Pediatric nephrology : journal of the International Pediatric Nephrology Association
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Case Reports
Extra corporeal membrane oxygenation and plasmapheresis for pulmonary hemorrhage in microscopic polyangiitis.
Early initiation of extracorporeal membrane oxygenation to treat acute hypoxemic respiratory failure secondary to massive pulmonary hemorrhage in microscopic polyangiitis in children can be life-saving while awaiting control of the autoimmune disease process by plasmapheresis and immunosuppression.
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Case Reports
Cerebral vasculitis and intracerebral hemorrhage in Henoch-Schönlein purpura treated with plasmapheresis.
Severe neurological complications in Henoch-Schonlein purpura (HSP) are rare. We report a 13-year-old girl with HSP receiving oral steroid therapy for HSP nephritis who presented with acute onset of headache, confusion, and loss of bilateral vision. ⋯ Therapeutic plasmapheresis was performed immediately and there was rapid clinical improvement. In conclusion, we propose that plasmapheresis is a reliable and effective therapy and may be considered as the first-line treatment in this critical condition to prevent massive intracerebral hemorrhagic complication of this immune complex-mediated cerebral vasculitis leading to irreversible neurological sequelae or mortality.
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Spinal epidural lipomatosis (SEL) in patients on steroid therapy may be explained by two hypotheses: (1) steroids induce SEL and (2) steroids cause the growth of a pre-existing SEL, especially in obese children. Steroid-induced SEL (SSEL) is rarely described in children, with only six cases reported elsewhere. However, we have already reported four nephrotic children with SSEL, including one child in another hospital, in addition to the six cases. ⋯ SSEL was detected in 5 of 125 children (4.0%). Of the 5 patients with SSEL, 3 had vertebral compression fractures, and all 5 patients were on methylprednisolone pulse therapy. Our study suggests that many more patients can be detected at the early stage of SSEL by performing spinal MRI for patients with early symptoms, obesity, and those who have received methylprednisolone pulse therapy.
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The immunosuppressive agents administered to maintain the remission of idiopathic nephrotic syndrome (INS) may have a deleterious effect on several cell types. The aim of this study was to analyze platelet activation and reactivity in children with INS treated with cyclosporin A (CyA). The study groups comprised 16 children with remission of INS induced by CyA and 16 children with glucocorticosteroid-induced remission 8 weeks from the onset of INS relapse. ⋯ No differences between groups were found in CD61, CD62P, and CD42b surface expression, but markers of the coagulation cascade and fibrinolysis or endothelial injury (F1+2 prothrombin fragments, tissue plasminogen activator inhibitor 1) were elevated in patients treated with CyA compared with children on steroids and healthy controls. No correlations between markers of platelet function and CyA concentration were found. We postulate that CyA administration in nephrotic patients causes an activation of thrombinogenesis but does not influence platelet activation and reactivity in INS.