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Background: Neonatal acute kidney injury (AKI) is a common yet underdiagnosed condition in neonates with significant implications for long-term kidney health. Lack of timely recognition and documentation of AKI contributes to missed opportunities for nephrology consultation and follow-up, potentially leading to adverse outcomes. Methods: We conducted a quality improvement (QI) project to address this by incorporating an automated real-time electronic medical record (EMR)-AKI alert system in the Neonatal Intensive Care Unit (NICU) at Le Bonheur Children's Hospital. Our primary objective was to improve documentation of neonatal AKI (defined as serum creatinine (SCr) > 1.5 mg/dL) by 25% compared to baseline levels. The secondary goal was to increase nephrology consultations and referrals to the neonatal nephrology clinic. We designed an EMR-AKI alert system to trigger for neonates with SCr > 1.5 mg/dL, automatically adding AKI diagnosis to the problem list. This prompted physicians to consult nephrology, refer neonates to the nephrology clinic, and consider medication adjustments. Results: Our results demonstrated a significant improvement in AKI documentation after implementing the EMR-AKI alert, reaching 100% compared with 7% at baseline (p < 0.001) for neonates with SCr > 1.5 mg/dL. Although the increase in nephrology consultations was not statistically significant (p = 0.5), there was a significant increase in referrals to neonatal nephrology clinics (p = 0.005). Conclusions: Integration of an EMR alert system with automated documentation offers an efficient and economical solution for improving neonatal AKI diagnosis and documentation. This approach enhances healthcare provider engagement, streamlines workflows, and supports QI. Widespread adoption of similar approaches can lead to improved patient outcomes and documentation accuracy in neonatal AKI care. [ABSTRACT FROM AUTHOR]

Background: Secondary pseudohypoaldosteronism (S-PHA) is a rare condition resulting from renal tubular resistance to aldosterone in children with urinary tract infection (UTI) and/or nephrourological malformations. It is characterized by nonspecific symptoms but with the potential for life-threatening complications. We aim to evaluate the clinical manifestations, diagnostic approach, and therapeutic interventions in children with S-PHA, along with a review of recent publications. Methods: A retrospective observational descriptive study was conducted on S-PHA cases diagnosed over the last 15 years at a tertiary pediatric nephrology unit. The literature for the last 10 years was reviewed. Results: Twelve patients (10 males, 6 days to 6 months) were identified. Weight loss was the main reason for consultation (50%). Ninety-two percent of patients had an underlying nephrourological pathology and 62% concomitant confirmed UTI. Seven out of 12 children were admitted to the PICU. A subsequent extrapontine myelinolysis was observed in one patient as neurological sequelae. Twenty-one articles related to S-PHA have been identified on PubMed and Embase. Conclusions: S-PHA should be considered in infants under 6 months of age with UTI and/or CAKUT. Obstructive anomalies and vesicoureteral reflux can be found, affecting both unilateral and bilateral systems. Early medical and surgical interventions are crucial and require close monitoring to avoid iatrogenic complications. [ABSTRACT FROM AUTHOR]

Background: Assessing bias (estimated – measured) is key to evaluating glomerular filtration rate (GFR). Stratification by subgroups can indicate where equations perform differently. However, there is a fallacy in the assessment of two instruments (e.g., eGFR and mGFR) when stratifying on the level of only one of those instruments. Here, we present statistical aspects of the problem and a solution for GFR stratification along with an empirical investigation using data from the CKiD study. Methods: Compared and contrasted biases (eGFR relative to mGFR) with 95% confidence intervals within strata of mGFR only, eGFR only, and the average of mGFR and eGFR using data from the Chronic Kidney Disease in Children (CKiD) study. Results: A total of 304 participants contributed 843 GFR studies with a mean mGFR of 48.46 (SD = 22.72) and mean eGFR of 48.67 (SD = 22.32) and correlation of 0.904. Despite strong agreement, eGFR significantly overestimated mGFR when mGFR < 30 (+ 6.2%; 95%CI + 2.9%, + 9.7%) and significantly underestimated when mGFR > 90 (–12.2%; 95%CI − 17.3%, − 7.0%). Significant biases in opposite direction were present when stratifying by eGFR only. In contrast, when stratifying by the average of eGFR and mGFR, biases were not significant (+ 1.3% and − 1.0%, respectively) congruent with strong agreement. Conclusions: Stratifying by either mGFR or eGFR only to assess eGFR biases is ubiquitous but can lead to inappropriate inference due to intrinsic statistical issues that we characterize and empirically illustrate using data from the CKiD study. Using the average of eGFR and mGFR is recommended for valid inferences in evaluations of eGFR biases. [ABSTRACT FROM AUTHOR]

Free Open-Access Medical Education (FOAMed) has transformed medical education in the past decade by complementing and substituting for traditional medical education when needed. The attractiveness of FOAMed resources is due to their inexpensive nature, wide availability, and user ability to access on demand across a variety of devices, making it easy to create, share, and participate. The subject of nephrology is complex, fascinating, and challenging. Traditional didactic lectures can be passive and ineffective in uncovering these difficult concepts and may need frequent revisions. Active teaching methods like flipped classrooms have shown some benefits, and these benefits can only be multifold with current social media tools. Social media will inspire the involvement of students and allow them to create and share educational content in a "trendy way," encouraging the participation of their peers and thus building an educational environment more conducive to them while promoting revision and retainment. FOAMed also promotes asynchronous learning, spaced learning, microlearning, and multimodal presentation with a meaningful variation. This article discusses the evolution of digital education, social media platforms, tools for creating and developing FOAMed resources, and digital scholarship. [ABSTRACT FROM AUTHOR]

Background: Oral health conditions are common in children and young people (CYP) with kidney disorders. There is currently limited literature on how confident paediatric nephrology teams feel to identify and manage oral health concerns for their patients. Method: An exploratory mixed-method survey was distributed across all 13 UK specialist paediatric nephrology centres with responses received from consultants, registrars, specialist nurses and special interest (SPIN) paediatricians. Results: Responses received from 109 multidisciplinary team members of 13/13 (100%) UK tertiary units. Ninety-two percent (n = 100) of respondents reported they had never received any training in oral health and 87% (n = 95) felt that further training would be beneficial to optimise care for patients and improve communication between medical and dental teams. Most respondents reported that they did not regularly examine, or enquire about, their patients' oral health. Only 16% (n = 17) reported that all their paediatric kidney transplant recipients underwent routine dental assessment prior to transplant listing. Severe adverse oral health outcomes were rarely reported and only 11% (n = 12) of respondents recalled having a patient who had a kidney transplant delayed or refused due to concerns about oral infection. Seventy-eight percent (n = 85) felt that joint working with a dental team would benefit patients at their unit; however, 17% (n = 18) felt that current infrastructure does not currently support effective joint working. Conclusions: Across the UK, paediatric kidney health professionals report lack of confidence and training in oral health. Upskilling subspecialty teams and creating dental referral pathways are recommended to maximise oral health outcomes for CYP with kidney diseases. [ABSTRACT FROM AUTHOR]

Background: The gastrointestinal (GI) tract represents one of the main targets of typical hemolytic uremic syndrome (HUS) in children. In this observational study, we tried to establish (1) the main features of GI complications during STEC-HUS and (2) the relationship between Escherichia coli serotypes and Shiga toxin (Stx) variants with hepatopancreatic involvement. Methods: A total of 79 STEC-HUS patients were admitted to our pediatric nephrology department between January 2012 and June 2021. Evidence of intestinal, hepatobiliary, and pancreatic involvements was reported for each patient, alongside demographic, clinical, and laboratory features. Frequency of gastrointestinal complications across groups of patients infected by specific E. coli serotypes and Stx gene variants was evaluated. Results: Six patients developed a bowel complication: two developed rectal prolapse, and four developed bowel perforation which resulted in death for three of them and in bowel stenosis in one patient. Acute pancreatitis was diagnosed in 13 patients. An isolated increase in pancreatic enzymes and/or liver transaminases was observed in 41 and 15 patients, respectively. Biliary sludge was detected in three, cholelithiasis in one. Forty-seven patients developed direct hyperbilirubinemia. Neither E. coli serotypes nor Shiga toxin variants correlated with hepatic or pancreatic involvement. Conclusions: During STEC-HUS, GI complications are common, ranging from self-limited elevation of laboratory markers to bowel perforation, a severe complication with a relevant impact on morbidity and mortality. Hepatopancreatic involvement is frequent, but usually short-lasting and self-limiting. [ABSTRACT FROM AUTHOR]

Background: We aimed to examine temporal changes in the annual rate of acute kidney injury (AKI) in Danish children and associated changes in patient characteristics including potential underlying risk factors. Methods: In this population-based cohort study, we used plasma creatinine measurements from Danish laboratory databases to identify AKI episodes in children aged 0–17 years from 2007 to 2021. For each child, the first AKI episode per calendar year was included. We estimated the annual crude and sex- and age-standardized AKI rate as the number of children with an AKI episode divided by the total number of children as reported by census numbers. Using Danish medical databases, we assessed patient characteristics including potential risk factors for AKI, such as use of nephrotoxic medication, surgery, sepsis, and perinatal factors. Results: In total, 14,200 children contributed with 16,345 AKI episodes over 15 years. The mean annual AKI rate was 148 (95% CI: 141–155) per 100,000 children. From 2007 to 2021, the annual AKI rate demonstrated minor year-to-year variability without any discernible overall trend. The highest AKI rate was recorded in 2007 at 174 (95% CI: 161–187) per 100,000 children, while the lowest rate occurred in 2012 at 129 (95% CI: 118–140) per 100,000 children. In 2021, the AKI rate was 148 (95% CI: 141–155) per 100,000 children. Characteristics of children with AKI were similar throughout the study period. Conclusion: The rate of AKI among Danish children was stable from 2007 to 2021 with little variation in patient characteristics over time. [ABSTRACT FROM AUTHOR]

The article focuses on pediatric nephrologists' perception and knowledge of evidence-based guideline development methodology. Topics discussed include the importance of guidelines in clinical practice, physicians' reliance on different guidance documents, and the need for improved understanding of Grading of Recommendations, Assessment, Development, and Evaluations (GRADE) methodology among pediatric nephrologists.

Background: Acute kidney injury (AKI) is a recognized comorbidity in pediatric diabetic ketoacidosis (DKA), although the exact etiology is unclear. The unique physiology of DKA makes dehydration assessments challenging, and these patients potentially receive excessive amounts of intravenous fluids (IVF). We hypothesized that dehydration is over-estimated in pediatric DKA, leading to over-administration of IVF and hyperchloremia that worsens AKI. Methods: Retrospective cohort of all DKA inpatients at a tertiary pediatric hospital from 2014 to 2019. A total of 145 children were included; reasons for exclusion were pre-existing kidney disease or incomplete medical records. AKI was determined by change in creatinine during admission, and comparison to a calculated baseline value. Linear regression multivariable analysis was used to identify factors associated with AKI. True dehydration was calculated from patients' change in weight, as previously validated. Fluid over-resuscitation was defined as total fluids given above the true dehydration. Results: A total of 19% of patients met KDIGO serum creatinine criteria for AKI on admission. Only 2% had AKI on hospital discharge. True dehydration and high serum urea levels were associated with high serum creatinine levels on admission (p = 0.042; p < 0.001, respectively). Fluid over-resuscitation and hyperchloremia were associated with delayed kidney recovery (p < 0.001). Severity of initial AKI was associated with cerebral edema (p = 0.018). Conclusions: Dehydration was associated with initial AKI in children with DKA. Persistent AKI and delay to recovery was associated with hyperchloremia and over-resuscitation with IVF, potentially modifiable clinical variables for earlier AKI recovery and reduction in long-term morbidity. This highlights the need to re-address fluid protocols in pediatric DKA. [ABSTRACT FROM AUTHOR]

Background: Diuretics are commonly used in neonatal AKI with the rationale to decrease positive fluid balance in critically sick neonates. The patterns of furosemide use vary among hospitals, which necessitates the need for a well-designed study. Methods: The TINKER (The Indian Iconic Neonatal Kidney Educational Registry) study provides a database, spanning 14 centres across India since August 2018. Admitted neonates (≤ 28 days) receiving intravenous fluids for at least 48 h were included. Neonatal KDIGO criteria were used for the AKI diagnosis. Detailed clinical and laboratory parameters were collected, including the indications of furosemide use, detailed dosing, and the duration of furosemide use (in days). Results: A total of 600 neonates with AKI were included. Furosemide was used in 8.8% of the neonates (53/600). Common indications of furosemide use were significant cardiac disease, fluid overload, oliguria, BPD, RDS, hypertension, and hyperkalemia. The odds of mortality was higher in neonates < 37 weeks gestational age with AKI who received furosemide compared to those who did not receive furosemide 3.78 [(1.60–8.94); p = 0.003; univariate analysis] and [3.30 (1.11–9.82); p = 0.03]; multivariate logistic regression]. Conclusions: In preterm neonates with AKI, mortality was independently associated with furosemide treatment. The furosemide usage rates were higher in neonates with associated co-morbidities, i.e. significant cardiac diseases or surgical interventions. Sicker babies needed more resuscitation at birth, and died early, and hence needed shorter furosemide courses. Thus, survival probability was higher in neonates treated with long furosemide courses vs. short courses. [ABSTRACT FROM AUTHOR]

The endogenous capacity of the kidney to repair is limited, and generation of new nephrons after injury for adequate function recovery remains a need. Discovery of factors that promote the endogenous regenerative capacity of the injured kidney or generation of transplantable kidney tissue represent promising therapeutic strategies. While several encouraging results are obtained after administration of stem or progenitor cells, stem cell secretome, or extracellular vesicles in experimental kidney injury models, very little data exist in the clinical setting to make conclusions about their efficacy. In this review, we provide an overview of the cutting-edge knowledge on kidney regeneration, including pre-clinical methodologies used to elucidate regenerative pathways and describe the perspectives of regenerative medicine for kidney patients. [ABSTRACT FROM AUTHOR]

An editorial is presented which expresses the views on the provision of pediatric nephrology services in low-middle-income countries (LMICs), highlighting the challenges and disparities in healthcare access. Topics include the impact of socio-economic conditions on public health systems, the unique challenges faced by LMICs in managing acute kidney injury (AKI) and chronic kidney disease (CKD) in children, and positive interventions such as telemedicine.

Background: The overall cost of managing chronic diseases is a significant barrier to accessing complete and timely healthcare, especially in rural and geographically isolated areas. This cost disparity becomes more pronounced in the case of children and more so in under-resourced regions of the world. In the era of COVID-19, as the need for physical distancing increased, there was a transition in approach to healthcare provision to telemedicine consultations. This study evaluates the cost saving using teleconsultations in a paediatric nephrology clinic. Methods: This prospective cohort study was conducted at AIIMS Jodhpur, a tertiary care centre in western Rajasthan from March 2021 to October 2022. All consecutive paediatric (29 days–18 years) patients attending telemedicine services for kidney-related illness were enrolled. Basic demographic details were collected. Cost analysis was done after 6 months, regarding perceived cost savings for the patient and family by using telehealth for follow-up during 6 months starting from enrolment. Results: A total of 112 patients were enrolled; 266 teleconsultations attended; 109 patients who could be followed up saved INR 457,900 during 6 months of follow-up. The average cost saving was INR − 1577/patient/visit. Patients saved 4.99% of the family income (median 2.16% (IQR 0.66–5.5)). The highest expenditure per visit was incurred for food and transport. The median distance from the residence to the clinic was 122.5 km (IQR 30–250). Over the 6-month study period, patients saved a travel distance of 83,274 km (743 km/patient). Conclusions: The use of telemedicine as a follow-up method helps save significant costs and distances travelled by patients. A higher-resolution version of the Graphical abstract is available as Supplementary information [ABSTRACT FROM AUTHOR]

Background: Eculizumab for the treatment of atypical hemolytic uremic syndrome (HUS) is a standard of care. Central nervous system (CNS) involvement in Shiga toxin–producing Escherichia coli (STEC)-HUS is associated with increased morbidity and mortality. There is no consensus on the use of plasma exchange and/or eculizumab. We report a series (n = 4) of children with CNS involvement in STEC-HUS with excellent outcomes after treatment with eculizumab only and supportive therapies. Methods: A retrospective chart review of patients with CNS involvement in STEC-HUS is managed with supportive therapies and eculizumab only. Results: Four patients (75% female) with a median age of 5 years and 11 months (IQR: 23.5–105.5 months) were admitted to a tertiary pediatric nephrology center with CNS involvement in STEC-HUS. Neurological symptoms presented between days 2 and 7 of illness and included ataxia, altered mental status, visual symptoms, and seizures. All had an abnormal MRI brain. All received two doses of eculizumab, 1 week apart (dosing according to weight). Resolution of neurological symptoms was evident at a mean of 60 h post-administration (range: 24–72 h). All patients have complete kidney and neurological recovery at 12-month follow-up. Conclusion: We present a case series of four children with STEC-HUS and CNS involvement, managed with eculizumab only, in lieu of plasma exchange (as per our previous policy). The marked improvement in symptoms in our cohort supports the use of eculizumab, rather than plasma exchange in the CNS involvement of STEC-HUS. [ABSTRACT FROM AUTHOR]

Biological and biomedical research using Drosophila melanogaster as a model organism has gained recognition through several Nobel prizes within the last 100 years. Drosophila exhibits several advantages when compared to other in vivo models such as mice and rats, as its life cycle is very short, animal maintenance is easy and inexpensive and a huge variety of transgenic strains and tools are publicly available. Moreover, more than 70% of human disease-causing genes are highly conserved in the fruit fly. Here, we explain the use of Drosophila in nephrology research and describe two kidney tissues, Malpighian tubules and the nephrocytes. The latter are the homologous cells to mammalian glomerular podocytes and helped to provide insights into a variety of signaling pathways due to the high morphological similarities and the conserved molecular make-up between nephrocytes and podocytes. In recent years, nephrocytes have also been used to study inter-organ communication as links between nephrocytes and the heart, the immune system and the muscles have been described. In addition, other tissues such as the eye and the reproductive system can be used to study the functional role of proteins being part of the kidney filtration barrier. [ABSTRACT FROM AUTHOR]

The International Study of Kidney Disease in Children (ISKDC), begun in 1966, conducted the first international collaborative randomized blinded controlled trial in pediatric nephrology and one of the first in either pediatrics or nephrology. For this trial, the ISKDC developed the criteria, such as those for response and relapse, used today to describe the clinical course of the nephrotic syndrome, and the trial generated the nephropathologic terminology and criteria which largely remain the current standards. Over an approximately 20-year span, the ISKDC followed the natural history and evaluated the therapeutic effectiveness of therapies in over 500 children with the nephrotic syndrome from three continents. It published 14 peer-reviewed studies and several reports and commentaries, many of which helped create current standards of practice for therapy of childhood nephrotic syndrome and consequently remain highly cited today. The ISKDC continues to be an important model for subsequent collaborative studies and was the impetus for the development of regional and national pediatric nephrology societies leading to the recognition and growth of pediatric nephrology as a separate subspecialty. [ABSTRACT FROM AUTHOR]

Background: Access to care for children with kidney disease is limited in less well-resourced regions of the world and paediatric nephrology (PN) workforce development with good practical skills is critical. Methods: Retrospective review of a PN training program and trainee feedback from 1999 to 2021, based at Red Cross War Memorial Children's Hospital (RCWMCH), University of Cape Town. Results: A regionally appropriate 1–2-year training program enrolled 38 fellows with an initial 100% return rate to their country of origin. Program funding included fellowships from the International Pediatric Nephrology Association (IPNA), International Society of Nephrology (ISN), International Society of Peritoneal Dialysis (ISPD), and the African Paediatric Fellowship Program (APFP). Fellows were trained on both in- and out-patient management of infants and children with kidney disorders. "Hands-on skills" training included examination, diagnosis and management skills, practical insertion of peritoneal dialysis catheters for management of acute kidney injury and kidney biopsies. Of 16 trainees who completed > 1 year of training, 14 (88%) successfully completed subspecialty exams and 9 (56%) completed a master's degree with a research component. PN fellows reported that their training was appropriate and enabled them to make a difference in their respective communities. Conclusions: This training program has successfully equipped African physicians with the requisite knowledge and skills to provide PN services in resource-constrained areas for children with kidney disease. The provision of funding from multiple organizations committed to paediatric kidney disease has contributed to the success of the program, along with the fellows' commitment to build PN healthcare capacity in Africa. [ABSTRACT FROM AUTHOR]

Background: IgA vasculitis is the most common vasculitis in children and is often complicated by acute nephritis (IgAVN). Risk of chronic kidney disease (CKD) among children with IgAVN remains unknown. This study aimed to describe the clinical management and kidney outcomes in a large cohort of children with IgAVN. Methods: This observational cohort study used the PEDSnet database to identify children diagnosed with IgAV between January 1, 2009, and February 29, 2020. Demographic and clinical characteristics were compared among children with and without kidney involvement. For children followed by nephrology, clinical course, and management patterns were described. Patients were divided into four categories based on treatment: observation, renin–angiotensin–aldosterone system (RAAS) blockade, corticosteroids, and other immunosuppression, and outcomes were compared among these groups. Results: A total of 6802 children had a diagnosis of IgAV, of whom 1139 (16.7%) were followed by nephrology for at least 2 visits over a median follow-up period of 1.7 years [0.4,4.2]. Conservative management was the most predominant practice pattern, consisting of observation in 57% and RAAS blockade in 6%. Steroid monotherapy was used in 29% and other immunosuppression regimens in 8%. Children receiving immunosuppression had higher rates of proteinuria and hypertension compared to those managed with observation (p < 0.001). At the end of follow-up, 2.6 and 0.5% developed CKD and kidney failure, respectively. Conclusions: Kidney outcomes over a limited follow-up period were favorable in a large cohort of children with IgAV. Immunosuppressive medications were used in those with more severe presentations and may have contributed to improved outcomes. [ABSTRACT FROM AUTHOR]

BACKGROUND: Eculizumab is approved for the treatment of atypical hemolytic uremic syndrome (aHUS). Its use off-label is frequently reported. The aim of this study was to describe the broader use and outcomes of a cohort of pediatric patients exposed to eculizumab. METHODS: A retrospective, cohort analysis was performed on the clinical and biomarker characteristics of eculizumab-exposed patients < 25 years of age seen across 21 centers of the Pediatric Nephrology Research Consortium. Patients were included if they received at least one dose of eculizumab between 2008 and 2015. Traditional summary statistics were applied to demographic and clinical data. RESULTS: A total of 152 patients were identified, mean age 9.1 (+/-6.8) years. Eculizumab was used off-label in 44% of cases. The most common diagnoses were aHUS (47.4%), Shiga toxin-producing Escherichia coli HUS (12%), unspecified thrombotic microangiopathies (9%), and glomerulonephritis (9%). Genetic testing was available for 60% of patients; 20% had gene variants. Dosing regimens were variable. Kidney outcomes tended to vary according to diagnosis. Infectious adverse events were the most common adverse event (33.5%). No cases of meningitis were reported. Nine patients died of noninfectious causes while on therapy. CONCLUSIONS: This multi-center retrospective cohort analysis indicates that a significant number of children and young adults are being exposed to C5 blockade for off-label indications. Dosing schedules were highly variable, limiting outcome conclusions. Attributable adverse events appeared to be low. Cohort mortality (6.6%) was not insignificant. Prospective studies in homogenous disease cohorts are needed to support the role of C5 blockade in kidney outcomes.

The authors talk about the International Pediatric Nephrology Association (IPNA)-European Society for Paediatric Nephrology (ESPN) Junior Master Class program. Topics include purpose of launching the program in 2014, components of the program, and demographic characteristics of IPNA-ESPN Junior Master Class participants. Participants' feedback evaluation is also mentioned.

The authors discuss barriers for clinical trials including the ethical approach to acquiring high level evidence for optimal decision making, lack of funding, paucity of partners, low interest from pharmaceutical industry, regulatory issues, under-recognition of children's rights and autonomy and attitudes of patients/caregivers and providers. Topics include differences between pediatric subspecialties, examples of some pediatric nephrology studies, and ethical considerations and harms.

Point-of-care ultrasound (POCUS) has evolved in recent years in clinical practice, helping in early bedside diagnosis of important etiologies. Many medical schools and training programs are integrating POCUS into their curriculum. Especially with the technological advances of newer handheld ultrasound devices, POCUS has now become a component adjunct to clinical examination, in the clinic and bedside in critical care units. The diagnostic utility of POCUS lies both in early identification of critical kidney disease, and also extra-renal pathologies from a focused cardiac ultrasound, lung ultrasound, and integrated fluid assessment. There is a need to incorporate POCUS in training in pediatric nephrology and establish competency standard criteria. This review shall cover how POCUS helps in enhancing patient care in pediatric kidney disorders and critical children, and the recent advances. [ABSTRACT FROM AUTHOR]

Background: Rhabdomyolysis, the breakdown of skeletal muscles following an insult or injury, has been established as a possible complication of SARS-CoV-2 infection. Despite being highly effective in preventing COVID-19-related morbidity and mortality, several cases of COVID-19 mRNA vaccination-induced rhabdomyolysis have been identified. We provide the second description of a pediatric case of severe rhabdomyolysis presenting after COVID-19 mRNA vaccination. Case: diagnosis/treatment A 16-year-old male reported to the emergency department with a 2-day history of bilateral upper extremity myalgias and dark urine 2 days after his first dose of COVID-19 vaccine (Pfizer-BioNtech). The initial blood work showed an elevated creatinine kinase (CK) of 141,300 units/L and a normal creatinine of 69 umol/L. The urinalysis was suggestive of myoglobinuria, with the microscopy revealing blood but no red blood cells. Rhabdomyolysis was diagnosed, and the patient was admitted for intravenous hydration, alkalinization of urine, and monitoring of kidney function. CK levels declined with supportive care, while his kidney function remained normal, and no electrolyte abnormalities developed. The patient was discharged 5 days after admission as his symptoms resolved. Conclusion: While vaccination is the safest and most effective way to prevent morbidity from COVID-19, clinicians should be aware that rhabdomyolysis could be a rare but treatable adverse event of COVID-19 mRNA vaccination. With early recognition and diagnosis and supportive management, rhabdomyolysis has an excellent prognosis. [ABSTRACT FROM AUTHOR]

Background: Pediatric chronic disease impacts the affected child and their family structure. There is limited literature investigating the psychosocial impact of nephrotic syndrome on families. Methods: Caregivers of children with nephrotic syndrome completed two validated surveys: (1) Impact on Family (IOF) that evaluates the family impact (degree to which family is affected by a pediatric chronic illness) and (2) Coping Health Inventory for Parents (CHIP) that examines the coping patterns used by caregivers. Linear regression models were utilized to determine predictors of perceived family impact and coping patterns. Results: Seventy-five caregivers of a child with nephrotic syndrome completed the surveys. On a scale from low impact to significant impact to very serious impact, results indicated that nephrotic syndrome had a significant impact on families (mean revised IOF total score 33.04 ± 9.38). Families in the steroid-resistant nephrotic syndrome (SRNS) group reported a higher financial impact compared to the steroid-sensitive nephrotic syndrome (SSNS) group (p = 0.03). Families in the frequently relapsing group (FRNS) reported a higher impact on the caregiver's ability to cope with the child's condition compared to the SRNS group (p = 0.02). Tacrolimus use was associated with increasing the perceived family impact (β = 4.76, p = 0.046). CHIP scores indicated that caregivers did not cope well with family integration (component I) but coped well with social support (component II) and communication (component III). Conclusions: Childhood nephrotic syndrome has a significant overall perceived impact on the family, and caregivers did not cope well regarding strengthening their family life. These findings can be used as outcome measures for future intervention studies to find solutions that would decrease the perceived family burden. A higher resolution version of the Graphical abstract is available as Supplementary information [ABSTRACT FROM AUTHOR]

Pediatric patients on kidney replacement therapy (KRT) are among the most vulnerable during large-scale disasters, either natural or man-made. Hemodialysis (HD) treatments may be impossible because of structural damage and/or shortage of medical supplies, clean water, electricity, and healthcare professionals. Lack of peritoneal dialysis (PD) solutions and increased risk of infectious/non-infectious complications may make PD therapy challenging. Non-availability of immunosuppressants and increased risk of infections may result in graft loss and deaths of kidney transplant recipients. Measures to mitigate these risks must be considered before, during, and after the disaster including training of staff and patients/caregivers to cope with medical and logistic problems. Soon after a disaster, if the possibility of performing HD or PD is uncertain, patients should be directed to other centers, or the duration and/or number of HD sessions or the PD prescription adapted. In kidney transplant recipients, switching among immunosuppressants should be considered in case of non-availability of the medications. Post-disaster interventions target treating neglected physical and mental problems and also improving social challenges. All problems experienced by pediatric KRT patients living in the affected area are applicable to displaced patients who may also face extra risks during their travel and also at their destination. The need for additional local, national, and international help and support of non-governmental organizations must be anticipated and sought in a timely manner. [ABSTRACT FROM AUTHOR]

Background: Initiation of continuous kidney replacement therapy (CKRT) greater than 20% fluid overload is associated with increased morbidity and mortality. We aimed to reduce the number of patients initiated on CKRT greater than 20% fluid overload by 50% in one year by implementation of a quality improvement initiative. Methods: This is a prospective quality improvement study set in a pediatric ICU of an urban children's hospital of patients initiated on CKRT over 2 years. The intervention included creation of an electronic health record order for daily calculation of net percent fluid overload, incorporation into daily rounds, and education programs tailored to physicians and bedside nursing. We measured adherence with the new order set, percent fluid overload at CKRT initiation, days on CKRT, timing of first nephrology consultation, and death prior to discharge. Results: A total of 32% of patients were initiated on CKRT greater than 20% fluid overload pre-initiative and 9% post-initiative, a 72% reduction over 13 months. Patients initiated on CKRT greater than 20% fluid overload had median CKRT course of 8 (IQR 4–14) vs. 22 days (IQR 13.5–62). Conclusion: Creating a system using EHR with education may reduce initiation of CKRT after development of severe fluid overload. A higher resolution version of the Graphical abstract is available as Supplementary information. [ABSTRACT FROM AUTHOR]

Learning health systems (LHS) align science, informatics, incentives, and culture for continuous improvement and innovation. In this organizational system, best practices are seamlessly embedded in the delivery process, and new knowledge is captured as an integral byproduct of the care delivery experience aimed to transform clinical practice and improve patient outcomes. The objective of this review is to describe how building better health systems that integrate clinical care, improvement, and research as part of an LHS can improve care within pediatric nephrology. This review will provide real-world examples of how this system can be established in a single center and across multiple centers as learning health networks. [ABSTRACT FROM AUTHOR]

The article highlights clinical guidelines for the management of nephrotic syndrome. Topics mentioned include the randomized controlled trials for the use of corticosteroid therapy in the management of first episode of nephrotic syndrome, the use of prednisone for the therapy of relapses, and the importance of managing patients with frequent relapse and who are dependent with steroid to lessen the risk of morbidity and the side effects of immunosuppresive medications.

Background: To improve pre-emptive kidney transplantation (PKT) in children and limit starting dialysis in an emergency, we aimed to describe nephrology care trajectories pre-CKD stage 5. Methods: We included all children in France who, between 2010 and 2016, started kidney replacement therapy (KRT): standard dialysis (reference group) and emergency dialysis or PKT. We identified four pre-CKD stage 5 nephrology care trajectories before KRT that were extracted from the national exhaustive medical-administrative database and used logistic regression to explore associations between patient characteristics, care trajectories, and KRT initiation. Results: Six hundred forty-three pediatric patients started KRT in France; 406 started dialysis and 30.5% emergency dialysis. The "optimal" care trajectory encompassed 179 patients, 82.7% with at least 18 months nephrology follow-up. Conversely, the "no care" trajectory encompassed 118 patients with no nephrology follow-up before KRT. The "severe" trajectory encompassed 128 patients; 93% hospitalized more than once a year and 18% in an intensive care unit. Finally, the "irregular" trajectory encompassed 127 patients, 77% and 46% with irregular laboratory monitoring and CKD drug delivery, respectively. With the "optimal" trajectory as the reference, probability of emergency dialysis was higher with the "irregular" and "no care" trajectories (odds ratio 3.02 [95% confidence interval 1.18–7.66] and 26.5 [10.8–64.8], respectively), and PKT was reduced with the "severe" trajectory (0.43 [0.23–0.82]). Conclusion: We identified a group of patients with irregular follow-up who may benefit the most from interventions aiming at improving adherence to treatment and earlier diagnosis of their CKD to improve access to PKT. A higher resolution version of the Graphical abstract is available as Supplementary information. [ABSTRACT FROM AUTHOR]

Background: In recent years, several studies have been published on the prognosis of children with congenital solitary kidney (CSK), with controversial results, and a worldwide consensus on management and follow-up is lacking. In this consensus statement, the Italian Society of Pediatric Nephrology summarizes the current knowledge on CSK and presents recommendations for its management, including diagnostic approach, nutritional and lifestyle habits, and follow-up. Summary of the recommendations: We recommend that any antenatal suspicion/diagnosis of CSK be confirmed by neonatal ultrasound (US), avoiding the routine use of further imaging if no other anomalies of kidney/urinary tract are detected. A CSK without additional abnormalities is expected to undergo compensatory enlargement, which should be assessed by US. We recommend that urinalysis, but not blood tests or genetic analysis, be routinely performed at diagnosis in infants and children showing compensatory enlargement of the CSK. Extrarenal malformations should be searched for, particularly genital tract malformations in females. An excessive protein and salt intake should be avoided, while sport participation should not be restricted. We recommend a lifelong follow-up, which should be tailored on risk stratification, as follows: low risk: CSK with compensatory enlargement, medium risk: CSK without compensatory enlargement and/or additional CAKUT, and high risk: decreased GFR and/or proteinuria, and/or hypertension. We recommend that in children at low-risk periodic US, urinalysis and BP measurement be performed; in those at medium risk, we recommend that serum creatinine also be measured; in high-risk children, the schedule has to be tailored according to kidney function and clinical data. [ABSTRACT FROM AUTHOR]

Background: Coronavirus disease-2019 (COVID-19) has been challenging for patients and medical staff. Radical changes have been needed to prevent disruptions in patient care and medical education. Methods: A web-based survey was sent to European Society for Pediatric Nephrology (ESPN) members via the ESPN mailing list to evaluate the effects of the COVID-19 pandemic on delivery of pediatric nephrology (PN) care and educational activities. There were ten questions with subheadings. Results: Seventy-six centers from 24 countries completed the survey. The time period was between the beginning of the pandemic and May 30, 2020. The number of patients admitted in PN wards and outpatient clinics were significantly decreased (2.2 and 4.5 times, respectively). Telemedicine tools, electronic prescriptions, online applications for off-label drugs, and remote access to laboratory/imaging results were used in almost half of the centers. Despite staff training and protective measures, 33% of centers reported COVID-19 infected staff, and 29% infected patients. Difficulties in receiving pharmaceuticals were reported in 25% of centers. Sixty percent of centers suspended living-related kidney transplantation, and one-third deceased-donor kidney transplantation. Hands-on education was suspended in 91% of medical schools, and face-to-face teaching was replaced by online systems in 85%. Multidisciplinary training in PN was affected in 54% of the centers. Conclusions: This survey showed a sharp decline in patient admissions and a significant decrease in kidney transplantation. Telemedicine and online teaching became essential tools, requiring integration into the current system. The prolonged and fluctuating course of the pandemic may pose additional challenges necessitating urgent and rational solutions. [ABSTRACT FROM AUTHOR]

Background: Genetic kidney diseases contribute a significant portion of kidney diseases in children and young adults. Nephrogenetics is a rapidly evolving subspecialty; however, in the clinical setting, increased use of genetic testing poses implementation challenges. Consequently, we established a national nephrogenetics clinic to apply a multidisciplinary model. Methods: Patients were referred from different pediatric or adult nephrology units across the country if their primary nephrologist suspected an undiagnosed genetic kidney disease. We determined the diagnostic rate and observed the effect of diagnosis on medical care. We also discuss the requirements of a nephrogenetics clinic in terms of logistics, recommended indications for referral, and building a multidisciplinary team. Results: Over 24 months, genetic evaluation was completed for a total of 74 unrelated probands, with an age range of 10 days to 72 years. The most common phenotypes included congenital anomalies of the kidneys and urinary tract, nephrotic syndrome or unexplained proteinuria, nephrocalcinosis/nephrolithiasis, tubulopathies, and unexplained kidney failure. Over 80% of patients were referred due to clinical suspicion of an undetermined underlying genetic diagnosis. A molecular diagnosis was reached in 42/74 probands, yielding a diagnostic rate of 57%. Of these, over 71% of diagnoses were made via next generation sequencing (gene panel or exome sequencing). Conclusions: We identified a substantial fraction of genetic kidney etiologies among previously undiagnosed individuals which influenced subsequent clinical management. Our results support that nephrogenetics, a rapidly evolving field, may benefit from well-defined multidisciplinary co-management administered by a designated team of nephrologist, geneticist, and bioinformatician. A higher resolution version of the Graphical abstract is available as Supplementary information [ABSTRACT FROM AUTHOR]

The authors reflect on the updates in the quest for precision medicine for individualized care of patients. Topics include the aim of precision medicine to identify the genetic and environmental factors contributing to the phenotypic severity, the issue of whether a kidney failure patient can be treated with peritoneal dialysis (PD), and the use of genome-wide association studies (GWAS) to identify the phenotypic diversity of a disorder.

Adult nephrologists often look after patients who have been diagnosed with kidney disease in childhood. This does present unique challenges to the adult nephrologist, who may be unfamiliar with the underlying cause of kidney disease as well as the complications of chronic kidney disease (CKD) that may have accumulated during childhood. This review discusses common causes of childhood CKD, in particular congenital anomalies of the kidney and urinary tract (CAKUT), autosomal dominant tubulointerstitial kidney disease (ADTKD), polycystic kidney disease, hereditary stone disease, nephrotic syndrome and atypical haemolytic uraemic syndrome. The long-term consequences of childhood CKD, such as the cardiovascular consequences, cognition and education as well as bone health, nutrition and growth are also discussed. [ABSTRACT FROM AUTHOR]

Background: Nearly 50% of the world population and 60% of children aged 0 to 14 years live in low- or lower-middle–income countries. Paediatric nephrology (PN) in these countries is not a priority for allocation of limited health resources. This article explores advancements made and persisting limitations in providing optimal PN services to children in such under-resourced areas (URA). Methods: Medline, PubMed and Google Scholar online databases were searched for articles pertaining to PN disease epidemiology, outcome, availability of services and infrastructure in URA. The ISN and IPNA offices were contacted for data, and two online questionnaire surveys of IPNA membership performed. Regional IPNA members were contacted for further detailed information. Results: There is a scarcity of published data from URA; where available, prevalence of PN diseases, managements and outcomes are often reported to be different from high income regions. Deficiencies in human resources, fluoroscopy, nuclear imaging, immunofluorescence, electron microscopy and genetic studies were identified. Several drugs and maintenance kidney replacement therapy are inaccessible to the majority of patients. Despite these issues, regional efforts with support from international bodies have led to significant advances in PN services and infrastructure in many URA. Conclusions: Equitable distribution and affordability of PN services remain major challenges in URA. The drive towards acquisition of regional data, advocacy to local government and non-government agencies and partnership with international support bodies needs to be continued. The aim is to optimise and achieve global parity in PN training, investigations and treatments, initially focusing on preventable and reversible conditions. [ABSTRACT FROM AUTHOR]

Background: Information on the course of SARS-CoV-2 infection in children with chronic kidney disease (CKD) is limited. Methods: We retrospectively reviewed the presentation and outcomes of SARS-CoV-2 infection in patients with CKD followed at any of the four pediatric nephrology centers in New Delhi from April 2020 to June 2021. Outcomes, including cardiopulmonary and renal complications, were reported in relation to underlying disease category and illness severity at presentation. Results: Underlying illness in 88 patients included nephrotic syndrome (50%), other CKD stages 1–4 (18.2%), CKD 5D (17%), and CKD 5T (14.8%). Thirty-two of 61 patients with symptomatic COVID-19 and 9/27 asymptomatic patients were admitted for median 10 (interquartile range 7–15) days. Seventeen (19.3%) patients developed moderate or severe COVID-19. Systemic complications, observed in 30 (34.1%), included acute kidney injury (AKI, 34.2%), COVID-19 pneumonia (15.9%), unrelated pulmonary disease (2.3%), and shock (4.5%). Nineteen (21.6%) had severe complications (AKI stage 2–3, encephalopathy, respiratory failure, shock). Eight (11%) of twelve (16.4%) patients with severe AKI required dialysis. Three (3.4%) patients, two with steroid-resistant nephrotic syndrome in relapse and one with CKD 1–4, died due to respiratory failure. Univariate logistic regression indicated that patients presenting with nephrotic syndrome in relapse or moderate to severe COVID-19 were at risk of AKI (respective odds ratio, 95%CI: 3.62, 1.01–12.99; 4.58, 1.06–19.86) and/or severe complications (respective odds ratio, 95%CI: 5.92, 1.99–17.66; 61.2, 6.99–536.01). Conclusions: Children with CKD presenting with moderate-to-severe COVID-19 or in nephrotic syndrome relapse are at risk of severe complications, including severe AKI and mortality. A higher resolution version of the Graphical abstract is available as Supplementary information [ABSTRACT FROM AUTHOR]

Background Although there are data showing that the frequency of hypertension increases in adults with migraine, there has been no study on this subject in children. In this study, we aimed to evaluate the presence of hypertension in children with migraine by performing ambulatory blood pressure monitoring (ABPM). Methods Thirty-seven children diagnosed with migraine and 30 healthy controls were evaluated between January 2015 and March 2016. Demographic data, clinical and laboratory features, and physical examination findings were recorded for both groups. Office blood pressure was measured for all children, and each also underwent ABPM. The two groups were compared in terms of ambulatory blood pressure parameters. Results The mean age was 13.3 and 13.1 years and the proportion of females was 73% and 60% in the migraine and control groups, respectively. Although the frequency of hypertension was not higher, abnormal ABPM patterns were found to be significantly more frequent in the migraine group (migraine, 45.9%; control, 16.7%; p, 0.018). Nighttime mean arterial blood pressure, nighttime diastolic blood pressure, and non-dipping pattern were higher in children with migraine than those in the control group (p < 0.05). Conclusions These results suggest that ambulatory blood pressure abnormalities may be present in almost half of patients with migraine. Therefore, we suggest that ABPM should be performed even if the office blood pressure measurements of children diagnosed with migraine are normal., Author(s): Songül Yilmaz [sup.1] , Sare Gülfem Özlü [sup.1] , Aysegül Nese Çitak Kurt [sup.2] Author Affiliations: (1) grid.449874.2, 0000 0004 0454 9762, Department of Pediatric Nephrology, Ankara Yildirim Beyazit [...]

Autosomal recessive polycystic kidney disease (ARPKD) is a rare but highly relevant disorder in pediatric nephrology. This genetic disease is mainly caused by variants in the PKHD1 gene and is characterized by fibrocystic hepatorenal phenotypes with major clinical variability. ARPKD frequently presents perinatally, and the management of perinatal and early disease symptoms may be challenging. This review discusses aspects of early manifestations in ARPKD and its clincial management with a special focus on kidney disease. [ABSTRACT FROM AUTHOR]

The article offers information on recent changes to Pediatric Nephrology, focusing on improvements to better serve the journal's readers, contributors, and the International Pediatric Nephrology Association. Topics include changes to article types, such as the retirement of Clinical Quizzes and Brief Reports, replaced by a new category named Clinical Insights.

Idiopathic nephrotic syndrome is the most frequent glomerular disease in children in most parts of the world. Children with steroid-sensitive nephrotic syndrome (SSNS) generally have a good prognosis regarding the maintenance of normal kidney function even in the case of frequent relapses. The course of SSNS is often complicated by a high rate of relapses and the associated side effects of repeated glucocorticoid (steroid) therapy. The following recommendations for the treatment of SSNS are based on the comprehensive consideration of published evidence by a working group of the German Society for Pediatric Nephrology (GPN) based on the systematic Cochrane reviews on SSNS and the guidelines of the KDIGO working group (Kidney Disease - Improving Global Outcomes). [ABSTRACT FROM AUTHOR]

Abstract title BONE MINERAL DENSITY OF CHILDREN WITH NEPHROTIC SYNDROME ADMITTED TO YANGON CHILDREN HOSPITAL [ABSTRACT FROM AUTHOR]

Onco-nephrology has been a growing field within the adult nephrology scope of practice. Even though pediatric nephrologists have been increasingly involved in the care of children with different forms of malignancy, there has not been an emphasis on developing special expertise in this area. The fast pace of discovery in this field, including the development of new therapy protocols with their own kidney side effects and the introduction of the CD19-targeted chimeric antigen receptor T cell (CAR-T) therapy, has introduced new challenges for general pediatric nephrologists because of the unique effects of these treatments on the kidney. Moreover, with the improved outcomes in children receiving cancer therapy come an increased number of survivors at risk for chronic kidney disease related to both their cancer diagnosis and therapy. Therefore, it is time for pediatric onco-nephrology to take its spot on the expanding subspecialties map in pediatric nephrology. [ABSTRACT FROM AUTHOR]

Pediatric nephrology has a history rooted in pediatric advocacy and has made numerous contributions to child health policy affecting pediatric kidney diseases. Despite this progress, profound social disparities remain for marginalized and socially vulnerable children with kidney disease. Different risk factors, such as genetic predisposition, environmental factors, social risk factors, or health care access influence the emergence and progression of pediatric kidney disease, as well as access to life-saving interventions, leading to disparate outcomes. This review will summarize the breadth of literature on social determinants of health in children with kidney disease worldwide and highlight policy-based initiatives that mitigate the adverse social factors to generate greater equity in pediatric kidney disease. [ABSTRACT FROM AUTHOR]

A quiz about the case of a 19-month-old girl with chronic kidney disease and hepatic dysfunction is presented.