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  • Rezk, Tamer  (25)
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  • 1
    Language: English
    In: Blood, 16 August 2018, Vol.132(7), pp.761-764
    Keywords: Immunoglobulin M ; Bendamustine Hydrochloride -- Administration & Dosage ; Immunoglobulin Light-Chain Amyloidosis -- Drug Therapy ; Rituximab -- Administration & Dosage
    ISSN: 00064971
    E-ISSN: 1528-0020
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  • 2
    Language: English
    In: Journal of the American College of Cardiology, 25 July 2017, Vol.70(4), pp.466-477
    Description: Cardiac transthyretin amyloidosis (ATTR) is an increasingly recognized cause of heart failure. Cardiac magnetic resonance (CMR), with late gadolinium enhancement (LGE) and T1 mapping, is emerging as a reference standard for diagnosis and characterization of cardiac amyloidosis. The authors used CMR with extracellular volume fraction (ECV) measurement to characterize cardiac involvement in relation to outcome in ATTR. Subjects comprised 263 patients with cardiac ATTR corroborated by grade 2 to 3 Tc-DPD ( Tc-3,3-diphosphono-1,2-propanodicarboxylic acid) cardiac uptake, 17 with suspected cardiac ATTR (grade 1 Tc-DPD), and 12 asymptomatic individuals with amyloidogenic transthyretin (TTR) mutations. Fifty patients with cardiac light-chain (AL) amyloidosis acted as disease comparators. Unlike cardiac AL amyloidosis, asymmetrical septal left ventricular hypertrophy (LVH) was present in 79% of patients with ATTR (70% sigmoid septum and 30% reverse septal contour), whereas symmetrical LVH was present in 18%, and 3% had no LVH. In patients with cardiac amyloidosis, the pattern of LGE was always typical for amyloidosis (29% subendocardial, 71% transmural), including right ventricular LGE (96%). During follow-up (19 ± 14 months), 65 patients died. ECV independently correlated with mortality and remained independent after adjustment for age, N-terminal pro-B-type natriuretic peptide, ejection fraction, E/E′, and left ventricular mass (hazard ratio: 1.164; 95% confidence interval: 1.066 to 1.271; p 〈 0.01). Asymmetrical hypertrophy, traditionally associated with hypertrophic cardiomyopathy, was the commonest pattern of ventricular remodeling in ATTR. LGE imaging was typical in all patients with cardiac ATTR. ECV correlated with amyloid burden and was an independent prognostic factor for survival in this cohort of patients.
    Keywords: Attr ; Extracellular Volume Fraction ; Late Gadolinium Enhancement ; Left Ventricular Hypertrophy ; N-Terminal Pro-B-Type Natriuretic Peptide ; Medicine
    ISSN: 0735-1097
    E-ISSN: 1558-3597
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  • 3
    Language: English
    In: Kidney International, December 2017, Vol.92(6), pp.1476-1483
    Description: Renal involvement causing progressive chronic kidney disease (CKD) is present in 70% of patients with systemic Ig light-chain (AL) amyloidosis at diagnosis. Chemotherapy that substantially suppresses free light chain production is associated with improved patient survival, but its benefit in delaying the onset of renal replacement therapy among patients who present with established advanced CKD has not been studied. To evaluate this, we studied 1000 patients enrolled in the prospective UK AL amyloidosis chemotherapy study (ALchemy). Of these, 84 patients had advanced amyloid-related CKD defined by an estimated glomerular filtration rate (eGFR) under 20 ml/min/1.73 m . We determined outcomes among these 84 patients, who had a median eGFR of 10 ml/min/1.73 m , in relation to response to chemotherapy evaluated at three, six, and 12 months from baseline. Patients who achieved suppression of 90% or more in their amyloidogenic free light chain (dFLC) within three months of baseline had significantly better overall survival, prolonged time to dialysis, and prolonged time to the composite endpoint of ‘death or dialysis’ compared to those who achieved lesser degrees of clonal response at the same time point. Even when this target of greater than 90% dFLC response was achieved but was delayed beyond 3 months, it was associated with worse outcomes. Cox regression analyses confirmed that a 90% or better dFLC response within 3 months was the only significant independent predictor of all three of these outcome measures. Thus, renal survival among patients with systemic immunologic light chain amyloidosis who present with advanced CKD is strongly dependent upon the magnitude and speed with which the underlying hematologic disorder is suppressed by chemotherapy.
    Keywords: Amyloid ; Amyloidosis ; Chemotherapy ; Chronic Kidney Disease ; Medicine
    ISSN: 0085-2538
    E-ISSN: 1523-1755
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  • 4
    Language: English
    In: Heart, April 1, 2017, Vol.103(7)
    Keywords: Amyloidosis -- Diagnosis ; Magnetic Resonance Imaging -- Usage ; Hypertrophic Cardiomyopathy -- Risk Factors
    ISSN: 1355-6037
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  • 5
    Language: English
    In: Heart April 2017, Vol.103(Suppl 1), p.A20
    Description: Cardiac transthyretin amyloidosis (ATTR amyloidosis) is an increasingly recognised cause of heart failure. Cardiovascular magnetic resonance (CMR) with late gadolinium enhancement (LGE) and T1 mapping is emerging as a reference standard for diagnosis and characterisation of cardiac amyloid.
    Keywords: Medicine;
    ISSN: 1355-6037
    ISSN: 13556037
    E-ISSN: 1468-201X
    E-ISSN: 1468201X
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  • 6
    In: Circulation, 2016, Vol.133(24), pp.2404-2412
    Description: BACKGROUND—: Cardiac transthyretin (ATTR) amyloidosis is a progressive and fatal cardiomyopathy for which several promising therapies are in development. The diagnosis is frequently delayed or missed because of the limited specificity of echocardiography and the traditional requirement for histological confirmation. It has long been recognized that technetium-labeled bone scintigraphy tracers can localize to myocardial amyloid deposits, and use of this imaging modality for the diagnosis of cardiac ATTR amyloidosis has lately been revisited. We conducted a multicenter study to ascertain the diagnostic value of bone scintigraphy in this disease. METHODS AND RESULTS—: Results of bone scintigraphy and biochemical investigations were analyzed from 1217 patients with suspected cardiac amyloidosis referred for evaluation in specialist centers. Of 857 patients with histologically proven amyloid (374 with endomyocardial biopsies) and 360 patients subsequently confirmed to have nonamyloid cardiomyopathies, myocardial radiotracer uptake on bone scintigraphy was 〉99% sensitive and 86% specific for cardiac ATTR amyloid, with false positives almost exclusively from uptake in patients with cardiac AL amyloidosis. Importantly, the combined findings of grade 2 or 3 myocardial radiotracer uptake on bone scintigraphy and the absence of a monoclonal protein in serum or urine had a specificity and positive predictive value for cardiac ATTR amyloidosis of 100% (positive predictive value confidence interval, 98.0–100). CONCLUSIONS—: Bone scintigraphy enables the diagnosis of cardiac ATTR amyloidosis to be made reliably without the need for histology in patients who do not have a monoclonal gammopathy. We propose noninvasive diagnostic criteria for cardiac ATTR amyloidosis that are applicable to the majority of patients with this disease.
    Keywords: Medicine ; Anatomy & Physiology;
    ISSN: 0009-7322
    E-ISSN: 15244539
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  • 7
    In: Arthritis & Rheumatology, October 2017, Vol.69(10), pp.2051-2051
    Keywords: Amyloid Neuropathies, Familial -- Diagnosis ; Carpal Tunnel Syndrome -- Pathology;
    ISSN: 2326-5191
    E-ISSN: 2326-5205
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  • 8
    In: European Heart Journal, 2018, Vol. 39(30), pp.2799-2806
    Description: Abstract Aims Cardiac transthyretin (ATTR) amyloidosis is an increasingly recognized, progressive, and fatal cardiomyopathy, the natural history of which remains unclear. We sought to establish and validate a new prognostic staging system applicable to patients with both wild-type ATTR (ATTRwt) and hereditary variant ATTR (ATTRv) amyloid cardiomyopathy. Methods and results Eight hundred and sixty-nine patients with cardiac ATTR amyloidosis (553 with ATTRwt and 316 with ATTRv) attending the UK National Amyloidosis Centre were stratified into three disease stages at baseline on the basis of cut points in two universally measured biomarkers, N-terminal pro-B-type natriuretic peptide (NT-proBNP) and estimated glomerular filtration rate (eGFR). Stage I was defined as NT-proBNP ≤3000 ng/L and eGFR ≥45 ml/min, Stage III was defined as NT-proBNP 〉3000 ng/L and eGFR 〈45 ml/min, and the remainder were Stage II. The staging system was validated in a cohort of 318 patients with cardiac ATTR amyloidosis from France. Median survival among 393 (45%) Stage I patients was 69.2 months, 334 (38%) Stage II patients was 46.7 months, and 142 (16%) Stage III patients was 24.1 months (P 〈 0.0001). After adjusting for age, compared with Stage I, the hazard ratio (HR) for death for Stage II was 2.05 [confidence interval (CI) 1.54–2.72, P 〈 0.001] and for Stage III was 3.80 (CI 2.73–5.28, P 〈 0.001). HRs and statistical significance were little altered by transthyretin genotype and were maintained in the validation cohort. Conclusion This simple, universally applicable staging system stratifies patients with both ATTRwt and ATTRv amyloid cardiomyopathy into prognostic categories. It will be of value in the design of forthcoming clinical trials of novel amyloid-specific therapies.
    Keywords: Amyloid ; Amyloidosis ; Transthyretin ; Ttr ; Cardiomyopathy ; Staging
    ISSN: 0195-668X
    E-ISSN: 1522-9645
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  • 9
    Language: English
    In: British journal of haematology, July 2018, Vol.182(1), pp.145-148
    Description: To purchase or authenticate to the full-text of this article, please visit this link: http://onlinelibrary.wiley.com/doi/10.1111/bjh.14747/abstract Byline: Tamer Rezk, Carol J. Whelan, Helen J. Lachmann, Marianna Fontana, Sajitha Sachchithanantham, Shameem Mahmood, Fakhar Khan, Raj Khiani, Joseph Tomson,Taryn Youngstein, Julian D. Gillmore, Philip N. Hawkins, Ashutosh D. Wechalekar Keywords: Cardiac AL amyloidosis; amyloidosis; arrhythmias; implantable cardioverter-defibrillator (ICD); infiltrative cardiomyopathies ***** No abstract is available for this article. *****
    Keywords: Cardiac AL Amyloidosis ; Amyloidosis ; Arrhythmias ; Implantable Cardioverter-Defibrillator (ICD) ; Infiltrative Cardiomyopathies
    ISSN: 00071048
    E-ISSN: 1365-2141
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  • 10
    In: Nephrology Dialysis Transplantation, 2018, Vol. 33(2), pp.241-247
    Description: Introduction: Renal biopsy series from North America suggest that leucocyte chemotactic factor 2 (ALECT2) amyloid is the third most common type of renal amyloid. We report the first case series from a European Centre of prevalence, clinical presentation and diagnostic findings in ALECT2 amyloidosis and report long-term patient and renal outcomes for the first time. Methods: We studied the clinical features, diagnostic investigations and the outcome of all patients with ALECT2 amyloidosis followed systematically at the UK National Amyloidosis Centre (NAC) between 1994 and 2015. Results: Twenty-four patients, all non-Caucasian, were diagnosed with ALECT2 amyloidosis representing 1.3% of all patients referred to the NAC with biopsy-proved renal amyloid. Diagnosis was made at median age of 62 years, usually from renal histology; immunohistochemical staining was definitive for ALECT2 fibril type. Median estimated glomerular filtration rate (GFR) at diagnosis was 33 mL/min/1.73 m2 and median proteinuria was 0.5 g/24 h. Hepatic amyloid was evident on serum amyloid P component (SAP) scintigraphy in 11/24 cases but was not associated with significant derangement of liver function. No patient had evidence of cardiac amyloidosis or amyloid neuropathy. Median follow-up was 4.8 (range 0.5–15.2) years, during which four patients died and four progressed to end-stage renal disease. The mean rate of GFR loss was 4.2 (range 0.5–9.6) mL/min/year and median estimated renal survival from diagnosis was 8.2 years. Serial SAP scans revealed little or no change in total body amyloid burden. Conclusions: ALECT2 amyloidosis is a relatively benign type of renal amyloid, associated with a slow GFR decline, which is reliably diagnosed on renal histology. Neither the molecular basis nor the factors underlying the apparent restriction of ALECT2 amyloidosis to non-Caucasian populations have been determined.
    Keywords: Amyloidosis ; Chronic Kidney Disease ; End - Stage Renal Disease ; Leucocyte Chemotactic Factor 2 Amyloidosis ; Proteinuria
    ISSN: 0931-0509
    E-ISSN: 1460-2385
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