Pediatric neurology
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Pediatric neurology · May 2007
Phase I trial of pirfenidone in children with neurofibromatosis 1 and plexiform neurofibromas.
We aimed to define the dose of pirfenidone in children and adolescents with neurofibromatosis 1 and plexiform neurofibromas that is pharmacokinetically comparable to the active adult dose. Pirfenidone was administered orally on a continuous dosing schedule. The starting dose level was 250 mg/m2/dose. ⋯ Dose-limiting toxicities were observed in 2 of 12 patients receiving 500 mg/m2 three times a day. The plasma pharmacokinetics of pirfenidone were highly variable, but not age dependent. The second dose level was the pharmacokinetically comparable dose and is being used in an ongoing phase II trial of pirfenidone for children with neurofibromatosis 1 and progressive plexiform neurofibroma.
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Pediatric neurology · May 2007
Case ReportsLupus anticoagulant and thrombosis following Henoch-Schonlein purpura.
A male adolescent developed a sinovenous thrombosis 4 weeks following a Henoch-Schonlein purpura episode. A hypercoagulation evaluation revealed a positive lupus anticoagulant. This suggests an association between Henoch-Schonlein purpura and antiphospholipid antibody syndrome and is the first report of sinovenous thrombosis after Henoch-Schonlein purpura that was likely due to elevated antiphospholipids. Children who develop Henoch-Schonlein purpura with neurologic features including headache should be evaluated for sinovenous thrombosis and a hypercoagulable state.