Objectives: To investigate the severe haematological involvement in children with SLE and assess its clinical associations, treatments, outcome and damage accrual.
Methods: The medical charts of children with SLE in whom haematological involvement was observed were reviewed. Severe haematological indices were defined as autoimmune haemolytic anaemia with a haemoglobin concentration < 8 g/dL, thrombocyte count < 30 000/µL, and neutrophil count < 500/µL.
Nephrol Dial Transplant
August 2024
Background: Emphysematous cystitis (EC) and emphysematous pyelonephritis (EPN) are rare urinary tract infections. They have a wide spectrum of clinical manifestations; ranging from asymptomatic to septic shock at presentation. In children, EC and EPN are rare complications of urinary tract infections (UTIs).
View Article and Find Full Text PDFBackground: Peritoneal dialysis (PD) is the most common kidney replacement therapy in children. Complications associated with PD affect treatment success and sustainability. The aim of this study was to investigate the frequency of PD-related non-infectious complications and the predisposing factors.
View Article and Find Full Text PDFPurpose: To evaluate the clinical characteristics, including spectral domain optical coherence tomography (SD-OCT) findings, of pediatric-onset Behçet's disease (PBD) patients.
Methods: Medical records of 23 PBD (15 males and 8 females) and 24 (15 males and 9 females) healthy subjects were evaluated retrospectively. The main outcomes were compared between PBD patients, with and without ocular involvement, and healthy subjects.
Goals: Although all children with nephrotic syndrome (NS) have similar biochemical abnormalities and clinical manifestations, they seem to have variable grades of steroid responsiveness and patterns of disease relapse. Therefore, this study aimed to examine whether steroid metabolism-related genetic polymorphisms, which are responsible for drug elimination, play a role for steroid response in children with NS.
Methods: The study population consisted of 53 children with idiopathic NS [45 steroid sensitive (SS) and 8 steroid resistant (SR) nephrotic patients] and 22 healthy children as the control group.
Background: To evaluate the clinical features of patients with multicystic dysplastic kidney (MCDK).
Methods: The medical files of children diagnosed with MCDK between January 2008 and November 2015 were retrospectively reviewed. The demographic, clinical, laboratory and radiological data were evaluated.
Background: The aim of present study was to evaluate the indications, complications and outcomes of acute peritoneal dialysis (APD) in neonates at a referral university hospital during the previous 8 years.
Methods: This retrospective analysis included a total of 52 newborn infants who underwent APD in a neonatal intensive care unit between January 2008 and March 2016. Demographic, clinical, laboratory and microbiological data were extracted from patients' medical files.
Clin J Am Soc Nephrol
October 2017
Background And Objectives: Infantile nephropathic cystinosis is a severe disease that occurs due to mutations in the cystinosis gene, and it is characterized by progressive dysfunction of multiple organs; >100 cystinosis gene mutations have been identified in multiple populations. Our study aimed to identify the clinical characteristics and spectrum of cystinosis gene mutations in Turkish pediatric patients with cystinosis.
Design, Setting, Participants, & Measurements: We identified the clinical characteristics and spectrum of cystinosis gene mutations in Turkish patients with cystinosis in a multicenter registry that was established for data collection.
Background And Objective: Urinary tract infections (UTIs) are common childhood bacterial infections that may involve renal parenchymal infection (acute pyelonephritis [APN]) followed by late scarring. Prompt, high-quality diagnosis of APN and later identification of children with scarring are important for preventing future complications. Examination via dimercaptosuccinic acid scanning is the current clinical gold standard but is not routinely performed.
View Article and Find Full Text PDFBackground: Predicting vesico-ureteral reflux (VUR) ≥3 at the time of the first urinary tract infection (UTI) would make it possible to restrict cystography to high-risk children. We previously derived the following clinical decision rule for that purpose: cystography should be performed in cases with ureteral dilation and a serum procalcitonin level ≥0.17 ng/mL, or without ureteral dilatation when the serum procalcitonin level ≥0.
View Article and Find Full Text PDFObjective: To assess the predictive value of procalcitonin, a serum inflammatory marker, in the identification of children with first urinary tract infection (UTI) who might have high-grade (≥3) vesicoureteral reflux (VUR).
Study Design: We conducted a meta-analysis of individual data, including all series of children aged 1 month to 4 years with a first UTI, a procalcitonin (PCT) level measurement, cystograms, and an early dimercaptosuccinic acid scan.
Results: Of the 152 relevant identified articles, 12 studies representing 526 patients (10% with VUR ≥3) were included.
Data on conservative treatment in children with urolithiasis are limited. The aim of the study was to determine the metabolic etiology and results of conservative treatment in children with urolithiasis. We evaluated the clinical presentation and metabolic features of 112 children with urolithiasis.
View Article and Find Full Text PDFBackground: The pathogenesis of edema in nephrotic syndrome is not entirely understood. The aim of this study was to contribute to the discussion on edema pathogenesis in nephrotic syndrome by following changes in volume and sodium retention for the course of the disease in children with steroid-sensitive nephrotic syndrome (SSNS).
Methods: Forty-one children with SSNS were included in the study.
The aim of this study was to investigate the levels of circulating endothelial microparticles (EMPs) in children with HSP and to determine whether there was a difference between patients with nephritis and those without nephritis. Twenty patients with HSP aged between 2.5 and 15 and 10 age-and sex-matched healthy controls were enrolled in the study.
View Article and Find Full Text PDFBackground: Determining uric acid : creatinine ratios in random urine samples may be useful to assess the excretion of uric acid in children. Because it was shown that urinary uric acid excretion varies with age and geographic area, it is important to have accurate reference values of uric acid excretion. The aim of the present study was therefore to obtain regional reference values for urinary uric acid : creatinine ratios in healthy Turkish children.
View Article and Find Full Text PDFBackground: The aim of the present study was not only to review clinical and demographic features of child-onset familial Mediterranean fever (FMF) patients but also to investigate whether there is a phenotype-genotype correlation in the same patient population.
Methods: The medical records of 102 patients with FMF were retrospectively reviewed. Patients were classified into three groups according to mutations: group 1, Met694Val-Met694Val (homozygote); group 2, Met694Val-other; and group 3, other-other.
Objective: The objective was to investigate the clinical features and metabolic and anatomic risk factors for kidney stone formation in our patient group.
Methods: Between 1998 and 2005, 179 children (94 girls, 85 boys) followed in our department because of urolithiasis were enrolled to participate in our study. Clinical presentation, urinary tract infection, stone localisation, positive family history, stone composition, presence of anatomic abnormalities and urinary metabolic risk factors, and treatment modality were evaluated retrospectively.