ABSTRACT
Colchicine is the mainstay of treatment for familial Mediterranean fever. We investigated the frequency of leukopenia in 213 patients with familial Mediterranean fever treated with standard doses of colchicine (0.5-2.0 mg/day). We found that 23 patients (10.8%) had reversible leukopenia, 3 moderate, and none severe and that their rate of infections was not increased.
Subject(s)
Colchicine/adverse effects , Familial Mediterranean Fever/drug therapy , Leukopenia/chemically induced , Tubulin Modulators/adverse effects , Case-Control Studies , Child , Child, Preschool , Colchicine/administration & dosage , Colchicine/pharmacology , Dose-Response Relationship, Drug , Female , Humans , Leukopenia/diagnosis , Leukopenia/drug therapy , Longitudinal Studies , Male , Tubulin Modulators/administration & dosage , Tubulin Modulators/pharmacologyABSTRACT
OBJECTIVE: To review the results of our treatment protocol in the last 7 years. STUDY DESIGN: Six patients (4 girls, 2 boys) with an age range of 12 to 17 years were diagnosed with Takayasu arteritis (TA) during this period. Patients were allocated to receive (1) oral steroids and methotrexate (MTX) (12.5 mg/m(2)/week) if they had disease limited to one side of the diaphragm only without pulmonary disease involvement (two patients); and (2) oral steroids and oral cyclophosphamide (CYC) (maximum total dose 150 mg/kg) followed by oral MTX for maintenance as above if the disease was more widespread (four patients). RESULTS: One patient died of pulmonary vasculitis during the first month of therapy. The remaining three patients with involvement of both the thoracic and abdominal aorta and branches received the second protocol for 12 to 18 months. All entered remission. Aortic bypass, aortorenal bypass, balloon dilatation, and unilateral nephrectomy were performed in these patients. CONCLUSIONS: The presented single-center experience suggests that CYC induction and corticosteroids followed by MTX is an effective and safe treatment for childhood TA.