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Phase 3 N=130 Randomized Double-blind Treatment

The Effect of Diflunisal on Familial Amyloidosis

Familial Amyloid Polyneuropathy · Familial Amyloidosis

Enrolled (actual)
130
Serious AEs
5.4%
Results posted
Mar 2017
Primary outcome: Primary: Neurologic Impairment Score + 7 (NIS+7) — 8.2; 26.3; 6.2; 12.5 units on a scale — p=<0.001

Study Design & Population

Study type
Interventional
Phase
Phase 3
Interventions
diflunisal (Drug); placebo (Other)
Age
Adult, Older Adult · 18+ yrs
Sex
All
Sponsor
Boston University
Primary completion
Dec 2012

Outcome Measures

OutcomeResultp-value
PRIMARY
Neurologic Impairment Score + 7 (NIS+7)
8.2; 26.3; 6.2; 12.5 <0.001 sig
SECONDARY
Kumamoto Neurologic Scale;
3.1; 8.0; 1.9; 4.1 0.002 sig
SECONDARY
Modified Body Mass Index (mBMI);
-33.7; -67.9; -18.7; -38.5 0.21
SECONDARY
Quality of Life Questionnaire: SF-36 Physical Component Score
1.2; -4.9; 0.7; -1.9 0.001 sig
SECONDARY
Quality of Life Questionnaire: SF-36 Mental Component Score
3.5; -0.9; 2.5; 0.8 0.06

Summary

The purpose of this study is to determine if diflunisal can prevent progressive lower leg nerve damage in patients with familial amyloidosis polyneuropathy. Funding Source - FDA Office of Orphan Products Development (OOPD); National Institute of Neurological Disorders and Stroke (NINDS)

Eligibility Criteria

Inclusion Criteria

  • Age 18 to 75 years
  • Biopsy proven amyloidosis
  • Genotyping of variant transthyretin
  • Signs of peripheral or autonomic neuropathy

Exclusion Criteria

  • Use of other non-steroidal anti-inflammatory drugs
  • Other causes of sensorimotor polyneuropathy
  • Anticipated survival <2 years or liver transplantation in <1 yr
  • Liver transplantation
  • Profound nerve, heart or kidney impairment
  • Pregnancy or unwillingness to use contraception by women of childbearing age
  • Active or recent gastrointestinal bleeding
  • Non-steroidal or aspirin drug allergy/hypersensitivity
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT00294671). Outcome figures and adverse-event rates are extracted automatically from the registry's posted results and are provided for clinician reference, not as a substitute for the primary publication.

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