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  • 1
    Language: English
    In: Kidney International, March 2015, Vol.87(3), pp.516-526
    Description: Amyloidosis results from protein misfolding, and ongoing amyloid deposition can ultimately lead to organ failure and death. Historically, this is a group of diseases with limited treatment options and frequently poor prognosis. However, there are now ‘targeted’ therapeutics emerging in the form of stabilizers of the precursor protein, inhibitors of fibrillogenesis, fibril disruptors, and blockers of protein translation, transcription, and immunotherapy. We review many of these approaches that are currently being assessed in clinical trials.
    Keywords: Amyloidosis ; Chemotherapy ; Diflunisal ; Eprodisate ; Immunotherapy ; Oligonucleotides ; Medicine
    ISSN: 0085-2538
    E-ISSN: 1523-1755
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  • 2
    In: Circulation, 2015, Vol.132(16), pp.1570-1579
    Description: BACKGROUND—: The prognosis and treatment of the 2 main types of cardiac amyloidosis, immunoglobulin light chain (AL) and transthyretin (ATTR) amyloidosis, are substantially influenced by cardiac involvement. Cardiovascular magnetic resonance with late gadolinium enhancement (LGE) is a reference standard for the diagnosis of cardiac amyloidosis, but its potential for stratifying risk is unknown. METHODS AND RESULTS—: Two hundred fifty prospectively recruited subjects, 122 patients with ATTR amyloid, 9 asymptomatic mutation carriers, and 119 patients with AL amyloidosis, underwent LGE cardiovascular magnetic resonance. Subjects were followed up for a mean of 24±13 months. LGE was performed with phase-sensitive inversion recovery (PSIR) and without (magnitude only). These were compared with extracellular volume measured with T1 mapping. PSIR was superior to magnitude-only inversion recovery LGE because PSIR always nulled the tissue (blood or myocardium) with the longest T1 (least gadolinium). LGE was classified into 3 patterns: none, subendocardial, and transmural, which were associated with increasing amyloid burden as defined by extracellular volume (P〈0.0001), with transitions from none to subendocardial LGE at an extracellular volume of 0.40 to 0.43 (AL) and 0.39 to 0.40 (ATTR) and to transmural at 0.48 to 0.55 (AL) and 0.47 to 0.59 (ATTR). Sixty-seven patients (27%) died. Transmural LGE predicted death (hazard ratio, 5.4; 95% confidence interval, 2.1–13.7; P〈0.0001) and remained independent after adjustment for N-terminal pro-brain natriuretic peptide, ejection fraction, stroke volume index, E/E′, and left ventricular mass index (hazard ratio, 4.1; 95% confidence interval, 1.3–13.1; P〈0.05). CONCLUSIONS—: There is a continuum of cardiac involvement in systemic AL and ATTR amyloidosis. Transmural LGE is determined reliably by PSIR and represents advanced cardiac amyloidosis. The PSIR technique provides incremental information on outcome even after adjustment for known prognostic factors.
    Keywords: Medicine ; Anatomy & Physiology;
    ISSN: 0009-7322
    E-ISSN: 15244539
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  • 3
    Language: English
    In: The International Journal of Artificial Organs, May 2012, Vol.35(5), pp.338-345
    Description: Introduction: Cardiovascular mortality remains high among peritoneal dialysis (PD) patients. Several small studies have suggested that PD patients are volume expanded, and as such this could be a cardiovascular risk factor. We therefore wished to investigate factors which could lead to extracellular water (ECW) expansion. Methods: Retrospective cross-sectional audit of 600 prevalent, adult PD patients attending two tertiary university PD centers, with corresponding assessments of PD adequacy, transport status, and multifrequency bioimpedance measurements of extracellular water to total body water (ECW/TBW). Results: 600 PD patients, median age 57.5 (46.9–67.9) years, 54% male, 31% diabetic, 47.6% Caucasoid, median PD vintage 16 (3.7–38) months, (64% prescribed icodextrin, 34% hypertonic glucose dialysates, and 74% antihypertensive medications). Mean ECW 15.1±0.2 L, ICW 20±0.2 L, ECW/TBW ratio 0.437±0.007. On multivariate analysis %ECW/TBW was associated with age (F=13.1 β=0.045 p=0.000), number of antihypertensive medications (F=10.3 β=0.43 p=0.001), log CRP (F=12.9 β=1.3 p=0.000), and negatively with serum albumin (F=25 β=-0.22 p=0.000), and residual renal function (urine volume mL F=9.96 β=-0.001 p=0.002) (weekly Kt/Vurine F=8.82 β=-2.05, p=0.003). Conclusions: Overhydration as assessed by ECW/TBW is prevalent in adult PD patients, and is associated with loss of residual renal function, inflammation, malnutrition and hypertension – as assessed by antihypertensive medications. As this was a retrospective cross-sectional audit, whether loss of residual renal function, inflammation, and protein energy wasting lead to volume expansion remains to be determined in prospective longitudinal studies.
    Keywords: Bio-Impedance ; Peritoneal Dialysis ; Residual Renal Function ; Icodextrin ; C-Reactive Protein ; Albumin ; Medicine
    ISSN: 0391-3988
    E-ISSN: 1724-6040
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  • 4
    In: Circulation, 2016, Vol.133(12), pp.e450-e451
    Description: We thank Aquaro, Cohen, and colleagues for their interest in our article.1 Cardiac involvement is a chief driver of prognosis in systemic amyloidosis, and the stratification of patients is essential for prognosis and choosing management strategies. Cardiovascular magnetic resonance with late gadolinium enhancement has good diagnostic accuracy for cardiac amyloidosis, but its prognostic impact was uncertain.2–6 This study confirms incremental prognostic information after adjusting for known prognosis factors. We note the comments of Aquaro and colleagues regarding cardiac biopsy, but point out that microscopic histological analyses of these tiny samples is not only open to sampling error, but, crucially, the presence of amyloid in heart muscle is not actually proof of cardiac amyloidosis. It is essential to make the distinction between the presence of amyloid deposits and the clinical syndromes of amyloidosis. Amyloid deposits occur widely throughout the tissues in patients with systemic amyloidosis, often without any clinical consequences, providing the basis for rectal, salivary gland, skin, and fat biopsies to support diagnosis. It is likely …
    Keywords: Medicine ; Anatomy & Physiology;
    ISSN: 0009-7322
    E-ISSN: 15244539
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  • 5
    Language: English
    In: Blood, 24 December 2015, Vol.126(26), pp.2805-10
    Description: Light chain deposition disease (LCDD) is characterized by the deposition of monotypic immunoglobulin light chains in the kidney, resulting in renal dysfunction. Fifty-three patients with biopsy-proven LCDD were prospectively followed at the UK National Amyloidosis Center. Median age at diagnosis was 56 years, and patients were followed for a median of 6.2 years (range, 1.1-14.0 years). Median renal survival from diagnosis by Kaplan-Meier analysis was 5.4 years, and median estimated patient survival was 14.0 years; 64% of patients were alive at censor. Sixty-two percent of patients required dialysis, and median survival from commencement of dialysis was 5.2 years. There was a strong association between hematologic response to chemotherapy and renal outcome, with a mean improvement in glomerular filtration rate (GFR) of 6.1 mL/min/year among those achieving a complete or very good partial hematologic response (VGPR) with chemotherapy, most of whom remained dialysis independent, compared with a mean GFR loss of 6.5 mL/min/year among those achieving only a partial or no hematologic response (P 〈 .009), most of whom developed end-stage renal disease (ESRD; P = .005). Seven patients received a renal transplant, and among those whose underlying clonal disorder was in sustained remission, there was no recurrence of LCDD up to 9.7 years later. This study highlights the need to diagnose and treat LCDD early and to target at least a hematologic VGPR with chemotherapy, even among patients with advanced renal dysfunction, to delay progression to ESRD and prevent recurrence of LCDD in the renal allografts of those who subsequently receive a kidney transplant.
    Keywords: Immunoglobulin Light Chains ; Kidney Failure, Chronic -- Etiology ; Paraproteinemias -- Pathology
    ISSN: 00064971
    E-ISSN: 1528-0020
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  • 6
    In: European Heart Journal, 2015, Vol. 36(18), pp.1098-1105
    Description: AIMS: AL amyloidosis may respond to chemotherapy but two-thirds of patients with severe cardiac involvement die within a year of diagnosis, purportedly from tachyarrhythmias or electromechanical dissociation. We sought to characterize the nature of cardiac arrhythmias in severe cardiac AL amyloidosis using implanted cardiac rhythm recorders.METHODS AND RESULTS: Implantable loop recorders (ILRs) were inserted within 24 h of baseline evaluation at the UK National Amyloidosis Centre, into 20 consecutive patients with newly diagnosed severe cardiac AL amyloidosis and symptoms of syncope or pre-syncope. Weekly ILR recordings and additional recordings at the time of symptoms were obtained. Median (range) follow-up from baseline was 308 (10-399) days. Thirteen patients died, and median survival in the whole cohort was 61 days from device insertion. In each of eight evaluable cases, death was heralded by bradycardia, usually associated with complete atrioventricular block (CAVB), followed shortly thereafter by pulseless electrical activity. Four patients received pacemakers, a median (range) of 7 (3-38) h after development of symptomatic CAVB, but these did not prevent rapid cardiac decompensation and death in three cases. Despite 272 loop recordings, there was only one episode of non-sustained ventricular tachycardia, which was preceded by severe bradycardia. Patients who died had significantly worse global left ventricular strain on echocardiography (P = 0.029) and reduced 6 min walk distance (P = 0.048) at baseline compared with survivors.CONCLUSIONS: The discovery that bradyarrhythmias heralded terminal cardiac decompensation in most patients with severe cardiac AL amyloidosis supports a study of prophylactic pacemaker insertion in this patient population.
    Keywords: Amyloidosis ; Cardiomyopathy ; Bradycardia ; Implantable Loop Recorder
    ISSN: 0195-668X
    E-ISSN: 1522-9645
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  • 7
    Language: English
    In: Circulation, Nov 25, 2014, Vol.130(22)
    Keywords: Electrocardiography – Usage ; Myocardial Diseases – Risk Factors ; Myocardial Diseases – Care and Treatment ; Heart Failure – Risk Factors ; Heart Failure – Care and Treatment ; Elderly – Health Aspects
    ISSN: 0009-7322
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  • 8
    In: Clinical Kidney Journal, 2013, Vol. 6(4), pp.433-435
    Description: IgG4-related tubulointerstitial nephritis is an uncommon cause of renal impairment. It has been associated with dysfunction in a number of other organs giving rise to the term IgG4-related systemic disease; organ involvement can occur metachronously, hence, making it more difficult to identify patients. The exact cause of this condition remains unknown. Here, we present a case of isolated renal involvement which demonstrates how particular biochemical, radiological and histopathological changes should raise the suspicion of IgG4-related nephropathy, especially when there is an absence of clues from any other organ.
    Keywords: Igg4 - Related Systemic Disease ; Isolated Renal Involvement ; Tubulointerstitial Nephritis
    ISSN: 2048-8505
    E-ISSN: 2048-8513
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  • 9
    Language: English
    In: Blood Purification, October 2011, Vol.32(3), pp.226-231
    Description: Background and Objectives: Residual renal function (RRF) is important for peritoneal dialysis (PD) technique failure and patient survival. Reduction in extracellular water (ECW) has been reported to reduce urine output. We audited volume status and RRF to determine whether ECW was linked to urinary output. Methods: We retrospectively audited PD patients who had RRF and multiple frequency bioimpedance spectroscopy assessments. Results: 550 patients were studied. Urine output was lower in males (p = 0.021), non-Caucasoids (p = 0.04), those prescribed antihypertensives (p 〈 0.001), and greater glucose dialysate usage (p 〈 0.001). Urine output was negatively associated with dialysis vintage (F = 40.7, β –0.627, p 〈 0.001), and ECW (F = 33.7, β –0.177, p 〈 0.001) and positively associated with intracellular volume (F = 34.6, β 0.11, p 〈 0.001). Conclusions: RRF is important for successful PD, and ECW volume expansion may lead to better preservation of RRF in PD patients compared to haemodialysis. However, in our retrospective cross-sectional study, ECW expansion was associated with reduced urine output.
    Keywords: Original Paper ; Residual Renal Function ; Icodextrin ; Bioimpedance ; Peritoneal Dialysis ; Medicine
    ISSN: 0253-5068
    E-ISSN: 1421-9735
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  • 10
    Language: English
    In: Clinical journal of the American Society of Nephrology : CJASN, October 2011, Vol.6(10), pp.2492-8
    Description: Peritoneal dialysis adequacy is typically assessed by urea clearance corrected for total body water (TBW) on the basis of anthropomorphic equations, which do not readily take into account changes in body composition, which may vary between ethnic groups. To determine whether ethnicity could affect estimates of peritoneal dialysis adequacy, we compared TBW estimated by anthropomorphic equations and that measured by multifrequency bioimpedance spectroscopy. We calculated TBW in 600 healthy adult peritoneal dialysis outpatient attending two tertiary university hospitals serving an inner-city multiethnic population who had TBW measured by multifrequency bioimpedance spectroscopy performed. 600 adult peritoneal dialysis patients were studied: mean age, 56.7 ± 0.6 years; 54.2% men; 29.7% diabetic; mean body mass index, 26.1 ± 0.2; 47.3% Caucasian; 29.2% South Asian; 12.8% African/Afro-Caribbean. Total body water was calculated using several anthropomorphic equations and was higher than that calculated MEASURED BY MF-BIS for all ethnic groups, apart from African/Afro-Caribbeans, with the greatest difference between Watson calculated TBW and multifrequency bioelectrical impedance spectroscopy 12.3 ± 0.6% for the South Asians, 9.0 ± 2.6% for Far Eastern Asians, 2.8 ± 0.6% Caucasians, and -0.2 ± 1.5% for African/Afro-Caribbeans. In this United Kingdom-based multiethnic population, body composition differed particularly for the South Asian patients compared with Caucasians and African/Afro-Caribbeans. Overestimation of TBW by anthropomorphic-based equations would lead to a lower calculation of Kt/V(urea), which may lead to changes in peritoneal dialysis prescription to achieve clinical standard targets and also affect studies examining the relationship between Kt/V and survival.
    Keywords: Body Composition ; Continental Population Groups ; Peritoneal Dialysis ; Body Water -- Metabolism
    ISSN: 15559041
    E-ISSN: 1555-905X
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