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Background Little is known about the long-term social and professional outcomes in adults after pediatric kidney replacement therapy (KRT). In this study, we described social and professional outcomes of adults after kidney failure during childhood and compared these outcomes with the general population. Methods We sent a questionnaire to 143 individuals registered in the Swiss Pediatric Renal Registry (SPRR) with KRT starting before the age of 18 years. In the questionnaire, we assessed social (partner relationship, living situation, having children) and professional (education, employment) outcomes. Logistic regression models adjusted for age at study and sex were used to compare outcomes with a representative sample of the Swiss general population and to identify socio-demographic and clinical characteristics associated with adverse outcomes. Results Our study included 80 patients (response rate 56%) with a mean age of 39 years (range 19-63). Compared to the general population, study participants were more likely to not have a partner (OR = 3.7, 95%CI 2.3-5.9), live alone (OR = 2.5, 95%CI 1.5-4.1), not have children (OR = 6.8, 95%CI 3.3-14.0), and be unemployed (OR = 3.9, 95%CI 1.8-8.6). No differences were found for educational achievement (p = 0.876). Participants on dialysis at time of study were more often unemployed compared to transplanted participants (OR = 5.0, 95%CI 1.2-21.4) and participants with > 1 kidney transplantation more often had a lower education (OR = 3.2, 95%CI 1.0-10.2). Conclusions Adults after pediatric kidney failure are at risk to experience adverse social and professional outcomes. Increased awareness among healthcare professionals and additional psycho-social support could contribute to mitigate those risks. Graphical abstract, Author(s): Guido F. Laube [sup.1] [sup.2] , Marc-Andrea Heinzelmann [sup.2] [sup.3] , Katharina Roser [sup.4] , Claudia E. Kuehni [sup.2] [sup.3] [sup.5] , Luzius Mader [sup.2] [sup.3] [sup.6] Author Affiliations: [...]

Background Little is known about health-related quality of life (HRQoL) in adults after kidney failure during childhood. In this study, we analyzed HRQoL of adults after pediatric kidney failure in Switzerland and investigated socio-demographic and clinical factors associated with HRQoL. Methods In this cohort study, we sent questionnaires to 143 eligible patients registered in the Swiss Pediatric Renal Registry with continuous kidney replacement therapy starting before the age of 18 years. We assessed HRQoL using the Short-Form 36 version 1, compared HRQoL scores between our sample and the Swiss general population, and used linear regression models to examine socio-demographic and clinical factors associated with HRQoL. Results We included 79 patients (response rate 55%) with a mean age of 38.6 years (range 19.4-63.1). Compared to the general population, HRQoL scores were lower for physical functioning (- 12.43, p < 0.001), role physical (- 13.85, p = 0.001), general health (- 14.42, p < 0.001), vitality (- 4.98, p = 0.035), and physical HRQoL (- 6.11, p < 0.001), but we found no difference in mental HRQoL (- 0.13, p = 0.932). The socio-demographic factors-lower education, unemployment, and not being in a relationship-were associated with lower HRQoL. The only clinical factor associated with HRQoL was the type of kidney disease. Patients with acquired kidney diseases had lower mental HRQoL than patients with congenital anomalies of the kidney and urinary tract (- 11.4, p = 0.007) or monogenetic hereditary diseases (- 9.5, p = 0.018). Conclusions Adults after pediatric kidney failure in Switzerland have lower physical, but similar mental HRQoL compared to the general population. Subgroups may require special attention with regard to their HRQoL. Graphical abstract, Author(s): Marc-Andrea Heinzelmann [sup.1] , Claudia E. Kuehni [sup.1] [sup.2] , Katharina Roser [sup.3] , Luzius Mader [sup.4] , Guido F. Laube [sup.5] Author Affiliations: (1) grid.5734.5, 0000 0001 0726 [...]

Aim: To investigate the evolution of clinical symptoms of COVID‐19 in children throughout the pandemic. Methods: In this national prospective surveillance study, symptoms in children hospitalised with COVID‐19 were collected from all paediatric hospitals in Switzerland between March 2020 and March 2023. Data was analysed across four time periods, according to the predominantly circulating SARS‐CoV‐2 variant: T1 (wild‐type), T2 (Alpha), T3 (Delta) and T4 (Omicron), as well as by age group. Results: The study included 1323 children. The proportion of children admitted to an intensive care unit remained stable throughout the pandemic. However, the pattern and frequency of clinical manifestations changed over time. Respiratory symptoms were less prevalent during T1 (wild‐type), fever during T2 (Alpha) and rash during T4 (Omicron). In contrast, fever and neurological symptoms were more prevalent during T4 (Omicron). Newly described symptoms during T4 (Omicron) included conjunctivitis, laryngotracheitis and seizures. Fever was more prevalent among neonates and infants whereas respiratory symptoms were more common among infants. Gastrointestinal symptoms were more frequent among toddlers, while both toddlers and school‐aged children presented with neurological symptoms more often than other age groups. Conclusion: Continuous surveillance is required to detect changes in manifestations and there by be prepared for the optimal management of complications in children with COVID‐19. [ABSTRACT FROM AUTHOR]

Background Infants with a body weight of less than 10 kg are often not considered to be suitable candidates for renal transplantation (RTx). The objective of this study was to evaluate this arbitrary weight threshold for pediatric RTx. Methods We conducted a multicenter, retrospective, match-controlled cohort study on infants weighing less than 10 kg at time of engrafting (low-weight group [LWG], n = 38) compared to a matched control group (n = 76) with a body weight of 10-15 kg, using data from the first 2 years post-transplant derived from the CERTAIN Registry. Results Patient survival was 97 and 100% in the LWG and control groups, respectively (P = 0.33), and death-censored graft survival was 100 and 95% in the LWG and control groups, respectively (P = 0.30). Estimated glomerular filtration rate at 2 years post-transplant was excellent and comparable between the groups (LWG 77.6 ± 34.9 mL/min/1.73 m.sup.2; control 74.8 ± 29.1 mL/min/1.73 m.sup.2; P = 0.68). The overall incidences of surgery-related complications (LWG 11%, control 23%; P = 0.12) and medical outcome measures (LWG 23%, control 36%, P = 0.17) were not significantly different between the groups. The medical outcome measures included transplant-related viral diseases (LWG 10%, control 21%; P = 0.20), acute rejection episodes (LWG 14%, control 29%; P = 0.092), malignancies (LWG 3%, control 0%; P = 0.33) and arterial hypertension (LWG 73%, control 67%; P = 0.57). Conclusions These data suggest that RTx in low-weight children is a feasible option, at least in selected centers with appropriate surgical and medical expertise., Author(s): Marcus Weitz [sup.1] , Guido F. Laube [sup.1] , Maria Schmidt [sup.1] , Kai Krupka [sup.2] , Luisa Murer [sup.3] , Dominik Müller [sup.4] , Bernd Hoppe [sup.5] , [...]

Ureteropelvic junction obstruction (UPJO) is the most common obstructive uropathy and its optimal management remains controversial. However, there is a current trend towards non-surgical management. We aimed to determine the effects of the non-surgical management in children with unilateral UPJO. For a systematic review, we searched MEDLINE, EMBASE, CENTRAL, clinical trials registries, and selected conference proceedings for eligible studies. Any type of study reporting the outcomes renal function, secondary surgical intervention, drainage pattern or hydronephrosis of non-surgical management in children with unilateral UPJO was included. Data from 20 studies were extracted and evaluated by two independent authors. The pooled prevalence was 21% for split renal function deterioration, 27.9% for secondary surgical intervention, 3.2% for progressive hydronephrosis, and 82.2% for improved drainage pattern. Not all patients with surgical intervention regained split renal function from enrolment. Renal imaging methods did not strongly correlate with each other. Many studies had to be excluded because of a lack of detection of an obstruction or mixed populations with bilateral UPJO or other uropathies. The variable definitions of UPJO, different criteria for surgical intervention, incongruity of management protocols, and the imprecise reporting of outcomes were limiting factors in the comparability of the results, leading to heterogeneity in meta-analyses. Although the available evidence cannot recommend or refute the current non-surgical management, the systematic review clarifies aspects of the ongoing controversy by providing realistic estimates for non-surgical management in children with unilateral UPJO. Additionally, it reveals unclear potential risks, particularly for long-term outcomes, which were rarely reported., Author(s): Marcus Weitz [sup.1] , Maria Schmidt [sup.1] , Guido Laube [sup.1] Author Affiliations: (1) Department of Nephrology, University Children's Hospital Zurich, 0000 0001 0726 4330, grid.412341.1, , Steinwiesstrasse 75, [...]

Background In children after haemolytic-uraemic syndrome (HUS), little is known about long-term health-related quality of life (HRQoL) and psychological adjustment as defined by behavioural problems, depressive symptoms and post-traumatic stress symptoms. Methods Sixty-two paediatric patients with a history of HUS were included in this study. Medical data of the acute HUS episode were retrieved retrospectively from hospital records. Data on the clinical course at study investigation were assessed by clinical examination and laboratory evaluation. HRQoL and psychological adjustment data were measured by standardised, parent- and self-reported questionnaires. Results Haemolytic-uraemic syndrome was diagnosed at a mean of 6.5 years before the initiation of the study (standard deviation 2.9, range 0.1-15.7) years. Among the preschool children, parents reported that their child was less lively and energetic (HRQoL emotional dimension), while no increased behavioural problems were reported. In the school-age children, self- and proxy-reported HRQoL was well within or even above the norms, while increased total behavioural problems were found. The school-age children reported no increased depression scores. Also none of the children met the criteria for full or partial HUS-associated posttraumatic stress disorder. Conclusions Healthcare providers should be particularly alert to behavioural problems in school-age children with a history of HUS and to lower HRQoL in preschool children., Author(s): Helene Werner [sup.1] [sup.2] , Kathrin Buder [sup.3] , Markus A. Landolt [sup.1] [sup.2] , Thomas J. Neuhaus [sup.4] , Guido F. Laube [sup.3] , Giuseppina Spartà [sup.3] Author [...]

Background Febrile urinary tract infections (fUTIs) are common after kidney transplantation (KTx); however, prospective data in a multicenter pediatric cohort are lacking. We designed a prospective registry to record data on fUTI before and after pediatric KTx. Methods Ninety-eight children (58 boys and 40 girls) [less than or equal to] 18 years from 14 mid-European centers received a kidney transplant and completed a 2-year follow-up. Results Posttransplant, 38.7 % of patients had at least one fUTI compared with 21.4 % before KTx (p = 0.002). Before KTx, fUTI was more frequent in patients with congenital anomalies of kidneys and urinary tract (CAKUT) vs. patients without (38 % vs. 12 %; p = 0.005). After KTx, fUTI were equally frequent in both groups (48.7 % vs. 32.2 %; p = 0.14). First fUTI posttransplant occurred earlier in boys compared with girls: median range 4 vs. 13.5 years (p = 0.002). Graft function worsened (p < 0.001) during fUTI, but no difference was recorded after 2 years. At least one recurrence of fUTI was encountered in 58 %. Conclusion This prospective study confirms a high incidence of fUTI after pediatric KTx, which is not restricted to patients with CAKUT; fUTIs have a negative impact on graft function during the infectious episode but not on 2-year graft outcome., Author(s): Friederike Weigel[sup.1] , Anja Lemke[sup.2] , Burkhard Tonshoff[sup.3] , Lars Pape[sup.4] , Henry Fehrenbach[sup.5] , Michael Henn[sup.6] , Bernd Hoppe[sup.7] , Therese Jungraithmayr[sup.8] , Martin Konrad[sup.9] , Guido Laube[sup.10] [...]

Background The oculocerebrorenal syndrome of Lowe (OCRL) is a rare X-linked multi-systemic disorder, almost always characterized by the triad of congenital cataract, cognitive and behavioral impairment and a proximal tubulopathy. Methods Twenty-eight novel patients with suspected Lowe syndrome were studied. Results All patients carried OCRL gene defects with mutational hot spots at CpG dinucleotides. Mutations previously unknown in Lowe syndrome were observed in ten of the 28 patients, and carriership was identified in 30.4 % of the mothers investigated. Mapping the exact breakpoints of a complete OCRL gene deletion revealed involvement of several flanking repeat elements. We noted a similar pattern of documented clinically relevant symptoms, and even though the patient cohort comprised relatively young patients, 32 % of these patients already showed advanced chronic kidney disease. Thrombocytopenia was seen in several patients, and hyperosmia and/or hyperacusis were reported recurrently. A p.Asp523Asn mutation in a Polish patient, associated with the typical cerebrorenal spectrum but with late cataract (10 year), was also evident in two milder affected Italian brothers with ocular involvement of similar progression. Conclusions We have identified clinical features in 28 patients with suspected Lowe syndrome that had not been recognized in Lowe syndrome prior to our study. We also provide further evidence that OCRL mutations cause a phenotypic continuum with selective and/or time-dependent organ involvement. At least some of these mutants might exhibit a genotype-phenotype correlation., Author(s): Florian Recker[sup.1] , Marcin Zaniew[sup.2] , Detlef Bockenhauer[sup.3] , Nunzia Miglietti[sup.4] , Arend Bokenkamp[sup.5] , Anna Moczulska[sup.6] , Anna Rogowska-Kalisz[sup.7] , Guido Laube[sup.8] , Valerie Said-Conti[sup.9] , Belde Kasap-Demir[sup.10] [...]

Background To investigate the long-term neurodevelopmental outcome in children after hemolytic uremic syndrome (HUS) and to compare outcome dependent on central nervous system (CNS) involvement during HUS. Methods A single-center retrospective cohort of 47 children was examined at a median age of 10.6 (range 6-16.9) years and a median follow-up of 7.8 (range 0.4-15.3) years after having had HUS. Intellectual performance was assessed with the German version of the Wechsler Intelligence Scale 4th version and neuromotor performance with the Zurich Neuromotor Assessment (ZNA). The occurrence of neurological symptoms during the acute phase of HUS was evaluated retrospectively. Results Mean IQ of the whole study population fell within the normal range (median full scale IQ 104, range 54-127). Neuromotor performance was significantly poorer in the domains 'adaptive fine,' 'gross motor,' 'static balance' (all p < 0.05) and 'associated movements' (p < 0.001); only the 'pure motor' domain was within the normal reference range. Neurological findings occurred in 16/47 patients (34%) during acute HUS. Neurodevelopmental outcome was not significantly different between children with or without CNS involvement. Conclusions Our follow-up of children after HUS showed a favorable cognitive outcome. However, neuromotor outcome was impaired in all study participants. Neurological impairment during acute HUS was not predictive of outcome. Keywords Intellectual * Motor * Neurocognitive outcome * Central nervous system involvement * Hemolytic uremic syndrome, Introduction Hemolytic uremic syndrome (HUS) is a multi-organ and life-threatening disease characterized by hemolytic anemia, thrombocytopenia and acute renal injury. HUS is also one of the most frequent causes of [...]