Paediatric nephrology

AIM: Rituximab (RTX) is a genetically engineered chimeric monoclonal antibody which binds directly to CD20 antigen and mediates inhibition of B cell development. Although RTX has been widely used in paediatric nephrology, there is no routine protocol for its use. In this study, paediatric nephrologists in Türkiye were asked to fill out a questionnaire to understand their practice in using RTX. This study aimed to determine common practices and clarify the uncertainties regarding the use of RTX in paediatric nephrology...

AIM: Acute kidney injury (AKI) in neonates is associated with longer hospital stays and higher mortality rates. However, there is significant variability in prevalence rates of AKI and the true burden is incompletely understood. In November 2020, the University of Iowa Stead Family Children's Hospital Neonatal Intensive Care Unit implemented a creatinine screening protocol to enhance kidney function monitoring. We sought to evaluate adherence to the protocol to determine if increased surveillance led to increased detection of AKI events...

BACKGROUND: Disturbances of sleep are prevalent among children with chronic kidney disease. However, the aetiology of sleep disorders in children particularly after kidney transplantation is not clear. We sought to ascertain the prevalence and type of sleep disturbances in paediatric kidney transplant recipients and to identify predictors of sleep disturbances in this population. METHODS: Caregivers of kidney transplant recipients completed online questionnaires about their child's sleep...

BACKGROUND: Despite the high perioperative risk profile, international guidelines for anesthesia and intensive care unit (ICU) care in pediatric kidney transplantation do not exist. Optimizing hemodynamics can be challenging in these patients, while scientific data to guide decisions in hemodynamic monitoring, hemodynamic targets, and perioperative fluid management are lacking. The limited annual number of pediatric kidney transplantations, even in reference centers, necessitates the urge for international collaboration to share knowledge and develop research and guidelines...

Systemic lupus erythematosus (SLE) is a multi-system disorder with a variety of clinical presentations. A wide range of renal vascular lesions (VL) is described predominantly in adult patients. The exact prevalence of renal VL in the pediatric SLE (pSLE) population is yet to be determined. A 10-year-old female patient with lupus nephritis (LN) presented with deteriorating kidney function. An exhaustive array of clinical-biochemical and pathological evaluations resulted in a diagnosis of class IV LN with thrombotic microangiopathy (TMA) associated with malignant hypertension and hypocomplementemia...

BACKGROUND: Disadvantaged socioeconomic position (SEP) is an important predictor of poor health in children with chronic kidney disease (CKD). The time course over which SEP influences the health of children with CKD and their carers is unknown. METHODS: This prospective longitudinal study included 377 children, aged 6-18 years with CKD (stages I-V, dialysis, and transplant), and their primary carers. Mixed effects ordinal regression was performed to assess the association between SEP and carer-rated child health and carer self-rated health over a 4-year follow-up...

AIMS: We propose using glomerular filtration rate (GFR) as the physiological basis for distinguishing components of renal clearance. METHODS: Gentamicin, amikacin and vancomycin are thought to be predominantly excreted by the kidneys. A mixed effects joint model of the pharmacokinetics of these drugs was developed, with a wide dispersion of weight, age, and serum creatinine. A dataset created from 18 sources resulted in 27,338 drug concentrations from 9,901 patients...

BACKGROUND: Sickle cell disease is the most common inherited blood disorder in humans and constitutes a major public health burden. It is a multisystemic condition with long-term renal complications. Early detection of sickle cell nephropathy and initiation of appropriate interventions are associated with improved survival and quality of life. This study aimed to compare the cystatin C-derived estimated glomerular filtration rate (GFR) of the study groups and also, to correlate the clinical features of chronic kidney disease (CKD) with decreased GFR in children with sickle cell anaemia (SCA)...

Kidney function is strongly influenced by genetic factors with both monogenic and polygenic factors contributing to kidney function. Monogenic disorders with primarily autosomal dominant inheritance patterns account for 10% of adult and 50% of paediatric kidney diseases. However, kidney function is also a complex trait with polygenic architecture, where genetic factors interact with environment and lifestyle factors. Family studies suggest that kidney function has significant heritability at 35-69%, capturing complexities of the genome with shared environmental factors...

BACKGROUND: Vesicoureteral reflux (VUR) is one of the most common risk factors of urinary tract infection (UTI) among children. Various treatment modalities including antibiotic prophylaxis, surgical or endoscopic corrections and conservative treatment were used depending on the severity of VUR. The aim of this study is to compare the effectiveness of these treatment modalities in children with VUR grades II-IV by conducting a systematic review and network meta-analysis. METHODS: A systematic search from different databases was performed from their earliest records to December 2022 without any language restriction...

Continuous renal replacement therapy (CRRT) is the preferred method for renal support in critically ill and hemodynamically unstable children in the pediatric intensive care unit (PICU) as it allows for gentle removal of fluids and solutes. The most frequent indications for CRRT include acute kidney injury (AKI) and fluid overload (FO) as well as non-renal indications such as removal of toxic metabolites in acute liver failure, inborn errors of metabolism, and intoxications and removal of inflammatory mediators in sepsis...

AIM: Technological advances and increased access have led to genomics expanding beyond the genetics clinic. Consequently, nephrologists can now order genomic testing for their patients. Consistent decision-making around patient and test selection is required to ensure equitable access while maximising the utility of genomic testing. However, there are currently no frameworks to guide decision-making for testing in this context. We aimed to develop an ethical decision-making framework for genomic testing in paediatric nephrology...

INTRODUCTION: Urolithiasis is the presence of mineral deposits in the urinary tract. It is rare in under-5 children and in Sub-Saharan Africa. Although metabolic abnormality is implicated in 50% of cases, infection, decreased urine volume and flow (dehydration state) have been implicated. We report a case of bilateral ureteric calculi in an infant with diarrhoea disease and dehydration. CASE REPORT: A.S, is an 8-month-old male with prolonged loose, large-volume stool, large-volume vomiting, high-grade fever, body weakness, and peri-orbital swelling that progressed to generalized body swelling and absent urine for 2 days...

INTRODUCTION: Children present with a spectrum of renal diseases depending on age, sex, and geographic location among other factors. With the absence of a paediatric renal registry in Nigeria, this will provide part of the regional data necessary for the Nigerian renal registry. METHODOLOGY: A retrospective study where cases of renal diseases that presented in a nephrology clinic over a 2-year period were retrieved from the nurses' and doctors' records and analysed...

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BACKGROUND: Therapeutic plasma exchange (TPE) is utilised in the management of a limited number of paediatric renal conditions. Despite its widespread acceptance and advancements in the practice of apheresis, there remains a paucity of data pertaining to paediatrics. We present a large retrospective review of our cohort of paediatric patients undergoing TPE for renal indications, outlining their outcomes and complications. METHODS: A retrospective chart review was conducted for all patients (under 16 years) undergoing TPE for renal conditions between January 2002 and June 2019 in Ireland...

Chronic kidney disease (CKD) in children, from birth to late adolescence, is a unique and highly challenging condition that requires epidemiological research and large-scale, prospective cohort studies. Since its first launch in 2007, the European Society for Paediatric Nephrology/European Renal Association (ESPN/ERA) Registry has collected data on patients on kidney replacement therapy (KRT). However, slowing the progression of CKD is of particular importance and thus the possibility to extend the current registry dataset to include patients in CKD stages 4-5 should be a priority...

INTRODUCTION: Impaired physical function is relatively common in children/adolescents with chronic kidney disease (CKD), affecting not only patients' daily lives but also the efficiency of later treatment and rehabilitation. Exercise is a recognised intervention to improve physical function and quality of life in adult patients with CKD. However, implementing this strategy in paediatric/adolescent populations remains relatively limited. For exercise training to be widely delivered as part of paediatric public health policy, a better understanding of the physical function and the impact of exercise interventions in children/adolescents with CKD is needed...

Diabetes mellitus, a disease that affects hundreds of millions of people worldwide, is increasing in prevalence in all age groups, including children and adolescents. Much of the morbidity and mortality associated with diabetes is closely related to hypertension, often coincident with diabetes. Comorbid hypertension and diabetes often worsen the outcomes of each other, likely rooted in some overlapping pathogenic mechanisms. In this educational review, we will discuss the shared pathophysiology of diabetes and hypertension, particularly in regard to inflammation and oxidative stress, the sympathetic nervous system, vascular remodeling, and the renin-angiotensin-aldosterone system (RAAS)...

BACKGROUND: Several equations for glomerular filtration rate (GFR) estimation based on serum creatinine (SCr) have been proposed for children, but most were developed among patients with kidney disease. The association between SCr and GFR may be distorted by kidney dysfunction and thus not applicable to healthy children. This study aimed to evaluate the applicability of existing SCr-based GFR estimation equations in healthy Chinese children. METHODS: GFR estimation equations that developed in healthy children were mainly analysed, including the Flanders Metadata (FM), simple height-independent (Simple), full age spectrum (FAS) and FAS-height equations...