The long-term results of pediatric patients with primary focal and segmental glomerulosclerosis

Focal and segmental glomerulosclerosis (FSGS) is a major cause of idiopathic steroid-resistant nephrotic syndrome (SRNS) and end-stage renal disease (ESRD). In this retrospective study, we report on 34 pediatric patients with FSGS who were diagnosed and treated from 1992 to 2006. The mean age at onset was 6.3 ± 4.3 years. All patients had nephrotic-range proteinuria. Micros-copic hematuria was seen in three patients and hypertension was seen in 15 patients at presentation. All patients were treated with steroids (oral and/or methylprednisolone), while 23 patients received cytotoxic therapy in addition. The mean follow-up period was 8.6 ± 3.3 years at the end of which, 59% of patients achieved complete or partial remission, 20.5% continued to have active renal di-sease while 20.5% of the patients developed CKD. Our study suggests that most of the patients with FSGS progress to renal insufficiency. Steroid therapy increases the chances of remission and pre-serves renal function in patients with sporadic primary FSGS

Unilateral double kidney with contralateral supernumerary kidney which found incidentally

A supernumerary kidney is one of the rarest congenital anomalies of urinary tract, approximately 70 cases have been reported. Supernumerary kidneys may be asymptomatic and associated with genital-urinary tract anomalies. A 6-year old girl was found to have supernumerary kidney with contralateral double kidney from recurrent urinary tract infections (UTI) in her past history while she had hospitalized for bronchopneumonia. Ultrasonography (USG), pyelography and scintigraphy had demonstrated an extra kidney in the left side with contralateral double kidney. USG is the most valuable method of early diagnosis this anomalie of urinary tract, especially after UTI. Herein, this rare urinary anomaly was discussed with literature.Supernumerary böbrek, literatürde yaklasık 70 vakada bildirilmis nadir üriner sistem anomalilerindendir. Supernumerary böbrekler genellikle asemptomatik olup basta genito-üriner sistemi ilgilendiren anomalilerle birliktelik gösterebilirler. Bronkopnömoni nedeniyle hospitalize edilmis 6 yasında kız olguda, özgeçmisindeki tekrarlayan idrar yolu enfeksiyonlarından (İYE) solda ‘supernumerary böbrek’ ve kontalateral ‘double böbrek’ anomalilierine ulasılmıstır. Ultrasonografi (USG), intravenöz pyelografi ve sintigrafi bulguları sol tarafta ekstra bir böbreği ve kontralateral double böbreği desteklemistir. Özellikle İYE’den sonra yapılması gereken USG bu tür genitoüriner sistem anomalilerinin tanısında oldukça değerlidir. Bu yazıda, son derece nadir görülen bu anomali literatür esliğinde tartısılmak istenmistir

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

Familial Mediterranean fever gene mutation frequencies and genotype-phenotype correlations in the Aegean region of Turkey

WOS: 000290989300013PubMed ID: 20217092Familial Mediterranean fever (FMF) is a disease characterized by recurrent, self-limiting fever and serositis and caused by altered pyrin due to mutated MEFV gene. The aim of this study was to investigate clinical manifestations and MEFV mutations among patients with FMF and healthy controls in the Aegean region of Turkey. This study included 308 patients and 164 healthy controls. Patients were divided into three groups according to Tel-Hashomer criteria; definitive, probable, and suspicious. Among the patients, 146 were women (47.4%) and 162 were men (52.6%). The mean age (+/- SD) of the patients at the diagnosis was 9.6 +/- A 3.95 (range 0.5-18). The mean age (+/- SD) at onset of the symptom was 6.2 +/- A 3.95 (range 1-18). Symptoms were seen earlier onset in definitive group than the suspicious group in our cohort (4.7 +/- A 3.9 years, 6.6 +/- A 3.9 years, respectively; P = 0.001). Clinical features were abdominal pain (83.1%), fever (55%), arthritis (17.1%), myalgia (4.5%), pleuritis (10%), and erysipelas-like erythema (7.7%). Fever, arthralgia, arthritis, chest pain, and amyloidosis were found statistically significant more in definitive group than suspicious group (P < 0.001, P < 0.001, P < 0.001, P < 0.05, and P < 0.001, respectively). MEFV gene mutations were identified in 199 patients (64.6%). The most commonly encountered MEFV mutation among the patients was M694V homozygote (25%). M694V homozygous mutation was found most frequently in definitive FMF group than other groups (49, 9, 8.9%, respectively). To our knowledge that FMF should be suspected in the case of non-specific but recurrent attacks of serositis and high fever, and molecular analysis should be performed in order to make diagnosis of FMF

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