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Torque teno (TTV) viral load as a biomarker of immunosuppressive strength after kidney transplantation in children
Ryszard Grenda
doi : 10.1007/s00467-020-04607-2
Pediatr Nephrol 36, 1–3 (2021).
Conversations for the future in the follow-up of antenatally diagnosed renal pelvicalyceal dilatation
Aniruddh V. Deshpande
doi : 10.1007/s00467-020-04766-2
Pediatr Nephrol 36, 5–8 (2021).
Mitochondrial DNA mutations in renal disease: an overview
Larissa P. GoversHakan R. TokaDetlef Bockenhauer
doi : 10.1007/s00467-019-04404-6
Pediatr Nephrol 36, 9–17 (2021).
Kidneys have a high energy demand to facilitate the reabsorption of the glomerular filtrate. For this reason, renal cells have a high density of mitochondria. Mitochondrial cytopathies can be the result of a mutation in both mitochondrial and nuclear DNA. Mitochondrial dysfunction can lead to a variety of renal manifestations. Examples of tubular manifestations are renal Fanconi Syndrome, which is often found in patients diagnosed with Kearns-Sayre and Pearson’s marrow-pancreas syndrome, and distal tubulopathies, which result in electrolyte disturbances such as hypomagnesemia. Nephrotic syndrome can be a glomerular manifestation of mitochondrial dysfunction and is typically associated with focal segmental glomerular sclerosis on histology. Tubulointerstitial nephritis can also be seen in mitochondrial cytopathies and may lead to end-stage renal disease. The underlying mechanisms of these cytopathies remain incompletely understood; therefore, current therapies focus mainly on symptom relief. A better understanding of the molecular disease mechanisms is critical in order to improve treatments.
Membranous nephropathy: diagnosis, treatment, and monitoring in the post-PLA2R era
Luisa Safar-BoueriAlbina PiyaRivka Ayalon
doi : 10.1007/s00467-019-04425-1
Pediatr Nephrol 36, 19–30 (2021).
Membranous nephropathy (MN) is an immune complex-mediated cause of the nephrotic syndrome that can occur in all age groups, from infants to the very elderly. However, nephrotic syndrome in children is more frequently caused by conditions such as minimal change disease or focal segmental glomerulosclerosis, and much less commonly by MN. While systemic conditions such as lupus or infections such as hepatitis B may more commonly be associated as secondary causes with MN in the younger population, primary or “idiopathic” MN has generally been considered a disease of adults. Autoantibodies both to the M-type phospholipase A2 receptor (PLA2R) and to thrombospondin type-1 domain-containing 7A (THSD7A), initially described in adult MN, have now been identified in children and adolescents with MN and serve as a useful diagnostic and monitoring tool in this younger population as well. Whereas definitive therapy for secondary forms of MN should be targeted at the underlying cause, immunosuppressive therapy is often necessary for primary disease. Rituximab has been successfully used in the treatment of MN, and is likely effective in children with MN as well, although dosing in the pediatric population is not well established. This review highlights the new findings in adult and pediatric MN since last reviewed in this journal.
Does HLA matching matter in the modern era of renal transplantation?
Jon Jin KimSusan V FuggleStephen D Marks
doi : 10.1007/s00467-019-04393-6
Pediatr Nephrol 36, 31–40 (2021).
Children with end-stage kidney disease should be offered the best chance for future survival which ideally would be a well-matched pre-emptive kidney transplant. Paediatric and adult practice varies around the world depending on geography, transplant allocation schemes and different emphases on living (versus deceased) donor renal transplantation. Internationally, paediatric patients often have priority in allocation schemes and younger donors are preferentially allocated to paediatric recipients. HLA matching can be difficult and may result in longer waiting times. Additionally, with improved surgical techniques and modern immunosuppressive regimens, how important is the contribution of HLA matching to graft longevity? In this review, we discuss the relative importance of HLA matching compared with donor quality; and long-term patient outcomes including re-transplantation rates. We share empirical evidence that will be useful for clinicians and families to make decisions about best donor options. We discuss why living donation still provides the best allograft survival outcomes and what to do in the scenario of a highly mismatched living donor.
CKD-MBD post kidney transplantation
Dieter HaffnerMaren Leifheit-Nestler
doi : 10.1007/s00467-019-04421-5
Pediatr Nephrol 36, 41–50 (2021).
Complications of chronic kidney disease-associated mineral and bone disorders (CKD-MBD) are frequently observed in pediatric kidney transplant recipients and are associated with high morbidity, including growth failure, leg deformities, bone pain, fractures, osteonecrosis, and vascular calcification. Post-transplant CKD-MBD is mainly due to preexisting renal osteodystrophy and cardiovascular changes at the time of transplantation, glucocorticoid treatment, and reduced graft function. In addition, persistent elevated levels of parathyroid hormone (PTH) and fibroblast growth factor 23 may cause hypophosphatemia, resulting in impaired bone mineralization. Patient monitoring should include assessment of growth, leg deformities, and serum levels of calcium, phosphate, magnesium, alkaline phosphatase, 25-hydroxyvitamin D, and PTH. Therapy should primarily focus on regular physical activity, preservation of transplant function, and steroid-sparing immunosuppressive protocols. In addition, adequate monitoring and treatment of vitamin D and mineral metabolism including vitamin D supplementation, oral phosphate, and/or magnesium supplementation, in case of persistent hypophosphatemia/hypomagnesemia, and treatment with active vitamin D in cases of persistent secondary hyperparathyroidism. The latter should be done using the minimum PTH-suppressive dosages aiming at the recommended CKD stage-dependent PTH target range. Finally, treatment with recombinant human growth hormone should be considered in patients lacking catch-up growth within the first year after transplantation.
Management of dyslipidemia in pediatric renal transplant recipients
Margret E. BockLeslie WallJens Goebel
doi : 10.1007/s00467-019-04428-y
Pediatr Nephrol 36, 51–63 (2021).
Dyslipidemia after kidney transplantation is a common complication that has historically been underappreciated, especially in pediatric recipients. It is also a major modifiable risk factor for cardiovascular disease, a top cause of morbidity and mortality of transplant patients. While most knowledge about post-transplant dyslipidemia has been generated in adults, recommendations and treatment strategies also exist for children and are presented in this review. Awareness of these applicable guidelines and approaches is required, but not sufficient, for the reliable management of dyslipidemia in our patients, and additional needs and opportunities for comprehensive care in this area (e.g., quality improvement) are outlined.
What does acute kidney injury and dark red-brown urine that appear after bone marrow transplantation tell us: Questions
Demet AlaygutG?kçen ErfidanBelde Kasap Demir
doi : 10.1007/s00467-020-04589-1
Pediatr Nephrol 36, 65–66 (2021).
What does acute kidney injury and dark red-brown urine that appear after bone marrow transplantation tell us: Answers
Demet AlaygutG?kçen ErfidanBelde Kasap Demir
doi : 10.1007/s00467-020-04592-6
Pediatr Nephrol 36, 67–70 (2021).
Hypercalcemia, hyperuricemia, and kidney dysfunction in a 35-month-old boy: Questions
Qiang LinYanhong LiXiaozhong Li
doi : 10.1007/s00467-020-04601-8
Pediatr Nephrol 36, 71–72 (2021).
Hypercalcemia, hyperuricemia, and kidney dysfunction in a 35-month-old boy: Answers
Qiang LinYanhong LiXiaozhong Li
doi : 10.1007/s00467-020-04603-6
Pediatr Nephrol 36, 73–76 (2021).
A rare cause of nephrocalcinosis in an infant: Questions
Francisco Antonio Nieto-VegaRafael Mart?n-MasotV?ctor Manuel Navas-L?pez
doi : 10.1007/s00467-020-04608-1
Pediatr Nephrol 36, 77–78 (2021).
A rare cause of nephrocalcinosis in an infant: Answers
Francisco Antonio Nieto-VegaRafael Mart?n-MasotV?ctor Manuel Navas-L?pez
doi : 10.1007/s00467-020-04615-2
Pediatr Nephrol 36, 79–81 (2021).
Efficacy and safety of an innovative prolonged-release combination drug in patients with distal renal tubular acidosis: an open-label comparative trial versus standard of care treatments
Aurélia Bertholet-ThomasCatherine GuittetLuc-André Granier
doi : 10.1007/s00467-020-04693-2
Pediatr Nephrol 36, 83–91 (2021).
Distal renal tubular acidosis (dRTA), due to impaired acid secretion in the urine, can lead to severe long-term consequences. Standard of care (SoC) oral alkalizers, requiring several daily intakes, are currently used to restore normal plasma bicarbonate levels. A new prolonged-release formulation, ADV7103, has been developed to achieve a sustained effect with an improved dosing scheme.
Impact of steroids and steroid-sparing agents on quality of life in children with nephrotic syndrome
Shawn KhullarTonny BanhRulan S. Parekh
doi : 10.1007/s00467-020-04684-3
Pediatr Nephrol 36, 93–102 (2021).
Steroids and/or steroid-sparing medications are commonly used for nephrotic syndrome treatment; however, the impact of these medications on health-related quality of life over time is not well described.
Fluid restriction versus volume expansion in children with diarrhea-associated HUS: a retrospective observational study
Pablo BonanyManuel D. BilkisEzequiel Monteverde
doi : 10.1007/s00467-020-04673-6
Pediatr Nephrol 36, 103–109 (2021).
Fifty percent of patients with typical diarrhea-associated hemolytic uremic syndrome (D+HUS) require kidney replacement therapy (KRT). In these patients, dehydration worsens disease prognosis. We evaluated dialysis requirement, presence of seizures, and mortality rate among patients diagnosed with D+HUS treated with volume expansion (VE) versus fluid restriction (FR).
Prediction of kidney failure in children with chronic kidney disease and obstructive uropathy
Yuri V. Sebasti?oJennifer N. CooperDaryl J. McLeod
doi : 10.1007/s00467-020-04661-w
Pediatr Nephrol 36, 111–118 (2021).
Obstructive uropathy (OU) is a leading cause of pediatric kidney injury. Accurate prediction of kidney disease progression may improve clinical outcomes. We aimed to examine discrimination and accuracy of a validated kidney failure risk equation (KFRE), previously developed in adults, in children with OU.
Pre-pregnancy obesity and risk of congenital abnormalities of the kidney and urinary tract (CAKUT)—systematic review, meta-analysis and ecological study
Lyda Jadresi?Howard AuDorothea Nitsch
doi : 10.1007/s00467-020-04679-0
Pediatr Nephrol 36, 119–132 (2021).
There is increasing evidence that maternal obesity is associated with several structural birth defects. Congenital abnormalities of the kidney and urinary tract (CAKUT) account for 30 to 50% of children starting kidney replacement therapy (KRT). We conducted a systematic review, meta-analysis and ecological study to explore the relationship between maternal obesity and CAKUT.
Phase 1, single-dose study to assess the safety, tolerability, pharmacokinetics, and pharmacodynamics of etelcalcetide in pediatric patients with secondary hyperparathyroidism receiving hemodialysis
Winnie SohnIsidro B. SaluskyBradley A. Warady
doi : 10.1007/s00467-020-04599-z
Pediatr Nephrol 36, 133–142 (2021).
Data on the safety, efficacy of etelcalcetide in children with secondary hyperparathyroidism (sHPT) are limited.
Effects of COVID-19 pandemic on pediatric kidney transplant in the United States
Olga CharnayaTeresa Po-Yu ChiangJacqueline M. Garonzik-Wang
doi : 10.1007/s00467-020-04764-4
Pediatr Nephrol 36, 143–151 (2021).
In March 2020, COVID-19 infections began to rise exponentially in the USA, placing substantial burden on the healthcare system. As a result, there was a rapid change in transplant practices and policies, with cessation of most procedures. Our goal was to understand changes to pediatric kidney transplantation (KT) at the national level during the COVID-19 epidemic.
Torque teno viral load reflects immunosuppression in paediatric kidney-transplanted patients—a pilot study
Phoebe UhlAndreas HeilosKrisztina Rusai
doi : 10.1007/s00467-020-04606-3
Pediatr Nephrol 36, 153–162 (2021).
Chronic deterioration of kidney graft function is related to inadequate immunosuppression (IS). A novel tool to assess the individual net state of IS in transplanted patients might be the monitoring of Torque teno virus (TTV) viral load. TTV is a non-pathogen virus detectable in almost all individuals. TTV level in the peripheral blood has been linked to the immune-competence of its host and should thus reflect IS after solid organ transplantation.
Be aware of acute kidney injury in critically ill children with COVID-19
Xiaowen WangXingfeng ChenJianbo Shao
doi : 10.1007/s00467-020-04715-z
Pediatr Nephrol 36, 163–169 (2021).
Acute kidney injury (AKI) is a common complication of critically ill adult patients with COVID-19. However, currently, no studies investigate kidney impairment in children with COVID-19. We investigated incidence and treatment of AKI in pediatric patients with COVID-19 in Wuhan Children’s Hospital during the early stages of the COVID-19 pandemic and discuss possible mechanisms of AKI related to SARS-CoV-2 infection.
Acute kidney injury among hospitalized children with cancer
Mengqi XiongLong WangFan Fan Hou
doi : 10.1007/s00467-020-04678-1
Pediatr Nephrol 36, 171–179 (2021).
Few studies to date have analyzed the epidemiology of acute kidney injury (AKI) in children with cancer in developing countries. The aim of this study was to assess the incidence, risk profile and outcomes of AKI in Chinese children hospitalized with cancer.
Cystatin C compared to serum creatinine as a marker of acute kidney injury in critically ill neonates
Eka Laksmi HidayatiMeita Dwi UtamiBambang Tridjaja
doi : 10.1007/s00467-020-04668-3
Pediatr Nephrol 36, 181–186 (2021).
Acute kidney injury (AKI) is one of the most common causes of neonatal morbidity and mortality. Diagnosing AKI in neonates is challenging as it lacks specific signs, symptoms, and biomarkers. However, detecting AKI in critically ill neonates is crucial to determine appropriate management and prevent complications. Cystatin C (CysC) has been recognized as a superior kidney biomarker reflecting kidney function in neonates. The objective of this study is to evaluate the diagnostic value of CysC as an AKI biomarker in critically ill neonates.
Delivery of a nutritional prescription by enteral tube feeding in children with chronic kidney disease stages 2–5 and on dialysis—clinical practice recommendations from the Pediatric Renal Nutrition Taskforce
Lesley ReesVanessa Shawon behalf of the Pediatric Renal Nutrition Taskforce
doi : 10.1007/s00467-020-04623-2
Pediatr Nephrol 36, 187–204 (2021).
The nutritional prescription (whether in the form of food or liquid formulas) may be taken orally when a child has the capacity for spontaneous intake by mouth, but may need to be administered partially or completely by nasogastric tube or gastrostomy device (“enteral tube feeding”). The relative use of each of these methods varies both within and between countries. The Pediatric Renal Nutrition Taskforce (PRNT), an international team of pediatric renal dietitians and pediatric nephrologists, has developed clinical practice recommendations (CPRs) based on evidence where available, or on the expert opinion of the Taskforce members, using a Delphi process to seek consensus from the wider community of experts in the field. We present CPRs for delivery of the nutritional prescription via enteral tube feeding to children with chronic kidney disease stages 2–5 and on dialysis. We address the types of enteral feeding tubes, when they should be used, placement techniques, recommendations and contraindications for their use, and evidence for their effects on growth parameters. Statements with a low grade of evidence, or based on opinion, must be considered and adapted for the individual patient by the treating physician and dietitian according to their clinical judgement. Research recommendations have been suggested. The CPRs will be regularly audited and updated by the PRNT.
COVID-19 associated with onset nephrotic syndrome in a pediatric patient: coincidence or related conditions?
Amado AlvaradoGabriela FranceschiNelson Orta
doi : 10.1007/s00467-020-04724-y
Pediatr Nephrol 36, 205–207 (2021).
COVID-19 is less frequent in children than in adults and affects the former less severely; despite the fact that respiratory symptoms are the most frequent, in some cases unusual manifestations can be seen.
SARS-CoV-2 infection associated with the recurrence of nephrotic syndrome in a Japanese boy
Takuji EnyaKeisuke Sugimoto
doi : 10.1007/s00467-020-04782-2
Pediatr Nephrol 36, 209 (2021).
Relapse rate of nephrotic syndrome in the time of COVID-19
Jérôme HarambatLise AllardAstrid Godron-Dubrasquet
doi : 10.1007/s00467-020-04814-x
Pediatr Nephrol 36, 211–212 (2021).
Correction to: A rare cause of nephrocalcinosis in an infant: Answers
Francisco Antonio Nieto-VegaRafael Mart?n-MasotV?ctor Manuel Navas-L?pez
doi : 10.1007/s00467-020-04651-y
Pediatr Nephrol 36, 213 (2021).
Correction to: Efficacy and safety of an innovative prolonged-release combination drug in patients with distal renal tubular acidosis: an open-label comparative trial versus standard of care treatments
Aurélia Bertholet-ThomasCatherine GuittetLuc-André Granier
doi : 10.1007/s00467-020-04776-0
Pediatr Nephrol 36, 215 (2021).
