We report a rare case of nephritic syndrome underlying dense deposit disease (DDD) with alternative complement pathway dysfunction explained with both C3 nephritic factor (C3NeF) antibodies and DDD associated polymorphism of factor H. An 8-year-old boy presented with macroscopic hematuria, hypertension and periorbital edema followed by persistently low C3 during the 8-week follow-up. Positive C3 staining on immunofluorescence microscopy, supported by dense deposits within the glomerular basement membrane on electron microscopy, confirmed the diagnosis of DDD. Preliminary tests for complement activation showed decreased classic pathway and deficient alternative complement pathway, as well as slightly positive C3NeF, supporting the diagnosis of DDD. Genetic analysis revealed a polymorphism of the complement factor H gene with an increased risk of developing DDD. Supportive therapy led to satisfactory recovery of renal function and normalization of C3. Given the poor prognosis of the disease, proper approach to such specific glomerulopathy is important to avoid or at least slow down progression to end-stage renal disease.
Methods A review of the available literature related to application of Octaplas in children.Results The use of Octaplas in severe patients reduces the possibility of immune and anaphylactic reactions and transfusioninduced acute lung injury. Also, a smaller volume of Octaplas compared to FFP is required to correct coagulopathy. No additional bleeding with reduction in the number of transfusions in liver transplant patients was observed. An increase in platelets has been demonstrated in patients with thrombotic thrombocytopenic purpura who were refractory to FFP. The survival rate in Pediatric Intensive Care Unit (PICU) was higher in children treated with Octaplas compared to FFP. Recent research has shown that in children who received Octaplas during cardiac surgery ACT values were lower with lower doses of heparin, and platelets, APTV, INR, fibrinogen values were higher compared to children who received FFP. Reduction in postoperative infections and shorter stay in PICU were observed. The latest prospective, multicenter study in the United States examined the safety, tolerability, and efficacy of Octaplas in the treatment of children who required replacement of multiple coagulation factors due to cardiac surgery, transplantation and/or liver dysfunction, with coagulopathy and sepsisrelated coagulopathy as well as hypoxic encephalopathy. No thromboembolic events associated with hyper fibrinolysis or treatment have been reported. The overall safety, tolerability and efficiency was defined as excellent. Hemostatic parameters measured with INR, PT, APTV, thromboelastography, or thromboelastometry were within the expected range. Conclusion The results of previous research support the use of Octaplas in children. Octaplas has an advantage over FFP due to its strong hemostatic effect, less frequent side effects, safety and in critically ill children may be associated with improved survival.
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