The roles of homocysteinemia and methylmalonic acidemia in kidney injury in atypical hemolytic uremic syndrome caused by cobalamin C deficiency
ConclusionWe question the roles of homocysteine and MMA as causative of aHUS/TMA in cblC deficiency as they remained elevated during follow-up but did not result in aHUS/TMA or AKI. Hyperhomocysteinemia and/or MMA caused by other metabolic diseases do not result in aHUS/TMA or AKI. This suggests that other nephrotoxic factors may trigger aHUS/TMA in cblC patients. (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - December 2, 2021 Category: Urology & Nephrology Source Type: research

Recurrent acute kidney injury and rhabdomyolysis: Answers
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - December 1, 2021 Category: Urology & Nephrology Source Type: research

Osteopetrosis and renal tubular acidosis: Answers
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - December 1, 2021 Category: Urology & Nephrology Source Type: research

Osteopetrosis and renal tubular acidosis: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - December 1, 2021 Category: Urology & Nephrology Source Type: research

A rare cause of membranoproliferative patterns of injury in siblings with steroid-resistant nephrotic syndrome: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - December 1, 2021 Category: Urology & Nephrology Source Type: research

Recurrent pain in a child with cerebral palsy: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - December 1, 2021 Category: Urology & Nephrology Source Type: research

Macroscopic hematuria in a child with portal hypertension: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - December 1, 2021 Category: Urology & Nephrology Source Type: research

Pancytopenia and acute glomerulonephritis in an adolescent: Answers
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - December 1, 2021 Category: Urology & Nephrology Source Type: research

Worsening anasarca on a child with severe steroid-dependent nephrotic syndrome without proteinuria: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - December 1, 2021 Category: Urology & Nephrology Source Type: research

Correction to: Different approaches to long-term treatment of aHUS due to MCP mutations: a multicenter analysis
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - December 1, 2021 Category: Urology & Nephrology Source Type: research

Correction to: An infant with hyperechoic cystic kidneys and congenital diaphragmatic hernia: Answers
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - December 1, 2021 Category: Urology & Nephrology Source Type: research

A child with persistent low-grade fever, urinary frequency, and hypertension: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - December 1, 2021 Category: Urology & Nephrology Source Type: research

Cystinuria: an update on pathophysiology, genetics, and clinical management
AbstractCystinuria is the most common genetic cause of nephrolithiasis in children. It is considered a heritable aminoaciduria as the genetic defect affects the reabsorption of cystine and three other amino acids (ornithine, lysine, and arginine) in the renal proximal tubule. Patients affected by this condition have elevated excretion of cystine in the urine, and because of this amino acid ’s low solubility at normal urine pH, patients tend to form cystine calculi. To date, two genes have been identified as disease-causative:SLC3A1 andSLC7A9, encoding for the two subunits of the heterodimeric transporter. The clinica...
Source: Pediatric Nephrology - November 23, 2021 Category: Urology & Nephrology Source Type: research

Structural brain abnormalities in children and young adults with severe chronic kidney disease
AbstractBackgroundThe pathophysiology of neurological dysfunction in severe chronic kidney disease (CKD) in children and young adults is largely unknown. We aimed to investigate brain volumes and white matter integrity in this population and explore brain structure under different treatment modalities.MethodsThis cross-sectional study includes 24 patients with severe CKD (eGFR  
Source: Pediatric Nephrology - November 20, 2021 Category: Urology & Nephrology Source Type: research

Nutcracker syndrome: a potentially underdiagnosed cause of proteinuria in children with familial Mediterranean fever
ConclusionNutcracker syndrome is the leading cause of proteinuria in children with FMF nowadays, and it should be kept in mind during the evaluation of proteinuria in these patients.Graphical AbstractA higher resolution version of the Graphical abstract is available asSupplementary information. (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - November 18, 2021 Category: Urology & Nephrology Source Type: research

Practice patterns and outcomes for pediatric ANCA vasculitis transplant recipients in a national cohort
ConclusionsKidney transplant is a safe treatment modality for children with ANCA-related kidney failure. ANCA patients have comparable graft survival when compared to the general transplant population with a low risk of recurrence. Thymoglobulin was used in a higher proportion within the ANCA group compared to the non-ANCA group. Tacrolimus, mycophenolate mofetil/mycophenolic acid, and steroids were the predominant maintenance immunosuppression used in both groups.Graphical abstractA higher resolution version of the Graphical abstract is available as Supplementary information. (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - November 18, 2021 Category: Urology & Nephrology Source Type: research

Low variability of plant protein intake in the CKiD cohort does not demonstrate changes in estimated GFR nor electrolyte balance
ConclusionsA diverse cohort of children with CKD has a narrow and homogeneous intake of vegetable protein. Due to the low variability of plant-based protein in the cohort, there were no associations between the percentage of plant protein intake and changes in eGFR nor electrolyte balance.A higher resolution version of the Graphical abstract is available asSupplementary information (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - November 18, 2021 Category: Urology & Nephrology Source Type: research

Determinants of medication adherence in childhood nephrotic syndrome and associations of adherence with clinical outcomes
AbstractBackgroundPediatric patients with nephrotic syndrome take medications long-term with significant toxicity and complex regimens, yet data on medication adherence are limited.MethodsIn a multicenter observational study of patients with nephrotic syndrome, NEPTUNE (NCT01209000), we surveyed caregivers of patients
Source: Pediatric Nephrology - November 18, 2021 Category: Urology & Nephrology Source Type: research

Clinical and neurophysiological characterization of early neuromuscular involvement in children and adolescents with nephropathic cystinosis
ConclusionsWe expanded the recognized phenotypes of cystinosis neuromuscular complications with early proximal skeletal myopathy and symptomatic motor and sensory axonal neuropathy. Early asymptomatic neuromuscular complications could develop in pediatric patients and would require neurophysiological studies for early detection prior to development of overt clinical manifestations. Prompt diagnosis and timely initiation of cysteamine therapy with recommended dose can delay the development of neuromuscular complications.Graphical abstractA higher resolution version of the Graphical abstract is available as Supplementary inf...
Source: Pediatric Nephrology - November 18, 2021 Category: Urology & Nephrology Source Type: research

An unusual case of dysuria, pollakisuria, and eosinophilia: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - November 18, 2021 Category: Urology & Nephrology Source Type: research

Recurrent hypocalcemic tetany presenting to the emergency room: Answers
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - November 18, 2021 Category: Urology & Nephrology Source Type: research

A 5-year-old boy with left flank mass and pain: Answers
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - November 18, 2021 Category: Urology & Nephrology Source Type: research

A 5-year-old boy with left flank mass and pain: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - November 18, 2021 Category: Urology & Nephrology Source Type: research

Recurrent hypocalcemic tetany presenting to the emergency room: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - November 18, 2021 Category: Urology & Nephrology Source Type: research

An unusual case of dysuria, pollakisuria, and eosinophilia: Answers
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - November 18, 2021 Category: Urology & Nephrology Source Type: research

Referees for 2021
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - November 18, 2021 Category: Urology & Nephrology Source Type: research

Response to Battaglia and Balestracci
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - November 18, 2021 Category: Urology & Nephrology Source Type: research

A novel prognostic index in hemolytic uremic syndrome related to Shiga toxin –producing Escherichia coli
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - November 18, 2021 Category: Urology & Nephrology Source Type: research

IgA nephropathy presenting as rapidly progressive glomerulonephritis following first dose of COVID-19 vaccine
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - November 16, 2021 Category: Urology & Nephrology Source Type: research

Long-term obesity prevalence and linear growth in children with idiopathic nephrotic syndrome: is normal growth and weight control possible with steroid-sparing drugs and low-dose steroids for relapses?
The objective of this study was to determine the prevalence of obesity and overweight and analyze linear growth in children with nephrotic syndrome.MethodsThe study involved 265 children treated with glucocorticoids for nephrotic syndrome for a mean duration of 43  months (range: 6–167, IQR: 17, 63.3). Height, weight, and BMI SDS were recorded at each visit. Rate of change between the final and initial height, weight, and BMI was calculated (Δ score). The cumulative steroid dose (mg/kg/day) during follow-up was calculated. Relapses without significant ede ma were treated with low-dose steroids and steroid-...
Source: Pediatric Nephrology - November 12, 2021 Category: Urology & Nephrology Source Type: research

Anti-glomerular basement membrane disease in children: a brief overview
AbstractAnti-glomerular basement membrane disease (Anti-GBM), previously known as Goodpasture syndrome, is an extremely rare cause of rapidly progressive glomerulonephritis and chronic kidney disease stage 5 (CKD5) in children. It is associated with acute pulmonary haemorrhage and it has a poor prognosis. It is classified as an autoimmune, small-vessel vasculitis caused by autoantibody formation against the alpha-3 chain in type IV collagen found in the glomerular basement membrane. Evidence of anti-GBM antibodies in serum or histologically are required for diagnosis. Treatment in children is based on very limited adult da...
Source: Pediatric Nephrology - November 12, 2021 Category: Urology & Nephrology Source Type: research

Whole exome sequencing identifies monogenic forms of nephritis in a previously unsolved cohort of children with steroid-resistant nephrotic syndrome and hematuria
ConclusionsWe confirmed that in cohorts of childhood-onset SRNS, patients with nephritic syndrome can be discerned by WES. The findings highlight the importance of clinical genetic testing for therapeutic and preventative measures in patients with proteinuria.Graphical abstractA higher resolution version of the Graphical abstract is available asSupplementary information. (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - November 11, 2021 Category: Urology & Nephrology Source Type: research

Large inter-assay difference of serum creatinine in pediatric population: a threat to accurate staging of chronic kidney disease
AbstractBackgroundSerum creatinine concentration is a primary component of Bedside Schwartz equation for estimated glomerular filtration rate (eGFR) in children. To standardize creatinine measurement, most manufacturers have adopted calibration procedures traceable to isotope dilution mass spectrometry (IDMS) using National Institute of Standards and Technology reference material. However, reference material representing much lower creatinine concentrations seen in children is not available and it is unclear how well commercial assays perform at pediatric levels.MethodsOne thousand nine hundred seventy-one specimens from consecutive children
Source: Pediatric Nephrology - November 10, 2021 Category: Urology & Nephrology Source Type: research

Development of a patient-reported outcome measure for the assessment of symptom burden in pediatric chronic kidney disease (PRO-Kid)
ConclusionsPRO-Kid is the first pediatric CKD/kidney failure-specific PRO tool to assess symptom burden. Future work should validate this tool in a larger cohort so that it may be used to improve the care of children living with CKD/kidney failure.Graphical abstractA higher resolution version of the Graphical abstract is available as Supplementary information (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - November 10, 2021 Category: Urology & Nephrology Source Type: research

When the progresses in neonatology lead to severe congenital nephron deficit: is there a pilot in the NICU?
AbstractAdvances in the care of neonates to the extreme limits of viability have increased the risk of severe comorbidities in surviving preemies. The respiratory and the neurodevelopmental consequences of premature birth and/or intra-uterine growth retardation have been well described. Because of the usual clinical silence of the kidney, the long-term renal consequences of low birth weight have not been as well studied. A case report illustrates the risk factors associated with low birth weight and prematurity and discusses the pathogenesis of the late consequences of the congenital nephron deficit associated with a low b...
Source: Pediatric Nephrology - November 10, 2021 Category: Urology & Nephrology Source Type: research

Interference of ketone bodies on laboratory creatinine measurement in children with DKA: a call for change in testing practices
ConclusionsCompared to the enzymatic methods, the Jaffe methods were much more susceptible to interference by acetone and acetoacetate, especially at lower Cr values which are commonly seen in pediatrics. Interpretation of changes in Cr concentration between different hospitals when transferring patients can become ambiguous and true kidney function unclear if different methods are used without awareness of method-specific biases. To improve DKA patient care, we recommend standardizing all of the Cr methods to an enzymatic method.Graphical abstractA higher resolution version of the Graphical abstract is available asSupplem...
Source: Pediatric Nephrology - November 10, 2021 Category: Urology & Nephrology Source Type: research

The relationship between hyperchloremia and acute kidney injury in pediatric diabetic ketoacidosis and its impact on clinical outcomes
ConclusionThe development of AKI in patients with DKA was accompanied by hyperchloremia, increased time to DKA resolution, and longer hospital stay.Graphical abstract“A higher resolution version of the Graphical abstract is available as Supplementary information.” (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - November 5, 2021 Category: Urology & Nephrology Source Type: research

What a paediatric nephrologist should know about preeclampsia and why it matters
AbstractPreeclampsia is a protean syndrome causing a kidney disease characterised by hypertension and proteinuria, usually considered transitory and reversible after delivery. Its prevalence ranges from 3 –5 to 10% if all the related disorders are considered. This narrative review, on behalf of the Kidney and Pregnancy Study Group of the Italian Society of Nephrology, focuses on three reasons why preeclampsia should concern paediatric nephrologists and how they can play an important role in its pre vention, as well as in the prevention of future kidney and cardiovascular diseases. Firstly, all diseases of the kidney ...
Source: Pediatric Nephrology - November 4, 2021 Category: Urology & Nephrology Source Type: research

Teenagers and young adults with a past of allogenic hematopoietic stem cell transplantation are at significant risk of chronic kidney disease
AbstractBackgroundAllogenic hematopoietic stem cell transplantation (aHSCT) remains the treatment of choice for some malignant hemopathies in children, albeit with the risk of long-term consequences, including chronic kidney disease (CKD).MethodsIn our single tertiary referral center, we retrospectively assessed the long-term renal outcome in a cohort of children and adolescents who had undergone aHSCT for malignant hemopathies between 2003 and 2017. We distinguished glomerular and tubular dysfunctions and assessed the accuracy of the most common formula(s) to estimate glomerular filtration rate (GFR) during standard clini...
Source: Pediatric Nephrology - November 4, 2021 Category: Urology & Nephrology Source Type: research

Prof. Man-Chun Chiu (1949 –2021)
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - November 4, 2021 Category: Urology & Nephrology Source Type: research

An initiative to improve pneumococcal immunization counseling in children with nephrotic syndrome
ConclusionBridging the knowledge gap in provider awareness and using specific EHR reminders can improve adherence to PPSV23 counseling in children with nephrotic syndrome. Such interventions could be applied to similar groups of immunocompromised patients in whom additional vaccines are indicated.Graphical abstractA higher resolution version of the Graphical abstract is available as Supplementary information. (Source: Pediatric Nephrology)
Source: Pediatric Nephrology - November 3, 2021 Category: Urology & Nephrology Source Type: research

A rare cause of chronic tubulointerstitial nephritis in childhood: Answers
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - November 3, 2021 Category: Urology & Nephrology Source Type: research

A rare cause of chronic tubulointerstitial nephritis in childhood: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - November 3, 2021 Category: Urology & Nephrology Source Type: research

Amenorrhea in a pediatric kidney transplant recipient: Answers
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - November 3, 2021 Category: Urology & Nephrology Source Type: research

Amenorrhea in a pediatric kidney transplant recipient: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - November 3, 2021 Category: Urology & Nephrology Source Type: research

Leveraging neuroimaging to understand the impact of chronic kidney disease on the brain
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - November 3, 2021 Category: Urology & Nephrology Source Type: research

Association between fluid overload and mortality in newborns: a systematic review and meta-analysis
AbstractFluid overload (FO) is associated with higher rates of mortality and morbidity in pediatric and adult populations. The aim of this systematic review and meta-analysis was to investigate the association between FO and mortality in critically ill neonates. Systematic search of Ovid MEDLINE, EMBASE, Cochrane Library, trial registries, and gray literature from inception to January 2021. We included all studies that examined neonates admitted to neonatal intensive care units and described FO and outcomes of interest. We identified 17 observational studies with a total of 4772 critically ill neonates who met the inclusio...
Source: Pediatric Nephrology - November 2, 2021 Category: Urology & Nephrology Source Type: research

Two cases of children presenting with polydipsia, polyuria, and malignant hypertension: Answers
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - November 2, 2021 Category: Urology & Nephrology Source Type: research

What is the cause of kidney dysfunction in a newborn with trisomy 21? Answers
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - November 2, 2021 Category: Urology & Nephrology Source Type: research

Two cases of children presenting with polydipsia, polyuria, and malignant hypertension: Questions
(Source: Pediatric Nephrology)
Source: Pediatric Nephrology - November 2, 2021 Category: Urology & Nephrology Source Type: research