Comprehensive evaluation of patients with primary hyperoxaluria type 1: A nationwide study.

Background: Primary hyperoxaluria type 1 (PH1) is characterized by increased endogenous oxalate production and deposition as calcium oxalate crystals. The main manifestations are nephrocalcinosis/nephrolithiasis, causing impaired kidney function. We aimed to evaluate the clinical characteristics and overall outcomes of paediatric PH1 patients in Turkey.

Comparison of infants and children with urolithiasis: a large case series.

We evaluated the demographic features, etiologic risk factors, treatment strategies, and outcome of the infants and children with urolithiasis (UL). A retrospective multicenter study was conducted including 23 Pediatric Nephrology centers in Turkey. The medical records of 2513 children with UL were reviewed.

Evaluation of subclinical cardiovascular risk and cardiac function in children with vesicoureteral reflux: a prospective study.

Background: Vesicoureteral reflux is a prominent congenital anomaly of the kidney and the urinary tract. Further, renal scarring is known to be related to chronic inflammation. However, there have been limited studies to date regarding the cardiovascular consequences of vesicoureteral reflux.

The association between the congenital heart diseases and congenital anomalies of the kidney and the urinary tract in nonsyndromic children.

Several congenital heart diseases (CHD) are present in children with congenital anomalies of the kidney and the urinary tract (CAKUT) in syndromic or nonsyndromic patterns. The purpose of this study was to identify nonsyndromic children with CAKUT who required echocardiographic evaluation and to find the prevalence of CHD in these patients. Retrospective chart review of nonsyndromic patients with CAKUT between 2012 and 2018 was conducted.

A nationwide retrospective study in Turkish children with nephrocalcinosis.

Background: Nephrocalcinosis (NC) is defined as calcium deposition in the kidney parenchyma and tubules. This study aims to determine the etiology, risk factors, and follow-up results of patients with NC in Turkey.

Methods: Patients diagnosed with NC in the pediatric nephrology Department Units of 19 centers from all geographical regions of Turkey over a 10-year period (2010-2019) were included in the study.

A rare congenital fusion anomaly of the urinary tract: Pancake kidney.

Pancake kidney is a rare subtype of cross fused renal ectopia. Fusion of both upper and lower poles of the kidney in pelvis results in a disc or cake shaped kidney appearence. In this report, we are presenting two cases (a 3-month-old male and a 3-year-old girl) with different presentations and their follow-up results.

Is voiding cystourethrography necessary for evaluating unilateral ectopic pelvic kidney?

Objective: The aim of this study is to evaluate if voiding cystourethrography (VCUG) is necessary for the evaluation of unilateral ectopic pelvic kidney (UEPK) in order to identify vesicoureteral reflux (VUR).

Material And Methods: Files of the patients who had been followed-up for ectopic pelvic kidney in two pediatric nephrology clinics between August 2011 and December 2017 were retrospectively reviewed. Other anomalies, such as crossed, fused, and bilateral pelvic ectopia were excluded.

Incidence of primary vesicoureteral reflux in patients with febrile convulsions.

The aim of this study is to evaluate the incidence of vesicoureteral reflux (VUR) in patients with febrile convulsion (FC). For this, patients that were diagnosed with FC in 2018 were retrospectively reviewed. Those with epilepsy, motor/mental retardation, or spina bifida were excluded.

Follow-up results of patients with ADCK4 mutations and the efficacy of CoQ10 treatment.

Background: ADCK4-related glomerulopathy is an important differential diagnosis in adolescents with steroid-resistant nephrotic syndrome (SRNS) and/or chronic kidney disease (CKD) of unknown origin. We screened adolescent patients to determine the frequency of ADCK4 mutation and the efficacy of early CoQ10 administration.

Methods: A total of 146 index patients aged 10-18 years, with newly diagnosed non-nephrotic proteinuria, nephrotic syndrome, or chronic renal failure and end-stage kidney disease (ESKD) of unknown etiology were screened for ADCK4 mutation.

Do JJ Stents Increase the Effectiveness of Extracorporeal Shock Wave Lithotripsy for Pediatric Renal Stones?

Purpose: We aimed to evaluate the effects of preoperative urinary catheterization in nephrolithiasis treatment with extracorporeal shock wave lithotripsy (SWL).

Methods: Patients admitted to the Department of Pediatric Surgery for renal stones between June 2012 and June 2014 were evaluated retrospectively. Patients were divided into 2 groups based on JJ stent placements.

Pediatric urolithiasis: metabolic risk factors and follow-up results in a Turkish region with endemic stone disease.

The goal of this study was to investigate the metabolic etiology, clinical findings and medical treatment of children with urolithiasis in an endemic region of Turkey. We retrospectively analyzed the medical records of 742 (437 males, 305 females) children with urolithiasis. Physical examination results, serum biochemistry and urine metabolic evaluation, including urinary citrate, oxalate, calcium, uric acid, cystine and magnesium levels were recorded.

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.