Juvenile dermatomyositis with a rare and remarkable complication: sinus bradycardia

WOS: 000243226400011PubMed ID: 16944159Juvenile dermatomyositis (JDM) is characterized by proximal muscle weakness, vasculopathy of the skin and muscles, and typical skin rash. Approximately, 70% of adult patients with dermatomyositis have evidence of cardiac damage. However, cardiac involvement, one of the important causes of death, has been reported rarely in children with JDM. Here, we describe an 11-year-old boy who presented with bradycardia as a result of sinus node involvement due to JDM and recovered by the treatment of JDM without any specific drug for bradycardia

Clinical outcome in children with Henoch-Schonlein nephritis

WOS: 000242513200012PubMed ID: 17024391Henoch-Schonlein purpura (HSP) is the most common vasculitis in childhood. The long-term prognosis is variable and depends on renal involvement. The aim of this study was to evaluate both clinical features of the children with HSP and the prognoses of short- and long-term outcome of patients diagnosed as HSP nephritis (HSN). This is a retrospective data study of all children with HSP hospitalized from January 1991 to December 2005. The patients with HSN were classified according to their initial presentation, histologic findings, type of treatment and clinical outcome. All patients have been evaluated once every 2 months. Fifty-three of the patients had kidney biopsies. The patient population consisted of 141 children included 78 boys (55.3%) and 63 girls (44.7%) ranging in age at disease onset from 2 to 17 (8.9 +/- 3.29) years. Renal involvement was determined in 58.1%. Nephrotic and/or nephritic syndrome were found to be an unfavorable predictor both for short and long-term outcome (P < 0.05). However, 35% of these patients and 62% of them showed complete remission after 6 months and long-term course. Overall prognosis of HSN is relatively good and long-term morbidity is predominantly associated with initial presentation and renal involvement

Treatment and outcome of pediatric patients with primary focal segmental glomerulosclerosis

43rd ERA-EDTA Congress -- JUL 15-18, 2006 -- Glasgow, SCOTLANDWOS: 000239919002205ERA, EDT

PPAR-gamma 2 gene Pro12 Ala mutation in Turkish children with metabolic syndrome

43rd ERA-EDTA Congress -- JUL 15-18, 2006 -- Glasgow, SCOTLANDWOS: 000239919002333ERA, EDT

TLR 4 gene polymorphism in paediatric renal transplantation patients in turkish population

43rd ERA-EDTA Congress -- JUL 15-18, 2006 -- Glasgow, SCOTLANDWOS: 000239919003376ERA, EDT

Juvenile psoriatic arthritis carrying familial Mediterranean fever gene mutations in a 14-year-old Turkish girl

WOS: 000245312300012PubMed ID: 17408446Juvenile psoriatic arthritis (JPsA) is characterized by asymmetric arthritis of big and small joints, enthesitis, dactylitis, psoriatic skin lesions and nail pitting. Investigators agree that JPsA is a relatively common chronic arthropathy of childhood that differs clinically, serologically, and genetically from both juvenile idiopathic arthritis and juvenile ankylosing spondylitis. Familial Mediterranean fever (FMF) is a multisystemic autosomal recessive disease occasionally accompanied by sacroiliitis. This is characterized by recurrent self-limited attacks of fever and accompanying abdominal, chest and arthricular pain. We present a 14-year-old Turkish girl with JPsA and carrying FMF gene mutations. In this patient, JPsA was diagnosed according to her physical, laboratory and skin biopsy findings and a treatment with methotrexate and sulfasalazine was started. As an inadequate clinical and laboratory response was obtained after the first month of therapy, the patient was investigated for FMF, and was diagnosed by molecular analyses of related gene (E148Q heterozygous/V726A homozygous mutation) besides clinical findings. After 2 weeks of the colchicine treatment, symptoms of the patient regressed and acute phase reactants decreased. To our knowledge, this is the first case presenting with psoriatic arthritis and FMF gene mutations together and responds to colchicine, methotrexate and sulfasalazine dramatically in clinical and laboratory findings. This case has been presented to remind that cases with psoriatic arthritis may also carry mutations in the MEFV gene