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OBJECTIVE To investigate the prevalence of MEFV gene mutations in Turkish patients with Henoch-Schönlein purpura (HSP) but with no symptoms of familial Mediterranean fever (FMF). In addition, we assessed the clinical and laboratory characteristics of HSP patients with and without MEFV mutations. METHODS Eighty pediatric patients with HSP (44 boys and 36(More)
Encapsulating peritoneal sclerosis (EPS) is a serious complication of chronic peritoneal dialysis (CPD). In contrast to the adult population, there are few studies regarding EPS in paediatric CPD patients, and the majority of reported patients are from Japan. The aim of the present report is to define the incidence of EPS in our paediatric CPD patients and(More)
Urofacial syndrome (UFS) (or Ochoa syndrome) is an autosomal-recessive disease characterized by congenital urinary bladder dysfunction, associated with a significant risk of kidney failure, and an abnormal facial expression upon smiling, laughing, and crying. We report that a subset of UFS-affected individuals have biallelic mutations in LRIG2, encoding(More)
The nutcracker phenomenon refers to compression of the left renal vein between the aorta and the superior mesenteric artery. Clinical features are hematuria, abdominal pain, left flank pain, pelvic or scrotal discomfort due to varicocele or ovarian vein syndrome. In this report, 2 patients with orthostatic proteinuria, in whom nutcracker phenomenon was(More)
AIM The purpose of our study was to evaluate and analyse the prevalence and association of acute kidney injury (AKI) as defined by paediatric Risk, Injury, Failure, Loss of kidney function and End-stage kidney disease (pRIFLE) and Acute Kidney Injury Network (AKIN) classifications in a paediatric intensive care unit (PICU). METHODS A prospective analysis(More)
OBJECTIVES Familial Mediterranean fever (FMF) is an autosomal recessive disease, characterised by recurrent, self limited attacks of fever with serositis. The aim of our study was to describe the demographic, clinical and genetic features of FMF patients who had early disease onset and to compare them with late onset patients. Our second aim was to(More)
The changing pattern of antimicrobial resistance in the causative microorganisms of urinary tract infection (UTI) in childhood is a growing problem. The aims of this study were to assess the resistance patterns of urinary isolates to commonly used antimicrobials and to evaluate the options for empirical treatment of UTI. A prospective cross-sectional(More)
OBJECTIVES Several sets of criteria mainly for adults have been proposed for the diagnosis of FMF. The aim of the present study is to validate the most widely used diagnostic 'Tel Hashomer' criteria in children and to establish a new set of criteria for use in childhood. METHODS The study group consisted of 170 recently diagnosed FMF patients who had(More)
OBJECTIVE The purpose of this study was to evaluate the pathophysiologic characteristics of nutcracker syndrome (NS) and to assess the role of upright position imaging and superior mesenteric artery (SMA) angle measurement in the diagnosis. METHODS Doppler sonographic findings in 23 children with NS and in 26 healthy control subjects were compared. The(More)
OBJECTIVES To test the hypothesis that alterations in the Mediterranean fever (MEFV) gene are a susceptibility factor for the development of polyarteritis nodosa (PAN) we investigated the prevalence of MEFV mutations in patients with PAN without any symptoms of familial Mediterranean fever (FMF). STUDY DESIGN Pediatric patients with PAN (n = 29) were(More)