Characterizing the frequency of modifiable histological changes observed on surveillance biopsies in pediatric kidney allograft recipients
ConclusionsSBs are safe and offer the opportunity to identify and treat modifiable histologic changes in the pediatric kidney transplant population. The performance of SBs for up to 2 years after transplantation can have meaningful clinical impact. (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 25, 2020 Category: Urology & Nephrology Source Type: research

Prevalence of arterial hypertension, hemodynamic phenotypes, and left ventricular hypertrophy in children after coarctation repair: a multicenter cross-sectional study
This study aimed to evaluate hemodynamic phenotypes and prevalence of left ventricular hypertrophy in children after coarctation repair with right arm and leg blood pressure difference
Source: Pediatric Nephrology - September 25, 2020 Category: Urology & Nephrology Source Type: research

Urine neutrophil gelatinase-associated lipocalin in girls with recurrent urinary tract infections
ConclusionIn this cohort of girls less than 6  years of age, there is no difference in urine NGAL concentrations between those with single and recurrent UTIs. (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 25, 2020 Category: Urology & Nephrology Source Type: research

Ambulatory blood pressure abnormalities in children with migraine
In this study, we aimed to evaluate the presence of hypertension in children with migraine by performing ambulatory blood pressure monitoring (ABPM).MethodsThirty-seven children diagnosed with migraine and 30 healthy controls were evaluated between January 2015 and March 2016. Demographic data, clinical and laboratory features, and physical examination findings were recorded for both groups. Office blood pressure was measured for all children, and each also underwent ABPM. The two groups were compared in terms of ambulatory blood pressure parameters.ResultsThe mean age was 13.3 and 13.1 years and the proportion of females ...
Source: Pediatric Nephrology - September 25, 2020 Category: Urology & Nephrology Source Type: research

Follow-up after neonatal heart disease repair: watch out for chronic kidney disease and hypertension!
AbstractBackgroundWith advances in care, neonates undergoing cardiac repairs are surviving more frequently. Our objectives were to 1) estimate the prevalence of chronic kidney disease (CKD) and hypertension 6  years after neonatal congenital heart surgery and 2) determine if cardiac surgery-associated acute kidney injury (CS-AKI) is associated with these outcomes.MethodsTwo-center prospective, longitudinal single-visit cohort study including children with congenital heart disease surgery as neonates between January 2005 and December 2012. CKD (estimated glomerular filtration rate
Source: Pediatric Nephrology - September 25, 2020 Category: Urology & Nephrology Source Type: research

Antenatally detected urinary tract dilatation: a 12 –15-year follow-up
ConclusionsWe conclude that in children with postnatal APD ≤ 7 mm, no calyceal dilatation, normal bladder, ureters, and kidney parenchyma, the outcome is excellent. There is no need for long-term follow-up in these patients. (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 25, 2020 Category: Urology & Nephrology Source Type: research

IPDN-China promotes the development of pediatric dialysis in China
AbstractBackgroundIn mainland China, dialysis for children with end-stage renal disease (ESRD) was not introduced until the 1980s. To describe the development of pediatric dialysis in different regions of China, a national pediatric dialysis network, namely, International Pediatric Dialysis Network-China (IPDN-China) (www.pedpd.org.cn), was launched in 2012.MethodsOriginal and updated information from the renal centers registered with the IPDN-China was collected between 2012 and 2016 from two sources, namely, the registry and the survey, and demographic features were analyzed.ResultsDue to promotion by the IPDN-China, the...
Source: Pediatric Nephrology - September 25, 2020 Category: Urology & Nephrology Source Type: research

Antihypertensive agents: a long way to safe drug prescribing in children
The objective of this article is to review the literature for safety and efficacy of commonly prescribed antihypertensive agents in pediatrics. Thus far, the most data to support use in children was found for angiotensin-converting enzyme inhibitors (ACE-I), angiotensin receptor blockers (A RB), and calcium channel blockers (CCB). Several gaps were noted in the literature, particularly for beta blockers, vasodilators, and the long-term safety profile of antihypertensive agents in children. Further clinical trials are needed to guide safe and effective prescribing in the pediatric popul ation. (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 25, 2020 Category: Urology & Nephrology Source Type: research

Rasburicase improves the outcome of acute kidney injury from typical hemolytic uremic syndrome
AbstractBackgroundTypical hemolytic uremic syndrome (HUS) causes acute kidney injury (AKI) and serious sequelae of chronic kidney disease (CKD) in some. Hyperuricemia is a common finding in typical HUS that may contribute to kidney damage. We explored whether aggressive management of hyperuricemia with rasburicase could improve outcomes in AKI patients with typical HUS.MethodsWe retrospectively analyzed medical records of children with typical HUS admitted to a tertiary center between 2005 and 2017. We compared clinical outcomes of hospitalization and 1-year post-discharge between those with rasburicase treatment (n = 13) ...
Source: Pediatric Nephrology - September 25, 2020 Category: Urology & Nephrology Source Type: research

Metabolic risk factors in children with kidney stone disease: an update
ConclusionsWe conclude that specific urinary metabolic risk factors can be found in most children with kidney stones, with hypercalciuria and hypocitraturia being the most common diagnoses.Graphical abstract. (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 25, 2020 Category: Urology & Nephrology Source Type: research

Persistent hyperkalemia in an otherwise healthy 4-month-old female: Answers
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 25, 2020 Category: Urology & Nephrology Source Type: research

Role of pediatric nephrologists in managing adults with AKI due to COVID-19
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 25, 2020 Category: Urology & Nephrology Source Type: research

A child with painful swollen joints, rash and proteinuria: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 25, 2020 Category: Urology & Nephrology Source Type: research

A child with painful swollen joints, rash and proteinuria: Answers
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 25, 2020 Category: Urology & Nephrology Source Type: research

Common presentation of micro hematuria and proteinuria in an uncommon disease: Answers
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 25, 2020 Category: Urology & Nephrology Source Type: research

Common presentation of micro hematuria and proteinuria in an uncommon disease: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 25, 2020 Category: Urology & Nephrology Source Type: research

Persistent hyperkalemia in an otherwise healthy 4-month-old female: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 25, 2020 Category: Urology & Nephrology Source Type: research

An unusual etiology of thrombotic microangiopathy in an adolescent male: Answers
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 18, 2020 Category: Urology & Nephrology Source Type: research

Clinical and histological differences between adults and children in new onset IgA nephropathy
We report higher eGFR at diagnosis in children compared to adults (89.5 vs. 64 ml/min/1.73 m2;p = 0.0001) but no difference in proteinuria. Histological analysis of kidney biopsy found higher proportions of mesangial (M1) and endocapillary (E1) hypercellularity in children compared with adults (M1 [80.7% vs. 27.9%,p = 0.0001]; E1 [71.3% vs. 30%,p = 0.0001]). Focal glomerulosclerosis (S1), tubular atrophy/interstitial fibrosis≥ 25% (T1), and P1 were more frequent in adults (S1 [81.5% vs. 61.3%,p = 0.0012], T1 [49.5% vs. 1.35%,p = 0.0001], P1 [33.8% vs. 16.4%,p = 0.008). Proteinuria associated with M1, E1, and C1 in child...
Source: Pediatric Nephrology - September 18, 2020 Category: Urology & Nephrology Source Type: research

A brief history of rickets
AbstractThe review provides a historical perspective on the convergence of our understanding of the physiology and pathophysiology of bone, calcium, phosphorus, vitamin D, parathyroid hormone, and FGF-23 their impact on rickets. (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 18, 2020 Category: Urology & Nephrology Source Type: research

Hypomagnesemia is underestimated in children with HNF1B mutations
AbstractBackgroundHypomagnesemia in patients with congenital anomalies of the kidneys and urinary tract or autosomal dominant tubulointerstitial kidney disease is highly suggestive ofHNF1B-associated disease. Intriguingly, the frequency of low serum Mg2+ (sMg) level varies and is lower in children than in adults withHNF1B mutations that could be partially due to application of inaccurate normal limit of sMg, irrespective of age and gender. We aimed to re-assess cross-sectionally and longitudinally the frequency of hypomagnesemia inHNF1B disease by using locally derived reference values of sMg.MethodsFourteen children withH...
Source: Pediatric Nephrology - September 18, 2020 Category: Urology & Nephrology Source Type: research

Dietary calcium intake does not meet the nutritional requirements of children with chronic kidney disease and on dialysis
AbstractBackgroundAdequate calcium (Ca) intake is required for bone mineralization in children. We assessed Ca intake from diet and medications in children with CKD stages 4 –5 and on dialysis (CKD4–5D) and age-matched controls, comparing with the UK Reference Nutrient Intake (RNI) and international recommendations.MethodsThree-day prospective diet diaries were recorded in 23 children with CKD4 –5, 23 with CKD5D, and 27 controls. Doses of phosphate (P) binders and Ca supplements were recorded.ResultsMedian dietary Ca intake in CKD4 –5D was 480 (interquartile range (IQR) 300–621) vs 724 (IQR 57...
Source: Pediatric Nephrology - September 18, 2020 Category: Urology & Nephrology Source Type: research

Nephrotoxins and nephrotoxic acute kidney injury
AbstractAlthough the concept of nephrotoxicity has been recognized for more than 80 years, interest in nephrotoxins has intensified dramatically over the past two decades. Much of this attention has rightfully been focused on pharmaceutical agents and iatrogenic harm; however, it is important for providers to recognize that nephrotoxins can be found in naturally occurring substances as well. Although nephrotoxins exist in a myriad of forms, the means by which they induce injury can be organized into a few categories. For most of these agents, regardless of the mechanism, the final common pathway is acute kidney injury (AKI...
Source: Pediatric Nephrology - September 18, 2020 Category: Urology & Nephrology Source Type: research

Kidney health for everyone everywhere —from prevention to detection and equitable access to care
AbstractThe global burden of chronic kidney disease (CKD) is rapidly increasing with a projection of becoming the 5th most common cause of years of life lost globally by 2040. Aggravatingly, CKD is a major cause of catastrophic health expenditure. The costs of dialysis and transplantation consume up to 3% of the annual healthcare budget in high-income countries. Crucially, however, the onset and progression of CKD are often preventable. In 2020, the World Kidney Day campaign highlights the importance of preventive interventions —be it primary, secondary, or tertiary. This complementing article focuses on outlining an...
Source: Pediatric Nephrology - September 18, 2020 Category: Urology & Nephrology Source Type: research

Mineralized tissues in hypophosphatemic rickets
AbstractHypophosphatemic rickets is caused by renal phosphate wasting that is most commonly due to X-linked dominant mutations inPHEX.PHEX mutations cause hypophosphatemia indirectly, through the increased expression of fibroblast growth factor 23 (FGF23) by osteocytes. FGF23 decreases renal phosphate reabsorption and thereby increases phosphate excretion. The lack of phosphate leads to a mineralization defect at the level of growth plates (rickets), bone tissue (osteomalacia), and teeth, where the defect facilitates the formation of abscesses. The bone tissue immediately adjacent to osteocytes often remains unmineralized ...
Source: Pediatric Nephrology - September 18, 2020 Category: Urology & Nephrology Source Type: research

A riddle wrapped in an enigma: acute kidney injury in a girl with Crohn ’s disease: Answers
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 18, 2020 Category: Urology & Nephrology Source Type: research

Congenital nephrotic syndrome: is early aggressive treatment needed? Yes
AbstractCongenital nephrotic syndrome (CNS) was primarily considered one disease entity. Hence, one treatment protocol was proposed in the beginning to all CNS patients. Today, with the help of gene diagnostics, we know that CNS is a heterogeneous group of disorders and therefore, different treatment protocols are needed. The most important gene defects causing CNS areNPHS1,NPHS2,WT1,LAMB2, andPLCE1. Before active treatment, all infants with CNS died. It was stated already in the mid-1980s that intensive medical therapy followed by kidney transplantation (KTx) should be the choice of treatment for infants with severe CNS. ...
Source: Pediatric Nephrology - September 18, 2020 Category: Urology & Nephrology Source Type: research

Predicting outcomes and improving care in children with congenital kidney anomalies
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 18, 2020 Category: Urology & Nephrology Source Type: research

A riddle wrapped in an enigma: acute kidney injury in a girl with Crohn ’s disease: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 18, 2020 Category: Urology & Nephrology Source Type: research

A child with familial glomerulonephritis: Answers
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 18, 2020 Category: Urology & Nephrology Source Type: research

Response to letter to the editor
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 18, 2020 Category: Urology & Nephrology Source Type: research

C3 levels and neurologic involvement in hemolytic uremic syndrome related to Shiga toxin –producing Escherichia coli
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 18, 2020 Category: Urology & Nephrology Source Type: research

An unusual etiology of thrombotic microangiopathy in an adolescent male: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 18, 2020 Category: Urology & Nephrology Source Type: research

Correction to: C3 levels and neurologic involvement in hemolytic uremic syndrome related to Shiga toxin –producing Escherichia coli
The original version of the letter unfortunately contained a mistake. (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 18, 2020 Category: Urology & Nephrology Source Type: research

A child with familial glomerulonephritis: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 18, 2020 Category: Urology & Nephrology Source Type: research

Aortic dilatation in children with chronic kidney disease
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 18, 2020 Category: Urology & Nephrology Source Type: research

Correction to: Aortic dilatation in children with mild to moderate chronic kidney disease
The original version of this article unfortunately contained a mistake. (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 18, 2020 Category: Urology & Nephrology Source Type: research

Characterizing dynamics of serum creatinine and creatinine clearance in extremely low birth weight neonates during the first 6 weeks of life
ConclusionsWe report the first mathematical model able to characterize Scr and CLcr in ELBW neonates during the first 6  weeks of life in a quantitative manner as a function of GA, MOD, and ibuprofen treatment. This model allows the derivation of GA-adjusted reference ranges for ELBW neonates and provides a rationale for normative Scr concentrations, and as such will help clinicians to further optimize monitoring an d treatment decisions in this vulnerable patient population. (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 17, 2020 Category: Urology & Nephrology Source Type: research

Comparing predictive values of carbohydrate antigen 19-9, neutrophil gelatinase –associated lipocalin, and kidney injury molecule-1 in 161 patients with ureteropelvic junction obstruction
ConclusionsOur evaluations demonstrate the higher efficacy of CA19-9 to predict the requirement of surgical intervention in comparison with the other biomarkers, as well as a significant correlation between kidney function deterioration and urinary CA19-9 and NGAL. The outcomes of this investigation could pave the way for more extensive clinical application of these urinary biomarkers, besides future research determining the association between markers and kidney fibrosis. (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 16, 2020 Category: Urology & Nephrology Source Type: research

Pediatric kidney transplantation in China: an analysis from the IPNA Global Kidney Replacement Therapy Registry
AbstractBackgroundThe International Pediatric Nephrology Association (IPNA) Global Kidney Replacement Therapy (KRT) Registry was established to evaluate the incidence and outcomes of kidney replacement therapy (dialysis and transplantation) provided to children worldwide. Analysis of registry data for separate regions is feasible.MethodsThree centers located in Shanghai, Guangzhou, and Zhengzhou, which have the greatest number of pediatric kidney transplantation cases in China, participated in this analysis of transplant data. Data were registered by each center for patients under the age of 19  years who received a s...
Source: Pediatric Nephrology - September 15, 2020 Category: Urology & Nephrology Source Type: research

Urine collection methods and dipstick testing in non-toilet-trained children
AbstractUrinary tract infection is a commonly occurring paediatric infection associated with significant morbidity. Diagnosis is challenging as symptoms are non-specific and definitive diagnosis requires an uncontaminated urine sample to be obtained. Common techniques for sampling in non-toilet-trained children include clean catch, bag, pad, in-out catheterisation and suprapubic aspiration. The pros and cons of each method are examined in detail in this review. They differ significantly in frequency of use, contamination rates and acceptability to parents and clinicians. National guidance of which to use differs significan...
Source: Pediatric Nephrology - September 12, 2020 Category: Urology & Nephrology Source Type: research

Resident foreign patients receive adequate dialysis but fewer preemptive transplantations: data from the Italian pediatric dialysis registry
AbstractBackgroundSociocultural issues play a key role in children needing kidney replacement therapy (KRT).MethodsData of incident patients
Source: Pediatric Nephrology - September 10, 2020 Category: Urology & Nephrology Source Type: research

Adherence to cysteamine in nephropathic cystinosis: A unique electronic monitoring experience for a better understanding. A prospective cohort study: CrYSTobs
ConclusionOur data are the first describing a rather good adherence to cysteamine, decreasing in adolescents and adults. We described a potential interest of the delayed release formulation. Our data highlight the need for a multidisciplinary approach including therapeutic education and individualized approaches in NC patients transitioning to adulthood.Graphical abstract (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 9, 2020 Category: Urology & Nephrology Source Type: research

Pathophysiology and consequences of arterial stiffness in children with chronic kidney disease
AbstractChanges in arterial structure and function are seen early in the course of chronic kidney disease (CKD) and have been causally associated with cardiovascular (CV) morbidity. Numerous potential injuries encompassing both traditional and uremia-specific CV risk factors can induce structural arterial changes and accelerate arterial stiffening. When the buffering capacity of the normally elastic arteries is reduced, damage to vulnerable microcirculatory beds can occur. Moreover, the resultant increase to cardiac afterload contributes to the development of left ventricular hypertrophy and cardiac dysfunction. Adult stud...
Source: Pediatric Nephrology - September 7, 2020 Category: Urology & Nephrology Source Type: research

Lower albumin level and longer disease duration are risk factors of acute kidney injury in hospitalized children with nephrotic syndrome
ConclusionsAKI is not uncommon in hospitalized children with NS, and its incidence in this nationwide study was 16.2%. Risk factors for AKI in hospitalized children with NS include longer disease duration, lower albumin level, and methylprednisolone pulse therapy. Pediatric NS patients with these characteristics should be under more strict scrutiny for the occurrence of AKI.Graphical abstract (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 5, 2020 Category: Urology & Nephrology Source Type: research

Therapy and outcomes of C3 glomerulopathy and immune-complex membranoproliferative glomerulonephritis
AbstractBackgroundData on therapy and outcome of dense deposit disease (DDD), C3 glomerulonephritis (C3GN), and immune-complex MPGN (IC-MPGN) in children are limited.MethodsIn this retrospective single-center study from 2007 to 2019, kidney biopsies were reviewed to include patients aged
Source: Pediatric Nephrology - September 4, 2020 Category: Urology & Nephrology Source Type: research

Bilateral native nephrectomies for severe hypertension in children with stage 5 chronic kidney disease leads to improved BP control following transplantation
ConclusionBNN for severe hypertension in CKD  5 is associated with resolution of hypertension prior to KT. It is also associated with a trend towards lower prevalence of hypertension and good graft function following KT. (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 3, 2020 Category: Urology & Nephrology Source Type: research

Transforming growth factor beta signaling functions during mammalian kidney development
AbstractAberrant transforming growth factor beta (TGF β) signaling during embryogenesis is implicated in severe congenital abnormalities, including kidney malformations. However, the molecular mechanisms that underlie congenital kidney malformations related to TGFβ signaling remain poorly understood. Here, we review current understanding of the linea ge-specific roles of TGFβ signaling during kidney development and how dysregulation of TGFβ signaling contributes to the pathogenesis of kidney malformation. (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 3, 2020 Category: Urology & Nephrology Source Type: research

Evolution of isolated systolic hypertension with normal central blood pressure in adolescents —prospective study
ConclusionsIn conclusion, after 1  year of non-pharmacological treatment, 23% of adolescents with sHT developed sustained hypertension, with the main predictor of cSBP change being the change in serum uric acid. (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - September 3, 2020 Category: Urology & Nephrology Source Type: research

Prematurity, perinatal inflammatory stress, and the predisposition to develop chronic kidney disease beyond oligonephropathy
AbstractPrematurity and perinatal stress, such as intrauterine growth restriction (IUGR) and chorioamnionitis, are pathological processes creating an impaired intrauterine environment. These intrauterine factors are associated with the development of proteinuria, hypertension, and chronic kidney disease (CKD) later in life. Initially, this was thought to be secondary to oligonephropathy, subsequent glomerular hypertrophy, and hyperfiltration, leading to glomerulosclerosis, a further decrease in nephron number, and finally CKD. Nowadays, there is increasing evidence that prematurity and perinatal stress affect not only neph...
Source: Pediatric Nephrology - September 3, 2020 Category: Urology & Nephrology Source Type: research