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Primary amyloidosis

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https://www.readbyqxmd.com/read/29770800/current-and-future-circulating-biomarkers-for-cardiac-amyloidosis
#1
REVIEW
Marco Luciani, Luca Troncone, Federica Del Monte
Cardiac amyloidosis (CA) comprises a heterogeneous group of medical conditions affecting the myocardium. It presents with proteinaceous infiltration with variable degrees of severity, prevalence and evolution. Despite this heterogeneity, erroneous protein folding is the common pathophysiologic process, yielding the formation of a single misfolded protein (monomer) that progressively evolves and ultimately forms amyloid fibers. Additionally, by seeding out from the organs of origin, intermediates called oligomers metastasize and restart the process...
May 17, 2018: Acta Pharmacologica Sinica
https://www.readbyqxmd.com/read/29756110/agenesis-of-the-isthmus-of-the-thyroid-gland
#2
Uğur Kesici, Sevgi Kesici
The thyroid is an endocrine gland composed of two lobes connected by the isthmus tissue. Thyroid isthmus agenesis is a rare condition, and only a few cases have been reported in the literature. Here, we discuss the case of a 56-year-old female patient in whom agenesis of the thyroid isthmus was discovered incidentally during surgery for a multinodular goitre. When agenesis of the isthmus is found, associated thyroid lobe agenesis and the presence of ectopic thyroid tissue must be considered. In addition, associated autoimmune thyroid nodule, thyroiditis, primary thyroid carcinoma, metastasis, and amyloidosis must be considered in the differential diagnosis...
2018: Turkish Journal of Surgery
https://www.readbyqxmd.com/read/29729178/-primary-renal-amyloidosis-feautures-of-disease-course-and-the-possibilities-of-in-time-diagnosis-clinical-case-report
#3
Ivan P Katerenchuk, Lidia A Tkachenko, Tetiana Yarmola, Oleksandr I Katerenchuk
The article presents a clinical case of primary renal amyloidosis that was diagnosed in female patient. Typically, the spectrum of clinical signs of primary amyloidosis is wide: from moderate symptoms in some patients having localized amyloidosis, to life-threatening conditions that require rapid diagnosis determination and aggressive treatment. Finding a diagnosis of primary renal amyloidosis is enough difficult as for family doctors as for well-skilled nephrologists. In some cases, the primary renal amyloidosis starts with atypical symptoms, and so, only doctor's sufficient clinical experience combined with the in-time usage of highly informative diagnostic methods (morphological and immunological evaluation of the renal biopsy), allows to make an in-time diagnosis of the disease and to prescribe specific therapy that can slow down the disease progression and delay the time of severe complications development...
2018: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/29719808/primary-endobronchial-amyloidosis-a-rare-case-of-endobronchial-tumor
#4
Amos Lal, Jamal Akhtar, Mohammad Saud Khan, Yayan Chen, Yaron Goldman
Primary localized endobronchial amyloidosis is a rare entity, as pulmonary amyloidosis most commonly occurs as a part of systemic AL amyloidosis. It can be asymptomatic or can present with nonspecific symptoms such as progressive dyspnea, cough, wheezing and rarely respiratory failure. It is frequently misdiagnosed as asthma, COPD or pneumonia. Solitary endobronchial amyloidosis having a nodular appearance can mimic endobronchial tumor. The diagnosis is usually delayed by 8-37 months. The average life expectancy for primary tracheobronchial amyloidosis is approximately 9 years, so the early diagnosis of this disease is very critical to improve the prognosis of patients...
2018: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/29719798/an-unusual-case-of-hoarseness-of-voice
#5
Ranjit Singh, Tiyas Sen, Sanjeev Pattari
Amyloidosis is a condition wherein there is an over-expression of specific proteins culminating in the extracellular deposition of insoluble beta pleated sheets of fibres. These deposits disrupt function of the target organ. Its aetiology remains unknown. Primary Amyloidosis localized to the trachea and bronchus is a rare entity. It produces tumour like lesion in the tracheo-bronchial tree. We hereby discuss a patient who presented with persistent hoarseness of voice who was referred to a tertiary care centre to rule out malignancy...
2018: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/29707074/hepatobiliary-manifestations-and-complications-in-inflammatory-bowel-disease-a-review
#6
REVIEW
Fotios S Fousekis, Vasileios I Theopistos, Konstantinos H Katsanos, Epameinondas V Tsianos, Dimitrios K Christodoulou
Liver and biliary track diseases are common extraintestinal manifestations of inflammatory bowel disease (IBD), reported both in Crohn's disease and ulcerative colitis, and may occur at any time during the natural course of the disease. Their etiology is mainly related to pathophysiological changes induced by IBD, and secondary, due to drugs used in IBD. Fatty liver is considered as the most frequent hepatobiliary manifestation in IBD, while primary sclerosing cholangitis (PSC) is the most correlated hepatobiliary disorder and is more prevalent in patients with ulcerative colitis...
April 2018: Gastroenterology Research
https://www.readbyqxmd.com/read/29692592/revisiting-renal-amyloidosis-with-clinicopathological-characteristics-grading-and-scoring-a-single-institutional-experience
#7
Abhiram Kalle, Archana Gudipati, Sree Bhushan Raju, Karthik Kalidindi, Swarnalatha Guditi, Gangadhar Taduri, Megha S Uppin
INTRODUCTION: Kidney involvement is a major cause of mortality in systemic amyloidosis. Glomerulus is the most common site of deposition in renal amyloidosis, and nephrotic syndrome is the most common presentation. Distinction between AA and AL is done using immunofluorescence (IF) and immunohistochemistry (IHC). Renal biopsy helps in diagnosis and also predicting the clinical course by applying scoring and grading to the biopsy findings. MATERIALS AND METHODS: The study includes all cases of biopsy-proven renal amyloidosis from January 2008 to May 2017...
April 2018: Journal of Laboratory Physicians
https://www.readbyqxmd.com/read/29630157/an-intensely-pruritic-pebbled-presentation
#8
Livia Francine Soriano, Conn McGrath, Mark Hawthorne, Arucha Linda Ekeowa-Anderson
Lichen amyloidosis (LA) is a form of primary localized cutaneous amyloidosis (PLCA) characterized by bilateral intensely itchy domed scaly hyperkeratotic papules. Lichen amyloidosis is rare and affects men more than women. It is uncommonly seen in the western world but more prevalent in Asia. These papules most typically affect the shins and occasionally the arms and torso. Lichen amyloidosis has been reported in association with autoimmune disorders and after prolonged exfoliation and friction of affected skin...
March 7, 2018: Dermatology Online Journal
https://www.readbyqxmd.com/read/29622069/-neurogenic-autonomic-dysfunction-in-primary-amyliodosis
#9
Astrid Juhl Terkelsen, John Hansen, Anja Klostergaard, Marit Otto, Henning Mølgaard, Christian Lodberg Hvas, Klaus Krogh, Hans Jørgen Kirkeby, Line Bille Madsen, Henning Andersen, Troels Staehelin Jensen
Neurogenic autonomic dysfunction (NAD) and polyneuropathy occur in common conditions like diabetes and alcoholism. However, it can also be seen in rare diseases like in this case report of amyloid light-chain amyloidosis: primary amyloidosis. A 56-year-old man presented with polyneuropathy, a sympathetic dysfunction causing orthostatic intolerance, syncope, parasympathetic dysfunction and involvement of the enteric nervous system. The report illustrates, that routine screening can be insufficient in diagnosing amyloidosis...
April 2, 2018: Ugeskrift for Laeger
https://www.readbyqxmd.com/read/29615179/pulmonary-nodular-amyloidosis-mimicking-metastatic-lung-lesion-in-a-patient-with-colon-cancer
#10
Waseem M Hajjar, Tariq E Al-Johani, Mohammed A Jameel, Othman T Almutairi, Iftikhar Ahmed, Adnan W Hajjar, Sami A Al-Nassar
Amyloidosis is defined as abnormal aggregation of amyloid proteins. Amyloidosis can be localised or systemic affecting the lung, heart, spleen, liver and kidneys. The most common form of pulmonary amyloidosis is the nodular type. The disease is idiopathic and linked to many systemic diseases. The clinical manifestations and prognosis of respiratory tract amyloidosis depends on its etiology and anatomical location. Radiologically, the nodular pulmonary amyloidosis may appear as single or multiple nodules in any lobe, and, therefore, may mimic as primary pulmonary or metastatic neoplasms...
April 2018: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/29607206/primary-lung-carcinoma-combined-with-pulmonary-amyloidosis-secondary-to-syphilis-infection
#11
Luo Zhao, Xiaohui Xu, Ye Zhang, Shanqing Li, Xiaohua Shi, Yushang Cui
A 55-year-old female patient was found to have a pulmonary nodule combined with multiple lung cysts detected on CT scan. Video-assisted thoracoscopic surgery (VATS) lobectomy was performed and the nodule showed adenocarcinoma while the whole left upper lobe showed a heavy deposition of amyloid. Syphilis infection was detected and was suspected contributing to secondary pulmonary amyloidosis. Although very rare, pulmonary amyloidosis should be added to the differential diagnosis for solid pulmonary nodules. Furthermore, widespread lung cysts located apart from pulmonary nodules is especially rare in pulmonary amyloidosis secondary to syphilis infection...
February 2018: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29594138/changes-in-the-spectrum-of-kidney-diseases-an-analysis-of-40-759-biopsy-proven-cases-from-2003-to-2014-in-china
#12
Jin-Hua Hou, Hui-Xian Zhu, Min-Lin Zhou, Wei-Bo Le, Cai-Hong Zeng, Shao-Shan Liang, Feng Xu, Dan-Dan Liang, Si-Jia Shao, Ye Liu, Zhi-Hong Liu
Objectives: To evaluate the changing spectrum of kidney diseases over time in China using renal biopsy-proven cases. Methods: All patients over the age of 14 years who were diagnosed with a kidney disease by renal biopsy in the Renal Biopsy Registry of the National Clinical Research Center of Kidney Diseases in Jinling Hospital, Nanjing, from 2003 to 2014 were included. Results: In total, 40,759 cases of renal biopsy were analyzed. The mean age of the patients was 36...
February 2018: Kidney Diseases
https://www.readbyqxmd.com/read/29589647/primary-systemic-amyloidosis-with-skin-and-cardiac-involvement-a-case-report
#13
Anja Trajber Horvat, Katarina Trčko, Vesna Jurčić, Pij Bogomir Marko
Primary systemic amyloidosis is characterized by the deposition of insoluble monoclonal immunoglobulin light chains in various tissues and is usually associated with an underlying plasma cell dyscrasia. In the early stage of the disease, dermatological findings can be the only manifestation, as opposed to organ involvement in the later stages. A dermatologist can diagnose amyloidosis early with a skin biopsy stained with Congo red dye and other appropriate investigations. This case report describes a female patient with primary systemic amyloidosis confirmed histologically from a skin biopsy...
March 2018: Acta Dermatovenerologica Alpina, Panonica, et Adriatica
https://www.readbyqxmd.com/read/29582184/small-bowel-amyloidosis
#14
REVIEW
Raghav Bansal, Umer Syed, Jacob Walfish, Joshua Aron, Aaron Walfish
PURPOSE OF REVIEW: The goal of this paper is to review the literature on small bowel amyloidosis. Our review focuses on the underlying etiology, histopathology, clinical features, endoscopic and radiologic findings, and the mainstay of management. RECENT FINDINGS: The latest research shows changing epidemiological trends of different types of amyloidosis. It also reveals a better understanding of its pathophysiology and shows improvement in treatment outcomes. Amyloidosis is a group of diseases of multiple etiologies and clinical presentations...
March 26, 2018: Current Gastroenterology Reports
https://www.readbyqxmd.com/read/29552568/response-of-bortezomib-chemotherapy-in-hepatic-amyloidosis
#15
Syed M Hasan, Nida N Ahmed, Zunirah Ahmed, Allan Seibert
Amyloidosis is a rare disorder with a wide spectrum of presentations and anomalies. It is subdivided into 2 broad categories based on protein deposition; primary and secondary amyloidosis. It can present as a single-organ involvement or as a diffuse infiltrative multi-organ process. Isolated hepatic amyloidosis presentation is a rare phenomenon that develops due to insoluble amyloid deposition in liver. Its clinical presentation is usually vague and ranges from mild hepatomegaly with elevated liver enzymes to acute liver failure and hepatic rupture...
January 2018: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/29552246/amyloidosis-of-the-renal-pelvis-presenting-as-flank-pain
#16
Rachel Shikhman, Jarrod Curry, Roland Gazaille
Amyloidosis is a rare disease defined by accumulation of extracellular amyloid systemically or within a specific organ. Localized amyloidosis of the genitourinary system is extremely rare, with the predominate location being the bladder. The imaging findings are often nonspecific and mimic urothelial carcinoma. We present a 49-year-old woman with a chief complaint of flank pain. A filling defect was discovered on radiological imaging. The defect was subsequently biopsied and proven to be a primary amyloidosis of the renal pelvis...
February 2018: Radiology Case Reports
https://www.readbyqxmd.com/read/29547468/primary-eyelid-amyloidosis-presenting-as-a-calcified-plaque-a-rare-presentation
#17
Swathi Kaliki, Ashi Morawala, Swarnalata Gowrishankar
No abstract text is available yet for this article.
March 14, 2018: Ophthalmic Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/29541594/primary-amyloidosis-of-the-bladder
#18
Adrian Ho, Melissa C Davies, Raluca Guran, James Brewin
No abstract text is available yet for this article.
March 2018: Urology Case Reports
https://www.readbyqxmd.com/read/29534248/truncation-of-adamts13-by-plasmin-enhances-its-activity-in-plasma
#19
Chantal C Clark, Mirjam M Mebius, Steven de Maat, Aloysius G M Tielens, Philip G de Groot, Rolf T Urbanus, Rob Fijnheer, Bouke P C Hazenberg, Jaap J van Hellemond, Coen Maas
ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) cleaves von Willebrand Factor (VWF) multimers to control their thrombogenicity. The fibrinolytic enzyme plasmin can cleave VWF in a similar manner. However, plasmin can also cleave ADAMTS13, which ultimately inactivates it. This leaves the overall role of plasmin in primary haemostasis uncertain.We investigated the combined molecular effects of plasmin on VWF and ADAMTS13. We first identified that plasmin destroys FRETS-VWF73 substrate by cleaving the ADAMTS13 binding region in a buffered system...
March 2018: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/29519356/spectrum-of-restrictive-and-infiltrative-cardiomyopathies-part-2-of-a-2-part-series
#20
REVIEW
Naveen L Pereira, Martha Grogan, G William Dec
Restrictive cardiomyopathies are the least common form of heart muscle disease. They are characterized as infiltrative and noninfiltrative, storage diseases, and endomyocardial disorders. Genetic diseases commonly present during childhood or adolescence. However, a growing percentage of elderly patients with heart failure with preserved ejection fraction are being recognized as having forms of restrictive cardiomyopathy, particularly cardiac amyloidosis. Noninvasive evaluation has replaced endomyocardial biopsy in the diagnostic evaluation of most suspected etiologies...
March 13, 2018: Journal of the American College of Cardiology
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