Page last updated: 2024-10-26

diflunisal and Amyloid Neuropathy Type 1

diflunisal has been researched along with Amyloid Neuropathy Type 1 in 35 studies

Diflunisal: A salicylate derivative and anti-inflammatory analgesic with actions and side effects similar to those of ASPIRIN.
diflunisal : An organofluorine compound comprising salicylic acid having a 2,4-difluorophenyl group at the 5-position.

Research Excerpts

ExcerptRelevanceReference
" Herein, we report the diflunisal plasma concentrations measured by extraction, reversed phase HPLC separation, and fluorescence detection after long-term 250 mg BID oral dosing in two groups: a placebo-controlled diflunisal clinical trial group and an open-label Japanese polyneuropathy treatment cohort."1.91Characterising diflunisal as a transthyretin kinetic stabilizer at relevant concentrations in human plasma using subunit exchange. ( Berk, JL; Jäger, M; Kelly, JW; Kline, GM; Nelson, LT; Powers, ET; Sekijima, Y; Tsai, FJ, 2023)
"Diflunisal is an approved non-steroidal anti-inflammatory drug that stabilizes TTR, with limited data available regarding effects on cardiac structure and function."1.56Stabilization of Cardiac Function With Diflunisal in Transthyretin (ATTR) Cardiac Amyloidosis. ( Berk, JL; Connors, LH; Fox, J; Gopal, DM; Hellawell, J; Lohrmann, G; Maurer, MS; Mussinelli, R; Pipilas, A; Ruberg, FL; Siddiqi, OK; Vellanki, N, 2020)

Research

Studies (35)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's2 (5.71)29.6817
2010's18 (51.43)24.3611
2020's15 (42.86)2.80

Authors

AuthorsStudies
Yokoyama, T3
Kosaka, Y1
Mizuguchi, M3
Connelly, S1
Mortenson, DE1
Choi, S1
Wilson, IA1
Powers, ET5
Kelly, JW8
Johnson, SM1
Miller, M1
Pal, A1
Albusairi, W1
Joo, H1
Pappas, B1
Haque Tuhin, MT1
Liang, D1
Jampala, R1
Liu, F1
Khan, J1
Faaij, M1
Park, M1
Chan, W1
Graef, I1
Zamboni, R1
Kumar, N1
Fox, J2
Sinha, U1
Alhamadsheh, M1
Kitakami, R1
Inui, K1
Nakagawa, Y1
Sawai, Y1
Katayama, W1
Okada, T1
Kanamitsu, K1
Nakagawa, S1
Toyooka, N1
Kashihara, M1
Villanueva, E1
Carretero, M1
Aguirre, MA1
Negro, A1
Belziti, CA1
Posadas-Martínez, ML1
Nucifora, EM1
Baratta, S1
Costabel, JP1
Higa, C1
Rivas, C1
Fernández, A1
Quiroga, A1
Dumont, CA1
Volberg, VI1
Streitenberg, GM1
Perez de Arenaza, D1
Tsai, FJ3
Nelson, LT4
Kline, GM3
Jäger, M3
Berk, JL7
Sekijima, Y11
Nuvolone, M1
Girelli, M1
Merlini, G3
Lohrmann, G1
Pipilas, A1
Mussinelli, R1
Gopal, DM1
Connors, LH2
Vellanki, N1
Hellawell, J1
Siddiqi, OK2
Maurer, MS3
Ruberg, FL1
Rubin, J1
Magrinelli, F1
Fabrizi, GM1
Santoro, L1
Manganelli, F1
Zanette, G1
Cavallaro, T1
Tamburin, S1
Lin, H1
Merkel, M1
Hale, C1
Marantz, JL1
Cristóbal Gutiérrez, H1
Pelayo-Negro, AL1
Gómez Gómez, D1
Martín Vega, MÁ1
Valero Domínguez, M1
Paxman, RJ1
Xu, J1
Webb, B1
Benbrahim, M1
Norman, K1
Sanchorawala, V1
Hughes, D1
Patel, RK1
Fontana, M1
Hawkins, PN1
Gillmore, J1
Ibrahim, M1
Saint Croix, GR1
Lacy, S1
Fattouh, M1
Barillas-Lara, MI1
Behrooz, L1
Mechanic, O1
Koike, H1
Katsuno, M1
Azorín, SE1
Cabib, CE1
Campistol, JM1
Klimtchuk, ES1
Prokaeva, T1
Frame, NM1
Abdullahi, HA1
Spencer, B1
Dasari, S1
Cui, H1
Kurtin, PJ1
Gursky, O1
Buxbaum, JN1
Adams, D2
Cauquil, C2
Théaudin, M2
Suhr, OB2
Obici, L2
Zeldenrust, SR2
Yamashita, T2
Heneghan, MA1
Gorevic, PD1
Litchy, WJ1
Wiesman, JF1
Nordh, E1
Corato, M1
Lozza, A1
Cortese, A1
Robinson-Papp, J1
Colton, T1
Rybin, DV1
Bisbee, AB2
Ando, Y2
Ikeda, S4
Seldin, DC1
Skinner, M2
Dyck, PJ2
Takahashi, R1
Ono, K1
Shibata, S1
Nakamura, K1
Komatsu, J1
Ikeda, Y1
Ikeda, T1
Samuraki, M1
Sakai, K1
Iwasa, K1
Kayano, D1
Yamada, M1
Vilaró, M1
Nieto, J1
La Parra, JR1
Almeida, MR1
Ballesteros, A1
Planas, A1
Arsequell, G1
Valencia, G1
Castaño, A1
Drachman, BM1
Judge, D1
Labeyrie, C1
Beaudonnet, G1
Algalarrondo, V1
Heneghan, M1
Gorevic, P1
Tojo, K1

Clinical Trials (2)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
Analysis of Lumbar Spine Stenosis Specimens for Identification of Transthyretin Cardiac Amyloidosis[NCT06034405]1,663 participants (Anticipated)Observational2023-09-30Not yet recruiting
The Effect of Diflunisal on Familial Amyloidosis[NCT00294671]Phase 2/Phase 3130 participants (Actual)Interventional2006-02-28Completed
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Trial Outcomes

Kumamoto Neurologic Scale;

Change from baseline of the Kumamoto Score (0-102 points, increasing with disease severity), a clinical neurologic scale of motor, sensory, and autonomic function combined with heart and kidney end organ measures developed to track disease progression in Familial Amyloid Polyneuropathy (ATTR-FAP) (NCT00294671)
Timeframe: Baseline, 1 and 2 years

,
Interventionunits on a scale (Mean)
Change from baseline to 2 yearsChange from baseline to 1 year
Diflunisal3.11.9
Placebo8.04.1

Modified Body Mass Index (mBMI);

The product of body mass index (BMI) and serum albumin level (g/L) [kg/M2xg/L]. (NCT00294671)
Timeframe: Baseline, 1 and 2 years

,
Interventionkg/M2xg/L (Mean)
Change from baseline to 2 yearsChange from baseline to 1 year
Diflunisal-33.7-18.7
Placebo-67.9-38.5

Neurologic Impairment Score + 7 (NIS+7)

The primary endpoint, the difference in polyneuropathy progression between treatments, was measured by the Neuropathy Impairment Score plus 7 nerve tests (NIS+7) which ranges from 0 (no neurologic deficits) to 270 points (no detectable peripheral nerve function). (NCT00294671)
Timeframe: Baseline, 1 and 2 years

,
Interventionunits on a scale (Mean)
Change from baseline to 2 yearsChange from baseline to 1 year
Diflunisal8.26.2
Placebo26.312.5

Quality of Life Questionnaire: SF-36 Mental Component Score

The 36 item short-form health survey (SF-36) was used to assess the difference between treatment groups for change of mental component scores over 2 years treatment. Range 0-100; lower scores reflect lower quality-of-life. (NCT00294671)
Timeframe: Baseline, 1 and 2 years

,
Interventionunits on a scale (Mean)
Change from baseline to 2 yearsChange from baseline to 1 year
Diflunisal3.52.5
Placebo-0.90.8

Quality of Life Questionnaire: SF-36 Physical Component Score

The 36 item short-form health survey (SF-36) was used to assess the difference between treatment groups for change of physical component scores over 2 years treatment. Range 0-100; lower scores reflect lower quality-of-life. (NCT00294671)
Timeframe: Baseline, 1 and 2 years

,
Interventionunits on a scale (Mean)
Change from baseline to 2 yearsChange from baseline to 1 year
Diflunisal1.20.7
Placebo-4.9-1.9

Reviews

15 reviews available for diflunisal and Amyloid Neuropathy Type 1

ArticleYear
Oral Therapy for the Treatment of Transthyretin-Related Amyloid Cardiomyopathy.
    International journal of molecular sciences, 2022, Dec-18, Volume: 23, Issue:24

    Topics: Amyloid; Amyloid Neuropathies, Familial; Cardiomyopathies; Diflunisal; Humans; Prealbumin

2022
Cardiac Amyloidosis: Overlooked, Underappreciated, and Treatable.
    Annual review of medicine, 2020, 01-27, Volume: 71

    Topics: Amyloid Neuropathies, Familial; Amyloidosis; Arrhythmias, Cardiac; Benzoates; Benzoxazoles; Biopsy;

2020
Pharmacological treatment for familial amyloid polyneuropathy.
    The Cochrane database of systematic reviews, 2020, Apr-20, Volume: 4

    Topics: Amyloid Neuropathies, Familial; Benzoxazoles; Diflunisal; Disease Progression; Humans; Oligonucleoti

2020
Overview of treatments used in transthyretin-related hereditary amyloidosis: a systematic review.
    European journal of hospital pharmacy : science and practice, 2020, Volume: 27, Issue:4

    Topics: Amyloid Neuropathies, Familial; Benzoxazoles; Diflunisal; Humans; Liver Transplantation; RNA, Small

2020
A Review of Novel Agents and Clinical Considerations in Patients With ATTR Cardiac Amyloidosis.
    Journal of cardiovascular pharmacology, 2021, 05-01, Volume: 77, Issue:5

    Topics: Amyloid Neuropathies, Familial; Animals; Benzoxazoles; Cardiomyopathies; Cardiovascular Agents; Difl

2021
The use of diflunisal for transthyretin cardiac amyloidosis: a review.
    Heart failure reviews, 2022, Volume: 27, Issue:2

    Topics: Adult; Amyloid Neuropathies, Familial; Diflunisal; Humans; Myocardium; Prealbumin

2022
The Ultrastructure of Tissue Damage by Amyloid Fibrils.
    Molecules (Basel, Switzerland), 2021, Jul-29, Volume: 26, Issue:15

    Topics: Alzheimer Disease; Amyloid; Amyloid Neuropathies, Familial; Benzoxazoles; Diflunisal; Humans; Immuno

2021
Treatment of hereditary and acquired forms of transthyretin amyloidosis in the era of personalized medicine: the role of randomized controlled trials.
    Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis, 2019, Volume: 26, Issue:2

    Topics: Amyloid Neuropathies; Amyloid Neuropathies, Familial; Benzoxazoles; Diflunisal; Female; Humans; Male

2019
[Familial amyloid polyneuropathies: therapeutic issues].
    Bulletin de l'Academie nationale de medecine, 2012, Volume: 196, Issue:7

    Topics: Amyloid Neuropathies, Familial; Antibodies, Monoclonal; Benzoxazoles; Clinical Trials as Topic; Difl

2012
Recent progress in the understanding and treatment of transthyretin amyloidosis.
    Journal of clinical pharmacy and therapeutics, 2014, Volume: 39, Issue:3

    Topics: Amyloid Neuropathies, Familial; Benzoxazoles; Diflunisal; Genetic Therapy; Humans; Liver Transplanta

2014
Natural history and therapy of TTR-cardiac amyloidosis: emerging disease-modifying therapies from organ transplantation to stabilizer and silencer drugs.
    Heart failure reviews, 2015, Volume: 20, Issue:2

    Topics: Amyloid Neuropathies, Familial; Benzoxazoles; Biomarkers; Cardiomyopathies; Cyclooxygenase Inhibitor

2015
Transthyretin (ATTR) amyloidosis: clinical spectrum, molecular pathogenesis and disease-modifying treatments.
    Journal of neurology, neurosurgery, and psychiatry, 2015, Volume: 86, Issue:9

    Topics: Amyloid Neuropathies, Familial; Benzoxazoles; Diflunisal; Humans; Mutation; Prealbumin

2015
[Clinical diversity, diagnosis and treatment of hereditary amyloid neuropathy].
    Rinsho shinkeigaku = Clinical neurology, 2014, Volume: 54, Issue:12

    Topics: Adult; Age of Onset; Aged; Aged, 80 and over; Amyloid; Amyloid Neuropathies, Familial; Benzoxazoles;

2014
TTR kinetic stabilizers and TTR gene silencing: a new era in therapy for familial amyloidotic polyneuropathies.
    Expert opinion on pharmacotherapy, 2016, Volume: 17, Issue:6

    Topics: Amyloid Neuropathies, Familial; Benzoxazoles; Clinical Trials, Phase III as Topic; Diflunisal; Disea

2016
[Therapeutic strategy for familial amyloid polyneuropathy (FAP)].
    Rinsho shinkeigaku = Clinical neurology, 2009, Volume: 49, Issue:11

    Topics: Amino Acid Substitution; Amyloid; Amyloid Neuropathies, Familial; Anti-Inflammatory Agents, Non-Ster

2009

Trials

3 trials available for diflunisal and Amyloid Neuropathy Type 1

ArticleYear
Experience of patisiran with transthyretin stabilizers in patients with hereditary transthyretin-mediated amyloidosis.
    Neurodegenerative disease management, 2020, Volume: 10, Issue:5

    Topics: Adult; Aged; Amyloid Neuropathies, Familial; Benzoxazoles; Diflunisal; Female; Humans; Male; Middle

2020
Repurposing diflunisal for familial amyloid polyneuropathy: a randomized clinical trial.
    JAMA, 2013, Dec-25, Volume: 310, Issue:24

    Topics: Aged; Amyloid Neuropathies, Familial; Anti-Inflammatory Agents, Non-Steroidal; Body Mass Index; Difl

2013
The Diflunisal Trial: study accrual and drug tolerance.
    Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis, 2012, Volume: 19 Suppl 1

    Topics: Adult; Aged; Amyloid Neuropathies, Familial; Anti-Inflammatory Agents, Non-Steroidal; Diflunisal; Fe

2012

Other Studies

17 other studies available for diflunisal and Amyloid Neuropathy Type 1

ArticleYear
Inhibitory activities of propolis and its promising component, caffeic acid phenethyl ester, against amyloidogenesis of human transthyretin.
    Journal of medicinal chemistry, 2014, Nov-13, Volume: 57, Issue:21

    Topics: Amyloid Neuropathies, Familial; Amyloidosis; Caffeic Acids; Coumaric Acids; Masoprocol; Phenylethyl

2014
Semi-quantitative models for identifying potent and selective transthyretin amyloidogenesis inhibitors.
    Bioorganic & medicinal chemistry letters, 2017, 08-01, Volume: 27, Issue:15

    Topics: Amyloid Neuropathies, Familial; Computer Simulation; Drug Design; Humans; Molecular Docking Simulati

2017
Enthalpy-Driven Stabilization of Transthyretin by AG10 Mimics a Naturally Occurring Genetic Variant That Protects from Transthyretin Amyloidosis.
    Journal of medicinal chemistry, 2018, 09-13, Volume: 61, Issue:17

    Topics: Administration, Oral; Amyloid Neuropathies, Familial; Animals; Benzoates; Biomimetics; Dogs; Entropy

2018
Inhibitory activities of anthraquinone and xanthone derivatives against transthyretin amyloidogenesis.
    Bioorganic & medicinal chemistry, 2021, 08-15, Volume: 44

    Topics: Amyloid Neuropathies, Familial; Anthraquinones; Crystallography, X-Ray; Dose-Response Relationship,

2021
Repositioning of the Anthelmintic Drugs Bithionol and Triclabendazole as Transthyretin Amyloidogenesis Inhibitors.
    Journal of medicinal chemistry, 2021, 10-14, Volume: 64, Issue:19

    Topics: Amyloid Neuropathies, Familial; Anthelmintics; Bithionol; Crystallography, X-Ray; Drug Repositioning

2021
[Specific pharmacological treatment guide for transthyretin amyloid cardiomyopathy, 2021].
    Medicina, 2022, Volume: 82, Issue:2

    Topics: Amyloid Neuropathies, Familial; Benzoxazoles; Cardiomyopathies; Diflunisal; Doxycycline; Humans; Pre

2022
Characterising diflunisal as a transthyretin kinetic stabilizer at relevant concentrations in human plasma using subunit exchange.
    Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis, 2023, Volume: 30, Issue:2

    Topics: Amyloid Neuropathies, Familial; Anti-Inflammatory Agents, Non-Steroidal; Diflunisal; Excipients; Hum

2023
Characterising diflunisal as a transthyretin kinetic stabilizer at relevant concentrations in human plasma using subunit exchange.
    Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis, 2023, Volume: 30, Issue:2

    Topics: Amyloid Neuropathies, Familial; Anti-Inflammatory Agents, Non-Steroidal; Diflunisal; Excipients; Hum

2023
Characterising diflunisal as a transthyretin kinetic stabilizer at relevant concentrations in human plasma using subunit exchange.
    Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis, 2023, Volume: 30, Issue:2

    Topics: Amyloid Neuropathies, Familial; Anti-Inflammatory Agents, Non-Steroidal; Diflunisal; Excipients; Hum

2023
Characterising diflunisal as a transthyretin kinetic stabilizer at relevant concentrations in human plasma using subunit exchange.
    Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis, 2023, Volume: 30, Issue:2

    Topics: Amyloid Neuropathies, Familial; Anti-Inflammatory Agents, Non-Steroidal; Diflunisal; Excipients; Hum

2023
Stabilization of Cardiac Function With Diflunisal in Transthyretin (ATTR) Cardiac Amyloidosis.
    Journal of cardiac failure, 2020, Volume: 26, Issue:9

    Topics: Aged; Aged, 80 and over; Amyloid Neuropathies, Familial; Cardiomyopathies; Diflunisal; Female; Heart

2020
Blinded potency comparison of transthyretin kinetic stabilisers by subunit exchange in human plasma.
    Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis, 2021, Volume: 28, Issue:1

    Topics: Amyloid; Amyloid Neuropathies, Familial; Benzoates; Benzoxazoles; Cardiomyopathies; Diflunisal; Huma

2021
Splenic regression of amyloid on multi-modality imaging in response to treatment with patisiran and diflunisal in hereditary transthyretin amyloidosis.
    Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis, 2021, Volume: 28, Issue:4

    Topics: Amyloid Neuropathies, Familial; Diflunisal; Humans; Multimodal Imaging; Prealbumin; RNA, Small Inter

2021
Diflunisal compassive use in transthyretin hereditary amyloid polyneuropathy: report of a first Spanish experience.
    Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis, 2017, Volume: 24, Issue:sup1

    Topics: Aged; Amyloid; Amyloid Neuropathies; Amyloid Neuropathies, Familial; Diflunisal; Female; Humans; Mal

2017
Unusual duplication mutation in a surface loop of human transthyretin leads to an aggressive drug-resistant amyloid disease.
    Proceedings of the National Academy of Sciences of the United States of America, 2018, 07-10, Volume: 115, Issue:28

    Topics: Amyloid; Amyloid Neuropathies, Familial; Diflunisal; Drug Resistance; Female; Humans; Male; Models,

2018
[Newly developed drug therapies for familial amyloid polyneuropathy: diflunisal and tafamidis].
    Brain and nerve = Shinkei kenkyu no shinpo, 2014, Volume: 66, Issue:7

    Topics: Amyloid Neuropathies, Familial; Benzoxazoles; Diflunisal; Humans; Liver Transplantation; Molecular T

2014
Efficacy of diflunisal on autonomic dysfunction of late-onset familial amyloid polyneuropathy (TTR Val30Met) in a Japanese endemic area.
    Journal of the neurological sciences, 2014, Oct-15, Volume: 345, Issue:1-2

    Topics: 3-Iodobenzylguanidine; Aged; Amyloid Neuropathies, Familial; Anti-Inflammatory Agents, Non-Steroidal

2014
Tuning transthyretin amyloidosis inhibition properties of iododiflunisal by combinatorial engineering of the nonsalicylic ring substitutions.
    ACS combinatorial science, 2015, Jan-12, Volume: 17, Issue:1

    Topics: Amyloid Neuropathies, Familial; Combinatorial Chemistry Techniques; Diflunisal; Humans; Structure-Ac

2015
[Familial amyloid polyneuropathy: diflunisal].
    Rinsho shinkeigaku = Clinical neurology, 2010, Volume: 50, Issue:11

    Topics: Amyloid Neuropathies, Familial; Clinical Trials, Phase II as Topic; Clinical Trials, Phase III as To

2010
Diflunisal stabilizes familial amyloid polyneuropathy-associated transthyretin variant tetramers in serum against dissociation required for amyloidogenesis.
    Neuroscience research, 2006, Volume: 56, Issue:4

    Topics: Adult; Aged; Amyloid beta-Peptides; Amyloid Neuropathies, Familial; Anti-Inflammatory Agents, Non-St

2006