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Primary amyloidosis

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https://www.readbyqxmd.com/read/28935176/current-insight-in-the-localized-insulin-derived-amyloidosis-lida-clinico-pathological-characteristics-and-differential-diagnosis
#1
REVIEW
Amir Mehdi Ansari, Lais Osmani, Aerielle E Matsangos, Qing K Li
BACKGROUND: In diabetic patients, subcutaneous insulin injection may cause several types of injection site-related lesions, such as lipoatrophy, insulin-induced cutaneous lipohypertrophy (IICL), allergic reaction, and iatrogenic localized insulin-derived amyloidosis (LIDA). Among these complications, both IICL and LIDA present as tumor-like and slow growing lesions; and they may be confused with one another. The clinical implication and management of IICL and LIDA are different. LIDA causes poor blood glycemic controls due to inadequate absorption of the insulin...
September 1, 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28912854/comparative-proteomics-analysis-of-primary-cutaneous-amyloidosis
#2
Daxing Cai, Yang Li, Chunlei Zhou, Yulin Jiang, Jian Jiao, Lin Wu
Primary cutaneous amyloidosis (PCA) is a localized skin disorder that is characterized by the abnormal deposition of amyloid in the extracellular matrix (ECM) of the dermis. The pathogenesis of PCA is poorly understood. The objective of the present study was to survey proteome changes in PCA lesions in order to gain insight into the molecular basis and pathogenesis of PCA. Total protein from PCA lesions and normal skin tissue samples were extracted and analyzed using the isobaric tags for relative and absolute quantitation technique...
October 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28910817/the-lung-in-dysregulated-states-of-humoral-immunity
#3
Yurdagül Uzunhan, Florence Jeny, Marianne Kambouchner, Morgane Didier, Diane Bouvry, Hilario Nunes, Jean-François Bernaudin, Dominique Valeyre
In common variable immunodeficiency, lung manifestations are related to different mechanisms: recurrent pneumonias due to encapsulated bacteria responsible for diffuse bronchiectasis, diffuse infiltrative pneumonia with various patterns, and lymphomas, mostly B cell extranodal non-Hodgkin type. The diagnosis relies on significant serum Ig deficiency and the exclusion of any primary or secondary cause. Histopathology may be needed. Immunoglobulin (IgG) replacement is crucial to prevent infections and bronchiectasis...
September 15, 2017: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/28903570/czech-registry-of-monoclonal-gammopathies-technical-solution-data-collection-and-visualisation
#4
L Brozova, D Schwarz, I Snabl, J Kalina, B Pavlickova, M Komenda, J Jarkovský, P Němec, D Horinek, Z Stefanikova, L Pour, R Hájek, V Maisnar
BACKGROUND: The Registry of Monoclonal Gammopathies (RMG) was established by the Czech Myeloma Group in 2007. RMG is a registry designed for the collection of clinical data concerning diagnosis, treatment, treatment results and survival of patients with monoclonal gammopathies. Data on patients with monoclonal gammopathy of undetermined significance (MGUS), Waldenström macroglobulinaemia (WM), multiple myeloma (MM) or primary AL ("amyloid light-chain") amyloidosis are collected in the registry...
2017: Klinická Onkologie: Casopis Ceské a Slovenské Onkologické Spolecnosti
https://www.readbyqxmd.com/read/28902953/dystrophic-calcification-in-a-patient-with-primary-localized-cutaneous-nodular-amyloidosis-an-uncommon-ultrasound-finding
#5
Sergi Planas Ciudad, Esther Roé Crespo, Juan Francisco Mir Bonafé, Fania Zamantta Muñoz-Garza, Luis Puig Sanz
No abstract text is available yet for this article.
September 13, 2017: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/28894337/primary-tracheobronchial-amyloidosis
#6
Pedro Paulo Teixeira E Silva Torres, Matheus Rabahi, Sebastião Alves Pinto, Karla Cristina de Morais Arantes Curado, Marcelo Fouad Rabahi
No abstract text is available yet for this article.
July 2017: Radiologia Brasileira
https://www.readbyqxmd.com/read/28893208/trial-design-and-rationale-for-apollo-a-phase-3-placebo-controlled-study-of-patisiran-in-patients-with-hereditary-attr-amyloidosis-with-polyneuropathy
#7
David Adams, Ole B Suhr, Peter J Dyck, William J Litchy, Raina G Leahy, Jihong Chen, Jared Gollob, Teresa Coelho
BACKGROUND: Patisiran is an investigational RNA interference (RNAi) therapeutic in development for the treatment of hereditary ATTR (hATTR) amyloidosis, a progressive disease associated with significant disability, morbidity, and mortality. METHODS: Here we describe the rationale and design of the Phase 3 APOLLO study, a randomized, double-blind, placebo-controlled, global study to evaluate the efficacy and safety of patisiran in patients with hATTR amyloidosis with polyneuropathy...
September 11, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28892914/primary-amyloidosis-in-a-case-with-normal-plasma-cell-counts
#8
Pooja Trehan, Mangala Gouri, Clement Wilfred, K Rashmi, Vijaya Mysorekar
Amyloidosis is a group of disease that is characterized by the deposition of extracellular abnormal proteinaceous material (amyloid), in various organs. Amyloidosis involving the liver is common and the radiological findings are often nonspecific. We present the case of a 40-year-old female who presented with abdominal pain. Ultrasound abdomen was reported as massive hepatomegaly with diffuse liver parenchymal disease. Bone marrow aspiration showed normomegaloblastic erythroid hyperplasia and plasma cells were within normal limits (5%)...
July 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28885307/the-treatment-of-paraprotein-related-kidney-disease
#9
Colin A Hutchison, Fei Xiong, Peter Mollee
PURPOSE OF REVIEW: Myeloma kidney and amyloid light-chain (AL) amyloidosis remain the principal kidney complications of paraproteins. In this review, we update readers to many of the recent advances which have occurred in the care and outcomes for patients with these presentations. RECENT FINDINGS: Myeloma kidney has historically caused a severe acute kidney injury with very poor outcomes. The combination of new diagnostic techniques, enabling a rapid diagnosis and novel chemotherapy agents has transformed these poor outcomes for the better...
September 6, 2017: Current Opinion in Nephrology and Hypertension
https://www.readbyqxmd.com/read/28877738/sclerosing-thymoma-like-thymic-amyloidoma-with-nephrotic-syndrome-a-case-report
#10
Yuto Kato, Miyuki Okuda, Koji Fukuda, Nobuya Tanaka, Akihiko Yoshizawa, Yoshinori Saika, Yoshisumi Haruna, Shouji Kitaguchi, Ryuji Nohara
BACKGROUND: Primary localized amyloidosis presenting as an isolated mediastinal mass is extremely rare, especially in the thymus. Sclerosing thymoma is also an extremely rare anterior mediastinal tumor, pathologically characterized by extensive sclerotic lesions with hyalinization and calcification. Only 14 cases of sclerosing thymoma and five cases of thymic amyloidosis have been reported to date. CASE PRESENTATION: A 78-year-old Japanese woman was diagnosed as having sclerosing thymoma (Masaoka stage IVa pericardial dissemination)-like thymic amyloidoma...
September 7, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28877709/prevalence-of-amyloidosis-in-korea
#11
Su Ra Seo, Shin Yi Jang, Ga Yeon Lee, Bareun Choi, Heeran Chun, Eun Jeong Cho, Sung-Il Cho
BACKGROUND: The aim of this study was to assess in amyloidosis prevalence in Korea between 2006 and 2015. METHODS: Primary diagnoses related to amyloidosis, regardless of subtype, were collected from the Korean National Health Insurance Service from 2006 through 2015. RESULTS: Overall, the age-standardized prevalence of amyloidosis was 0.93 (95% confidence interval (CI) 0.81, 1.04) persons per 100,000 persons in 2006 and 1.91 (95% CI 1.78, 2...
September 6, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28861330/a-plasma-cell-dyscrasia-presenting-as-amyloid-cardiomyopathy-and-autonomic-dysfunction-in-a-healthy-patient
#12
Rabih Tabet, Julie Zaidan, Boutros Karam, Samer Saouma, Foad Ghavami
Systemic amyloidosis is a rare multisystem disease caused by incorrectly folded proteins that deposit pathologically in different tissues and organs of the human body. It has a very wide spectrum of clinical presentations according to the affected organ(s), and its diagnosis is commonly delayed. Cardiac involvement is the leading cause of morbidity and mortality and carries a poor prognosis, especially in primary light chain amyloidosis. Therefore any delay in the diagnosis can result in devastating outcomes for the patient...
June 29, 2017: Curēus
https://www.readbyqxmd.com/read/28832315/amyloidosis-and-acute-hemorrhage-of-the-kidney-ureter-and-bladder
#13
Dany N Hanna, Jason A Levy, Jonah S Marshall
Gross hematuria is a common occurrence in adults. The differential diagnosis is extensive, including: malignancy, trauma, inflammation of the urinary tract, and stones. While, urinary tract amyloidosis represents only a small percentage of causative gross hematuria, it is concerning because of its superficial resemblance to malignant processes. We report the case of an 82-year-old male with concurrent primary amyloidosis of the kidney, ureter and bladder in the setting of acute hemorrhage. Histopathological examination of several biopsied samples confirmed our diagnosis...
August 2017: Canadian Journal of Urology
https://www.readbyqxmd.com/read/28808991/light-chain-al-amyloidosis-the-journey-to-diagnosis
#14
Kristen L McCausland, Michelle K White, Spencer D Guthrie, Tiffany Quock, Muriel Finkel, Isabelle Lousada, Martha S Bayliss
BACKGROUND: Light chain (AL) amyloidosis is a rare, complex disease associated with significant morbidity and mortality. Delays in diagnosis are common and may have detrimental consequences on patients' prognosis. Too little is known regarding the patient journey to diagnosis. OBJECTIVE: The objective of this study was to describe the patient-reported journey to a correct diagnosis for AL amyloidosis. METHODS: Using a mixed-methods approach, data were collected from clinician (n = 4) and patient (n = 10) interviews and a survey of community-based patients with AL amyloidosis (n = 341)...
August 14, 2017: Patient
https://www.readbyqxmd.com/read/28808514/nodular-pulmonary-amyloidosis-with-primary-pulmonary-malt-lymphoma-masquerading-as-metastatic-lung-disease
#15
Sunil Upadhaya, Mohd Baig, Basim Towfiq, Samer Al Hadidi
Nodular pulmonary amyloidosis is a very rare form of localized amyloidosis involving the lung, with very little known about its nature. It is usually associated with indolent B cell lymphoproliferative disorder and also connective tissue disorders. No definite treatment guideline exists. Many patients respond to chemotherapy with low risk of progression and a 'wait and watch' strategy is also considered a valid treatment option. In this report the authors present a case of nodular pulmonary amyloidosis with pulmonary mucosa associated lymphoid tissue (MALT) lymphoma that presented with features of metastatic malignant disease and after definitive diagnosis decided not to undergo treatment...
July 2017: Journal of Community Hospital Internal Medicine Perspectives
https://www.readbyqxmd.com/read/28798722/autonomic-neuropathy-and-albuminocytologic-dissociation-in-cerebrospinal-fluid-as-the-presenting-features-of-primary-amyloidosis-a-case-report
#16
Jingjing Li, Yi Li, Hongbing Chen, Shihui Xing, Huiyu Feng, Dawei Liu, Dilong Wang, Jinsheng Zeng, Yuhua Fan
OBJECTIVE: Primary amyloidosis is a disease with a poor prognosis and multi-organ involvement. Here, we report the clinical and pathological features of a patient with primary amyloidosis featuring autonomic neuropathy as the initial symptom and albuminocytologic dissociation in the cerebrospinal fluid (CSF). METHODS: The patient was a 60-year-old Chinese male with numbness, orthostatic hypotension, and gastrointestinal symptoms. For diagnosis, we performed an electromyogram (EMG), lumbar puncture, Bence Jones protein urine test, serum electrophoresis blood test, sural nerve and rectal membrane biopsies, transthyretin (TTR) gene sequencing, and bone marrow puncture...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28774673/can-videolaryngoscopy-be-a-first-option-in-a-patient-with-laryngeal-amyloidosis
#17
L España Fuente, G Mella Pérez, B Laserna Cocina, J L González González
Amyloidosis is a term that involves a group of diseases characterised by deposition of extracellular monoclonal light-chain fibrillar immunoglobulin aggregates in the body, including many organs, with the larynx among them. A case is presented of a 78 year-old man who was referred to our institution for strangulated umbilical hernia treatment. He suffered from progressive hoarseness and dysphagia for 5months. He had a history of primary laryngeal amyloidosis. Awake intubation was performed successful with the King Vision(®) video-laryngoscopy...
July 31, 2017: Revista Española de Anestesiología y Reanimación
https://www.readbyqxmd.com/read/28772045/translational-registry-for-cardiomyopathies-torch-rationale-and-first-results
#18
Claudia Seyler, Benjamin Meder, Tanja Weis, Thea Schwaneberg, Kerstin Weitmann, Wolfgang Hoffmann, Hugo A Katus, Andreas Dösch
AIMS: Non-ischemic cardiomyopathies (CMPs) comprise heart muscle disorders of different causes with high variability in disease phenotypes and clinical progression. The lack of national structures for the efficient recruitment, clinical and molecular classification, and follow-up of patients with non-ischemic CMPs limit the thorough analysis of disease mechanisms and the evaluation of novel diagnostic and therapeutic strategies. This paper describes a national, prospective, multicenter registry for patients with non-ischemic CMPs...
August 2017: ESC Heart Failure
https://www.readbyqxmd.com/read/28763127/european-veterinary-renal-pathology-service-a-survey-over-a-7-year-period-2008-2015
#19
L Aresu, V Martini, S L Benali, C Brovida, R E Cianciolo, R Dalla Riva, D Trez, J J Van Der Lugt, A Van Dongen, E Zini
BACKGROUND: The European Veterinary Renal Pathology Service (EVRPS) is the first Web-based registry for canine renal biopsy specimens in Europe. HYPOTHESIS/OBJECTIVES: The aim was to verify whether differences exist between the clinical and laboratory presentation of dogs with nephropathy according to renal pathological findings, as defined by light and electron microscopy of renal biopsy specimens submitted to EVRPS. ANIMALS: Renal biopsy specimens of dogs were collected from the archive of the service (n = 254)...
September 2017: Journal of Veterinary Internal Medicine
https://www.readbyqxmd.com/read/28761861/primary-cutaneous-nodular-amyloidosis-a-rare-disease
#20
Debajyoti Chatterjee, Anshul Radotra, Sendhil M Kumaran
No abstract text is available yet for this article.
July 2017: Indian Dermatology Online Journal
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