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light chain deposition disease

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https://www.readbyqxmd.com/read/28926601/utility-of-abdominal-skin-plus-subcutaneous-fat-and-rectal-mucosal-biopsy-in-the-diagnosis-of-al-amyloidosis-with-renal-involvement
#1
Ting Li, Xianghua Huang, Shuiqin Cheng, Liang Zhao, Guisheng Ren, Wencui Chen, Qingwen Wang, Caihong Zeng, Zhihong Liu
OBJECTIVES: Skin fat biopsy of the abdominal wall is a simple and safe method for detecting amyloidosis, and rectal mucosal biopsy is also frequently used for screening for the disease; however, the sensitivity of these approaches has not been fully studied. The aim of this study was to evaluate the efficacy of skin fat biopsy combined with rectal mucosal biopsy as a screening procedure for the diagnosis of systemic immunoglobulin light-chain (AL) amyloidosis. METHODS: We retrospectively analyzed 224 AL amyloidosis patients confirmed by renal biopsy, including a test group of 165 patients and validation group of 59 patients...
2017: PloS One
https://www.readbyqxmd.com/read/28910817/the-lung-in-dysregulated-states-of-humoral-immunity
#2
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/28903572/biomarkers-in-immunoglobulin-light-chain-amyloidosis
#3
Z Kufová, T Sevcikova, K Growkova, P Vojta, J Filipová, Z Adam, L Pour, M Penka, R Rysava, P Němec, L Brozova, P Vychytilova, A Jurczyszyn, S Grosicki, A Barchnicka, M Hajdúch, M Simicek, R Hájek
Immunoglobulin light chain amyloidosis (AL amyloidosis - ALA) is a monoclonal gammopathy characterized by presence of aberrant plasma cells producing amyloidogenic immunoglobulin light chains. This leads to formation of amyloid fibrils in various organs and tissues, mainly in heart and kidney, and causes their dysfunction. As amyloid depositing in target organs is irreversible, there is a big effort to identify biomarker that could help to distinguish ALA from other monoclonal gammopathies in the early stages of disease, when amyloid deposits are not fatal yet...
2017: Klinická Onkologie: Casopis Ceské a Slovenské Onkologické Spolecnosti
https://www.readbyqxmd.com/read/28875827/multiple-myeloma-associated-intestinal-amyloidosis-intestinal-pseudo-obstruction-falsely-considered-as-an-ascites
#4
Vladimir Milivojevic, Maja Stojanovic, Ivan Rankovic, Marjan Micev, Rada Jesic Vukicevic, Tomica Milosavljevic
INTRODUCTION: Amyloidosis is a group of diseases pathohistologicaly diagnosed by characteristic extracellular deposition of an abnormal fibrillary protein (i.e. amyloid) into organs, leading to organ dysfunction secondary to destruction of normal tissue architecture. METHOD: Case-report of a 44 year-old female, presenting with massive abdominal distension clinically suspected of ascites. RESULTS: On admission, the patient was complaining of nausea, vomiting, abdominal pain, distension and bloating associated with weight loss and diarrhea...
August 29, 2017: Reviews on Recent Clinical Trials
https://www.readbyqxmd.com/read/28865972/high-dose-melphalan-and-stem-cell-transplantation-in-patients-on-dialysis-due-to-al-amyloidosis-and-monoclonal-immunoglobulin-deposition-disease
#5
Felipe Batalini, Laura Econimo, Karen Quillen, J Mark Sloan, Shayna Sarosiek, Dina Brauneis, Andrea Havasi, Lauren Stern, Laura Dember, Vaishali Sanchorawala
The kidney is the most common organ affected by immunoglobulin light-chain (AL) amyloidosis and monoclonal immunoglobulin deposition disease (MIDD), often leading to end stage renal disease (ESRD). High-dose melphalan and stem cell transplantation (HDM/SCT) is effective for selected patients with AL amyloidosis, with high rates of complete hematologic responses and potential for improvement in organ dysfunction. Data on tolerability and response to HDM/SCT in patients with ESRD due to AL amyloidosis and MIDD are limited...
August 30, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28861330/a-plasma-cell-dyscrasia-presenting-as-amyloid-cardiomyopathy-and-autonomic-dysfunction-in-a-healthy-patient
#6
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/28822451/-the-value-of-serum-free-light-chain-in-differential-diagnosis-of-monoclonal-gammopathy-of-renal-significance
#7
C Li, Y B Wen, H Li, W Su, J Li, J F Cai, L M Chen, X M Li, X W Li
Objective: To investigate the value of serum free light chain (FLC) in differential diagnosis of monoclonal gammopathy of renal significance (MGRS). Methods: Forty-nine hospitalized patients who underwent renal biopsy in Peking Union Medical College Hospital between January 2013 and December 2015 were included. Monoclonal gammopathy was detected by serum protein electrophoresis (SPE), serum immunofixation electrophoresis (IFE), urine IFE and serum FLC. All patients were classified as MGRS (n=32) and monoclonal gammopathy of undetermined significance (MGUS) (n=17)...
August 8, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28809601/detection-and-treatment-of-monoclonal-gammopathy-of-renal-significance-with-progression-to-light-chain-deposition-disease
#8
Gheorghe Ciprian Cazan, Kirk Foster, Matthew A Lunning, Ketki Tendulkar
No abstract text is available yet for this article.
August 15, 2017: Journal of Oncology Practice
https://www.readbyqxmd.com/read/28796568/contribution-of-human-smooth-muscle-cells-to-amyloid-angiopathy-in-al-light-chain-amyloidosis
#9
Moiz Vora, Christopher G Kevil, Guillermo A Herrera
OBJECTIVE: Amyloid light-chain (AL) amyloidosis is a disease process that often compromises the peripheral vascular system and leads to systemic end-organ dysfunction. Although amyloid formation in vessel walls is a multifaceted process, the assembly of the native light chains (LCs) into amyloid fibrils is central to its pathogenesis. Recent evidence suggests that endocytosis and endolysosomal processing of immunoglobin LCs by host cells is essential to the formation of amyloid fibrils that are deposited in at least some tissues...
August 10, 2017: Ultrastructural Pathology
https://www.readbyqxmd.com/read/28790241/-amyloidoma-adjacent-to-the-thoracic-vertebral-body-developed-into-systemic-amyloid-light-chain-amyloidosis
#10
Kyoko Tanaka, Hideto Goto, Shuhei Ikeda, Nami Masumoto, Katsuya Watanabe, Motofumi Tsubakihara
Amyloidoma is very rare case of amyloidosis, and seldom develop into systemic disease. We report a case of an 82-year-old man who was referred to our hospital because of an oppressive feeling in the upper chest. Chest computed tomography showed a tumor on thoracic vertebral body. Percutaneous needle biopsy showed pathological findings of AL amyloidosis. No obvious systemic finding was confirmed, and the tumor was diagnosed as amyloidoma. After a 1-year observation, heart amyloidosis was appeared, then 6 months later he died...
August 2017: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/28774673/can-videolaryngoscopy-be-a-first-option-in-a-patient-with-laryngeal-amyloidosis
#11
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/28758811/novel-pathologic-scoring-tools-predict-end-stage-kidney-disease-in-light-chain-al-amyloidosis
#12
Samuel Rubinstein, Robert F Cornell, Liping Du, Beatrice Concepcion, Stacey Goodman, Shelton Harrell, Sara Horst, Daniel Lenihan, David Slosky, Agnes Fogo, Anthony Langone
BACKGROUND AND OBJECTIVES: Light chain (AL) amyloidosis frequently involves the kidney, causing significant morbidity and mortality. A pathologic scoring system with prognostic utility has not been developed. We hypothesized that the extent of amyloid deposition and degree of scarring injury on kidney biopsy, could provide prognostic value, and aimed to develop pathologic scoring tools based on these features. METHODS: This is a case-control study of 39 patients treated for AL amyloidosis with biopsy-proven kidney involvement at a large academic medical center...
July 31, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28739313/cardiac-amyloidosis-an-update-on-pathophysiology-diagnosis-and-treatment
#13
REVIEW
Omar K Siddiqi, Frederick L Ruberg
The amyloidoses are a group of systemic diseases characterized by organ deposition of misfolded protein fragments of diverse origins. The natural history of the disease, involvement of other organs, and treatment options vary significantly based on the protein of origin. In AL amyloidosis, amyloid protein is derived from immunoglobulin light chains, and most often involves the kidneys and the heart. ATTR amyloidosis is categorized as mutant or wild-type depending on the genetic sequence of the transthyretin (TTR) protein produced by the liver...
July 13, 2017: Trends in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28725015/all-printed-highly-sensitive-2d-mos2-based-multi-reagent-immunosensor-for-smartphone-based-point-of-care-diagnosis
#14
Memoon Sajid, Ahmed Osman, Ghayas Uddin Siddiqui, Hyun Bum Kim, Soo Wan Kim, Jeong Bum Ko, Yoon Kyu Lim, Kyung Hyun Choi
Immunosensors are used to detect the presence of certain bio-reagents mostly targeted at the diagnosis of a condition or a disease. Here, a general purpose electrical immunosensor has been fabricated for the quantitative detection of multiple bio-reagents through the formation of an antibody-antigen pair. The sensors were fabricated using all printing approaches. 2D transition metal dichalcogenide (TMDC) MoS2 thin film was deposited using Electrohydrodynamic atomization (EHDA) on top of an interdigitated transducer (IDT) electrode fabricated by reverse offset printing...
July 19, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28716013/long-term-renal-survival-of-%C3%AE-3-heavy-chain-deposition-disease-a-case-report
#15
Takayuki Katsuno, Shige Mizuno, Masatsuna Mabuchi, Naotake Tsuboi, Atsushi Komatsuda, Shoichi Maruyama
BACKGROUND: Monoclonal immunoglobulin deposition disease (MIDD) is characterized by the non-amyloid deposition of monoclonal immunoglobulin fragments in the basement membranes. Heavy chain deposition disease (HCDD) is a type of MIDD. HCDD is an extremely rare disease, and only three cases have been reported in Japan up to the present. The prognosis of HCDD is very poor, and optimal treatment has not been established. Only a few cases of HCDD with favorable long-term renal prognosis have been reported to date...
July 17, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28701686/congestive-heart-failure-with-preserved-ejection-fraction-in-a-patient-with-light-chain-al-amyloidosis-and-multiple-myeloma
#16
Edgar Francisco Carrizales-Sepúlveda, Alejandro Ordáz-Farías, Raymundo Vera-Pineda, Mario Alberto Benavides-González, Ramiro Flores-Ramírez
BACKGROUND Amyloidosis is characterized by tissue deposition of insoluble fibrillar proteins and it affects almost every organ; there are many types and the heart can be affected in all of them. CASE REPORT Our report describes a middle-aged man who presented to the Emergency Department with congestive heart failure. Clinical, electrocardiographic, and echocardiographic findings suggested the presence of an infiltrative disease, so an abdominal fat tissue biopsy was performed. A final diagnosis of systemic amyloidosis with heart involvement was made...
July 13, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28694632/late-onset-familial-amyloidotic-polyneuropathy-with-bence-jones-proteinuria-and-cardiomyopathy
#17
Sira Carrasco García de León, Amalia Hernández González, Carmen Orellana Alonso, Laura Burriel Lobo
Familial amyloidotic polyneuropathy is a genetically determined disease characterized by deposition of an anomalous transthyretin. A high index of suspicion is needed for this multisymptomatic and lethal disease to be diagnosed. The patient was a 70-year-old male examined due to hypesthesia in the hands and feet, plus difficulty walking. A neurophysiological study delivered the diagnosis of axonal sensorimotor polyneuropathy. He later developed cardiac symptoms and diarrhea. Urine laboratory analyses revealed a monoclonal spike of light chains (kappa)...
July 2017: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/28669250/primary-cns-nonamyloidogenic-light-chain-deposition-disease-case-report-and-brief-review
#18
Juan Jose Mercado, James M Markert, William Meador, Philip Chapman, Arie Perry, James R Hackney
The true incidence of light chain deposition disease (LCDD) restricted to the central nervous system (CNS) is unknown. To our knowledge only 7 cases of LCDD restricted to the brain have been previously reported. We herein describe an unusual example. A 44-year-old man presented with a history of ischemic retinopathy in 2004 and left lower extremity hypoesthesia in 2007 that progressed gradually to left-sided weakness and numbness in the 2 years prior to his hospitalization in 2015. A stereotactic brain biopsy was performed, displaying nonspecific hyaline deposits of amorphous "amyloid-like" material involving deep brain white matter and vessels...
June 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28648829/novel-pharmacotherapies-for-cardiac-amyloidosis
#19
REVIEW
Kevin M Alexander, Avinainder Singh, Rodney H Falk
Amyloidosis refers to a range of protein misfolding disorders that can cause organ dysfunction through progressive fibril deposition. Cardiac involvement often leads to significant morbidity and mortality and increasingly has been recognized as an important cause of heart failure. The two main forms of cardiac amyloidosis, light chain (AL) and transthyretin (ATTR) amyloidosis, have distinct mechanisms of pathogenesis. Recent insights have led to the development of novel pharmacotherapies with the potential to significantly impact each disease...
June 22, 2017: Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/28647518/increasing-the-accuracy-of-proteomic-typing-by-decellularisation-of-amyloid-tissue-biopsies
#20
P Patrizia Mangione, Giuseppe Mazza, Janet A Gilbertson, Nigel B Rendell, Diana Canetti, Sofia Giorgetti, Luca Frenguelli, Marco Curti, Tamer Rezk, Sara Raimondi, Mark B Pepys, Philip N Hawkins, Julian D Gillmore, Graham W Taylor, Massimo Pinzani, Vittorio Bellotti
Diagnosis and treatment of systemic amyloidosis depend on accurate identification of the specific amyloid fibril protein forming the tissue deposits. Confirmation of monoclonal immunoglobulin light chain amyloidosis (AL), requiring cytotoxic chemotherapy, and avoidance of such treatment in non-AL amyloidosis, are particularly important. Proteomic analysis characterises amyloid proteins directly. It complements immunohistochemical staining of amyloid to identify fibril proteins and gene sequencing to identify mutations in the fibril precursors...
August 8, 2017: Journal of Proteomics
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