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A rare lymphoproliferative disorder involving thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), renal dysfunction (R), and organomegaly (O), called TAFRO syndrome, was first reported in 2010. Considered a variant of idiopathic multicentric Castleman's disease, the recent discovery and rarity of this syndrome pose challenges to diagnosis and management. Herein, we review three pediatric cases, including an infant, that illustrate the heterogeneity of TAFRO syndrome. Despite differences in presentation and treatment responses, all patients experienced excellent outcomes. This multi-institutional case series highlights the need to work toward earlier diagnosis and improved long-term management recommendations for patients with TAFRO syndrome.
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BACKGROUND: Obesity is associated with increased complications, rejection, and graft loss after kidney transplantation in adult and pediatric recipients. Elevated body mass index (BMI) is a common contraindication to transplant at adult kidney transplant programs; however, there is no data on such limitations for pediatric patients. METHODS: Between October and December 2022, we conducted a survey of Pediatric Nephrology Research Consortium centers assessing the use of BMI in pediatric kidney transplant evaluation. Centers reporting utilization of BMI cutoffs were invited to submit patient-level data on children declined for active transplant listing due to BMI. RESULTS: Thirty-nine centers responded to the survey (42% response rate); 51% include BMI in their written listing criteria, with a median BMI "cutoff" of 39 kg/m2 (range 30-50 kg/m2). Between January 1, 2016, and December 31, 2021, 30 children at 15 transplant centers were declined for listing status due to BMI. Patient-level data was provided for 19 children (63%) who were denied active listing status; median BMI was 42 kg/m2 (range 35.8-49.4 kg/m2) and 84% were on dialysis. One year after evaluation, seven patients (37%) had proceeded to active wait list status. Eight (42%) remained in inactive status and four (21%) were unlisted; ten of these 12 patients (83%) were on dialysis. CONCLUSIONS: The use of BMI in pediatric kidney transplant evaluation and listing varies among centers, but BMI limits access to transplant for some children. More information is needed on the outcomes of obese pediatric kidney candidates who are and are not transplanted, to guide development of national and international consensus.
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As outcomes and survival for children with chronic kidney disease (CKD) have improved over the last 30 years, there is an emerging need to characterize and understand later educational and employment outcomes across the spectrum of pediatric CKD severity-ranging from mild CKD to requirement for dialysis and kidney transplantation. Although large-scale research on the topic of long-term educational and employment outcomes in the pediatric CKD population is relatively scarce, the existing literature does support that children across the spectrum of CKD severity are at risk for education-related difficulties including chronic school absenteeism. These education-related difficulties are compounded by well-described neurocognitive deficits-particularly in the domain of executive functioning-that may potentially perpetuate the risk for academic underachievement. This is particularly concerning given that data from the general pediatric population suggest that childhood academic underachievement is associated with higher likelihood of un-/underemployment in adulthood. This review highlights what is known about educational and employment outcomes among persons with a history of childhood CKD, as well as suggestions for interventions to improve educational outcomes for this population.
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Chemical recycling of polyurethane (PU) waste is essential to displace the need for virgin polyol production and enable sustainable PU production. Currently, less than 20% of PU waste is downcycled through rebinding to lower value products than the original PU. Chemical recycling of PU waste often requires significant input of materials like solvents and slow reaction rates. Here, we report the fast (<10 min) and solvent-free acidolysis of a model toluene diisocyanate (TDI)-based flexible polyurethane foam (PUF) at <200 °C using maleic acid (MA) with a recovery of recycled polyol (repolyol) in 95% isolated yield. After workup (hydrolysis of repolyl ester and separations), the repolyol exhibits favorable physical properties that are comparable to the virgin polyol; these include 54.1 mg KOH/g OH number and 624 cSt viscosity. Overall, 80% by weight of the input PUF is isolated into two clean-cut fractions containing the repolyol and toluene diamine (TDA). Finally, end-of-life (EOL) mattress PUF waste is recycled successfully with high recovery of repolyol using MA acidolysis. The solvent-free and fast acidolysis with MA demonstrated in this work with both model and EOL PUF provides a potential pathway for sustainable and closed-loop PU production.
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Polyurethane (PU) is the sixth most used plastic in the world. Because many PU derived materials are thermosets and the monomers are valuable, chemical recycling to recover the polyol component is the most viable pathway to utilizing postconsumer PU waste in a closed-loop fashion. Acidolysis is an effective method to recover polyol from PU waste. Previous studies of PU acidolysis rely on the use of dicarboxylic acid (DCA) in high temperature reactions (>200 °C) in the liquid phase and result in unwanted byproducts, high energy consumption, complex separations of excess organic acid, and an overall process that is difficult to scale up. In this work, we demonstrate selective PU acidolysis with DCA vapor to release polyol at temperatures below the melting points of the DCAs (<150 °C). Notably, acidolysis with DCA vapor adheres to the principles of green chemistry and prevents in part esterification of the polyol product, eliminating the need for additional hydrolysis/processing to obtain the desired product. The methodology was successfully applied to a commercial PU foam (PUF) postconsumer waste.
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INTRODUCTION: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and IgG4-related disease (IgG4-RD) are distinct immune disorders with overlapping clinical and laboratory features. While ANCA positivity excludes IgG4-RD in the 2019 ACR/EULAR classification, this criterion is not uniformly applied, and AAV can form inflammatory masses in various organs and show increase in IgG4 + plasma cells, similar to IgG4-RD. CASE DIAGNOSIS/TREATMENT: A 5-year-old female with history of orbital mass diagnosed as IgG4-RD presents with acute kidney injury. She has a myeloperoxidase ANCA, and kidney biopsy shows pauci-immune crescentic glomerulonephritis and acute tubulointerstitial nephritis with increased IgG4 + plasma cells and tubular basement membrane (TBM) deposits. CONCLUSION: In isolation, TBM deposits and increased IgG4 + plasma cells are suggestive of IgG4-RD. In the context of a positive ANCA and pauci-immune crescentic glomerulonephritis, however, increased IgG4 + plasma cells due to AAV are favored. In cases with features of IgG4-RD, ANCA positivity suggests an alternate diagnosis of AAV to be more likely.
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Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos , Glomerulonefritis , Enfermedad Relacionada con Inmunoglobulina G4 , Nefritis Intersticial , Seudotumor Orbitario , Femenino , Humanos , Preescolar , Anticuerpos Anticitoplasma de Neutrófilos , Seudotumor Orbitario/patología , Enfermedad Relacionada con Inmunoglobulina G4/diagnóstico , Riñón/patología , Nefritis Intersticial/complicaciones , Nefritis Intersticial/diagnóstico , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/complicaciones , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/diagnóstico , Inmunoglobulina G , Glomerulonefritis/complicaciones , Glomerulonefritis/diagnósticoRESUMEN
We present the complete chloroplast genome sequence of an endophytic Ostreobium sp. isolated from a 19th-century coralline red algal specimen from St. Croix, U.S. Virgin Islands. The chloroplast genome is 84,848 bp in length, contains 114 genes, and has a high level of gene synteny to other Ostreobiaceae.
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BACKGROUND: Adolescents who have received a kidney transplant are at high risk of graft rejection and transplant-related comorbidities around the time of transition from pediatric to adult care. While there has been a progress in tracking transition readiness, further work is needed to prepare adolescents for healthcare transitions. We describe a longitudinal cohort-based transition curriculum designed to prepare kidney transplant recipients for adult transplant care. METHODS: Adolescent kidney transplant recipients aged 17 and older participated in the pilot cohort of the 2-year transition curriculum. Session topics included communication with the healthcare team, insurance, job skills, reflective practice, reproductive health, medications, and adult clinic introduction. Surveys were given to obtain narrative feedback, assess participant self-management behavior, and track curriculum knowledge. RESULTS: Each participant attended an average of two sessions, with 18 out of 30 eligible adolescents participating in at least one session. After transitioning to a virtual platform, there was increased attendance of participants who live greater than 150 miles from the transplant center. Adolescents highlighted the value of the program's group structure to relate to and learn from other participants. CONCLUSIONS: The pilot transition program successfully provided adolescent kidney transplant recipients the opportunity to learn alongside their peers and gain interdisciplinary knowledge to prepare for healthcare transition. The program converted to a virtual platform during the COVID-19 pandemic, with increased accessibility for participants who live further from the transplant center. Group-based programming for adolescents should be enhanced to further prepare them for transitions to adult medicine.
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COVID-19 , Trasplante de Riñón , Transición a la Atención de Adultos , Adulto , Adolescente , Humanos , Niño , Pandemias , Encuestas y Cuestionarios , Receptores de TrasplantesRESUMEN
The inclusion of body mass index (BMI) as a criterion for determining kidney transplant candidacy in children raises clinical and ethical challenges. Childhood obesity is on the rise and common among children with kidney failure. In addition, obesity is reported as an independent risk factor for the development of CKD and kidney failure. Resultantly, more children with obesity are anticipated to need kidney transplants. Most transplant centers around the world use high BMI as a relative or absolute contraindication for kidney transplant. However, use of obesity as a relative or absolute contraindication for pediatric kidney transplant is controversial. Empirical data demonstrating poorer outcomes following kidney transplant in obese pediatric patients are limited. In addition, pediatric obesity is distributed inequitably among groups. Unlike adults, most children lack independent agency to choose their food sources and exercise opportunities; they are dependent on their families for these choices. In this paper, we define childhood obesity and review (1) the association and impact of obesity on kidney disease and kidney transplant, (2) existing adult guidelines and rationale for using high BMI as a criterion for kidney transplant, (3) the prevalence of childhood obesity among children with kidney failure, and (4) the existing literature on obesity and pediatric kidney transplant outcomes. We then discuss ethical considerations related to the use of obesity as a criterion for kidney transplant.
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Trasplante de Riñón , Obesidad Infantil , Insuficiencia Renal , Adulto , Niño , Humanos , Índice de Masa Corporal , Contraindicaciones , Análisis ÉticoRESUMEN
BACKGROUND: Children with chronic kidney disease (CKD) are at risk for neurocognitive deficits while simultaneously being at risk for chronic school absenteeism (≥ 18 school days per school year). Chronic school absenteeism compounds the negative impacts of CKD on academic achievement. In this study, we examined patient- and caregiver-reported factors associated with school absenteeism in children with non-dialysis- or transplant-dependent CKD in order to help identify which factors could be modifiable and ultimately improve school attendance. METHODS: We utilized a combination of chart review and questionnaires distributed in person to patients and caregivers at a pediatric nephrology clinic between November 2018 and August 2019 to gather data. We used descriptive statistics to illustrate clinical characteristics of the children included in the study, caregiver characteristics, and examined reported reasons for missing school. RESULTS: Twenty-one percent of participants (10/48) missed 18 full days of school or more, categorizing them as chronically absent. The top three reasons for missing school were doctor appointments, feeling sick, and being bullied. More specific sequelae of CKD were not highly reported as reasons for missing school. CONCLUSIONS: Chronic absenteeism is a highly reported phenomenon among children with pediatric CKD. Given that missing school for doctor appointments was a top reason for absenteeism, this data suggests alternative appointment hours and virtual appointments may reduce chronic school absenteeism in children, and by extension improve their health, behavioral, and academic outcomes. A higher resolution version of the Graphical abstract is available as Supplementary information.
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Cuidadores , Insuficiencia Renal Crónica , Niño , Humanos , Absentismo , Instituciones Académicas , EscolaridadRESUMEN
Abandoned, lost, or otherwise discarded fishing gear (ALDFG) is a major contributor to ocean pollution, with extensive social, economic, and environmental impacts. However, quantitative ALDFG estimates are dated and limited in scope. To provide current global estimates, we interviewed fishers around the world about how much fishing gear they lose annually and multiplied reported losses by global fishing effort data. We estimate that nearly 2% of all fishing gear, comprising 2963 km2 of gillnets, 75,049 km2 of purse seine nets, 218 km2 of trawl nets, 739,583 km of longline mainlines, and more than 25 million pots and traps are lost to the ocean annually. These estimates represent critical baselines that can inform solutions targeted to ALDFG reduction strategies.
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BACKGROUND: Acute kidney injury (AKI) is seen in one-fifth of pediatric patients with COVID-19 requiring hospital admission, and is associated with increased morbidity, mortality, and residual kidney impairment. The majority of kidney pathology data in patients with COVID-19 is derived from adult case series and there is an overall lack of histologic data for most pediatric patients with COVID-19. METHODS: We assembled a multi-institutional cohort of five unvaccinated pediatric patients with COVID-19 and associated kidney dysfunction with available histology. RESULTS: Three complex patients with current or prior SARS-CoV-2 infection had multifactorial thrombotic microangiopathy with clinical features of hemolytic uremic syndrome (in two) or disseminated intravascular coagulation (in one); one died and another developed chronic kidney disease stage 5. Two with recently preceding SARS-CoV-2 infection presented with nephrotic syndrome; one had IgA vasculitis and one had minimal change disease. Within a short follow-up time, none has returned to baseline kidney function. CONCLUSION: Although uncommon, COVID-19-associated kidney injury can have significant morbidity in the unvaccinated pediatric and adolescent population. A higher resolution version of the Graphical abstract is available as Supplementary information.
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Lesión Renal Aguda , COVID-19 , Vasculitis por IgA , Lesión Renal Aguda/etiología , Lesión Renal Aguda/patología , Adolescente , Adulto , COVID-19/complicaciones , Niño , Humanos , Riñón/patología , SARS-CoV-2RESUMEN
BACKGROUND: Neutropenia is common in the first year after pediatric kidney transplant and is associated with an increased risk of infection, allograft loss, and death. Granulocyte colony-stimulating factor (G-CSF) increases neutrophil production, but its use in pediatric solid organ transplant recipients remains largely undescribed. METHODS: We performed a multicenter retrospective cohort study of children with neutropenia within the first 180 days after kidney transplant. Multivariable linear regression and Poisson regression were used to assess duration of neutropenia and incidence of hospitalization, infection, and rejection. RESULTS: Of 341 neutropenic patients, 83 received G-CSF during their first episode of neutropenia. Median dose of G-CSF was 5 mcg/kg for 3 (IQR 2-7) doses. G-CSF use was associated with transplant center, induction immunosuppression, steroid-free maintenance immunosuppression, hospitalization, and decreases in mycophenolate mofetil, valganciclovir, and trimethoprim-sulfamethoxazole dosing. Absolute neutrophil count nadir was also significantly lower among those treated with G-CSF. G-CSF use was not associated with a shorter duration of neutropenia (p = .313) and was associated with a higher rate of neutropenia relapse (p = .002) in adjusted analysis. G-CSF use was associated with a decreased risk of hospitalization (aIRR 0.25 (95%CI 0.12-0.53) p < .001) but there was no association with incidence of bacterial infection or rejection within 90 days of neutropenic episode. CONCLUSION: G-CSF use for neutropenia in pediatric kidney transplant recipients did not shorten the overall duration of neutropenia but was associated with lower risk of hospitalization. Prospective studies are needed to determine which patients may benefit from G-CSF treatment.
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Trasplante de Riñón , Nefrología , Neutropenia , Niño , Factor Estimulante de Colonias de Granulocitos/uso terapéutico , Humanos , Trasplante de Riñón/efectos adversos , Neutropenia/complicaciones , Estudios RetrospectivosRESUMEN
In the age of the Anthropocene, the ocean has typically been viewed as a sink for pollution. Pollution is varied, ranging from human-made plastics and pharmaceutical compounds, to human-altered abiotic factors, such as sediment and nutrient runoff. As global population, wealth and resource consumption continue to grow, so too does the amount of potential pollution produced. This presents us with a grand challenge which requires interdisciplinary knowledge to solve. There is sufficient data on the human health, social, economic, and environmental risks of marine pollution, resulting in increased awareness and motivation to address this global challenge, however a significant lag exists when implementing strategies to address this issue. This review draws upon the expertise of 17 experts from the fields of social sciences, marine science, visual arts, and Traditional and First Nations Knowledge Holders to present two futures; the Business-As-Usual, based on current trends and observations of growing marine pollution, and a More Sustainable Future, which imagines what our ocean could look like if we implemented current knowledge and technologies. We identify priority actions that governments, industry and consumers can implement at pollution sources, vectors and sinks, over the next decade to reduce marine pollution and steer us towards the More Sustainable Future. Supplementary Information: The online version contains supplementary material available at 10.1007/s11160-021-09674-8.
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BACKGROUND: Hypertension is common among children with chronic kidney disease (CKD), and dihydropyridine calcium channel blockers (dhCCBs) are frequently used as treatment. The impact of dhCCBs on proteinuria in children with CKD is unclear. METHODS: Data from 722 participants in the Chronic Kidney Disease in Children (CKiD) longitudinal cohort with a median age of 12 years were used to assess the association between dhCCBs and log transformed urine protein/creatinine levels as well as blood pressure control measured at annual visits. Angiotensin-converting enzyme inhibitor (ACEi) and angiotensin receptor blocker (ARB) use was evaluated as an effect measure modifier. RESULTS: Individuals using dhCCBs had 18.8% higher urine protein/creatinine levels compared to those with no history of dhCCB or ACEi and ARB use. Among individuals using ACEi and ARB therapy concomitantly, dhCCB use was not associated with an increase in proteinuria. Those using dhCCBs had higher systolic and diastolic blood pressures. CONCLUSIONS: Use of dhCCBs in children with CKD and hypertension is associated with higher levels of proteinuria and was not found to be associated with improved blood pressure control.
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Bloqueadores de los Canales de Calcio , Canales de Calcio Tipo L , Proteinuria , Insuficiencia Renal Crónica , Bloqueadores de los Canales de Calcio/farmacología , Canales de Calcio Tipo L/efectos de los fármacos , Niño , Humanos , Hipertensión/tratamiento farmacológico , Estudios Longitudinales , Proteinuria/tratamiento farmacológico , Insuficiencia Renal Crónica/tratamiento farmacológico , Insuficiencia Renal Crónica/orinaRESUMEN
Abandoned, lost or discarded fishing gear (ALDFG) comprises a significant amount of global marine debris, with diverse impacts to marine environments, wildlife, and the fishing industry. Building evidence on ALDFG is critical to holistically understand the marine debris issue, and to inform the development of solutions that reduce amounts of ALDFG sources and recover existing gear. Substantial work has been and continues to be undertaken around the world to collect data on ALDFG, much of which remains unpublished. To provide a global picture of data on ALDFG, we organized a technical session that brought together seven ALDFG leaders to share their expertise in data collection, retrieval, and awareness-raising. This paper summarizes the technical session to highlight: 1) case studies that feature innovative approaches to ALDFG data collection and retrieval; 2) examples of opportunities to fill data gaps and improve our understanding of wildlife ingestion of and entanglement in ALDFG; and 3) awareness-raising through the development of a publicly accessible global ALDFG database.
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Equipos y Suministros , Explotaciones Pesqueras , Contaminación del Agua/prevención & control , Congresos como Asunto , AmbienteRESUMEN
We evaluated the frequency of chronic school absenteeism (≥18 missed school days per year) among children with mild-to-moderate chronic kidney disease. Chronic absenteeism was present in 17.3% of children with chronic kidney disease, compared with 2.7% of children in the US National Health and Nutrition Examination Survey.
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Absentismo , Insuficiencia Renal Crónica , Adolescente , Niño , Estudios Transversales , Femenino , Encuestas Epidemiológicas , Humanos , Masculino , Índice de Severidad de la Enfermedad , Estados UnidosRESUMEN
OBJECTIVES: Acute kidney injury (AKI) is common in hospitalized children. The impact of AKI following hospitalization is not fully understood, particularly the impact on health related quality of life (HRQOL). The goal of this study was to determine the relationship between hospitalization-associated AKI and HRQOL in a pediatric population. STUDY DESIGN: We conducted a retrospective cohort study of children with hospitalization-associated AKI. Eligible children were 1-19 years old with AKI defined by kidney disease improving global outcomes (KDIGO) criteria and had at least one completed pediatric quality of life (PedsQL) 4.0 Generic Core Scale survey (N = 139). Participants completed up to three surveys to reflect baseline, admission and follow-up status. We categorized children as having mild AKI (KDIGO stage 1, N = 73) or severe AKI (KDIGO stage 2 or 3, N = 66). Mean PedsQL scores were compared by AKI group. Those with both baseline and follow-up surveys were analyzed to determine the proportion who returned to their baseline level of function within 8 weeks of discharge. RESULTS: Children with mild and severe AKI had similar baseline and admission PedsQL scores. Although children with severe AKI had lower follow-up scores, the results were not statistically significant (78.9 vs. 85.8, p = 0.11). Of those with severe AKI, 48% returned to their baseline level of physical functioning by follow-up, compared to 73% with mild AKI (p = 0.05). CONCLUSIONS: This is the first study of HRQOL following hospitalization-associated AKI. We found that children with severe AKI had depressed physical functioning after discharge when compared to children with mild AKI.
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Lesión Renal Aguda/fisiopatología , Calidad de Vida , Lesión Renal Aguda/terapia , Adolescente , Niño , Preescolar , Comorbilidad , Femenino , Hospitalización , Humanos , Lactante , Masculino , Recuperación de la Función , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Encuestas y CuestionariosRESUMEN
Fisheries observer data recorded between 2003 and 2015 on-board purse seine and longline vessels operating in the Western and Central Pacific Ocean reported more than 10 000 pollution incidents within the exclusive economic zones (EEZs) of 25 Pacific countries and territories, and in international waters. A majority of the reported purse seine pollution incidents related to dumping of plastics waste. Other common pollution incidents related to oil spillages and to abandoned, lost or dumped fishing gear. Data analysis highlighted the need for increased monitoring, reporting, and enforcement of pollution violations by all types of fishing vessels operating in the Pacific region; a regional outreach and compliance assistance programme on marine pollution prevention and improvements in Pacific port waste reception facilities.
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Explotaciones Pesqueras , Navíos/estadística & datos numéricos , Contaminación del Agua/estadística & datos numéricos , Océanos y MaresRESUMEN
Patients with chronic idiopathic Parkinson's disease are at a high risk for fractures, particularly of the hip. The causes may be multifactorial, including poor balance and other forms of neurological dysfunction. Osteopenia and osteoporosis leading to decreased bone mass are common in these patients. We report a case of a male patient with a long-standing history of Parkinson's disease on chronic high-dose carbidopa-levodopa who suffered a hip fracture. The relationship of Parkinson's disease and carbidopa-levodopa use with osteopenia and hip fractures is discussed, emphasizing the crucial need for prevention in this patient population along with medical and surgical treatment.