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Aim: The aim of this review is to provide clinicians with characteristics of children with nephrotic syndrome and cerebral sinovenous thrombosis (CSVT). Methods: We have reviewed 37 articles of pediatric cases and provided 1 new case. PRISMA guidelines were followed. Results: Sixty-two patients were included in the review. CSVT was more common in males, usually occurred within 6 months of nephrotic syndrome onset and was found more often in outpatients. The superior sagittal sinus was the most common sinus affected. Non-contrast computed tomography was the most frequent radiologic study performed, with 30% of results negative for CSVT. Headache and vomiting were the most common symptoms while neurologic symptoms were less frequent. Anticoagulation treatment was strongly inconsistent throughout the literature. Thrombosis outcomes were favorable. The most common possible risk factors were corticosteroid treatment, proteinuria and hypoalbuminemia. Four children had a genetic predisposition diagnosed after thrombosis. No markers for anticoagulation prophylaxis seemed to be relevant for the majority of thrombosis occurring in outpatients. Conclusion: Prophylactic anticoagulation does not seem reasonable to prevent CSVT. Knowledge of nonspecific symptoms and of nephrotic syndrome being a state of hypercoagulation and early use of appropriate radiologic study seem to be of major importance.
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BACKGROUND: Immunoglobulin A vasculitis is the most common vasculitis in children. It is usually a self-limiting condition, and the long-term prognosis depends on the severity of renal involvement. Although cyclosporin A is not generally recommended for the management of moderate immunoglobulin A vasculitis nephritis, a few previous reports showed its efficacy. Our aim was to determine whether the treatment with cyclosporin A in combination with corticosteroids is safe and effective for moderate pediatric immunoglobulin A vasculitis nephritis. METHODS: Nine children underwent treatment. Mean follow-up was 3.1±1.6 (1.4-5.8) years. RESULTS: All children (seven females and two males) reached complete remission (65.8±27.6 [24-99]) days. No patient had relapse, one patient had slightly impaired kidney function (glomerular filtration rate 84.4 mL/min/1.73 m2), and two patients had microscopic hematuria without proteinuria at last follow-up. One patient with delayed treatment had microscopic hematuria at last follow-up and developed early albuminuria after cessation of immunosuppression. We observed no serious complications or side effects of the treatment. CONCLUSIONS: Cyclosporin A in combination with corticosteroids seems to be a safe and effective treatment for moderate immunoglobulin A vasculitis nephritis. More studies with cyclosporin A should be conducted to better determine the best therapeutic approach.
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The number of pediatric solid organ transplantations is growing. This therapy leads often to better quality of life but also brings some specific complications. Our review summarizes practical recommendations for long-time care of the children after kidney and liver transplantation. The knowledge of the issues related to transplantation is essential for the first contact physicians, whose cooperation with transplant centre contributes highly to adequate management of these children.
