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- W3032251607 abstract "Objective To analyse clinical characteristics of neurofibromatosis type 2 (NF2) and evaluate clinical predictors of the risk of mortality in patients of NF2.Methods A total of 175 cases of NF2 patients,confirmed with Manchester criteria for clinical diagnosis of NF2,were retrospectively studied for the clinical manifestations and imaging.Average age of this group was 29.5 year-old,including 92 males and 83 females.27 cases were inherited cases and 148 were sporadic cases.During the follow-up from 1-255 months,154 NF2 cases still survive while 21 cases died.The median survival time was 208.4 ± 10.6 months (95% CI,187.6,229.1).The Kaplan-Meier survival analysis were performed for the survive time,examined with the covariates of age at onset of symptoms,age at diagnosis,sex,inheritance (new mutation or inherited case),presence each type of NF2 lesions (intracranial meningiomas,spinal tumors,peripheral neuropathy and ocular disorders).Results Group of aged at onset of symptoms groups > 19 years was significantly higher survival rate than group of aged ≤ 19 years (P =0.006) ; Patients without spinal tumors had significantly higher survival rate than patients with spinal tumors (P =0.009).Cox proportional hazards regression analysis indicated that presence with spinal tumors was the influencing factors for prognosis of NF2 patients.Conclusions NF2 is a clinically hetereogeneous disease with different phenotypes,presence with spinal tumors was associated with poor survival.Key words: Neurofibromatosis type 2; Clinical characteristics; Prognosis" @default.
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- W3032251607 date "2013-10-28" @default.
- W3032251607 modified "2023-09-28" @default.
- W3032251607 title "Analysis clinical predictors of the risk of mortality in patients with neurofibromatosis type 2" @default.
- W3032251607 doi "https://doi.org/10.3760/cma.j.issn.1001-2346.2013.10.004" @default.
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