Search

Background: The International Pediatric Nephrology Association (IPNA) Global Kidney Replacement Therapy (KRT) Registry was established to evaluate the incidence and outcomes of kidney replacement therapy (dialysis and transplantation) provided to children worldwide. Analysis of registry data for separate regions is feasible. Methods: Three centers located in Shanghai, Guangzhou, and Zhengzhou, which have the greatest number of pediatric kidney transplantation cases in China, participated in this analysis of transplant data. Data were registered by each center for patients under the age of 19 years who received a single-organ kidney transplant for the first time between 2011 and 2018. Results: In total, 415 patients (59.8% male) aged 1.4–18.7 (median 12.1) years were followed for 0.3–97.1 (median 27.7) months. The number of kidney transplants increased from a total of 129 during 2011–2014 to 286 cases during 2015–2018. 85.8% of patients received the transplanted kidney from a pediatric (age < 19 years) donor, and deceased donors accounted for 94% of all donors. 8.0% of grafts were lost. One and 5-year patient survival rates were 97.6% and 95.5%, respectively. The major cause of death was infection (7/14). Similar graft and patient survival rates were observed for organs from pediatric and adult donors in 6–11 and 12–18 year recipient age groups, whereas recipients < 6 years showed inferior patient and graft survival. Conclusions: Pediatric kidney transplantation shows favorable short-term and medium-term outcomes in China. Our experience supports use of pediatric donors in pediatric kidney transplantation, but attention directed to the outcome of recipients aged under 6 is necessary. [ABSTRACT FROM AUTHOR]

Coronavirus disease 2019 (COVID-19) has rapidly spread not only in China but throughout the world. Children with kidney failure (chronic kidney disease (CKD) stage 5) are at significant risk for COVID-19. In turn, a set of recommendations for the prevention and control of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and COVID-19 in pediatric hemodialysis (HD) centers and in home peritoneal dialysis (PD) settings have been proposed. The recommendations are based on the epidemiological features of the SARS-CoV-2 virus and COVID-19 disease, susceptibility factors, and preventive and control strategies. These recommendations will be updated as new information regarding SARS-CoV-2 and COVID-19 becomes available. [ABSTRACT FROM AUTHOR]

Nephrogenesis requires a fine balance of many factors that can be disturbed by intrauterine growth restriction (IUGR), leading to a low nephron endowment. The aim of this study was to test the hypothesis that IUGR affects expression of key proteins that regulate nephrogenesis, by a comparative proteomic approach. IUGR was induced in Sprague–Dawley (SD) rats by isocaloric protein restriction in pregnant dams. A series of methods, including two-dimensional gel electrophoresis (2-DE), silver staining, mass spectrometry and database searching was used. After silver staining, 2-DE image analysis detected an average 730 ± 58 spots in the IUGR group and 711 ± 73 spots in the control group. The average matched rate was 86% and 81%, respectively. The differential proteomic expression analysis found that 11 protein spots were expressed only in the IUGR group and one in the control group. Seven protein spots were up-regulated more than fivefold and two were down-regulated more than fivefold in the IUGR group compared with those in control group. These 21 protein spots were preliminarily identified and were structural molecules, including vimentin, perlecan, gamma-actin and cytokeratin 10, transcription regulators, transporter proteins, enzymes, and so on. These proteins were involved primarily in energy metabolism, oxidation and reduction, signal transduction, cell proliferation and apoptosis. Data from this study may provide, at least partly, evidence that abnormality of metabolism, imbalance of redox and apoptosis, and disorder of cellular signal and cell proliferation may be the major mechanisms responsible for abnormal nephrogenesis in IUGR. [ABSTRACT FROM AUTHOR]

Background : Most of the available epidemiological data on peritonitis have been derived from developed countries. Limited data from China have been reported. Methods: An 18-year (2001–2018) peritoneal dialysis (PD) program at the Children's Hospital of Fudan University was described, and data on peritonitis were retrospectively analyzed. Results: Since 2001, a program with a comprehensive PD care bundle has been developed, and 283 patients (53.7% male, median age 9.3 years) were enrolled between 2001 and 2018. Among these patients, 117 peritonitis episodes occurred in 68 (24.0%) patients over 4896 patient-months. The peritonitis rate decreased 20-fold from 2.2 episodes per patient-year in 2003 to 0.11 episodes in 2018. The culture-negative rate decreased from 68.7% during 2001–2006 to 18.5% during 2013–2018, and the proportion of gram-negative and fungal infections increased significantly from 6.6 to 33.8% and 0 to 9.2%, respectively (p < 0.001). Short stature as height ≤ − 2 SD (OR 2.35, 95% CI 1.30–4.24, p = 0.005) and PD duration ≥ 1 year (OR 3.38, 95% CI 1.76–6.49, p < 0.001) were independently associated with a higher risk of developing peritonitis. Of the 117 peritonitis episodes, 9.4% required permanent removal of the catheter, among which half were fungal infections. Patients with peritonitis had a higher risk for PD technique failure (p = 0.006), but there was no difference in estimated patient survival rates and no patient death due to peritonitis. Conclusions: With the successful development of the PD program and care bundles per the International Society of Pediatric Dialysis (ISPD) guidelines, peritonitis rates have been tremendously reduced in the most active pediatric PD center in China. Growth deficits and a long PD duration were risk factors for developing peritonitis, requiring further close monitoring for a better outcome. A higher resolution version of the Graphical abstract is available as Supplementary information. [ABSTRACT FROM AUTHOR]

Background: Genetic kidney disease is well established as an important cause of pediatric kidney failure, and genetic testing might increase diagnostic accuracy, but evidence is limited. This study was conducted to determine the diagnostic yield and clinical impact of genetic testing for children with kidney failure. Methods: Patients who were diagnosed with kidney failure before 19 years of age at Children's Hospital of Fudan University from 2009 to 2018 and received next-generation sequencing (NGS) were enrolled. The results for likely pathogenic variants in genes known to cause chronic kidney disease (CKD) were analyzed. Results: A molecular diagnosis was identified in 39.9% (75/188) of children with kidney failure. Specific subtype of clinical category was discerned in 54 (72.0%) patients, kidney disease was reclassified in 7 (9.3%) patients, the unknown etiology of 5 (6.7%) patients was molecularly diagnosed, and the clinical diagnoses of the other 9 (12.0%) patients were confirmed. In addition, genetic diagnosis was considered to have contributed to clinical management, including negating the need for kidney biopsy (26/75, 34.7%), avoiding immunosuppressive therapy (24/75, 32.0%), changing surveillance (48/75, 64.0%), guiding specific treatment (21/75, 28.0%), and guiding peri-transplant management and options for kidney transplantation (12/75, 16.0%). Furthermore, cascade testing was subsequently offered to 34.7% (26/75) of families. Conclusions: Genetic testing identified a molecular diagnosis in nearly 40% of children with kidney failure. Our results confirm that in children with kidney failure, genetic testing can not only establish a specific molecular diagnosis, but has a significant impact on clinical management. [ABSTRACT FROM AUTHOR]

Children with chronic kidney disease (CKD) are at risk for 'cachexia' or 'protein-energy wasting' (PEW). These terms describe a pathophysiologic process resulting in the loss of muscle, with or without loss of fat, and involving maladaptive responses, including anorexia and elevated metabolic rate. PEW has been defined specifically in relation to CKD. We review the diagnostic criteria for cachexia and PEW in CKD and consider the limitations and applicability of these criteria to children with CKD. In addition, we present an overview of the manifestations and mechanisms of cachexia and PEW. A host of pathogenetic factors are considered, including systemic inflammation, endocrine perturbations, and abnormal neuropeptide signaling, as well as poor nutritional intake. Mortality risk, which is 100- to 200-fold higher in patients with end-stage renal disease than in the general population, is strongly correlated with the components of cachexia/PEW. Further research into the causes and consequences of wasting and growth retardation is needed in order to improve the survival and quality of life for children with CKD. [ABSTRACT FROM AUTHOR]