Kidney transplantation in children and adolescents with C3 glomerulopathy or immune complex membranoproliferative glomerulonephritis: a real-world study within the CERTAIN research network.

Background: Complement 3 glomerulopathy (C3G) and immune complex membranoproliferative glomerulonephritis (IC-MPGN) are ultra-rare chronic kidney diseases with an overall poor prognosis, with approximately 40-50% of patients progressing to kidney failure within 10 years of diagnosis. C3G is characterized by a high rate of disease recurrence in the transplanted kidney. However, there is a lack of published data on clinical outcomes in the pediatric population following transplantation.

The role of anticomplement therapy in the management of the kidney allograft.

As the number of patients living with kidney failure grows, the need also grows for kidney transplantation, the gold standard kidney replacement therapy that provides a survival advantage. This may result in an increased rate of transplantation from HLA-mismatched donors that increases the rate of antibody-mediated rejection (AMR), which already is the leading cause of allograft failure. Plasmapheresis, intravenous immunoglobulin therapy, anti-CD20 therapies (i.

COVID-19 in pediatric nephrology centers in Turkey.

Background/aim: There is limited data on COVID-19 disease in children with kidney disease. We aimed to investigate the characteristics and prognosis of COVID-19 in pediatric nephrology patients in Turkey.

Materials And Methods: This was a national, multicenter, retrospective cohort study based on an online survey evaluating the data between 11 March 2020 and 11 March 2021 as an initial step of a detailed pediatric nephrology COVID-19 registry.

Clinical and molecular evaluation of MEFV gene variants in the Turkish population: a study by the National Genetics Consortium.

Familial Mediterranean fever (FMF) is a monogenic autoinflammatory disorder with recurrent fever, abdominal pain, serositis, articular manifestations, erysipelas-like erythema, and renal complications as its main features. Caused by the mutations in the MEditerranean FeVer (MEFV) gene, it mainly affects people of Mediterranean descent with a higher incidence in the Turkish, Jewish, Arabic, and Armenian populations. As our understanding of FMF improves, it becomes clearer that we are facing with a more complex picture of FMF with respect to its pathogenesis, penetrance, variant type (gain-of-function vs.

Improving the urine spot protein/creatinine ratio by the estimated creatinine excretion to predict proteinuria in pediatric kidney transplant recipients.

Background: Since the daily creatinine excretion rate (CER) is directly affected by muscle mass, which varies with age, gender, and body weight, using the spot protein/creatinine ratio (Spot P/Cr) follow-up of proteinuria may not always be accurate. Estimated creatinine excretion rate (eCER) can be calculated from spot urine samples with formulas derived from anthropometric factors. Multiplying Spot P/Cr by eCER gives the estimated protein excretion rate (ePER).

Macrophage Activation Syndrome in a Child with Juvenile Idiopathic Arthritis Secondary to SARS-CoV-2.

Severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) has caused a pandemic affecting many countries and millions of people. Physicians have encountered some rare and challenging cases related to SARS-CoV-2, a novel virus with still many unknowns. In order to share our experience of a such clinical picture, we present here a child with SARS-CoV-2-induced macrophage activation syndrome in the setting of juvenile idiopathic arthritis.

Rituximab treatment for difficult-to-treat nephrotic syndrome in children: a multicenter, retrospective study.

Background/aim: This study aimed to evaluate the efficacy of rituximab in children with difficult-to-treat nephrotic syndrome, considering the type of disease (steroid-sensitive or –resistant) and the dosing regimen.

Materials And Methods: This multicenter retrospective study enrolled children with difficult-to-treat nephrotic syndrome on rituximab treatment from 13 centers. The patients were classified based on low (single dose of 375 mg/m2) or high (2-4 doses of 375 mg/m2) initial dose of rituximab and the steroid response.

Is there a unique symptom in lower urinary tract dysfunction in children?

Objectives: Lower urinary tract symptoms (LUTS), particularly urgency, incontinence and intermittency are common in children and it is suggested that the specific symptoms may be used for definite diagnosis for LUT dysfunction (LUTD). This study was performed to investigate the relationship between each LUTD and its associated symptoms, using uroflowmetry/electromyography (UF/EMG) as a diagnostic tool.

Methods: Each patient was categorized into one of four LUT conditions which were overactive bladder (OAB), dysfunctional voiding (DV), underactive bladder and primary bladder neck dysfunction (PBND), according to UF/EMG results.

Symptomatology and Clinic of Hydronephrosis Associated With Uretero Pelvic Junction Anomalies.

The most common cause of hydronephrosis in the pediatric age group is ureteropelvic junction-type hydronephrosis (UPJHN). Since the advent of widespread maternal ultrasound screening, clinical presentation of hydronephrosis associated with UPJ anomalies has changed dramatically. Today most cases are diagnosed in the prenatal period, and neonates present without signs or symptoms.

Ambulatory arterial stiffness index is increased in obese children.

Background And Objectives: One way to measure arterial stiffness is the ambulatory arterial stiffness index (AASI), which is the relationship between diastolic and systolic ambulatory blood pressure (BP) over 24-hours.

Methods: We studied the difference in AASI between obese and lean children. AASI was calculated from 24- hour ambulatory blood pressure monitoring in 53 obese children (33 girls) and compared with age-matched 42 healthy subjects (20 girls).

Vascular Access Choice, Complications, and Outcomes in Children on Maintenance Hemodialysis: Findings From the International Pediatric Hemodialysis Network (IPHN) Registry.

Rationale & Objective: Arteriovenous fistulas (AVFs) have been recommended as the preferred vascular access for pediatric patients on maintenance hemodialysis (HD), but data comparing AVFs with other access types are scant. We studied vascular access choice, placement, complications, and outcomes in children.

Study Design: Prospective observational cohort study.