Collapsing glomerulopathy (CG) is a proliferative disease characterized by segmental or global wrinkling of the glomerular basement membrane and the formation of pseudocrescents, whereas focal segmental glomerulosclerosis (FSGS) is characterized by podocytopenia, and focal and segmental sclerosis of the glomeruli. Mutations in NPHS1, NPHS2, WT1, PLCE1, CD2AP, ACTN4, and TRPC6 have been reported in steroid-resistant FSGS patients. The mutations p.
View Article and Find Full Text PDFClin Pediatr (Phila)
October 2024
We aimed to evaluate the clinical parameters, histopathological findings of nephrotic syndrome (NS) patients, and independent factors predicting steroid resistance in a single tertiary center. One hundred and sixty-two children (57 girls and 105 boys) with NS who were followed between 1998 and 2018 were analyzed in this retrospective cohort. The median (interquartile range; range) age and follow-up time were 4.
View Article and Find Full Text PDFBackground/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.
Background: Compared with the general population, the immune response to COVID-19 mRNA vaccines is lower in adult kidney transplant recipients (KTRs). However, data is limited for pediatric KTRs. In this study, we aimed to assess humoral and cellular immune responses to the COVID-19 mRNA vaccine in pediatric KTRs.
View Article and Find Full Text PDFIntroduction: Bartter syndrome (BS) is a group of autosomal-recessive tubular disorders and it is classified into five genetic subtypes. BS can also be classified by phenotype (antenatal, classic). Patients with mutations in the same gene can present different phenotypes.
View Article and Find Full Text PDFBackground: Cubilin is one of the receptor proteins responsible for reabsorption of albumin in proximal tubules and is encoded by the CUBN gene. We aimed to evaluate clinical and genetic characterization of six patients with proteinuria who had CUBN mutations.
Methods: Patients' characteristics, serum creatinine, albumin, vitamin B levels, urine analysis, spot urine protein/creatinine, microalbumin/creatinine, beta-2 microglobulin/creatinine ratios, estimated glomerular filtration rates (eGFR), treatments, kidney biopsies, and genetic analyses were evaluated.
Objective: We aimed to present the characteristics, genetic analysis results, long-term progno- sis of our patients with distal kidney tubular acidosis, and the relationship between hyperam- monemia and distal kidney tubular acidosis.
Materials And Methods: Biochemical, clinical, and imaging findings were collected at presen- tation and the last clinic visit, and results of the genetic analysis were recorded.
Results: Our study included 9 patients (3 female, 33%).
Background: There are various oral symptoms related to the disease and its management in individuals with chronic kidney disease (CKD). The aim of the study was to investigate the oral health status of children with different stages of CKD, kidney transplant recipients (KTR), and healthy children.
Methods: A total of seventy-one children diagnosed with CKD and fifty-two healthy children were included in the study.
Objective: Failure to achieve high levels of medication adherence in obstructive lung diseases is a major cause of uncontrolled disease. The purpose of this study is to reveal clinicians' opinions on the level of patient adherence and the change in adherence during the COVID-19 pandemic.
Methods: A questionnaire containing multiple-choice questions about treatment adherence in patients with obstructive lung diseases was voluntarily applied to doctors working in a tertiary hospital for chest diseases.
The study aims to present the incidence of COVID-19 in pediatric patients undergoing renal replacement therapy (RRT) and to compare the severity and outcomes of the disease between the dialysis and kidney transplant (KTx) groups. This multicenter observational study was conducted between 1 April and 31 December 2020 in Istanbul. Members of the Istanbul branch of the Turkish Pediatric Nephrology Association were asked to report all confirmed cases of COVID-19 who were on RRT, as well as the number of prevalent RRT patients under the age of 20.
View Article and Find Full Text PDFBackground/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.
Introduction: Damage to the glycosaminoglycan layer of the urothelium, which is composed of hyaluronic acid (HA), may increase the possibility of bacterial adherence and infections. Patients with neurogenic bladder (NB) who perform clean intermittent catheterization (CIC) 4-6 times a day are also under great risk for recurrent urinary tract infections (RUTIs).
Objective: The aim of this study was to assess the efficacy and safety of intravesical HA in reducing the frequency of RUTIs in patients with spina bifida (SB) and NB, who perform CIC.
Aim: Functional iron deficiency seco ndary to inflammation and increased serum hepcidin lead to erythropoietin-resistant anemia in children with chronic kidney disease. Vitamin D deficiency, parathyroid hormone, and phosphate can also participate in chronic inflammation and anemia. The aim of this study was to evaluate the association between hepcidin, bone mineral metabolism, and anemia in non-dialysis pediatric patients with chronic kidney disease.
View Article and Find Full Text PDFIntroduction: Prevalence of isolated nocturnal hypertension (INH) and isolated daytime hypertension (IDH) is around 10% in adults. Data in children, especially in chronic kidney disease (CKD), are lacking. The aim of this cross-sectional multicenter cohort study was to define the prevalence of INH and IDH and its association with cardiovascular morphology and function, that is, pulse wave velocity (PWV), carotid intima-media thickness (cIMT), or left ventricular mass index (LVMI) in children with CKD.
View Article and Find Full Text PDFKaradeniz-Cerit K, Thomas DT, Ergun R, Yıldız N, Alpay H, İnanır S, Dağlı ET, Tuğtepe H. Positional installation of contrast (PIC) and Redo-PIC cystography for diagnosis of occult vesicoureteral reflux. Turk J Pediatr 2018; 60: 180-187.
View Article and Find Full Text PDFInvasive aspergillosis caused by A. Fumigatus, almost occurs in immunocompromised hosts and has a poor prognosis. We report a case of invasive Aspergillosis in a 15-year-old boy with ESRD.
View Article and Find Full Text PDFBackground: The causative agent spectrum and resistance patterns of urinary tract infections in children are affected by many factors.
Aims: To demonstrate antibiotic resistance in urinary tract infections and changing ratio in antibiotic resistance by years.
Study Design: Retrospective cross-sectional study.
Background/aim: We analyzed the clinical and pathological features and prognosis of 106 children with persistent asymptomatic microscopic hematuria (PAMH) with or without mild proteinuria.
Materials And Methods: This was a retrospective study of 106 children who were referred to our clinics from 2000 to 2013 for evaluation of PAMH.
Results: Among the 106 patients, 69 (65%) were female and 37 (35%) were male.