Evaluation of hematological parameters in children with FMF.

In this study, we aimed to investigate whether neutrophil/lymphocyte ratio (NLR), platelet/lymphocyte ratio (PLR), and mean platelet volume (MPV) might be helpful in the diagnosis of subclinical inflammation of familial Mediterranean fever (FMF). Clinical, laboratory, and genetic results of the patients who were followed up with the diagnosis of FMF were obtained retrospectively from the hospital files and recorded to standardized form. Age- and sex-matched healthy subjects were included as the control group.

Association of vesicoureteral reflux and renal scarring in urinary tract infections.

Introduction: The aim was to investigate the relationship between vesicoureteral reflux (VUR) and renal damage in non-febrile, febrile for the first time and recurrent urinary tract infection (UTI) patients. The secondary aim was to determine whether C-reactive protein (CRP) in febrile UTIs could be a predictor of renal scarring.

Population And Methods: This prospective study included non-febrile, febrile for the first time and recurrent pediatric UTI cases.

Occult Disseminated Tuberculosis with Holocord Longitudinally Extensive Transverse Myelitis: A Rare Phenomenon in a Child.

Longitudinally extensive transverse myelitis (LETM) is defined as an inflammatory lesion of the spinal cord that extends to three or more segments. LETM is a commonly characteristic feature of neuromyelitis optica (NMO) or various autoimmune diseases. Manifestation of (MTB) infection with LETM are rare and usually in the cervicothoracic spinal cord.

A novel assessment tool for clinical care of patients with autoinflammatory disease: juvenile autoinflammatory disease multidimensional assessment report.

Objectives: To develop and test a new multidimensional questionnaire for assessment of children with auto-inflammatory disease (AID) such as FMF, PFAPA, HIDS, TRAPS in standard clinical care.

Methods: The juvenile auto-inflammatory disease multidimensional assessment report (JAIMAR) includes 16 parent or patient-centered measures and four dimensions that assess functional status, pain, therapeutic compliance and health-related quality of life (physical, social, school, emotional status) with disease outcome. It is proposed for use as both a proxy-report and a patient self-report, with the suggested age range of 8-18 years for use as a self-report.

Comparison of the efficacy of once- and twice-daily colchicine dosage in pediatric patients with familial Mediterranean fever--a randomized controlled noninferiority trial.

Background: In this study, we examined the efficacy and safety of a once-daily dosage schema of colchicine compared with a twice-daily dosage schema in pediatric patients with familial Mediterranean fever (FMF).

Methods: In this 24-week, multicenter, randomized controlled noninferiority trial, pediatric patients newly diagnosed with FMF carrying a homozygous or compound heterozygous mutation and not receiving any treatment were included. Patients were randomly assigned using a block randomization method to receive treatment with a once- or twice-daily dosage.

Development of a medication adherence scale for familial Mediterranean fever (MASIF) in a cohort of Turkish children.

Objectives: To develop and assess the validity and reliability of an adherence scale concerning medical treatment in paediatric FMF patients.

Methods: The Medication Adherence Scale in FMF Patients (MASIF) is a 18-item questionnaire that evaluates adherence to medication in four domains. Validation of the instrument was accomplished in paediatric FMF patients (aged 2-18 years) under medication at least for 6 months.

Kocher-Debré-Semelaigne syndrome with rhabdomyolysis and increased creatinine.

Association of Kocher-Debré-Semelaigne syndrome-a myopathy of hypothyroidism in childhood characterized by muscular hypertrophy, with rhabdomyolysis is very rare. We present a case of Kocher-Debré-Semelaigne syndrome with rhabdomyolysis secondary to Hashimoto's thyroiditis. He had muscular symptoms simulating poly/dermatomyositis, massively elevated creatine kinase (CK) levels and high creatinine levels.

Chondrocalcinosis related to familial hypomagnesemia with hypercalciuria and nephrocalcinosis.

Calcium pyrophosphate dehydrate (CPPD) crystal deposition disease (also known as chondrocalcinosis, CC) is a rare metabolic arthropathy mostly seen in elderly patients. Chondrocalcinosis may be associated with metabolic diseases such as hypomagnesemia when it occurs in young people. We report here a case with hypomagnesemia due to familial hypomagnesemia with hypercalciuria and nephrocalcinosis (FHHNC) who developed CC during clinical follow-up.

Does oxybutynin hydrochloride cause arrhythmia in children with bladder dysfunction?

Aim: Pediatric surgeons frequently encounter children presenting with voiding dysfunction symptoms, including urgency, frequency, and incontinence. Antimuscarinic agents Oxybutynin) are the main drugs used to treat patients with overactive bladder (OAB) syndrome, defined as urgency, with or without urgency incontinence, usually with increased daytime frequency and nocturia. Increased QT dispersion is known to be the cause of ventricular arrhythmia in various systemic diseases and leads to increased mortality and morbidity.

P-wave duration and dispersion in children with uncomplicated familial Mediterranean fever.

Objectives: This was a prospective controlled study to determine the P-wave duration (Pdu) and P-wave dispersion (Pd) in patients with familial Mediterranean fever (FMF).

Methods: The study group consisted of 26 children with uncomplicated FMF and 25 age- and sex-matched healthy controls. We performed electrocardiography (ECG) with Doppler echocardiography on patients and controls.

Evaluation of the current disease severity scores in paediatric FMF: is it necessary to develop a new one?

Objectives: Modified adult disease severity scoring systems are being used for childhood FMF. We aim to test the clinical consistency of two common severity scoring systems and to evaluate the correlation of scores with the type of FMF mutations in paediatric FMF patients since certain mutations are prone to severe disease.

Methods: Two hundred and fifty-eight children with FMF were cross-sectionally studied.

Etiology and outcome of acute kidney injury in children.

The aim of this prospective, multicenter study was to define the etiology and clinical features of acute kidney injury (AKI) in a pediatric patient cohort and to determine prognostic factors. Pediatric-modified RIFLE (pRIFLE) criteria were used to classify AKI. The patient cohort comprised 472 pediatric patients (264 males, 208 females), of whom 32.

Sulphasalazine treatment in protracted familial Mediterranean fever arthritis.

Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by self-limited attacks of fever and polyserositis. Articular involvement in early-onset FMF is a common finding characterized by non-erosive, generally asymmetric monoarthritis in large joints. Protracted FMF arthritis was reported in 2.

Chronic kidney disease in children in Turkey.

To determine the incidence, etiology and treatment patterns of chronic kidney disease (CKD) in children a questionnaire was sent to pediatric nephrology centers in Turkey, asking them to report patients under the age of 19 years who had estimated glomerular filtration rates (GFRs) of View Article and Find Full Text PDF

Left ventricular diastolic function evaluated with tissue Doppler imaging in children with familial Mediterranean fever.

The aim of this study was to assess the left ventricular diastolic function using conventional echocardiography and tissue Doppler imaging in children with familial Mediterranean fever. This study included 29 (13 males and 16 females) patients and 30 healthy subjects as controls. Body mass index was calculated and arterial blood pressure was monitored.

The relationship between urinary tract infections and vesicoureteral reflux in Turkish children.

Early determination of renal scar development risk in children following first urinary tract infection (UTI) and early detection and treatment of vesicoureteral reflux (VUR) are important to prevent renal functional impairment. The aim of this study was to determine the prevalence of VUR and associated renal scar formation, in children who had acute pyelonephritis (APN), first afebrile UTI, and recurrent afebrile UTIs. Patient records of 642 children having UTI were scrutinized and 278 out of 642 were enrolled in this study.

CTLA-4 +49 A/G genotype and HLA-DRB1 polymorphisms in Turkish patients with Henoch-Schönlein purpura.

The pathogenesis of Henoch-Schönlein purpura (HSP) remains unknown; however, it is generally considered to be an immune complex-mediated disease. Cytotoxic T lymphocyte-associated protein 4 (CTLA-4) is expressed on activated T cells, and, thus, it is critically involved in the immune response. We aimed to investigate the possible influence of CTLA-4 polymorphisms for susceptibility to HSP and determine if there were associations with human leukocyte antigen (HLA)-DRB1 genotypes.

HLA-DRB1 alleles and Henoch-Schönlein purpura: susceptibility and severity of disease.

Objective: The genetic basis of susceptibility to Henoch-Schönlein purpura (HSP) may be conferred by a number of gene loci, including the MHC. Associations between human leukocyte antigen (HLA) and disease can help to establish a basis for susceptibility and assist in the prediction of the outcome and clinical heterogeneity. We aimed to investigate the implications of the HLA-DRB1 locus and the susceptibility to HSP, and to determine if there are associations with joint, gastrointestinal, and renal manifestations of the disease.

An unusual association between familial mediterranean fever and IgM nephropathy.

Objective: To report a case with the diagnosis of IgM nephropathy and familial Mediterranean fever (FMF).

Clinical Presentation And Intervention: A 9-year-old boy was admitted to our hospital with recurrent abdominal pain since the age of 4 years. Laboratory investigations revealed a sedimentation rate of 88 mm/h, C-reactive protein: 83.

Henoch Schonlein purpura in childhood: clinical analysis of 254 cases over a 3-year period.

We aimed to evaluate the patients who were diagnosed as Henoch Schonlein purpura (HSP) for disease characteristics and prognosis of those with joint, gastrointestinal (GI), and renal involvement. Two hundred and fifty-four children who were followed up with the diagnosis of HSP in the Pediatric Nephrology Clinics of Meram Medical Faculty of Selcuk University and Medical Faculty of Gazi University between January 2003 and June 2006 were retrospectively evaluated. The clinical follow-up and treatment regimens of patients in whom renal biopsy was performed were evaluated in detail.

Familial hypomagnesemia with hypercalciuria and nephrocalcinosis: report of three Turkish siblings.

Familial hypomagnesemia with hypercalciuria and nephrocalcinosis (FHHNC), an autosomal recessive renal tubular disorder is characterized by the impaired tubular reabsorption of magnesium and calcium in the thick ascending limb of the loop of Henle. This disease is caused by mutations in the claudin-16 gene (CLDN16), which encodes the tight junction protein, claudin-16. Claudin-16 belongs to the claudin family and regulates the paracellular transport of magnesium and calcium.

The evaluation of carotid intima-media thickness in children with familial Mediterranean fever.

The aim is to investigate whether pediatric familial Mediterranean fever (FMF) patients have an increased risk of premature atherosclerosis and to determine the possible strength of association between atherosclerosis and Mediterranean fever (MEFV) gene mutation gene type. Demographic characteristics and MEFV mutations were defined in 49 children diagnosed with FMF (26 female, 23 male; mean age, 10.71 +/- 3.

A case of homozygous familial hypercholesterolemia with focal segmental glomerulosclerosis.

Familial hypercholesterolemia (FH) is a common autosomal dominant inherited disorder characterized by increased levels of circulating plasma low-density lipoprotein cholesterol (LDL-C), tendon xanthomas, and premature atherosclerotic cardiovascular disease. Homozygous FH occurs in only one in a million people. Focal segmental glomerulosclerosis (FSGS) is clinically characterized by proteinuria, which is marked in the majority of cases and accompanied by nephrotic syndrome, high incidence of hypertension, and progression to renal failure.