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chronic kidney disease in pediatrics

Daniela A Braun, Jillian K Warejko, Shazia Ashraf, Weizhen Tan, Ankana Daga, Ronen Schneider, Tobias Hermle, Tilman Jobst-Schwan, Eugen Widmeier, Amar J Majmundar, Makiko Nakayama, David Schapiro, Jia Rao, Johanna Magdalena Schmidt, Charlotte A Hoogstraten, Hannah Hugo, Sevcan A Bakkaloglu, Jameela A Kari, Sherif El Desoky, Ghaleb Daouk, Shrikant Mane, Richard P Lifton, Shirlee Shril, Friedhelm Hildebrandt
Background: Nephrotic syndrome (NS), a chronic kidney disease, is characterized by significant loss of protein in the urine causing hypoalbuminemia and edema. In general, ∼15% of childhood-onset cases do not respond to steroid therapy and are classified as steroid-resistant NS (SRNS). In ∼30% of cases with SRNS, a causative mutation can be detected in one of 44 monogenic SRNS genes. The gene LAMA5 encodes laminin-α5, an essential component of the glomerular basement membrane. Mice with a hypomorphic mutation in the orthologous gene Lama5 develop proteinuria and hematuria...
March 9, 2018: Nephrology, Dialysis, Transplantation
Wesley Hayes, Fabio Paglialonga
Dysregulation of intravascular fluid leads to chronic volume overload in children with end-stage kidney disease (ESKD). Sequelae include left ventricular hypertrophy and remodeling and impaired cardiac function. As a result, cardiovascular complications are the commonest cause of mortality in the pediatric dialysis population. The clinical need to optimize intravascular volume in children with ESKD is clear; however, its assessment and management is the most challenging aspect of the pediatric dialysis prescription...
March 9, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Smitha R Vidi
PURPOSE OF REVIEW: Hypertension is an independent risk factor for progression of chronic kidney disease (CKD) in children. Children with early CKD develop hypertension secondary to renal disease. This review aims to highlight recent advances that help us better understand the current role of hypertension in progression of CKD in children. RECENT FINDINGS: There is increasing evidence that children with CKD who have hypertension develop early atherosclerosis and cardiac adaptive changes...
April 2018: Current Opinion in Pediatrics
Alexander J Skokan, Robert Caleb Kovell
PURPOSE OF REVIEW: As long-term survival with complex congenital and pediatric diseases has improved, more patients with congenital genitourinary conditions are living into adulthood. These patients can continue to face lifelong issues related to their conditions, including urinary incontinence, recurrent urinary tract infections, chronic kidney disease, and difficulties with sexual health and function. RECENT FINDINGS: The practice of transitional urology continues to grow nationwide and abroad, and it will be important to develop evidence-based practices for coordinated transition of these patients into the adult health care setting...
March 7, 2018: Current Urology Reports
Jason H Greenberg, Prasad Devarajan, Heather R Thiessen-Philbrook, Catherine Krawczeski, Chirag R Parikh, Michael Zappitelli
BACKGROUND: We previously reported that children undergoing cardiac surgery are at high risk for long-term chronic kidney disease (CKD) and hypertension, although postoperative acute kidney injury (AKI) is not a risk factor for worse long-term kidney outcomes. We report here our evaluation of renal injury biomarkers 5 years after cardiac surgery to determine whether they are associated with postoperative AKI or long-term CKD and hypertension. METHODS: Children aged 1 month to 18 years old undergoing cardiopulmonary bypass were recruited to this prospective cohort study...
March 6, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Hans Pottel, Laurence Dubourg, Karolien Goffin, Pierre Delanaye
The bedside Schwartz equation has long been and still is the recommended equation to estimate glomerular filtration rate (GFR) in children. However, this equation is probably best suited to estimate GFR in children with chronic kidney disease (reduced GFR) but is not optimal for children with GFR >75 mL/min/1.73 m2 . Moreover, the Schwartz equation requires the height of the child, information that is usually not available in the clinical laboratory. This makes automatic reporting of estimated glomerular filtration rate (eGFR) along with serum creatinine impossible...
January 2018: Advances in Chronic Kidney Disease
Akash Deep, Romit Saxena, Bipin Jose
Acute kidney injury (AKI) is a common accompaniment in patients with liver disease. The causes, risk factors, manifestations and management of AKI in these patients vary according to the liver disease in question (acute liver failure, acute-on-chronic liver failure, post-liver transplantation or metabolic liver disease). There are multiple causes of AKI in patients with liver disease-pre-renal, acute tubular necrosis, post-renal, drug-induced renal failure and hepatorenal syndrome (HRS). Definitions of AKI in liver failure are periodically revised and updated, but pediatric definitions have still to see the light of the day...
March 1, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
M S Ansari, Sandeep Kumar Nunia, Ankur Bansal, Prempal Singh, Virender Sekhon, Dharmveer Singh, Rahul Soni, Priyank Yadav
INTRODUCTION: Posterior urethral valve (PUV) is the most common cause of pediatric end stage renal disease (ESRD), imposing a major health burden on medical community caregivers and adversely affecting the quality of life of patients. Chronic kidney disease (CKD) stage III or estimated GFR of <60 mL/min/1.73 m2 is known to be associated with more adverse renal, cardiovascular, and clinical outcomes. Thus, it is desirable to identify factors predicting the rapid and early progression of disease...
November 22, 2017: Journal of Pediatric Urology
Ida Giardino, Maria D'Apolito, Michael Brownlee, Angela Bruna Maffione, Anna Laura Colia, Michele Sacco, Pietro Ferrara, Massimo Pettoello-Mantovani
Chronic kidney disease in children is an irreversible process that may lead to end-stage renal disease. The mortality rate in children with end-stage renal disease who receive dialysis increased dramatically in the last decade, and it is significantly higher compared with the general pediatric population. Furthermore, dialysis and transplant patients, who have developed end-stage renal disease during childhood, live respectively far less as compared with age/race-matched populations. Different reports show that cardiovascular disease is the leading cause of death in children with end-stage renal disease and in adults with childhood-onset chronic kidney disease, and that children with chronic kidney disease are in the highest risk group for the development of cardiovascular disease...
December 2017: Türk Pediatri Arşivi
Andrew Mantulak, Susan Cadell
Parenting a child with chronic kidney disease has a profound impact on the parental caregivers across social, emotional, and physical functioning. As the survival rates for children with chronic kidney disease increase, the demands on parents caring for these children intensify. The aim of this study was to understand the lived experience of being a mother of a child who has undergone kidney transplantation. Seven mothers caring for children with chronic kidney disease in Ontario, Canada participated in in-depth interviews that were analyzed according to the principles of hermeneutic phenomenology...
February 26, 2018: Journal of Social Work in End-of-life & Palliative Care
Kerry A McBrien, Noah Ivers, Lianne Barnieh, Jacob J Bailey, Diane L Lorenzetti, David Nicholas, Marcello Tonelli, Brenda Hemmelgarn, Richard Lewanczuk, Alun Edwards, Ted Braun, Braden Manns
BACKGROUND: People with chronic diseases experience barriers to managing their diseases and accessing available health services. Patient navigator programs are increasingly being used to help people with chronic diseases navigate and access health services. OBJECTIVE: The objective of this review was to summarize the evidence for patient navigator programs in people with a broad range of chronic diseases, compared to usual care. METHODS: We searched MEDLINE, EMBASE, CENTRAL, CINAHL, PsycINFO, and Social Work Abstracts from inception to August 23, 2017...
2018: PloS One
Robert S Venick
PURPOSE OF REVIEW: The review aims to describe in detail contemporary medical, nutritional, and psychosocial results in pediatric intestinal transplant (ITx) recipients greater than 10 years removed from their transplant, and to highlight significant long-term post-ITx challenges and complications. The review will draw from recent publications in the field, the ITx registry as well as detailed unpublished data from a large, well established single center. RECENT FINDINGS: The majority of long-term ITx survivors are off parenteral nutrition altogether, but a significant number require supplemental tube feeds to gain weight and grow properly...
February 15, 2018: Current Opinion in Organ Transplantation
Kamal Kumar Goyal, Abhijeet Saha, Puneet Kaur Sahi, Manpreet Kaur, Nand Kishore Dubey, Parul Goyal, Ashish Dutt Upadhayay
BACKGROUND: Hepcidin is the main regulator of hepcidin-ferroportin axis and is elevated in children with chronic kidney disease (CKD). Anemia of CKD and its relation to hepcidin, interleukin-6 (IL-6), tumor necrosis factor-α (TNF-α), and high-sensitivity C-reactive protein (hs-CRP) in iron- and erythropoietin (EPO)-naïve, non-dialyzed children with CKD is under-studied. MATERIALS AND METHODS: This case-control study aimed to study the levels of hepcidin and other proinflammatory markers (IL-6, TNF-α, hs-CRP) and their relation with anemia in iron- and erythropoietin-naïve, non-dialysis CKD (stage 3 - 5) patients...
February 16, 2018: Clinical Nephrology
Moglie Le Quintrec, Anne-Laure Lapeyraque, Arnaud Lionet, Anne-Laure Sellier-Leclerc, Yahsou Delmas, Véronique Baudouin, Eric Daugas, Stéphane Decramer, Leila Tricot, Mathilde Cailliez, Philippe Dubot, Aude Servais, Catherine Mourey-Epron, Franck Pourcine, Chantal Loirat, Véronique Frémeaux-Bacchi, Fadi Fakhouri
BACKGROUND: Cases reports and small series of patients with C3 glomerulopathy have reported variable efficacy of eculizumab. STUDY DESIGN: Case series of C3 glomerulopathy. SETTING & PARTICIPANTS: Pediatric and adult patients with C3 glomerulopathy treated with eculizumab between 2010 and 2016 were identified through the C3 glomerulopathy French registry database, and a questionnaire was sent to participating French pediatric and adult nephrology centers, as well as one pediatric referral center in Québec, Canada...
February 8, 2018: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
Kristen Sgambat, Sarah Clauss, Asha Moudgil
Children are at increased risk of developing metabolic syndrome (MS) after kidney transplantation, which contributes to long-term cardiovascular (CV) morbidities and decline in allograft function. While MS in the general population occurs due to excess caloric intake and physical inactivity, additional chronic kidney disease and transplant-related factors contribute to the development of MS in transplant recipients. Despite its significant health consequences, the interplay of the individual components in CV morbidity in pediatric transplant recipients is not well understood...
February 2018: Clinical Kidney Journal
David T Selewski, Dylan M Hyatt, Kevin M Bennett, Jennifer R Charlton
PURPOSE OF REVIEW: Despite abundant evidence in adults, the relationship between acute kidney injury (AKI) and chronic kidney disease (CKD) remains unanswered in pediatrics. Obstacles to overcome include the challenges defining these entities and the lack of long-term follow-up studies. This review focuses on pediatric populations at high-risk for AKI, the evidence of the long-term effect of AKI on renal health, and biomarkers to detect renal disease. RECENT FINDINGS: AKI in critically ill children and neonates is common and independently associated with adverse outcomes...
April 2018: Current Opinion in Pediatrics
Vaka K Sigurjonsdottir, Swasti Chaturvedi, Cherry Mammen, Scott M Sutherland
Acute kidney injury (AKI) is characterized clinically as an abrupt decline in renal function marked by reduced excretion of waste products, disordered electrolytes, and disrupted fluid homeostasis. The recent development of a standardized AKI definition has transformed our understanding of AKI epidemiology and outcomes. We now know that in the short term, children with AKI experience greater morbidity and mortality; additionally, observational studies have established that chronic renal sequelae are far more common after AKI events than previously realized...
January 26, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Akira Ashida, Hideki Matsumura, Toshihiro Sawai, Rika Fujimaru, Yuko Fujii, Akihiko Shirasu, Hyogo Nakakura, Kazumoto Iijima
BACKGROUND: Thrombotic microangiopathy (TMA) includes hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP). This study examined the epidemiological characteristics of pediatric patients with TMA classified according to etiology. METHODS: The survey evaluated 258 Japanese pediatric patients diagnosed with TMA between 2012 and 2015. RESULTS: The primary diseases responsible for TMA were categorized as TTP (15 cases), Shiga toxin-producing Escherichia coli-associated HUS (STEC-HUS) (166 cases), atypical HUS (aHUS) (40 cases), and secondary TMA (27 cases)...
January 19, 2018: Clinical and Experimental Nephrology
Shannon B Donnola, Connie M Piccone, Lan Lu, Joshua Batesole, Jane Little, Katherine M Dell, Chris A Flask
Chronic kidney disease (CKD) occurs in over one-third of patients with sickle cell disease (SCD) and can progress to end-stage renal disease. Unfortunately, current clinical assessments of kidney function are insensitive to early-stage CKD. Previous studies have shown that diffusion magnetic resonance imaging (MRI) can sensitively detect regional renal microstructural changes associated with early-stage CKD. However, previous MRI studies in patients with SCD have been largely limited to the detection of renal iron deposition assessed by T2 * relaxometry...
January 19, 2018: NMR in Biomedicine
Júlia Dauernheimer Machado, Eduardo Guimarães Camargo, Roberta Boff, Laís da Silva Rodrigues, Joíza Lins Camargo, Ariana Aguiar Soares, Sandra Pinho Silveiro
AIM: To evaluate the accuracy of creatinine and cystatin C (cysC) equations to estimate glomerular filtration rate (GFR) in type 2 diabetes mellitus (DM) patients and healthy adults. METHODS: Case-control study including 84 patients with type 2 DM and 100 healthy adults with measured GFR (mGFR)≥60mL/min/1.73m2. GFR was measured by 51Cr-EDTA and estimated (eGFR) by the following equations using creatinine, cysC or both markers: Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI), Caucasian Asian Pediatrics and Adults (CAPA), CKD-EPI creatinine-cystatin C (CKDEPI-CC), and CKD-EPI cystatin C (CKDEPIcysC)...
January 10, 2018: Clinical Biochemistry
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