Amyloidosis of the Colon and Clinical Features of Intestinal Obstruction

Publication date: May 2018Source: Cirugía Española (English Edition), Volume 96, Issue 5Author(s): Xavier Pérez, Elena Ramírez-Maldonado, Carmen Martín, Víctor Fumanal, Iván Urra
Source: Cirugia Espanola - Category: Surgery Source Type: research

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Conclusions99mTc-DPD scan detects cardiac involvement in subjects with TTR gene mutation earlier than ECG, echocardiography and biochemical markers, occurring some years before the fulfillment of current diagnostic criteria for cardiac amyloidosis. A positive99mTc-DPD scan predicts cardiac symptoms onset.
Source: Journal of Nuclear Cardiology - Category: Nuclear Medicine Source Type: research
We studied the response of mast cells in mouse liver to experimental amyloidosis modeled by administration of aqueous solution of soy cream substitute and its correction by spontaneous dry red wine intakeper os. Mast cells in mice with amyloidosis and intact mice were presented by α-ortho- and β1-metachromatic non-degranulating forms. In mice with amyloidosis, the relative content of β1-metachromatic mast cells in the capsule and parenchyma of the liver was by 1.8 and 1.5 times higher than in intact animals, respectively. In mice with amyloidosis receiving dry red wine, the proportions of mast cells with di...
Source: Bulletin of Experimental Biology and Medicine - Category: Biology Source Type: research
In this issue of the International Journal of Cardiology, Dr. Maurizi and colleagues report on the higher than expected prevalence of cardiac amyloidosis in patients undergoing evaluation for hypertrophic cardiomyopathy (HCM) in a tertiary referral center [1]. This carefully performed analysis highlights the clinical overlap of cardiac amyloidosis in a substantial percentage (a total of 9%) of patients who display a phenotype of hypertrophic cardiomyopathy and emphasizes the need to consider cardiac amyloidosis in any patient with ventricular hypertrophy without obvious inciting factor or known hypertrophic mutation.
Source: International Journal of Cardiology - Category: Cardiology Authors: Tags: Editorial Source Type: research
Transthyretin amyloid deposition is present in 17% of autopsies of patients with heart failure and a preserved ejection fraction (HFpEF). 99mTechnetium-pyrophosphate scintigraphy (99mTc-PYP) is sensitive and specific to diagnose cardiac transthyretin amyloid deposition (ATTR). The prevalence of ATTR by 99mTc-PYP was evaluated along with echocardiographic parameters in patients with HFpEF. One-hundred consecutive patients with HFpEF, who had 99mTc-PYP, were retrospectively evaluated. Echocardiographic variables were analyzed to compare patients with positive versus negative ATTR infiltration. Myocardial ATTR was present in ...
Source: Critical Pathways in Cardiology - Category: Cardiology Tags: Original Articles Source Type: research
Abstract Transthyretin amyloidosis (ATTR) is a progressive life-threatening disease characterized by the deposition of transthyretin (TTR) amyloid fibrils. Several pathogenic variants have been shown to destabilize TTR tetramers, leading to aggregation of misfolded TTR fibrils. However, factors that underlie the differential age of disease onset amongst amyloidogenic TTR variants remain elusive. Here, we examined the biological properties of various TTR mutations and found that the cellular secretory pattern of the wild-type (WT) TTR was similar to those of the late-onset mutant (Ala97Ser, p. Ala117Ser), stable mu...
Source: Cellular and Molecular Life Sciences : CMLS - Category: Cytology Authors: Tags: Cell Mol Life Sci Source Type: research
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Source: Amyloid: The Journal of Protein Folding Disorders - Category: Biochemistry Authors: Source Type: research
We present a case of primary amyloidosis of the colon in a patient affected by polymyositis who underwent Hartmann ’s procedure after a spontaneous colonic perforation. After 2 months of well-being, the patient underwent two consecutive surgical procedures for stenosis of the ostomy orifice.Areas CoveredA review of the literature has been performed, gathering case reports highlighting the distribution of this disease by age, gender, location, and treatment when available.Expert CommentaryGastrointestinal amyloid disease is a rare condition, and it could be considered among the rare causes of intestinal perforati...
Source: Digestive Diseases and Sciences - Category: Gastroenterology Source Type: research
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Source: Amyloid: The Journal of Protein Folding Disorders - Category: Biochemistry Authors: Source Type: research
Conditions:   Hemodialysis-Associated Amyloidosis;   Dialysis Amyloidosis;   Quality of Life Interventions:   Device: Theranova 400;   Device: Elisio-19H Sponsor:   Selayang Hospital Not yet recruiting
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
Modern Pathology, Published online: 13 November 2019; doi:10.1038/s41379-019-0407-5Amyloidosis in surgically resected atrial appendages: a study of 345 consecutive cases with clinical implications
Source: Modern Pathology - Category: Pathology Authors: Source Type: research
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