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Sundström, TorbjörnORCID iD iconorcid.org/0000-0002-9071-8993
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Publications (10 of 24) Show all publications
Unéus, E. I., Wilhelmsson, C., Bäckström, D., Anan, I., Wixner, J., Pilebro, B., . . . Sundström, T. (2022). Cerebellar and Cerebral Amyloid Visualized by [18F]flutemetamol PET in Long-Term Hereditary V30M (p.V50M) Transthyretin Amyloidosis Survivors. Frontiers in Neurology, 13, Article ID 816636.
Open this publication in new window or tab >>Cerebellar and Cerebral Amyloid Visualized by [18F]flutemetamol PET in Long-Term Hereditary V30M (p.V50M) Transthyretin Amyloidosis Survivors
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2022 (English)In: Frontiers in Neurology, E-ISSN 1664-2295, Vol. 13, article id 816636Article in journal (Refereed) Published
Abstract [en]

Introduction: Hereditary transthyretin (ATTRv) amyloidosis caused by the V30M (p. V50M) mutation is a fatal, neuropathic systemic amyloidosis. Liver transplantation has prolonged the survival of patients and central nervous system (CNS) complications, attributed to amyloid angiopathy caused by CNS synthesis of variant transthyretin, have emerged. The study aimed to ascertain amyloid deposition within the brain in long-term ATTRv amyloidosis survivors with neurological symptoms from the CNS.

Methods: A total of 20 patients with ATTR V30M having symptoms from the CNS and a median disease duration of 16 years (8–25 years) were included in this study. The cognitive and peripheral nervous functions were determined for 18 patients cross-sectionally at the time of the investigation. Amyloid brain deposits were examined by [18F]flutemetamol PET/CT. Five patients with Alzheimer's disease (AD) served as positive controls.

Result: 60% of the patients with ATTRv had a pathological Z-score in the cerebellum, compared to only 20% in the patients with AD. 75% of the patients with transient focal neurological episodes (TFNEs) displayed a pathological uptake only in the cerebellum. Increased cerebellar uptake was related to an early age of onset of the ATTRv disease. 55% of the patients with ATTRv had a pathological Z-score in the global cerebral region compared to 100% of the patients with AD.

Conclusion: Amyloid deposition within the brain after long-standing ATTRv amyloidosis is common, especially in the cerebellum. A cerebellar amyloid uptake profile seems to be related to TFNE symptoms.

Place, publisher, year, edition, pages
Frontiers Media S.A., 2022
Keywords
amyloid angiopathy, amyloidosis-hereditary, positron emission tomography, transthyretin, [18F]flutemetamol
National Category
Neurology
Identifiers
urn:nbn:se:umu:diva-193803 (URN)10.3389/fneur.2022.816636 (DOI)000773941500001 ()35317351 (PubMedID)2-s2.0-85127418033 (Scopus ID)
Funder
Swedish Heart Lung Foundation, 20160787Region VästerbottenThe Swedish Brain Foundation
Available from: 2022-05-06 Created: 2022-05-06 Last updated: 2023-08-28Bibliographically approved
Weidung, B., Hemmingsson, E.-S., Olsson, J., Sundström, T., Blennow, K., Zetterberg, H., . . . Lövheim, H. (2022). VALZ-Pilot: High-dose valacyclovir treatment in patients with early-stage Alzheimer's disease. Alzheimer’s & Dementia: Translational Research & Clinical Interventions, 8(1), Article ID e12264.
Open this publication in new window or tab >>VALZ-Pilot: High-dose valacyclovir treatment in patients with early-stage Alzheimer's disease
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2022 (English)In: Alzheimer’s & Dementia: Translational Research & Clinical Interventions, E-ISSN 2352-8737, Vol. 8, no 1, article id e12264Article in journal (Refereed) Published
Abstract [en]

Introduction: Herpes simplex virus (HSV) may be involved in Alzheimer's disease (AD) pathophysiology. The antiviral valacyclovir inhibits HSV replication.

Methods: This phase-II pilot trial involved valacyclovir administration (thrice daily, 500 mg week 1, 1000 mg weeks 2–4) to persons aged ≥ 65 years with early-stage AD, anti-HSV immunoglobulin G, and apolipoprotein E ε4. Intervention safety, tolerability, feasibility, and effects on Mini-Mental State Examination (MMSE) scores and cerebrospinal fluid (CSF) biomarkers were evaluated.

Results: Thirty-two of 33 subjects completed the trial on full dosage. Eighteen percent experienced likely intervention-related mild, temporary adverse events. CSF acyclovir concentrations were mean 5.29 ± 2.31 μmol/L. CSF total tau and neurofilament light concentrations were unchanged; MMSE score and CSF soluble triggering receptor expressed on myeloid cells 2 concentrations increased (P = .02 and .03).

Discussion: Four weeks of high-dose valacyclovir treatment was safe, tolerable, and feasible in early-stage AD. Our findings may guide future trial design.

Place, publisher, year, edition, pages
John Wiley & Sons, 2022
Keywords
Alzheimer's disease, apolipoprotein E ε4, feasibility study, herpes simplex, pilot project, valacyclovir
National Category
Neurology Neurosciences
Identifiers
urn:nbn:se:umu:diva-203082 (URN)10.1002/trc2.12264 (DOI)000908294000001 ()35310522 (PubMedID)2-s2.0-85137403065 (Scopus ID)
Funder
Swedish Research Council, 2018‐02532EU, Horizon 2020, 860197
Available from: 2023-01-16 Created: 2023-01-16 Last updated: 2023-09-05Bibliographically approved
Löfbacka, V., Axelsson, J., Pilebro, B., Suhr, O. B., Lindqvist, P. & Sundström, T. (2021). Cardiac transthyretin amyloidosis 99mTc-DPD SPECTcorrelates with strain echocardiography and biomarkers. European Journal of Nuclear Medicine and Molecular Imaging, 48, 1822-1832
Open this publication in new window or tab >>Cardiac transthyretin amyloidosis 99mTc-DPD SPECTcorrelates with strain echocardiography and biomarkers
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2021 (English)In: European Journal of Nuclear Medicine and Molecular Imaging, ISSN 1619-7070, E-ISSN 1619-7089, Vol. 48, p. 1822-1832Article in journal (Refereed) Published
Abstract [en]

Purpose: Hereditary transthyretin-amyloid amyloidosis (ATTRv) is an underdiagnosed condition commonly manifesting as congestive heart failure. Recently, scintigraphy utilizing DPD as a tracer was shown to identify ATTRv and wild-type ATTR cardiomyopathy. The aim of this study was to determine the value of quantified scintigraphy utilizing 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid (DPD) single-photon emission computed tomography (SPECT)/CT, and to correlate its uptake with well-established cardiac functional parameters.

Methods: Forty-eight patients with genetically verified ATTRv type-A fibril composition, positive 99mTc-DPD SPECT/CT, were retrospectively analyzed. Manual mapping of volumes of interest (VOIs) on DPD SPECT/CT examinations was used to quantify heart uptake. DPD mean and maximum uptake together with a calculated DPD-based amyloid burden (DPDload) was correlated with echocardiographic strain values and cardiac biomarkers.

Results: Statistically significant correlations were seen in VOIs between DPD uptakes and the corresponding echocardiographic strain values. Furthermore, DPDload had a strong correlation with echocardiographic strain parameters and also correlated with biomarkers troponin T and logarithmic NT-ProBNP.

Conclusions: In patients with ATTRv cardiomyopathy, DPD SPECT/CT measures the amyloid distribution and provides information on cardiac amyloid load. DPD amyloid load correlates with functional cardiac parameters.

Place, publisher, year, edition, pages
Springer, 2021
Keywords
Amyloidosis-hereditary, Cardiomyopathy, Transthyretin amyloidosis, Tc-99m-DPD scintigraphy, 2D speckle tracking strain, SPECT
National Category
Radiology, Nuclear Medicine and Medical Imaging
Identifiers
urn:nbn:se:umu:diva-178531 (URN)10.1007/s00259-020-05144-8 (DOI)000602293500001 ()33367948 (PubMedID)2-s2.0-85098069527 (Scopus ID)
Funder
Swedish Heart Lung Foundation, 20160787
Available from: 2021-01-15 Created: 2021-01-15 Last updated: 2023-03-24Bibliographically approved
Löfbacka, V., Suhr, O. B., Pilebro, B., Wixner, J., Sundström, T., Lindmark, K., . . . Lindqvist, P. (2021). Combining ECG and echocardiography to identify transthyretin cardiac amyloidosis in heart failure. Clinical Physiology and Functional Imaging, 41(5), 408-416
Open this publication in new window or tab >>Combining ECG and echocardiography to identify transthyretin cardiac amyloidosis in heart failure
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2021 (English)In: Clinical Physiology and Functional Imaging, ISSN 1475-0961, E-ISSN 1475-097X, Vol. 41, no 5, p. 408-416Article in journal (Refereed) Published
Abstract [en]

AIMS/BACKGROUND: Transthyretin amyloid (ATTR) amyloidosis cardiomyopathy is an underdiagnosed, causatively treatable cause of heart failure. The aim of this study was to evaluate the efficacy of electrocardiography (ECG) and echocardiography on patients with increased interventricular septum diameter (IVSd) to identify ATTR cardiac amyloidosis (ATTR-CA) patients.

METHODS: We investigated 58 patients with heart failure and an IVSd >14mm. Included were 33 ATTR-CA patients and 25 controls that consisted of non-amyloidosis heart failure (HF) patients with negative 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid (DPD) scintigraphy. We used echocardiography including 2D speckle tracking strain and a 12-lead ECG to test the accuracy to differentiate the groups.

RESULTS: We found high diagnostic accuracy (98%) for differentiating ATTR-CA from HF controls using a combination of R amplitude in -aVR from ECG and relative wall thickness acquired from echocardiography. With this combined model (RWT/ R in -aVR), the sensitivity was 100% and specificity was 95% using a cut off value of 0.90. Furthermore, the area under the curve was 99% and the negative predictive value was 100%.

CONCLUSION: We found that a simple combination of ECG and echocardiographic parameters used in clinical settings was able to differentiate ATTR-CA from other etiologies of HF with increased interventricular septum thickness. The high sensitivity and negative predictive value render the algorithm useful for selection of patients for further diagnostic procedures for ATTR-CA.

Place, publisher, year, edition, pages
John Wiley & Sons, 2021
Keywords
Cardiac amyloidosis, ECG, Echocardiography, Heart failure, Transthyretin amyloidosis
National Category
Cardiac and Cardiovascular Systems
Identifiers
urn:nbn:se:umu:diva-183897 (URN)10.1111/cpf.12715 (DOI)000661468500001 ()34033209 (PubMedID)2-s2.0-85107933221 (Scopus ID)
Funder
Swedish Heart Lung Foundation, 20160787Swedish Heart Lung Foundation, 20200160Swedish Research Council, 2019-01338
Available from: 2021-06-03 Created: 2021-06-03 Last updated: 2022-03-04Bibliographically approved
Lindmark, K., Pilebro, B., Sundström, T. & Lindqvist, P. (2021). Prevalence of wild type transtyrethin cardiac amyloidosis in a heart failure clinic. ESC Heart Failure, 8(1), 745-749
Open this publication in new window or tab >>Prevalence of wild type transtyrethin cardiac amyloidosis in a heart failure clinic
2021 (English)In: ESC Heart Failure, E-ISSN 2055-5822, Vol. 8, no 1, p. 745-749Article in journal (Refereed) Published
Abstract [en]

Aims: Wild type transthyretin amyloidosis (ATTRwt) has gained interest during recent years due to better diagnostic tools and the emergence of treatment options. Little is known about the prevalence of the disease. We aimed to investigate the prevalence in a heart failure population with myocardial hypertrophy.

Methods and results: All patients with an ICD code of heart failure living within the catchment area of Umeå University hospital and intraventricular septum >14 mm were offered screening with 3,3‐diphosphono‐1,2‐propanodicarboxylic acid (DPD) scan and a clinical work up. Out of 2238 patients with heart failure, 174 patients were found to have a septum >14 mm. Ten patients were already diagnosed with hereditary ATTR cardiomyopathy, 12 patients had ATTRwt cardiomyopathy, 12 patients had known HCM, one patient had AL amyloidosis, and four patients had already undergone a negative DPD scan (DPD uptake grade 0 and 1) within the last 3 years. This left 134 patients who we tried to contact for screening, but 48 patients had either died or declined to participate. Out of 86 screened patients, 13 had a DPD uptake of grade 2 or 3 without other amyloid disease making the total number of patients with ATTRwt in this population 25.

Conclusions: Approximately 20% of investigated patients in a cohort with heart failure and increased myocardial wall thickness has ATTRwt. Calculated for the whole population of heart failure patients, the prevalence is just over 1.1%. Comparing this number to the total population would give an estimated prevalence of 1:6000.

Place, publisher, year, edition, pages
John Wiley & Sons, 2021
Keywords
ATTRwt, Cardiac amyloidosis, Amyloidosis, Heart failure, Transthyretin amyloidosis
National Category
Cardiac and Cardiovascular Systems
Identifiers
urn:nbn:se:umu:diva-177241 (URN)10.1002/ehf2.13110 (DOI)000589923100001 ()33205581 (PubMedID)2-s2.0-85096650668 (Scopus ID)
Available from: 2020-12-14 Created: 2020-12-14 Last updated: 2022-05-03Bibliographically approved
Möckelind, S., Axelsson, J., Pilebro, B., Lindqvist, P., Suhr, O. B. & Sundström, T. (2020). Quantification of cardiac amyloid with [18F]Flutemetamol in patients with V30M hereditary transthyretin amyloidosis. Amyloid: Journal of Protein Folding Disorders, 27(3), 191-199
Open this publication in new window or tab >>Quantification of cardiac amyloid with [18F]Flutemetamol in patients with V30M hereditary transthyretin amyloidosis
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2020 (English)In: Amyloid: Journal of Protein Folding Disorders, ISSN 1350-6129, E-ISSN 1744-2818, Vol. 27, no 3, p. 191-199Article in journal (Refereed) Published
Abstract [en]

Background: Hereditary transthyretin amyloid (ATTRv) is a systemic amyloidosis with mainly neurological and cardiac symptoms. The aim of this study was to evaluate the outcome of [18F]Flutemetamol PET/CT-scan of the heart in long-term survivors with ATTRV30M amyloidosis.

Methods: Twenty-one patients with ATTRV30M amyloidosis and predominantly neurological symptoms, mainly negative on cardiac 99mtechnetium-3,3-diphosphono-1,2-propanodicarboxylic acid (DPD)-scintigraphy, were examined with a dynamic [18F]Flutemetamol PET/CT-scan. Five patients suffering from Alzheimer’s disease and one healthy individual served as controls. Volumes of interests were drawn over the intraventricular septum, lateral wall of the left ventricle and free wall of the right ventricle. Clinical records were reviewed for data from previous completed DPD-scintigraphy of the heart and echocardiography.

Results: Patients with ATTRv amyloidosis had a higher cardiac uptake than the control-group in all analysed regions of the heart and could be identified with high accuracy (sensitivity 88%, specificity 100%) in static image acquisition at 30 or 60 min. We found no correlation between cardiac [18F]Flutemetamol uptake and clinical variables.

Conclusion: In this small study of selected patients, cardiac [18F]Flutemetamol PET/CT could differentiate between healthy individuals and patients with ATTRV30M. [18F]Flutemetamol PET/CT imaging of amyloidosis in patients with a negative DPD-scintigraphy has a potential as a diagnostic method.

Place, publisher, year, edition, pages
Taylor & Francis, 2020
Keywords
Amyloidosis-hereditary, cardiomyopathy, [18F]Flutemetamol, positron emission tomography, transthyretin
National Category
Radiology, Nuclear Medicine and Medical Imaging
Identifiers
urn:nbn:se:umu:diva-171783 (URN)10.1080/13506129.2020.1760237 (DOI)000534784400001 ()32400202 (PubMedID)2-s2.0-85084853450 (Scopus ID)
Available from: 2020-06-15 Created: 2020-06-15 Last updated: 2021-05-07Bibliographically approved
Unéus, E., Wilhelmsson, C., Suhr, O., Anan, I., Wixner, J., Pilebro, B., . . . Sundström, T. (2019). Visualisation of amyloid deposition within the brain of long-term hereditary transthyretin amyloidosis survivors by 18F-flutemetamol positron emission tomography. Paper presented at 5th Congress of the European Academy of Neurology (EAN), June 29 – July 2, 2019, Oslo, Norway. European Journal of Neurology, 26(S1), 287-287, Article ID EPR3027.
Open this publication in new window or tab >>Visualisation of amyloid deposition within the brain of long-term hereditary transthyretin amyloidosis survivors by 18F-flutemetamol positron emission tomography
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2019 (English)In: European Journal of Neurology, ISSN 1351-5101, E-ISSN 1468-1331, Vol. 26, no S1, p. 287-287, article id EPR3027Article in journal, Meeting abstract (Other academic) Published
Abstract [en]

Background and aims: Hereditary transthyretin amyloid (ATTRv) amyloidosis caused by the transthyretin (TTR) Val30Met (p.V50M) mutation is characterised by peripheral neuropathy, and central nervous (CNS) complications has rarely been reported. However, liver transplantation has prolonged the patients’ survival, and CNS complications attributed to amyloid angiopathy caused by CNS synthesis of variant TTR have been reported. The aim of the study was to ascertain CNS amyloid deposition in long-term ATTRv survivors.

Methods: 20 ATTR Val30Met patients with symptoms from the CNS and a median disease duration of 16 years (9-25 years) together with five Alzheimer (AD) patients, who served as positive controls were included in the study. Amyloid CNS deposits were assessed by 18F- flutemetamol PET/CT examination utilising relative z scores with pons as reference.

Results: Expectedly, all Alzheimer patients had an clearly increased global composite z score above 2.0 compared with 55% of the ATTRv patients. There was an increased local uptake corresponding to cerebellum in 12 ATTRv patients compared to only one in the AD group (fig 1). Four of these ATTRv patients had a global composite z score within the normal range. No correlation between duration after 9 years and amyloid CNS deposition was noted.

Conclusion: Amyloid deposition within the brain after long-standing ATTRv amyloidosis is increased and is often noted in the cerebellum. However, not all patient display amyloid CNS deposition, thus, additional causes for CNS complications should always be considered.

Place, publisher, year, edition, pages
John Wiley & Sons, 2019
National Category
Neurology
Identifiers
urn:nbn:se:umu:diva-161913 (URN)10.1111/ene.14018 (DOI)000474481001092 ()
Conference
5th Congress of the European Academy of Neurology (EAN), June 29 – July 2, 2019, Oslo, Norway
Available from: 2019-08-12 Created: 2019-08-12 Last updated: 2023-03-07Bibliographically approved
Pilebro, B., Arvidsson, S., Lindqvist, P., Sundström, T., Westermark, P., Antoni, G., . . . Sörensen, J. (2018). Positron emission tomography (PET) utilizing Pittsburgh compound B (PIB) for detection of amyloid heart deposits in hereditary transthyretin amyloidosis (ATTR). Journal of Nuclear Cardiology, 25(1), 240-248
Open this publication in new window or tab >>Positron emission tomography (PET) utilizing Pittsburgh compound B (PIB) for detection of amyloid heart deposits in hereditary transthyretin amyloidosis (ATTR)
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2018 (English)In: Journal of Nuclear Cardiology, ISSN 1071-3581, E-ISSN 1532-6551, Vol. 25, no 1, p. 240-248Article in journal (Refereed) Published
Abstract [en]

Background: DPD scintigraphy has been advocated for imaging cardiac amyloid in ATTR amyloidosis. PET utilizing 11C-Pittsburgh compound B (PIB) is the gold standard for imaging brain amyloid in Alzheimer’s disease. PIB was recently shown to identify cardiac amyloidosis in both AL and ATTR amyloidosis. In the ATTR population, two types of amyloid fibrils exist, one containing fragmented and full-length TTR (type A) and the other only full-length TTR (type B). The aim of this study was to further evaluate PIB-PET in patients with hereditary ATTR amyloidosis.

Methods: Ten patients with biopsy-proven V30M ATTR amyloidosis and discrete or no signs of cardiac involvement were included. Patients were grouped according to TTR-fragmentation. All underwent DPD scintigraphy, echocardiography, and PIB-PET. A left ventricular PIB-retention index (PIB-RI) was established and compared to five normal volunteers.

Results: PIB-RI was increased in all patients (P < 0.001), but was significantly higher in type B than in type A (0.129 ± 0.041 vs 0.040 ± 0.006 min−1, P = 0.009). Cardiac DPD uptake was elevated in group A and absent in group B.

Conclusion: PIB-PET, in contrast to DPD scintigraphy, has the potential to specifically identify cardiac amyloid depositions irrespective of amyloid fibril composition. The heart appears to be a target organ for amyloid deposition in ATTR amyloidosis.

Place, publisher, year, edition, pages
Springer, 2018
Keywords
Cardiomyopathy, amyloidosis, Pittsburgh compound B
National Category
Cardiac and Cardiovascular Systems
Identifiers
urn:nbn:se:umu:diva-127300 (URN)10.1007/s12350-016-0638-5 (DOI)000423585200038 ()27645889 (PubMedID)2-s2.0-84988421982 (Scopus ID)
Available from: 2016-11-07 Created: 2016-11-07 Last updated: 2019-05-21Bibliographically approved
Englund, H., Lidén K., K., Lind, T., Sundström, T. & Karling, P. (2017). Radiation exposure in patients with inflammatory bowel disease and irritable bowel syndrome in the years 2001-2011. Scandinavian Journal of Gastroenterology, 52(3), 300-305
Open this publication in new window or tab >>Radiation exposure in patients with inflammatory bowel disease and irritable bowel syndrome in the years 2001-2011
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2017 (English)In: Scandinavian Journal of Gastroenterology, ISSN 0036-5521, E-ISSN 1502-7708, Vol. 52, no 3, p. 300-305Article in journal (Refereed) Published
Abstract [en]

OBJECTIVES: To compare cumulative ionizing radiation in patients with inflammatory bowel disease (IBD) and irritable bowel syndrome (IBS) for the years 2001-2011. To study how radiation exposure change over time in patients with newly diagnosed IBD and factors associated with radiation exposure.

MATERIAL AND METHODS: All radiological investigations performed between 1 January 2001 and 31 December 2011 were retrospectively recorded in patients with Crohn's disease (CD) (n = 103), ulcerative colitis (UC) (n = 304) and IBS (n = 149). Analyses were done with Mann-Whitney and Chi-Square test.

RESULTS: The median total cumulative radiation exposure in mSv for CD (20.0, inter quartile range (IQR) 34.8), UC (7.01, IQR 23.8), IBS (2.71, IQR 9.15) and the proportion of patients who had been exposed for more than 50 mSv during the study period (CD 19%, UC 11%, IBS 3%) were significantly higher in the patients with CD compared to patients with UC (p < .001) and IBS (p < .001), respectively. In turn, patients with UC had significantly higher doses than patients with IBS (p = .005). Risk factors for radiation exposure were female gender (CD), early onset (UC), ileocolonic location (CD), previous surgery (CD and UC), depression (IBS) and widespread pain (IBS). In newly diagnosed CD, there was a significant decline in median cumulative radiation dose in mSv (17.2 vs. 12.0; p = .048) during the study period.

CONCLUSIONS: Patients with CD are at greatest risk for high cumulative radiation exposure, but there is a decline in exposure during the late 2000s. Non-colectomized patients with UC and patients with IBS have a relatively low risk of cumulative radiation exposure.

Keywords
IBD-clinical, small-intestinal disorders, functional disorders, computer-technology, colonic disorders
National Category
Gastroenterology and Hepatology
Identifiers
urn:nbn:se:umu:diva-128592 (URN)10.1080/00365521.2016.1252945 (DOI)000392488800008 ()27832710 (PubMedID)2-s2.0-84994831358 (Scopus ID)
Available from: 2016-12-07 Created: 2016-12-07 Last updated: 2024-07-02Bibliographically approved
Pilebro, B., Suhr, O. B., Näslund, U., Westermark, P., Lindqvist, P. & Sundström, T. (2016). 99mTC-DPD uptake reflects amyloid fibril composition in hereditary transthyretin amyloidosis. Upsala Journal of Medical Sciences, 121(1), 17-24
Open this publication in new window or tab >>99mTC-DPD uptake reflects amyloid fibril composition in hereditary transthyretin amyloidosis
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2016 (English)In: Upsala Journal of Medical Sciences, ISSN 0300-9734, E-ISSN 2000-1967, Vol. 121, no 1, p. 17-24Article in journal (Refereed) Published
Abstract [en]

Aims In transthyretin amyloid (ATTR) amyloidosis various principal phenotypes have been described: cardiac, neuropathic, or a mixed cardiac and neuropathic. In addition, two different types of amyloid fibrils have been identified (type A and type B). Type B fibrils have thus far only been found in predominantly early-onset V30M and in patients carrying the Y114C mutation, whereas type A is noted in all other mutations currently examined as well as in wild-type ATTR amyloidosis. The fibril type is a determinant of the ATTR V30M disease phenotype. Tc-99m-DPD scintigraphy is a highly sensitive method for diagnosing heart involvement in ATTR amyloidosis. The objective of this study was to determine the relationship between ATTR fibril composition and Tc-99m-DPD scintigraphy outcome in patients with biopsy-proven ATTR amyloidosis. Methods Altogether 55 patients with biopsy-proven diagnosis of ATTR amyloidosis and amyloid fibril composition determined were examined by Tc-99m-DPD scintigraphy. The patients were grouped and compared according to their type of amyloid fibrils. Cardiovascular evaluation included ECG, echocardiography, and cardiac biomarkers. The medical records were scrutinized to identify subjects with hypertension or other diseases that have an impact on cardiac dimensions. Results A total of 97% with type A and none of the patients with type B fibrils displayed Tc-99m-DPD uptake at scintigraphy (p < 0.001). Findings from analyses of cardiac biomarkers, ECG, and echocardiography, though significantly different, could not differentiate between type A and B fibrils in individual patients. Conclusion In ATTR amyloidosis, the outcome of Tc-99m-DPD scintigraphy is strongly related to the patients' transthyretin amyloid fibril composition.

Place, publisher, year, edition, pages
Taylor & Francis, 2016
Keywords
transthyretin, Amyloidosis hereditary, echocardiography, scintigraphy, amyloid cardiomyopathy
National Category
Clinical Medicine
Identifiers
urn:nbn:se:umu:diva-119085 (URN)10.3109/03009734.2015.1122687 (DOI)000372123700003 ()26849806 (PubMedID)2-s2.0-84958093369 (Scopus ID)
Available from: 2016-04-19 Created: 2016-04-11 Last updated: 2023-03-24Bibliographically approved
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ORCID iD: ORCID iD iconorcid.org/0000-0002-9071-8993

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