RESUMO
Familial Mediterranean fever (FMF) is an autosomal recessive disorder characterized by recurrent and self-limited attacks of serosal inflammation with abdominal pain, chest pain, and arthritis usually accompanied by fever. Different vasculitides such as polyarteritis nodosa (PAN) and Henoch-Schönlein syndrome (HSS) may be associated with FMF. We report two sisters of a Turkish family with FMF who developed distinct vasculitides. The younger sister developed severe PAN with perirenal hematoma at the age of 13 years, the older sister presented with severe HSS and acute renal failure at the age of 19 years. Neither sister developed amyloidosis until the age of 30 years. This observation suggests that early events in the pathogenesis of PAN and HSS are generally quite similar.
Assuntos
Febre Familiar do Mediterrâneo/complicações , Vasculite/etiologia , Adolescente , Adulto , Aneurisma/diagnóstico por imagem , Aneurisma/epidemiologia , Angiografia , Criança , Colchicina/uso terapêutico , Febre Familiar do Mediterrâneo/tratamento farmacológico , Febre Familiar do Mediterrâneo/patologia , Feminino , Supressores da Gota/uso terapêutico , Humanos , Vasculite por IgA/etiologia , Imuno-Histoquímica , Poliarterite Nodosa/etiologia , Pele/patologia , Vasculite/diagnóstico por imagem , Vasculite/patologiaRESUMO
BACKGROUND: Out of various high-dose 5-fluorouracil (5-FU) regimens given with or without folinic acid (FA), the optimal 5-FU schedule has still to be defined as treatment for metastatic colorectal cancer (CRC). Consequently, we compared toxicity, response and survival following two FA/5-FU regimens in 55 CRC patients refractory to bolus FA/5-FU. METHODS: Twenty-eight patients (group A) received 5-FU (60 mg/kg body weight) for 48 h, and 27 (group B) received 2-hour infusions of FA (500 mg/m(2)) and 24-hour infusions of 5-FU (2600 mg/m(2)) until disease progression. RESULTS: Both groups were adequately matched with respect to patient characteristics. While overall toxicities were rare, hand-foot syndrome was more common in A. Tumor control was achieved in 57 and 44%, for A and B, respectively. Survival times were 16 months in A and 9 months in B. CONCLUSIONS: Since both 5-FU infusion protocols showed equivalent palliative effects, FA may be questioned in second-line 5-FU regimens.
