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Results 24 patients (20 males) affected by FAP associated with 7 different mutations were treated for a median of 24 months (range 12-60). Median age at baseline was 69 years (range 57-82), disease duration 43 months (range 17-90), PND score IIIA (range I-IV), Kumamoto score 25 (2-39), BMI 890 (range 604-1458). 21 patients presented with heart involvement. Median NT-proBNP was 728 pg/ml (range 141-5965), cTnI 0.04 ng/ml (range 0.029-0.65), mLVW 14.2 mm (range 12.5-17.5). PND increased by 1 point from baseline in 8/18 patients. mBMI remained stable during treatment. Mean change in Kumamoto score was 2.9/year (95% CI -0.3 to 4.8). Cardiac progression occurred only in 2/21 patients. One patient discontinued due to renal failure and three presented with a mild increase in serum creatinine. One patient discontinued after 3 years of treatment due to asymptomatic TnI increase that improved following discontinuation. No GI events were recorded.

Diflunisal in late-onset FAP patients with moderate to severe neuropathy