Infants with congenital nephrotic syndrome have comparable outcomes to infants with other renal diseases

BackgroundChildren with congenital nephrotic syndrome (CNS) commonly develop end stage renal failure in infancy and require dialysis, but little is known about the complications and outcomes of dialysis in these children.MethodsWe conducted a retrospective case note review across members of the European Society for Pediatric Nephrology Dialysis Working Group to evaluate dialysis management, complications of dialysis, and outcomes in children with CNS.ResultsEighty children (50% male) with CNS were identified form 17 centers over a 6-year period. Chronic dialysis was started in 44 (55%) children at a median age of 8 (interquartile range 4-14) months. Of these, 17 (39%) were on dialysis by the age of 6months, 30 (68%) by 1year, and 40 (91%) by 2years. Peritoneal dialysis (PD) was the modality of choice in 93%, but 34% switched to hemodialysis (HD), largely due to catheter malfunction (n=5) or peritonitis (n=4). The peritonitis rate was 0.77 per patient-year. Weight and height SDS remained static after 6months on dialysis. In the overall cohort, at final follow-up, 29 children were transplanted, 18 were still on dialysis (15 PD, 3 HD), 19 were in pre-dialysis chronic kidney disease (CKD), and there were 14 deaths (8 on dialysis). Median time on chronic dialysis until transplantation was 9 (6-18) months, and the median age at transplantation was 22 (14-28) months.ConclusionsInfants with CNS on dialysis have a comparable mortality, peritonitis rate, growth, and time to transplantation as infants with other primary renal diseases reported in international registry data.Peer reviewe

Rituximab-associated hypogammaglobulinemia in children with idiopathic nephrotic syndrome: results of an ESPN survey.

BackgroundThere is paucity of information on rituximab-associated hypogammaglobulinemia (HGG) and its potential infectious consequences in children treated for idiopathic nephrotic syndrome (INS).MethodsA survey was distributed by the European Society Pediatric Nephrology to its members. It addressed the screening and management practices of pediatric nephrology units for recognizing and treating RTX-associated HGG and its morbidity and mortality. Eighty-four centers which had treated an overall 1328 INS children with RTX responded.ResultsThe majority of centers administered several courses of RTX and continued concomitant immunosuppressive therapy. Sixty-five percent of centers routinely screened children for HGG prior to RTX infusion, 59% during, and 52% following RTX treatment. Forty-seven percent had observed HGG prior to RTX administration, 61% during and 47% >9 months following treatment in 121, 210, and 128 subjects respectively. Thirty-three severe infections were reported among the cohort of 1328 RTX-treated subjects, of whom 3 children died. HGG had been recognized in 30/33 (80%) of them.ConclusionsHGG in steroid-dependent/frequently relapsing nephrotic syndrome (SDNS/FRNS) children is probably multifactorial and can be observed prior to RTX administration in children with SDNS/FRNS. Persistent HGG lasting >9 months from RTX infusion is not uncommon and may increase the risk of severe infections in this cohort. We advocate for the obligatory screening for HGG in children with SDNS/FRNS prior to, during, and following RTX treatment. Further research is necessary to identify risk factors for developing both HGG and severe infections before recommendations are made for its optimal management. A higher resolution version of the Graphical abstract is available as Supplementary information

Diagnosis, management and treatment of the Alport syndrome - 2024 guideline on behalf of ERKNet, ERA and ESPN

Glomerular nephropathy resulting from the genetic defects in COL4A3/4/5 genes including the classical Alport syndrome (AS) is the second commonest hereditary kidney disease characterized by persistent haematuria progressing to the need of kidney replacement therapy, frequently associated with sensorineural deafness, and occasionally with ocular anomalies. Diagnosis and management of COL4A3/4/5 glomerulopathy is a great challenge due to its phenotypic heterogeneity, multiple modes of inheritance, variable expressivity, and disease penetrance of individual variants as well as imperfect prognostic and progression factors and scarce and limited clinical trials, especially in children. As a joint initiative of the European Rare Kidney disease reference Network (ERKNet), European Renal Association (ERA Genes&Kidney) and European Society for Paediatric Nephrology (ESPN) Working Group Hereditary Kidney Disorders, a team of experts including adult and paediatric nephrologists, kidney geneticists, audiologists, ophthalmologists and a kidney pathologist were selected to perform a systematic literature review on 21 clinically relevant PICO (Patient or Population covered, Intervention, Comparator, Outcome) questions. The experts formulated recommendations and formally graded them at a consensus meeting with input from patient representatives and a voting panel of nephrologists representing all regions of the world. Genetic diagnostics comprising joint analysis of COL4A3/4/5 genes is the key diagnostic test already during the initial evaluation of an individual presenting with persistent haematuria, proteinuria, kidney failure of unknown origin, focal segmental sclerosis of unknown origin and possibly cystic kidney disease. Early renin-angiotensin system blockade is the standard of care therapy; sodium-glucose cotransporter-2 inhibitors may be added in adults with proteinuria and chronic kidney disease. Relatives with heterozygous COL4A3/4/5 variants should only be considered as the last possible resource for living kidney donation. This guideline provides guidance for the diagnosis and management of individuals with pathogenic variants in COL4A3/4/5 genes

In-play betting, sport broadcasts, and gambling severity: a survey study of Spanish sports bettors on the risks of gambling on sport while watching it

Fans watching live sport events, both mediated or in stadia, have witnessed an increase in sports betting products. Most of these products feature in-play betting, that is, the ability to bet on a game once it has started while watching it. In-play betting has raised many concerns among responsible gambling advocates due to its perceived relationship with problem gambling behaviour. This study explored the association between in-play betting and problem gambling. More specifically, the study examined how motives for consuming sport and how involved sports fans were in watching sport affected their gambling. Also, adjacent risk behaviours to in-play betting (such as consuming junk food and alcohol) during live sports betting were examined. Using a survey comprising 659 sports bettors from Spain, the study found that compared to participants not engaging in in-play betting, in-play bettors reported higher (i) problem gambling severity, (ii) sport watching involvement, (iii) consumption of sport to escape from everyday preoccupations, and (iv) consumption of junk food and/or alcohol while watching sport. These findings make the case that in-play betting regulators and providers should be cognizant of the interplay of sport-specific, media-related, and other risks, involved in the act of in-play betting while watching live sport

Score a goal for climate: Assessing the carbon footprint of travel patterns of the English Premier League clubs

Football is the most popular sport, globally and in the United Kingdom. However it generates a range of negative environmental impacts, such as climate change, due to an extensive amount of travel involved. The growing contribution of football clubs to the global carbon footprint has been recognised, but never consistently assessed. This study assesses the carbon footprint of the English Premier League (EPL)clubs, using the patterns of their domestic travel in the 2016/2017 season as a proxy for analysis. The study shows that, within the 2016/17 season, the EPL clubs produced circa 1134 tonnes of CO 2- eq. as a result of their travel, where transportation accounts for 61% of the carbon footprint. To reduce this carbon footprint, a careful review of the current corporate travel and procurement practices in the EPL clubs is necessary. This is in order to optimise the travel itineraries, prioritise more climate-benign modes of transport and contract budget accommodation providers with the ‘green’ credentials

Raw video of the 1979 Springfield College Gymnastic Homeshow (video 4 of 4)

This is raw video of the Springfield College Gymnastic Homeshow held in 1979 at the Civic Center in Springfield Massachusetts. The video was created and used for an airing of the show on ESPN. The Homeshow took place on November 10, 1979. The video is around 51 minutes long. The video starts with still images of Olympic and SC banners and images. At around 5 minutes there are interviews of Frank Wolcott, Edward S. Steitz, and Art Linkletter. The interview with Art Linkletter includes him telling his story of how he became involved with Springfield College. The interviews include multiple takes of the same questions and answers. The video also shows the starting ceremonies of the Homeshow and there are performances of gymnasts performing on the balance beam, the rings, the parachute and dance routines. These performances are shown from different camera angles, including from afar and close-up. The ESPN commentators can be heard at times. The video is the fourth video, marked "D" on the case, of four videos of the Homeshow made on a U-matic video cassette.Technician who digitized this video wrote, "Tape came apart at leader and needed spliced. Tape begins with olympic still. Audio is on right channel at low levels. Picture is soft. Mild dropout. Tape has same setup as others in series"; Video's case is labeled, saying "Springfield College Gymnastics Exhibition Tape D."

Raw video of the 1979 Springfield College Gymnastic Homeshow (video 1 of 4)

This is raw video of the Springfield College Gymnastic Homeshow held in 1979 at the Civic Center in Springfield Massachusetts. The video was created and used for a prod cast of the show on ESPN. The Homeshow took place on November 10, 1979. The video is 55 minutes long. There are performances of gymnasts performing on the parallel bars, the high bars, and triple balance among others. There are also dance routines, clowns, and Tableaux performances. These performances are shown from different camera angles, including from afar and close-up. Art Linkletter is the MC and is shown close-up talking from a podium. There is sound but it is often light and hard to hear. The ESPN commentators, Richard Kentwell and Kitty Kjeldsen, can be heard at times. The video is the first video, marked "A" on the case, of four videos of the Homeshow made on a U-matic video cassette.Technician who digitized this video wrote, "Tape begins in red. Audio is on right channel at low levels. Picture is soft. Mild dropout. Tape has same setup as others in series"; Case for video reads, "Springfield College Gymnastics Exhibition Tape A" and "Tape ok, Guide plate missing on case."

Raw video of the 1979 Springfield College Gymnastic Homeshow (video 3 of 4)

This is raw video of the Springfield College Gymnastic Homeshow held in 1979 at the Civic Center in Springfield Massachusetts. The video was created and used for an airing of the show on ESPN. The Homeshow took place on November 10, 1979. The video is 26 minutes long. The video starts with still images of Olympic and SC banners and images. The vast majority of the video are speaking parts of ESPN commentators, Richard Kentwell and Kitty Kjeldsen. In addition, there are performances of gymnasts performing vaulting over the horse and much of the opening ceremonies of the Homeshow. The video is the third video, marked "C" on the case, of four videos of the Homeshow made on a U-matic video cassette.Technician who digitized this video wrote, "Tape begins with olympic still. Audio is on right channel at low levels. Picture is soft. Mild dropout. Tape has same setup as others in series"; Video's case is labeled, saying "Springfield College Gymnastics Exhibition Tape C.