One-year access to more than 500 world journals available in the system
-
http://medilib.ir
- Duration of Time : 365 Day
Price : 300$- Special Price : 100$
Importance of clinical practice guidelines to practicing pediatric nephrologists and IPNA survey
Pankaj Hari, Khalid Alhasan, Arvind Bagga, Melvin Bonilla-Felix, Paula Alejandra Coccia, Ali Duzova, Ill-So Ha, Giovanni Montini, Koichi Nakanishi, Susan Samuel, Hong Xu, Olivia Boyer & Dieter Haffner
doi : 10.1007/s00467-021-05105-9
Pediatric Nephrology volume 36, pages3493–3497 (2021)
Clinical practice guidelines (CPGs) are systematically developed statements backed by scientific evidence to assist practitioners in management in clinical practice. An international cross-sectional survey was conducted by the IPNA to examine the perceptions of pediatric nephrologists on guidelines and their usage and to identify important diseases for future clinical practice guidelines (CPGs). The survey found that the majority of pediatric nephrologists find CPGs useful in clinical practice and admitted to using them most of the time. Developing CPGs is challenging and there are standards available to develop trustworthy guidelines. While evidence-based global guidelines are ideal, pediatric nephrologists expressed the desire that they address regional differences. Most respondents (89.2%) to the survey agreed that adult guidelines did not cover the pediatric perspective adequately and 71.4% opined that consensus-based pediatric guidelines can be developed when evidence for the pediatric population is lacking. The development of high-quality practice guidelines requires substantial resources and may not be feasible in resource-poor countries. Adaptation of an existing guideline has been suggested as an alternative and the ADAPTE collaboration provides a systematic approach to adapting guidelines. Several diseases where pediatric guidelines are needed as a priority including IgA and C3 glomerulopathy were identified in the survey. Implementation of guideline-based care is challenging and the survey found that lack of availability of guidelines (43%) and resources (22.8%) are important reasons for poor implementation in lower-middle and low-income countries. Perceived complexity of guidelines, physician attitudes, and lack of training also contribute to non-adherence to guidelines.
Risk stratification for children with a solitary functioning kidney
Sander Groen in’t Woud, Loes F. M. van der Zanden & Michiel F. Schreuder
doi : 10.1007/s00467-021-05168-8
Pediatric Nephrology volume 36, pages3499–3503 (2021)
Cytopenia in autosomal dominant polycystic kidney disease (ADPKD): merely an association or a disease-related feature with prognostic implications?
Pieter Schellekens, Willem Roosens, Isabelle Meyts, Rudi Vennekens, Bert Bammens & Djalila Mekahli
doi : 10.1007/s00467-021-04937-9
Pediatric Nephrology volume 36, pages3505–3514 (2021)
Autosomal dominant polycystic kidney disease (ADPKD) is associated with distinct cytopenias in observational studies; the most consistent and strongest association is seen with alternations in the lymphocytic lineages. Although the underlying mechanism of these associations is unclear, it has been hypothesized to be secondary to sequestration of white blood cells in cystic organs, or related to the uremic environment in chronic kidney disease (CKD). However, since mutations in PKD1 or -2 affect several immunomodulating pathways, cytopenia may well be an unrecognized extrarenal manifestation of ADPKD. Furthermore, many important questions on the clinical implications of this finding and the effect on the disease course in these patients are unanswered. In this review article, we provide an overview of the current evidence on cytopenia in ADPKD and explore the underlying mechanisms of this association and its potential prognostic implications. Based on the current literature, we hypothesize that polycystin deficiency can disturb immune cell homeostasis and that cytopenia is thus an intrinsic feature of ADPKD, related to genetic factors. Taken together, these findings warrant further investigation to establish the exact etiology and role of cytopenia in patients with ADPKD.
Primary coenzyme Q10 nephropathy, a potentially treatable form of steroid-resistant nephrotic syndrome
Weizhen Tan & Rannar Airik
doi : 10.1007/s00467-020-04914-8
Pediatric Nephrology volume 36, pages3515–3527 (2021)
Steroid-resistant nephrotic syndrome (SRNS) is a genetically heterogeneous kidney disease that is the second most frequent cause of kidney failure in the first 2 decades of life. Despite the identification of mutations in more than 39 genes as causing SRNS, and the localization of its pathogenesis to glomerular podocytes, the disease mechanisms of SRNS remain poorly understood and no universally safe and effective therapy exists to treat patients with this condition. Recently, genetic research has identified a subgroup of SRNS patients whose kidney pathology is caused by primary coenzyme Q10 (CoQ10) deficiency due to recessive mutations in genes that encode proteins in the CoQ10 biosynthesis pathway. Clinical and preclinical studies show that primary CoQ10 deficiency may be responsive to treatment with CoQ10 supplements bypassing the biosynthesis defects. Coenzyme Q10 is an essential component of the mitochondrial respiratory chain, where it transports electrons from complexes I and II to complex III. Studies in yeast and mammalian model systems have recently identified the molecular functions of the individual CoQ10 biosynthesis complex proteins, validated these findings, and provided an impetus for developing therapeutic compounds to replenish CoQ10 levels in the tissues/organs and thus prevent the destruction of tissues due to mitochondrial OXPHOS deficiencies. In this review, we will summarize the clinical findings of the kidney pathophysiology of primary CoQ10 deficiencies and discuss recent advances in the development of therapies to counter CoQ10 deficiency in tissues.
The use of diagnostic tools for pediatric AKI: applying the current evidence to the bedside
Dana Fuhrman
doi : 10.1007/s00467-021-04940-0
Pediatric Nephrology volume 36, pages3529–3537 (2021)
Given the known deleterious consequences of acute kidney injury (AKI), exciting recent research efforts have focused on developing strategies for the earlier recognition of AKI in the pediatric population. Recognizing the limitations of serum creatinine, investigators have focused on the study of novel biomarkers and practical bedside tools for identifying patients at risk for AKI prior to a rise in serum creatinine. In PubMed, there are presently over 30 original research papers exploring the use of pediatric AKI risk prediction tools in just the last 2 years. The following review highlights the most recent advances in the literature regarding opportunities to refine our ability to detect AKI early. Importantly, this review discusses how prediction tools including novel urine and serum biomarkers, practical risk stratification tests, renal functional reserve, and electronic medical record alerts may ultimately be applied to routine clinical practice.
Sirtuins play critical and diverse roles in acute kidney injury
Kevin Peasley, Takuto Chiba, Eric Goetzman & Sunder Sims-Lucas
doi : 10.1007/s00467-020-04866-z
Pediatric Nephrology volume 36, pages3539–3546 (2021)
Acute kidney injury (AKI) is an extremely common medical affliction affecting both adult and pediatric patients resulting from hypoxic, nephrotoxic, and septic insults affecting approximately 20% of all hospital patients and up to 50% of patients in the intensive care unit. There are currently no therapeutics for patients who suffer AKI. Much recent work has focused on designing and implementing therapeutics for AKI. This review focuses on a family of enzymes known as sirtuins that play critical roles in regulating many cellular and biological functions. There are 7 mammalian sirtuins (SIRT1–7) that play roles in regulating the acylation of a wide variety of pathways. Furthermore, all but one of the mammalian sirtuins have been shown to play critical roles in mediating AKI based on preclinical studies. These diverse enzymes show exciting potential for therapeutic manipulation. This review will focus on the specific roles of each of the investigated sirtuins and the potential for manipulation of the various sirtuins and their effector pathways in mediating kidney injury.
Attention deficit and hyperactivity disorder and nocturnal enuresis co-occurrence in the pediatric population: a systematic review and meta-analysis
Ana Cecília de Sena Oliveira, Bruno da Silva Athanasio, Flávia Cristina de Carvalho Mrad, Monica Maria de Almeida Vasconcelos, Maicon Rodrigues Albuquerque, Débora Marques Miranda & Ana Cristina Simões e Silva
doi : 10.1007/s00467-021-05083-y
Pediatric Nephrology volume 36, pages3547–3559 (2021)
Attention deficit and hyperactivity/impulsivity disorder (ADHD) and enuresis are common behavioral disorders in childhood, impacting adolescence and adult life. Enuresis (NE) is an incontinence disorder frequently observed in children with ADHD. The relationship between ADHD and NE has been a matter of debate.
Early clinical management of autosomal recessive polycystic kidney disease
Max Christoph Liebau
doi : 10.1007/s00467-021-04970-8
Pediatric Nephrology volume 36, pages3561–3570 (2021)
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.
Neurological involvement in monogenic podocytopathies
Olivia Boyer, Géraldine Mollet & Guillaume Dorval
doi : 10.1007/s00467-020-04903-x
Pediatric Nephrology volume 36, pages3571–3583 (2021)
Genetic studies of hereditary nephrotic syndrome (NS) have identified more than 50 genes that, if mutated, are responsible for monogenic forms of steroid-resistant NS (SRNS), either isolated or syndromic. Most of these genes encode proteins expressed in the podocyte with various functions such as transcription factors, mitochondrial proteins, or enzymes, but mainly structural proteins of the slit diaphragm (SD) as well as cytoskeletal binding and regulator proteins. Syndromic NS is sometimes associated with neurological features. Over recent decades, various studies have established links between the physiology of podocytes and neurons, both morphologically (slit diaphragm and synapse) and functionally (signaling platforms). Variants in genes expressed in different compartments of the podocyte and neurons are responsible for phenotypes associating kidney lesions with proteinuria (mainly Focal and Segmental Glomerulosclerosis (FSGS) or Diffuse Mesangial Sclerosis (DMS)) and central and/or peripheral neurological disorders. The Galloway-Mowat syndrome (GAMOS, OMIM#251300) associates neurological defects, microcephaly, and proteinuria and is caused by variants in genes encoding proteins of various functions (microtubule cytoskeleton regulation (WDR73), regulation of protein synthesis via transfer RNAs (KEOPS and WDR4 complexes)). Pierson syndrome (OMIM#609049) associating congenital nephrotic syndrome and central neurological and ophthalmological anomalies is secondary to variants in LAMB2, involved in glomerular and ocular basement membranes. Finally, Charcot-Marie-Tooth-FSGS (OMIM#614455) combines peripheral sensory-motor neuropathy and proteinuria and arises from INF2 variants, resulting in cytoskeletal polymerization defects. This review focuses on genetic syndromes associating nephrotic range proteinuria and neurological involvement and provides the latest advances in the description of these neuro-renal disorders.
Substance use among adolescents and young adults with chronic kidney disease or kidney failure
Nianzhou Xiao, Hua Chai & Abiodun Omoloja
doi : 10.1007/s00467-021-05001-2
Pediatric Nephrology volume 36, pages3585–3593 (2021)
Substance use, a significant public health issue, is well described in the adult chronic kidney disease (CKD) population. Knowledge about substance use in the adolescent and young adult (AYA) CKD population such as prevalence, impact on kidney function, medication adherence, and psychosocial well-being remain largely unknown. Awareness of and inquiring about substance use is paramount to providing evidence-based care and preparation to transition to adult-focused health services. The authors in this review identify commonly used substances (alcohol, tobacco, marijuana, etc.) and how they impact kidney function and care of the AYA with CKD or kidney failure. Recommendations for screening and intervention strategies are provided.
Kidney disease in children with heart or liver transplant
Amrit Kirpalani, Chia Wei Teoh, Vicky Lee Ng, Anne I Dipchand & Mina Matsuda-Abedini
doi : 10.1007/s00467-021-04949-5
Pediatric Nephrology volume 36, pages3595–3605 (2021)
Over the past few decades, there has been increasing recognition of kidney disease in children with non-kidney solid organ transplantation. The risk of kidney disease in children undergoing heart or liver transplantation is higher than the general population as the underlying disease and its associated management may directly impair kidney function. Both heart and liver failures contribute to hypoperfusion and kidney ischemia before patients reach the point of transplant. The transplant surgery itself can often be complicated by acute kidney injury (AKI), which may be further exacerbated by a complicated postoperative course. In the short- and long-term post-transplant period, these children are at risk of acute illness, exposed to nephrotoxic medications, and susceptible to rare but severe infections and immunologic insults that may contribute to AKI and chronic kidney disease (CKD). In some, CKD can progress to kidney failure with replacement therapy (KFRT). CKD and KFRT are associated with increased morbidity and mortality in this patient population. Therefore, it is critical to monitor for and recognize the risk factors for kidney injury in this population and mitigate these risks. In this paper, the authors provide an overview of kidney disease pertaining to heart and liver transplantation in children with guidance on monitoring, diagnosis, prevention, and management.
Neonatal acute kidney injury: a case-based approach
Michelle C. Starr & Shina Menon
doi : 10.1007/s00467-021-04977-1
Pediatric Nephrology volume 36, pages3607–3619 (2021)
Neonatal acute kidney injury (AKI) is increasingly recognized as a common complication in critically ill neonates. Over the last 5–10 years, there have been significant advancements which have improved our understanding and ability to care for neonates with kidney disease. A variety of factors contribute to an increased risk of AKI in neonates, including decreased nephron mass and immature tubular function. Multiple factors complicate the diagnosis of AKI including low glomerular filtration rate at birth and challenges with serum creatinine as a marker of kidney function in newborns. AKI in neonates is often multifactorial, but the cause can be identified with careful diagnostic evaluation. The best approach to treatment in such patients may include diuretic therapies or kidney support therapy. Data for long-term outcomes are limited but suggest an increased risk of chronic kidney disease (CKD) and hypertension in these infants. We use a case-based approach throughout this review to illustrate these concepts and highlight important evidence gaps in the diagnosis and management of neonatal AKI.
Management of severe polyuria in idiopathic Fanconi syndrome
Guido Filler, Rishika Geda, Fabio Salerno, Yun Cong Zhang, Maria E Díaz-González de Ferris & Christopher William McIntyre
doi : 10.1007/s00467-021-05213-6
Pediatric Nephrology volume 36, pages3621–3626 (2021)
Polyuria is a common problem in patients with tubular diseases, especially for those with CKD and high-output Fanconi syndrome. There are currently no guidelines on how to treat debilitating polyuria, in children or adults, and vasopressin is usually not effective.
Hypokalemia and hypertensive urgency in a 10-year-old boy: Questions
Ozlem Yuksel Aksoy, Funda Bastug, Binnaz Celik & Salih Uytun
doi : 10.1007/s00467-021-05058-z
Pediatric Nephrology volume 36, pages3627–3628 (2021)
Hypokalemia and hypertensive urgency in a 10-year-old boy: Answers
Ozlem Yuksel Aksoy, Funda Bastug, Binnaz Celik & Salih Uytun
doi : 10.1007/s00467-021-05072-1
Pediatric Nephrology volume 36, pages3629–3631 (2021)
Hypokalemic paralysis and discolored teeth in a 12-year-old girl: Questions
Aakash Chandran Chidambaram, Sriram Krishnamurthy, Bobbity Deepthi, Narayanasamy Rajavelu Thiagarajan & Pediredla Karunakar
doi : 10.1007/s00467-021-05065-0
Pediatric Nephrology volume 36, pages3633–3634 (2021)
Hypokalemic paralysis and discolored teeth in a 12-year-old girl: Answers
Aakash Chandran Chidambaram, Sriram Krishnamurthy, Bobbity Deepthi, Narayanasamy Rajavelu Thiagarajan & Pediredla Karunakar
doi : 10.1007/s00467-021-05073-0
Pediatric Nephrology volume 36, pages3635–3638 (2021)
Kidney involvement in a child with autoimmune disease: Questions
Abir Boussetta, Manel Jellouli, Kawla Isa, Haythem Bacherouch, Keriima Sherayet, Raja A. Trabelsi, Rym Goucha & Tahar Gargah
doi : 10.1007/s00467-021-05021-y
Pediatric Nephrology volume 36, pages3639–3641 (2021)
Kidney involvement in a child with autoimmune disease: Answers
Abir Boussetta, Manel Jellouli, Kawla Isa, Haythem Bacherouch, Keriima Sherayet, Raja A Trabelsi, Rym Goucha & Tahar Gargah
doi : 10.1007/s00467-021-05034-7
Pediatric Nephrology volume 36, pages3643–3644 (2021)
Unexpected cause and successful management of typical urinary tract infection symptoms: Questions
Demet Alaygut, Özgür Özdemir-?im?ek, Fatma Ceren Sar?oglu, Seçil Arslansoyu-Çamlar, Fatma Mutluba? & Belde Kasap-Demir
doi : 10.1007/s00467-021-05090-z
Pediatric Nephrology volume 36, pages3645–3646 (2021)
Unexpected cause and successful management of typical urinary tract ?nfection symptoms: Answers
Demet Alaygut, Özgür Özdemir-?im?ek, Fatma Ceren Sarioglu, Seçil Arslansoyu-Çamlar, Fatma Mutluba? & Belde Kasap-Demir
doi : 10.1007/s00467-021-05091-y
Pediatric Nephrology volume 36, pages3647–3651 (2021)
Diagnostic and clinical utility of genetic testing in children with kidney failure
Jing Chen, Fang Lin, Yihui Zhai, Chunyan Wang, Bingbing Wu, Duan Ma, Jia Rao, Jiaojiao Liu, Jialu Liu, Minghui Yu, Qian Shen & Hong Xu
doi : 10.1007/s00467-021-05141-5
Pediatric Nephrology volume 36, pages3653–3662 (2021)
Genetic kidney disease is well established as an important cause of pediatric kidney failure, and genetic testing might increase diagnostic accuracy, but evidence is limited. This study was conducted to determine the diagnostic yield and clinical impact of genetic testing for children with kidney failure.
Long-term outcome of biopsy-proven idiopathic tubulointersitial nephritis with or without uveitis in children—a nationwide follow-up study
Sari Rytkönen, Juuso Tainio, Ville Saarela, Kira Endén, Janne Kataja, Pekka Arikoski, Matti Nuutinen & Timo Jahnukainen
doi : 10.1007/s00467-021-05060-5
Pediatric Nephrology volume 36, pages3663–3671 (2021)
Only a few studies reporting the long-term outcome of children with idiopathic tubulointerstitial nephritis (TIN) and uveitis syndrome (TINU) are available. We studied the long-term kidney and ocular outcome in a nationwide cohort of children with TIN or TINU.
Outcomes of solitary functioning kidneys—renal agenesis is different than multicystic dysplastic kidney disease
Douglas G. Matsell, Carol Bao, Teagan Po White, Ella Chan, Eli Matsell, Dan Cojocaru & Marisa Catapang on behalf of the Pediatric Nephrology Clinical Pathway Development Team
doi : 10.1007/s00467-021-05064-1
Pediatric Nephrology volume 36, pages3673–3680 (2021)
Multicystic dysplastic kidney (MCDK) disease and unilateral renal agenesis (URA) are well-known causes of a solitary functioning kidney (SFK) and are associated with long-term kidney injury. The aims of this study were to characterize the natural history of SFK at our center, define the risk factors associated with chronic kidney injury, and identify distinguishing features between URA and MCDK that predict outcome.
Oral steroids for reducing kidney scarring in young children with febrile urinary tract infections: the contribution of Bayesian analysis to a randomized trial not reaching its intended sample size
Liviana Da Dalt, Silvia Bressan, Floriana Scozzola, Enrico Vidal, Monia Gennari, Claudio La Scola, Mauro Anselmi, Elisabetta Miorin, Pietro Zucchetta, Danila Azzolina, Dario Gregori & Giovanni Montini
doi : 10.1007/s00467-021-05117-5
Pediatric Nephrology volume 36, pages3681–3692 (2021)
This study aimed to evaluate the effect of oral dexamethasone in reducing kidney scars in infants with a first febrile urinary tract infection (UTI).
Maturational changes in sodium metabolism in periviable infants
Jeffrey L Segar, Connie C Grobe & Justin L Grobe
doi : 10.1007/s00467-021-05119-3
Pediatric Nephrology volume 36, pages3693–3698 (2021)
Sodium depletion results in impaired somatic growth. The sodium requirements of extremely preterm (periviable) infants early in life are not known. We therefore investigated sodium homeostasis in this population over the first 10 weeks following birth.
Risk factors for post-nephrectomy hypotension in pediatric patients
Kentaro Nishi, Koichi Kamei, Masao Ogura, Mai Sato, Sho Ishiwa, Yoko Shioda, Chikako Kiyotani, Kimikazu Matsumoto, Kandai Nozu, Kenji Ishikura & Shuichi Ito
doi : 10.1007/s00467-021-05115-7
Pediatric Nephrology volume 36, pages3699–3709 (2021)
Although hypotension is a life-threatening complication of nephrectomy in children, risk factors for its development remain unknown. We evaluated the incidence, clinical course, and associated risk factors of pediatric post-nephrectomy hypotension in an observational study.
The influence of hyperbilirubinemia on indexes of kidney function in neonates
Huiying Wang, Min Su, Bo Yang, Yi Ren, Li Li, Dandan Zhao, Di Huang & Xiangyu Gao
doi : 10.1007/s00467-021-05151-3
Pediatric Nephrology volume 36, pages3711–3716 (2021)
To study the influence of hyperbilirubinemia on indexes of neonatal kidney function.
Increasing prevalence of hypertension during long-term follow-up in children with autosomal dominant polycystic kidney disease
Tomáš Seeman, Petr Jansky, Fencl Filip, Kv?ta Bláhová & Adam Jaroš
doi : 10.1007/s00467-021-05104-w
Pediatric Nephrology volume 36, pages3717–3723 (2021)
Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease. Kidney cysts form over the course of the disease and kidney function slowly declines, usually leading to kidney failure in middle to late adulthood. However, some symptoms, such as hypertension or proteinuria, can be present at an earlier age. In this study, we aimed to quantify early complications in children over time.
Chronic haemodialysis in infants and children less than 15 kg
Jean Grandy & Felipe Veloso
doi : 10.1007/s00467-021-05146-0
Pediatric Nephrology volume 36, pages3725–3732 (2021)
Peritoneal dialysis (PD) is the most commonly used kidney replacement therapy in infants and young children with chronic kidney disease (CKD) stage 5. Chronic haemodialysis (cHD) is the alternative treatment when PD is not possible for technical reasons; however, the difficulties that may be encountered are challenging and require clinicians with specialist training and experience. This study aims to describe the clinical history, complications and outcomes in children < 15 kg on cHD.
Nutritional status and volume control in adolescents on chronic hemodialysis
Fabio Paglialonga, Silvia Consolo, Marta Brambilla, Olga Caporale, Alejandro Cruz Gual, Maria Rosa Grassi & Giovanni Montini
doi : 10.1007/s00467-021-05089-6
Pediatric Nephrology volume 36, pages3733–3740 (2021)
Pediatric patients on maintenance hemodialysis (HD) are at risk of both malnutrition and fluid overload. This pilot study aimed to assess correlates of normalized protein catabolic rate (nPCR) in adolescents on chronic HD, in particular fluid status markers.
Effect of L-carnitine supplementation on lipid profile and apolipoproteins in children on hemodialysis: a randomized placebo-controlled clinical trial
Fatemeh Hamedi-Kalajahi, Meysam Zarezadeh, Sayed Yousef Mojtahedi, Sakineh Shabbidar, Dariyoosh Fahimi & Hossein Imani
doi : 10.1007/s00467-021-05080-1
Pediatric Nephrology volume 36, pages3741–3747 (2021)
Cardiovascular disease (CVD) is the leading cause of death in children with chronic kidney disease (CKD) and accounts for 40% of all deaths among pediatric patients with stage 5 chronic kidney disease (CKD 5). Dyslipidemia is common in children with CKD and is considered one of the major causes of CVD in these patients. As carnitine plays a key role in lipid metabolism and because plasma levels are reduced in hemodialysis patients, the aim of this study was to determine the effects of L-carnitine supplementation on serum lipid profiles, apolipoproteins, and free carnitine (FC) levels.
Incidence and risk factors for chronic kidney disease in patients with congenital heart disease
Nai-Wen Fang, Yu-Chieh Chen, Shih-Hsiang Ou, Chun-Hao Yin, Jin-Shuen Chen & Yee-Hsuan Chiou
doi : 10.1007/s00467-021-05129-1
Pediatric Nephrology volume 36, pages3749–3756 (2021)
Chronic kidney disease (CKD) is underdiagnosed in children with congenital heart disease (CHD). Our aim was to study the incidence of CKD in CHD children and identify risk factors for CKD.
A critical re-analysis of cases of post-transplantation recurrence in genetic nephrotic syndrome
Anna E. Mason, Moin A. Saleem & Agnieszka Bierzynska
doi : 10.1007/s00467-021-05134-4
Pediatric Nephrology volume 36, pages3757–3769 (2021)
Genetic defects in podocyte proteins account for up to 30% of steroid-resistant nephrotic syndrome (SRNS) in the paediatric population. Most children with genetic SRNS are resistant to immunosuppression and at high risk of progression to stage 5 chronic kidney disease. Kidney transplantation is often the treatment of choice. The possibility of post-transplantation disease recurrence in genetic SRNS remains controversial, and poses fundamental questions about disease biology.
Favipiravir use in children with COVID-19 and acute kidney injury: is it safe?
Yasemin Ozsurekci, Pembe Derin Oygar, Sibel Laçinel Gürlevik, Selman Kesici, Seza Ozen, Eda Didem Kurt Sukur, Bora Gülhan, Rezan Topaloglu, Benan Bayrakci & Ali Bülent Cengiz
doi : 10.1007/s00467-021-05111-x
Pediatric Nephrology volume 36, pages3771–3776 (2021)
The rising number of infections due to Severe Acute Respiratory Syndrome Coronavirus-2 (popularly known as COVID-19) has brought to the fore new antiviral drugs as possible treatments, including favipiravir. However, there is currently no data regarding the safety of this drug in patients with kidney impairment. The aim of this paper, therefore, is to share our experience of the use of favipiravir in pediatric patients affected by COVID-19 with any degree of kidney impairment.
Hemoconcentration and predictors in Shiga toxin-producing E. coli-hemolytic uremic syndrome (STEC-HUS)
Sebastian Loos, Jun Oh, Laura van de Loo, Markus J. Kemper, Martin Blohm & Raphael Schild
doi : 10.1007/s00467-021-05108-6
Pediatric Nephrology volume 36, pages3777–3783 (2021)
Hemoconcentration has been identified as a risk factor for a complicated course in Shiga toxin-producing E. coli-hemolytic uremic syndrome (STEC-HUS). This single-center study assesses hemoconcentration and predictors at presentation in STEC-HUS treated from 2009–2017.
Prevalence and impact of acute kidney injury in hospitalized pediatric patients with acute pancreatitis
Aravind Thavamani, Krishna Kishore Umapathi & Senthilkumar Sankararaman
doi : 10.1007/s00467-021-05106-8
Pediatric Nephrology volume 36, pages3785–3788 (2021)
The incidence of acute pancreatitis (AP) in pediatric patients is rising with accompanying increased hospitalizations. Acute kidney injury (AKI) is associated with worse clinical outcomes in adults, and similar data in the pediatric age group is limited.
Kidney involvement and histological findings in two pediatric COVID-19 patients
Jessica Serafinelli, Antonio Mastrangelo, William Morello, Valeria Fanny Cerioni, Adib Salim, Manuela Nebuloni & Giovanni Montini
doi : 10.1007/s00467-021-05212-7
Pediatric Nephrology volume 36, pages3789–3793 (2021)
Histological findings of kidney involvement have been rarely reported in pediatric patients with SARS-CoV-2 infection. Here, we describe clinical, laboratory, and histological findings of two pediatric cases with almost exclusive kidney involvement by SARS-CoV-2.
Cholelithiasis in infants with congenital nephrotic syndrome of the Finnish type
Taishi Nada, Mai Sato, Takahisa Yoshikawa, Masao Ogura & Koichi Kamei
doi : 10.1007/s00467-021-05225-2
Pediatric Nephrology volume 36, pages3795–3798 (2021)
Congenital nephrotic syndrome of the Finnish type (CNF) caused by NPHS1 mutations is a rare disease. Infants with CNF can develop many complications, but hepatobiliary complications are uncommon. CNF is not reported as a risk factor of cholelithiasis, which is also a rare disease in the pediatric population. We herein present the cases of three infants with CNF diagnosed with gallstones among a total of seven children with CNF who were treated between 2010 and 2020 in our hospital.
The effect of levamisole on kidney function in children with steroid-sensitive nephrotic syndrome
Lieke A. Hoogenboom, Hazel Webb, Kjell Tullus & Aoife Waters
doi : 10.1007/s00467-021-05231-4
Pediatric Nephrology volume 36, pages3799–3802 (2021)
Levamisole is frequently used as a steroid-sparing agent in children with steroid-sensitive nephrotic syndrome. Side effects, such as neutropenia, gastro-intestinal upset and skin rash, have been reported. We noted an increase in creatinine in some of our patients, but literature on the effect of levamisole on kidney function is lacking.
Correction to: H. William Schnaper—Life course journey of a true Mensch
Laurence Greenbaum, Victoria Norwood, Eileen Brewer, William Smoyer, Marva Moxey-Mims, Joseph Flynn, Barbara Fivush, Patrick Brophy, Brad Warady, Sandra Watkins, Isidro Salusky & Rick Kaskel
doi : 10.1007/s00467-021-05241-2
Pediatric Nephrology volume 36, page3803 (2021)
Correction to: Risk factors for post-nephrectomy hypotension in pediatric patients
Kentaro Nishi, Koichi Kamei, Masao Ogura, Mai Sato, Sho Ishiwa, Yoko Shioda, Chikako Kiyotani, Kimikazu Matsumoto, Kandai Nozu, Kenji Ishikura & Shuichi Ito
doi : 10.1007/s00467-021-05198-2
Pediatric Nephrology volume 36, pages3805–3806 (2021)
