava syndrome * Obstructive sleep apnea * Glaucoma * Pleural effusion * Protein-losing enteropathy * Increased intracranial pressure, Introduction Central venous catheter (CVC) is the most frequently used access device for pediatric patients on chronic hemodialysis (HD) [1]. Although peritoneal dialysis is often the preferred mode of treatment [...]

A heterozygosity mapping strategy was developed to determine the mutations in an uncharacterized gene, DHDPSL, on chromosome 10 which cause a third type of primary hyperoxaluria PH (PH III). The developed approach facilitated us to determine an allelic fragment shared by all patients of Ashkenazi Jewish descent and bearing a 3 bp deletion in DHDPSL and revealed the possible contribution of DHDPSL as the gene encoding 4-hydroxy-2-oxoglutarate aldolase which catalyzes the final step in the metabolic pathway of hydroxypyroline.

Aim: Blood culture contamination (BCC) can cause unnecessary hospitalisations and inappropriate use of antibiotics. The aim of this study was to find risk factors associated with BCCs in children and to compare contamination rates between open and closed blood culture collection systems. Methods: Data were prospectively collected regarding blood cultures obtained in the paediatric emergency department from February 26, 2020, to September 30, 2020, based on the method of drawing blood reported by the obtaining physician. A comparison between contaminated and non‐contaminated blood cultures was performed. We also compared the composition of the contaminations in the study period to the same period in 2019. Results: A total of 512 blood cultures were included, 33 (6.4%) of which were contaminated. The only parameter that was associated with an increased rate of contamination by 2.34 fold (95% CI 1.1–4.99, P = 0.028) was obtaining blood through an 'open' system, using a syringe connected to a needle in order to draw blood from an open ended needle. The proportion of contaminations originating from oral flora decreased in the study period by 44.7% as compared to the same period in the previous year (13% vs. 23.5%, P = 0.056). Conclusions: 'Open system' method, which is commonly used in paediatric emergency departments for blood culture obtainment, was associated with an increase in BCC. Adherence to blood cultures obtainment guidelines, even at the price of two different blood tests, is important in order to reduce BCC rates in children. [ABSTRACT FROM AUTHOR]