In our ten pediatric patients with aHUS who did not respond to PE, eculizumab was a lifesaving therapy and improved their quality of life. Early eculizumab use was a rescue therapy for renal function. Our results show that eculizumab is an effective treatment for aHUS. However, more studies are needed on the long-term efficacy and safety of eculizumab in children with aHUS and to determine the optimal duration of treatment.

These findings demonstrated that the signs of myocardial damage due to perinatal hypoxia still present at first month.

No abstract

When conventional methods for treating complicated problems such as acute and chronic renal failure or metabolic diseases fail, the therapy of choice is peritoneal dialysis (PD) in neonatal period. However, in cases that involve technical difficulties, such as bulky lesions in the abdomen or complications from previous abdominal surgeries, it is not always possible to place a peritoneal catheter. In such situations, continuous venovenous hemodiafiltration (CVVHDF) can be effective. This case series presents our experience in 2013 with the administration of CVVHDF to four patients in our neonatal intensive care unit who could not undergo PD for various reasons.

No abstract

No abstract

Objective: Minimal change disease (MCD) is the most common cause of nephrotic syndrome in childhood but its pathogenesis remains poorly understood. A T-cell-derived factor or factors were initially alleged as contributing to the disease pathogenesis. However, podocyte CD80 expression is now a commonly discussed theory. The aim of this study was to investigate the pathogenesis of MCD by determining urine interleukin-13, CD80, CD28, matrix metalloproteinase-2 (MMP-2), and granzyme B levels.Methods: Thirty patients and thirty healthy children were evaluated in this study. Six patients had biopsy proven MCD. The remaining patients were considered to have MCD because of their age at time of diagnosis; response to steroid treatment, absence of gross hematuria, hypocomplementemia or renal failure; and normal blood pressures in the active stage. The nephrotic-phase urine interleukin-13, CD80, CD28, MMP-2, and granzyme B levels of all patients were compared with the remission-stage urine levels of the same patients and control subjects. The urine samples were collected immediately before the application of immunosuppressive drugs or other treatment modalities.Results: Significantly higher interleukin-13, CD80, CD28, and MMP-2 levels were observed in the relapse period compared with both the remission period and control subjects. There was a significant positive correlation between the active-stage urine interleukin-13 and CD80 levels (r=0.619, p<0.001). Conclusion:Interleukin-13, CD80, CD28, and MMP-2 seem to have roles in the pathogenesis of MCD and using inhibitors of these molecules in treatment of steroid and immunosuppressive-resistant MCD cases can be thought. J Clin Exp Invest 2014; 5 (3): 354-361 ÖZET Amaç: Minimal değişiklik hastalığı çocuklarda nefrotik sendromun en sık nedenidir ve patogenezi hala aydınlatılamamıştır. Başlangıçta T hücresi kaynaklı faktör ya da faktörlerin hastalığın patogenezinden sorumlu olduğu ileri sürülmüştür. Fakat günümüzde hastalığın patogenezinde podosit CD80 ekspresyonu ön plana çıkmıştır. Bizim bu çalışmada amacımız hastaların idrar interlökin-13, CD80, CD28, matriks metaloproteinaz-2 ve granzim b düzeylerine bakarak hastalığın patogenezini anlamaya çalışmaktır.Yöntemler: Bu amaçla 30 hasta ve 30 sağlıklı çocuktan oluşan kontrol grubu ile çalıştık. 6 hastanın biyopsi sonucu minimal değişiklik hastalığı idi. Diğer hastalara hastalığın ilk aktif dönemindeki başlangıç yaşı, steroid tedavisine verdikleri cevap, hastalığın tanısı sırasında gros hematüri, hipokomplementemi, renal yetmezlik ve hipertansiyon olmaması nedeniyle minimal değişiklik hastalığı tanısı konuldu. Hastaların aktif faz idrar interlökin-13, CD80, CD28, matriks metaloproteinaz-2 ve granzim b düzeyleri aynı hastaların remisyon dönemi ve kontrol grubu ile karşılaştırıldı. İmmünsüpresif ilaçlar ya da diğer tedavi modaliteleri başlamadan hemen önce idrar örnekleri alındı. Bulgular:Hastalığın aktif döneminde remisyon ve kontrol grubuna göre istatistiksel olarak anlamlı idrar interlökin-13, CD80, CD28 ve MMP-2 yüksekliği sapta...

No abstract