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Background: Hypertension is frequent in chronic hemodialyzed patients and usually treated by reducing extracellular fluid. Probing dry weight only based on a clinical evaluation may be hazardous, especially in case of volume independent hypertension. Methods: We performed a 1-year retrospective study in three pediatric centers to define the relation between blood pressure (BP) and hydration status, assessed by whole-body bioimpedance spectroscopy (BIS). We analyzed 463 concomitant measurements of BP, relative overhydration (rel.OH), and plasma sodium (Napl) in 23 children (mean age 13.9 ± 5.1 years). Results: Pre-dialytic under-hydration (rel.OH < −7 %) was present in 5.4 % of the sessions, out of which 24 % showed hypertension. Normohydration (rel.OH −7 - +7 %) was observed in 62.4 % of the sessions, 45.3 % of them revealed hypertension. Moderate OH (rel.OH +7 - +15 %) was present in 21 % of the sessions, 47.4 % of them showed normal BP. In 11.2 % of the sessions, severe overhydration (rel.OH > +15 %) was assessed, however, the majority (73 %) showed normal BP. Patient-specific Napl setpoint could not be described. Mean dialysate sodium concentration was higher than mean Napl. Conclusions: Hypertension is not always related to overhydration. Therefore, BIS should restrict the practice of 'probing dry weight' in hypertensive children. Moreover, sodium dialytic balance needs to be considered to improve BP management. [ABSTRACT FROM AUTHOR]

Background: ABO-incompatible renal transplantation (ABOi-RTx) following preconditioning with immunoadsorption (IA) and rituximab is a promising approach to facilitate living-related RTx. However, clinical experience is limited in pediatric patients. Methods: Three patients underwent living-related ABOi-RTx in our center. Preoperative IA was performed six, ten and 11 times in patient one, two and three, respectively, to achieve isoagglutinin titers of ≤1:8 on the day of transplantation; rituximab was administered once. The immunosuppressive regimen further comprised tacrolimus, mycophenolate, methylprednisolone and basiliximab; immunoglobulin G (IgG) was infused on the day of ABOi-RTx. Results: All three patients achieved normal renal function within 2-6 days post-RTx. Major postoperative bleeding occurred in two patients, with one requiring repeated blood transfusions and the other a surgical revision 4 h after RTx, despite local citrate anticoagulation use during the preoperative IA procedures in the latter patient. A pyelonephritis-associated increase of the isoagglutinin IgG/IgM titers to 1:64/1:128 led to a biopsy-proven acute humoral rejection in the third patient, which was treated successfully with plasma exchange and methylprednisolone pulses. The estimated glomerular filtration rate at 18, 8 and 23 months post-RTx was 96, 52 and 74 ml/min/1.73 m, respectively. Conclusions: ABOi-RTx can successfully be performed in pediatric patients after preconditioning with quadruple immunosuppression, rituximab and IA. Caution is required regarding bleeding complications, which are most likely due to the unspecific binding of coagulation factors during repeated IA. [ABSTRACT FROM AUTHOR]

Estimation of peritoneal mass transport by three-pore model in children. Background. Computerized modeling is increasingly used to optimize the efficacy of peritoneal dialysis (PD). The Personal Dialysis Capacity (PDC) test is a new tool to model PD efficacy based on the three-pore model of peritoneal mass transport. We sought to evaluate (i ) whether the PDC test is applicable to children on chronic PD, and (ii ) whether the physiological mass transport coefficients defined in the three pore model are dependent on age or body size in childhood. Methods. A validation study was performed in 32 pediatric chronic PD patients. Twenty tests were performed using a standard CAPD regimen, and 22 tests using a simplified automated PD (APD) protocol. Test accuracy and precision were evaluated by comparison of predicted with measured 24-hour dialysate clearances of urea, creatinine, β2 -microglobulin and albumin and ultrafiltration rates. Long-term reproducibility was assessed in 16 patients by repeated clearance studies after a median time interval of 10 weeks. Results. While daily clearances of urea and creatinine were predicted with good precision and accuracy with both test protocols (concordance correlation coefficients 0.90 to 0.98, mean difference predicted-calculated -0.6 to +0.6 ml/min/1.73 m2 ), ultrafiltration rates were predicted more closely by the APD (r = 0.97) than by the CAPD test (0.80). Middle and large molecule clearances were predicted less precisely in both test settings (r = 0.48 to 0.83). Re-test reproducibility was slightly lower than the predictive precision observed in the original test (r = 0.80 to 0.91). The calculated total peritoneal pore area increased in absolute terms, decreased with body size when standardized to weight, and was independent of body size when normalized to body surface area. The body size-normalized fluid reabsorption rate was slightly increased in young infants compared to older children or... [ABSTRACT FROM AUTHOR]

In the European Union (EU), rare diseases (RDs) are diseases that affect no more than 5 in 10,000 people. Due to their rarity, clinical expertise and quality-assured care structures are scarce, and research is more difficult compared to other diseases. However, these problems can be overcome by means of national and transnational RD care networks. Data and expertise are pooled in these networks.In the EU, the European Reference Networks (ERNs) for Rare and Complex Diseases cooperate across borders. Important services provided by ERNs using health data include diagnostic coding of RDs, conducting virtual cross-border case conferences, and establishing European registries that are used to measure and improve the quality of care. In ERNs, local data generation and documentation combine with network-wide data infrastructures. This paper describes the data-based services in and for RD healthcare networks: (1) diagnostic coding, (2) cross-border case conferences, and (3) ERN registries for RD patient care. The final section discusses the integration of the networks into national healthcare systems.In order to achieve the best possible benefit for SE patients, ERN activities and structures need to be better integrated into national healthcare systems. In Germany, the Medical Informatics Initiative and the German Reference Networks play a central role in this regard., (© 2022. The Author(s).)

Background: Twenty-four-hour ambulatory blood pressure monitoring (ABPM) is an essential tool in the diagnosis and therapeutic monitoring of arterial hypertension in children. The statistical use of pediatric ABPM reference values has been compromised by the non-Gaussian distribution of 24-h blood pressure (BP) in children., Objective: To develop distribution-adjusted pediatric ABPM reference tables., Methods: From cross-sectional ABPM data obtained in 949 healthy children and adolescents aged 5-20 years, a set of reference tables was developed for 24-h, daytime and night-time mean values of systolic, diastolic, mean arterial BP and heart rate, utilizing the LMS method to account for the variably skewed distribution of ABPM data. Age- and gender-specific estimates of the distribution median (M), coefficient of variation (S) and degree of skewness (L) were obtained by a maximum-likelihood curve-fitting technique. The estimates of, and can be used to normalize ABPM data to gender and age or height., Results: Re-application of the established, and values in the reference population confirmed appropriate normalization of ABPM values. Height standard deviation scores (SDS), body mass index (BMI) SDS and heart rate SDS were independent positive predictors of 24-h systolic BP SDS. Diastolic 24-h mean BP SDS showed a weak correlation with BMI SDS only., Conclusions: The use of LMS reference tables permits calculation of appropriate SDS values for ABPM in children. Whereas systolic 24-h BP is independently correlated with age, relative height and obesity, diastolic values are almost independent of age and relative height, and weakly associated with relative obesity.