Cystic kidney diseases associated with mutations in phosphomannomutase 2 promotor: a large spectrum of phenotypes
ConclusionThese data show that thePMM2 promotor variation,in trans of aPMM2 coding mutation, is associated with a wide spectrum of kidney phenotypes, and is not always associated with extra-renal symptoms. When present, extra-renal defects may include COACH-like syndrome. These data prompt screening ofPMM2 in unresolved cases of fetal hyperechogenic/cystic kidneys as well as in cystic kidney disease in children and adults.Graphical Abstract (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - July 6, 2021 Category: Urology & Nephrology Source Type: research

Malignancies after pediatric solid organ transplantation
AbstractAs life expectancy among pediatric solid organ transplant recipients (SOTRs) improves, the risk of comorbid conditions such as malignancy post-transplantation has also increased. SOTRs are at elevated risks of post-transplantation lymphoproliferative disorders (PTLDs), and skin and solid cancers. PTLDs typically occur early following transplantation, while skin and solid cancers frequently arise in young adulthood (25 –40 years). By 30 years following transplantation, 26–41% of pediatric SOTRs have developed cancer. Different risk factors exist for PTLD, and skin and solid cancers, which are modified by...
Source: Pediatric Nephrology - July 6, 2021 Category: Urology & Nephrology Source Type: research

Seasonal variation of blood pressure in children
AbstractSeasonal blood pressure (BP) variation is mostly found between the summer and winter months. Guidelines for diagnosis and treatment of hypertension in children have not considered this variation until recently. This review aims to present an overview of seasonal BP variation in childhood along with potential underlying pathophysiological mechanisms and long-term implications as well as conclusions for future studies. In pediatric cohorts, seven studies investigated seasonal changes in BP. These changes amount to 3.4 –5.9 mmHg (or 0.5–1.5 mmHg per − 1 °C difference in envi...
Source: Pediatric Nephrology - July 6, 2021 Category: Urology & Nephrology Source Type: research

Reducing the burden of cardiovascular disease in children with chronic kidney disease: prevention vs. damage limitation
AbstractCardiovascular disease (CVD) is a life-limiting condition in patients with chronic kidney disease (CKD) and is rapidly progressive, especially in those with stage 5 CKD and on dialysis. Cardiovascular mortality, although reducing, remains at least 30 times higher than in the general pediatric population. The American Heart Association guidelines for cardiovascular risk reduction in high-risk pediatric patients has stratified pediatric CKD patients in the “high risk” category for the development of CVD, with associated pathological and/or clinical evidence for manifest coronary disease before 30 years of...
Source: Pediatric Nephrology - July 6, 2021 Category: Urology & Nephrology Source Type: research

Metabolic programming of nephron progenitor cell fate
AbstractMetabolic pathways are one of the first responses at the cellular level to maternal/fetal interface stressors. Studies have revealed the previously unrecognized contributions of intermediary metabolism to developmental programs. Here, we provide an overview of cellular metabolic pathways and the cues that modulate metabolic states. We discuss the developmental and physiological implications of metabolic reprogramming and the key role of metabolites in epigenetic and epiproteomic modifications during embryonic development and with respect to kidney development and nephrogenesis. (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - July 6, 2021 Category: Urology & Nephrology Source Type: research

Eculizumab exposure in children and young adults: indications, practice patterns, and outcomes —a Pediatric Nephrology Research Consortium study
AbstractBackgroundEculizumab is approved for the treatment of atypical hemolytic uremic syndrome (aHUS). Its use off-label is frequently reported. The aim of this study was to describe the broader use and outcomes of a cohort of pediatric patients exposed to eculizumab.MethodsA retrospective, cohort analysis was performed on the clinical and biomarker characteristics of eculizumab-exposed patients
Source: Pediatric Nephrology - July 6, 2021 Category: Urology & Nephrology Source Type: research

Predicting acute rejection in children, adolescents, and young adults with a kidney transplant by assessing barriers to taking medication
ConclusionsPediatric and adolescent kidney transplant recipients with identified adherence barriers are at increased risk for acute rejection. Barriers to adherence provide a potentially modifiable therapeutic target that can be assessed in clinic to guide targeted interventions. (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - July 6, 2021 Category: Urology & Nephrology Source Type: research

APOL1 in an ethnically diverse pediatric population with nephrotic syndrome: implications in focal segmental glomerulosclerosis and other diagnoses
ConclusionsNovel discoveries include a substantial prevalence of HRG among patients self-reported White, worse kidney outcomes in HRGversus LRG children in the FSGS subgroup, and a trend of higher CKD progression risk associated with a single risk variant in the SRNS cohort. These findings suggestAPOL1-associated NS extends beyond patients self-reported non-White, the HRG effect is independent of FSGS, and a single risk variant may have a detrimental impact in children with NS. (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - July 6, 2021 Category: Urology & Nephrology Source Type: research

Transplantation outcomes in patients with primary hyperoxaluria: a systematic review
AbstractBackgroundPrimary hyperoxaluria type 1 (PH1) is characterized by hepatic overproduction of oxalate and often results in kidney failure. Liver-kidney transplantation is recommended, either combined (CLKT) or sequentially performed (SLKT). The merits of SLKT and the place of an isolated kidney transplant (KT) in selected patients are unsettled. We systematically reviewed the literature focusing on patient and graft survival rates in relation to the chosen transplant strategy.MethodsWe searched MEDLINE and Embase using a broad search string, consisting of the terms ‘transplantation’ and ‘hyperoxaluri...
Source: Pediatric Nephrology - July 6, 2021 Category: Urology & Nephrology Source Type: research

An unusual cause of immune complex-mediated membranoproliferative glomerulonephritis in a child: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - July 6, 2021 Category: Urology & Nephrology Source Type: research

Rickets, elevated fibroblast growth factor-23 and mild anemia: Answers
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - July 6, 2021 Category: Urology & Nephrology Source Type: research

Experience with telemedicine in paediatric nephrology during the COVID pandemic
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - July 6, 2021 Category: Urology & Nephrology Source Type: research

The red lipstick mentor
AbstractProf. Lesley Rees belongs to the women who changed science and has inspired so many young researchers, including myself. These inspiring women deserve more credit and recognition than they get because they serve as a role model for us. Indeed, despite advances towards women empowerment, progress has been slow, and discrepancy persists around the world. Unfortunately, science is not immune to such inequalities, and the voices of female leaders are important in cracking this gender filter. Women represent only a third of researchers globally and often face gender-based discrimination and lack equal opportunities. In ...
Source: Pediatric Nephrology - July 6, 2021 Category: Urology & Nephrology Source Type: research

An unusual cause of immune complex-mediated membranoproliferative glomerulonephritis in a child: Answers
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - July 6, 2021 Category: Urology & Nephrology Source Type: research

Urinary stone and infection does not always mean a ‘chicken and egg dilemma’: Answers
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - July 6, 2021 Category: Urology & Nephrology Source Type: research

Rickets, elevated fibroblast growth factor-23 and mild anemia: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - July 6, 2021 Category: Urology & Nephrology Source Type: research

Correction to: The association of alcohol, cigarette, e-cigarette, and marijuana use with disease severity in adolescents and young adults with pediatric chronic kidney disease
A Correction to this paper has been published:https://doi.org/10.1007/s00467-021-05131-7 (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - July 6, 2021 Category: Urology & Nephrology Source Type: research

Festschrift for Professor Lesley Rees MD FRCPCH
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - July 6, 2021 Category: Urology & Nephrology Source Type: research

Urinary stone and infection does not always mean a ‘chicken and egg dilemma’: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - July 6, 2021 Category: Urology & Nephrology Source Type: research

Topical Collection: Festschrift for Professor Lesley Rees MD FRCPCH
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - July 6, 2021 Category: Urology & Nephrology Source Type: research

To biopsy or not to biopsy: Henoch-Sch önlein nephritis in children, a 5-year follow-up study
ConclusionsChildren with early KB at diagnosis seem to have a better renal outcome at 5 years compared to those without an early biopsy at diagnosis or biopsied later. However, this is a small patient cohort and data are missing. Further work is needed to build consensual guidelines on the management of HSPN in children.Graphical abstract (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - July 5, 2021 Category: Urology & Nephrology Source Type: research

Tubulopathy and hepatomegaly in a 2-year-old boy: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - June 2, 2021 Category: Urology & Nephrology Source Type: research

A 17-year-old girl with primary amenorrhea: Questions
Abstract"Note: This is mandatory. Please provide." (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - June 2, 2021 Category: Urology & Nephrology Source Type: research

Correction to: Urine collection methods and dipstick testing in non-toilet-trained children
The original version of the letter unfortunately contained a mistake. The catheterisation cost cited in the paper should be £49.39 instead of £25.98. The corrected paragraph is shown below. The original article was corrected. (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - June 2, 2021 Category: Urology & Nephrology Source Type: research

A case with kidney transplant and cystinosis: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - June 2, 2021 Category: Urology & Nephrology Source Type: research

A case of painless exercise-induced gross hematuria in a 9-year-old boy: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - June 2, 2021 Category: Urology & Nephrology Source Type: research

An unusual complication of peritoneal dialysis: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - June 2, 2021 Category: Urology & Nephrology Source Type: research

A rare cause of childhood hypertension detected in a school screening program: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - June 2, 2021 Category: Urology & Nephrology Source Type: research

An unusual cause of hypertension: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - June 2, 2021 Category: Urology & Nephrology Source Type: research

A rare cause of transitory hematuria and urinary tract dysfunction in children: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - June 2, 2021 Category: Urology & Nephrology Source Type: research

A rare cause of subnephrotic proteinuria in an adolescent: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - June 2, 2021 Category: Urology & Nephrology Source Type: research

A child with a severe headache: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - June 2, 2021 Category: Urology & Nephrology Source Type: research

Hypokalemic metabolic alkalosis in an adolescent female: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - June 2, 2021 Category: Urology & Nephrology Source Type: research

Two faces of lupus nephritis? Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - June 2, 2021 Category: Urology & Nephrology Source Type: research

An uncommon cause of nephrocalcinosis in an infant: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - June 2, 2021 Category: Urology & Nephrology Source Type: research

The dietary management of potassium in children with CKD stages 2 –5 and on dialysis—clinical practice recommendations from the Pediatric Renal Nutrition Taskforce
We describe the assessment of dietary potassium intake, requirements for potassium in healthy children, and the dietary management of hypo- and hyperkalemia in children with CKD2–5D. Common potassium containing foods are described and approaches to adjusting potas sium intake that can be incorporated into everyday practice discussed. Given the poor quality of evidence available, a Delphi survey was conducted to seek consensus from international experts. Statements with a low grade or those that are opinion-based must be carefully considered and adapted to ind ividual patient needs, based on the clinical judgment of t...
Source: Pediatric Nephrology - April 30, 2021 Category: Urology & Nephrology Source Type: research

Eosinophilic peritonitis in children on chronic peritoneal dialysis
AbstractBackgroundEosinophilic peritonitis (EP) is sometimes difficult to distinguish from bacterial peritonitis (BP) at onset, as they are often overlapping. Previous reports show EP occurs more frequently in infants, although the reason is unknown.MethodsThe study population was 77 pediatric patients receiving chronic peritoneal dialysis (PD) in our center. We compared clinical and laboratory data at onset of EP with those of BP. We also investigated age distribution at onset of EP and PD-related surgery.ResultsEleven patients developed EP (18 episodes) and 19 patients developed BP (38 episodes). EP patients showed lower...
Source: Pediatric Nephrology - April 30, 2021 Category: Urology & Nephrology Source Type: research

Rituximab in children with steroid sensitive nephrotic syndrome: in quest of the optimal regimen
AbstractRituximab has emerged as an effective and important therapy in children with complicated frequently relapsing and steroid-dependent nephrotic syndrome to induce long-term disease remission and avoid steroid toxicities. The optimal rituximab regimen is not totally well defined, and there are many varying practices worldwide. We will in this review describe how patient factors, rituximab dose, and use of maintenance immunosuppression affect treatment outcomes. Specifically, low-dose rituximab without concomitant immunosuppression is associated with shorter relapse-free duration while other regimens have comparable ou...
Source: Pediatric Nephrology - April 30, 2021 Category: Urology & Nephrology Source Type: research

Maturation of glomerular filtration rate in neonates and infants: an overview
AbstractGlomerular filtration rate (GFR) increases progressively throughout fetal life, matures rapidly after birth according to gestational and post-menstrual age, and reaches adult values by 1-year post-natal age. GFR is considered the best marker of kidney function, and in clinical practice, estimated GFR is useful to anticipate complications, establish prognosis, and facilitate treatment decisions. This review article summarizes the maturation of glomerular filtration and the factors and conditions that modulate and impair developing glomerular filtration, and discusses the techniques available to assess GFR in neonate...
Source: Pediatric Nephrology - April 30, 2021 Category: Urology & Nephrology Source Type: research

Macroscopic hematuria in a patient with leukemia: Answers
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - April 30, 2021 Category: Urology & Nephrology Source Type: research

Rocky road —an uncommon reason for kidney allograft dysfunction: Answers
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - April 30, 2021 Category: Urology & Nephrology Source Type: research

Rocky road —an uncommon reason for kidney allograft dysfunction: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - April 30, 2021 Category: Urology & Nephrology Source Type: research

H. William Schnaper - Life course journey of a true Mensch
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - April 30, 2021 Category: Urology & Nephrology Source Type: research

Metabolic acidosis with increased anion gap, oxaluria, and acute kidney injury: Answers
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - April 30, 2021 Category: Urology & Nephrology Source Type: research

Treatment strategy for Streptococcus pneumoniae -associated hemolytic uremic syndrome
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - April 30, 2021 Category: Urology & Nephrology Source Type: research

Macroscopic hematuria in a patient with leukemia: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - April 30, 2021 Category: Urology & Nephrology Source Type: research

Regarding: A global anti-B cell strategy combining obinutuzumab and daratumumab in severe pediatric nephrotic syndrome
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - April 30, 2021 Category: Urology & Nephrology Source Type: research

Metabolic acidosis with increased anion gap, oxaluria, and acute kidney injury: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - April 30, 2021 Category: Urology & Nephrology Source Type: research

Response to Majeranowski
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - April 30, 2021 Category: Urology & Nephrology Source Type: research

Correction to: Childhood risk factors for adulthood chronic kidney disease
The authors regret that the name of the author Michal Stern-Zimmer was incorrectly rendered as “Michal Stern Zimmer.” (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - April 30, 2021 Category: Urology & Nephrology Source Type: research