Pediatric chronic kidney disease

Anemia is a common complication of chronic kidney disease (CKD) in children, and dysregulation of iron homeostasis plays a central role in its pathogenesis. Optimizing iron status is a prerequisite for effective treatment of anemia. Insufficient iron can lead to inappropriate escalation of the erythropoiesis-stimulating agent (ESA) dose, which is associated with adverse outcomes. Excess iron supplementation also has negative sequelae including free radical tissue damage and increased risk of systemic infection...

Background: Current treatment options for chronic hyperkalemia in children with chronic kidney disease include dietary restrictions or enteral sodium polystyrene sulfonate (SPS); however, dietary restrictions may compromise adequate nutrition and enteral SPS may be limited by palatability, adverse effects and feeding tube obstruction. A potentially safer alternative is to pretreat enteral nutrition (EN) with SPS prior to consumption. The purpose of this study was to evaluate the efficacy and safety of pretreating EN with SPS in pediatric patients with hyperkalemia...

BACKGROUND/AIMS: Primary distal renal tubular acidosis (dRTA) in children is a rare genetic disorder, and three causative mutated genes have been identified: SLC4A1, ATP6V1B1, and ATP6V0A4. We analyzed the prevalence and phenotypic differences of genetic mutations in children with dRTA. METHODS: A total of 17 children with dRTA were enrolled in the study. All patients underwent genetic testing for all three candidate genes. RESULTS: Pathogenic mutations, including six novel mutations, were detected in 15 (88...

OBJECTIVES: To investigate the etiologies and outcomes of rapidly progressive glomerulonephritis (RPGN) in pediatric patients at King Abdulaziz University Hospital (KAUH) in Jeddah, Saudi Arabia. METHODS: A retrospective study was conducted in 19 pediatric patients who were diagnosed with RPGN between 2006 and 2016 at the Department of Pediatric Medicine at KAUH. Associations between variables were evaluated using independent t-test, one-way analysis of variance (ANOVA) and Chi-squared tests...

Background: The prevalence of glomerular hyperfiltration and chronic kidney disease is increasing worldwide in parallel with obesity hypertension epidemic. The effect of increases in glomerular filtrations (GFR) in children with metabolic syndrome has not been studied. The purpose of the present study is to investigate the relationship between GFR and cardiometabolic risk factors in a large sample of pediatric population. Methods: In this nationwide survey, 3800 participants were selected by cluster random sampling from 30 provinces in Iran...

BACKGROUND: Children with chronic kidney disease (CKD) have increased cardiovascular mortality. Identifying high-risk children who may benefit from further therapeutic intervention is difficult as cardiovascular abnormalities are subtle. Although transthoracic echocardiography may be used to detect sub-clinical abnormalities, it has well-known problems with reproducibility that limit its ability to accurately detect these changes. Cardiovascular magnetic resonance (CMR) is the reference standard method for assessing blood flow, cardiac structure and function...

Background: Recent studies in adults have found an incidence of acute kidney injury (AKI) in patients treated with a combination of vancomycin and piperacillin-tazobactam (TZP) that is greater than that expected with either medication alone. The purpose of this study was to determine whether combination therapy with vancomycin and TZP is associated with an incidence of AKI in pediatric patients higher than that in those on combination therapy with vancomycin and cefepime. Methods: We performed a retrospective single-center matched-cohort study of pediatric patients who received vancomycin in combination with TZP or cefepime between January 2015 and June 2016...

Anemia is a common complication of chronic kidney disease (CKD) in adult and pediatric patients. It has traditionally been treated with erythropoietin therapy and iron supplementation, with great success. With the discovery of the major transcription factor hypoxia inducible factor (HIF) for the erythropoietin gene in 1992, molecules were created that inhibit the HIF prolyl-hydroxylase enzyme. This new class of drug-called HIF stabilizers, or HIF prolyl-hydroxylase inhibitors-prevents the proteasomal degradation of HIF-α, thereby inducing upregulation of the erythropoietin gene...

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...

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...

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...

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...

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...

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...

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...

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...

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...

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...

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...

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...