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. The study group consisted of 254 children, 147 boys (57.8%) and 107 girls (42.2%), and the ratio of boys to girls was 1.37. The percentages of skin, joint, GI, and renal manifestations were 100%, 66%, 56%, and 30%, respectively. Eight patients had intussusception. Five of them recovered with steroid treatment only while three patients were operated on. Sixty-four patients (44%) with GI involvement had severe disease and were successfully treated with steroids. Renal biopsy was performed in 26 patients. Among those 26 patients, two of them recovered spontaneously within 3 and 4 weeks. Ten patients improved with only steroid treatment while 12 patients recovered with steroid and cyclophosphamide treatment. Two patients were resistant to steroid and cyclophosphamide treatment and were treated with cyclosporine A. We believe that steroid therapy given to the HSP patients with GI manifestations might be helpful to prevent probable complications such as GI bleeding and intussusception. In addition, combined therapy with steroid and cyclophosphamide can usually be an appropriate treatment for patients with nephrotic proteinuria.

ADCK4 mutations are one of the most common causes of adolescent-onset albuminuria and/or CKD of unknown etiology in Turkey. CoQ10 supplementation appears efficacious at reducing proteinuria, and may thereby be renoprotective.

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 +/- 3.69 years). Twenty-six age-, sex-, and body-mass-index-matched healthy children constituted the control group. We evaluated the blood counts and acute-phase proteins during attack-free periods. Mean C-reactive protein (CRP), serum amyloid-A (SAA), homocysteine (Hcy), lipoprotein-a (Lp-a), and common carotid artery intima-media thickness (CCA-IMT) were 10.75 +/- 15.29 vs 4.03 +/- 1.20, 23.22 +/- 41.94 vs 3.53 +/- 1.04, 10.36 +/- 3.36 vs 8.64 +/- 3.15, 20.84 +/- 23.89 vs 8.56 +/- 7.48, and 0.038 +/- 0.007 vs 0.032 +/- 0.004, respectively, and significantly higher than the mean values of control group (p < 0.05). However, no correlation was found between CCA-IMT and CRP, SAA, Hcy, and Lp-a. Twenty-nine patients had M694V mutation, and 13 patients had other mutations. There was no correlation between CCA-IMT and MEFV mutation subgroups. In conclusion, because of the nature of the disease, FMF patients should be considered to have an increased risk of early vascular alteration and atherosclerosis. For this reason, CCA-IMT measurement can be recommended as a noninvasive and early diagnostic method.

No abstract

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. We obtained follow-up records in 316 patients to evaluate the association between stone recurrence and metabolic risk factors. The mean age at diagnosis was 2.6 ± 3.4 (0.1-17.0) years. Male-to-female ratio was 1.4:1. A family history of stone disease was found in 76.5 % of patients and 41 % of parents had consanguineous marriage. The most common presenting symptoms were urinary tract infection (UTI, 23.9 %) and hematuria (23.6 %). Metabolic abnormalities were found in 588 (79.2 %) patients, including hypercalciuria in 31.5 %, hypocitraturia in 24.2 %, hyperoxaluria in 11.4 %, hyperuricosuria in 9.1 %, hypomagnesuria in 3.9 %, and cystinuria in 3.1 % of patients. The frequency of hyperoxaluria and hypocitraturia were significantly higher in patients with new stone formation. Follow-up records of 316 (42.6 %) patients (192 males, 124 females) were available. Urolithiasis was shown in 135 (42.7 %) of the patients on control ultrasonography, and 61.5 % of these patients had a stone size ≤ 3 mm. Hyperoxaluria and cystinuria were significantly higher in patients with stone persistence. The main goal of management for children with urolithiasis should be identification of risk factors.