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amyloidosis kidney

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https://www.readbyqxmd.com/read/29775082/profile-of-renal-aa-amyloidosis-in-older-and-younger-individuals-a-single-centre-experience
#1
Siyar Erdogmus, Zeynep Kendi Celebi, Serkan Akturk, Gizem Kumru, Neval Duman, Kenan Ates, Sehsuvar Erturk, Gokhan Nergizoglu, Sim Kutlay, Sule Sengul, Kenan Keven
OBJECTIVE: In epidemiological studies of amyloid A (AA) amyloidosis from Turkey, the most frequently cause was familial Mediterranean fever (FMF) and it occurs generally in young age population. However, there are no sufficient data regarding aetiology, clinical presentation and prognosis of renal AA amyloidosis in advanced age patients. In this study, we aimed to investigate demographic, clinical presentation, aetiology and outcomes of adults aged 60 years or older patients with biopsy-proven renal AA amyloidosis...
May 18, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29774542/accelerated-pre-senile-systemic-amyloidosis-in-pacap-knockout-mice-a-protective-role-of-pacap-in-age-related-degenerative-processes
#2
Dora Reglodi, Adel Jungling, Rémi Longuespée, Joerg Kriegsmann, Rita Casadonte, Mark Kriegsmann, Tamas Juhasz, Sebastian Bardosi, Andrea Tamas, Balazs Daniel Fulop, Krisztina Kovacs, Zsuzsanna Nagy, Jason Sparks, Attila Miseta, Gabriel Mazzucchelli, Hitoshi Hashimoto, Attila Bardosi
Dysregulation of neuropeptides may play an important role in aging-induced impairments. Among them, pituitary adenylate cyclase activating polypeptide (PACAP) is a potent cytoprotective peptide that provides an endogenous control against a variety of tissue-damaging stimuli. We hypothesized that the progressive decline of PACAP throughout life, and the well-known general cytoprotective effects of PACAP lead to age-related pathophysiological changes in PACAP deficiency, supported by the increased vulnerability to various stressors of animals partially or totally lacking PACAP...
May 17, 2018: Journal of Pathology
https://www.readbyqxmd.com/read/29770283/cardiac-amyloidosis-diagnosis-with-magnetic-resonance-imaging-a-case-report
#3
Meidi El Issa, Malik El Issa, Besma Sidia
Amyloidosis is a rare disease, which can affect various organs, such as the kidneys, heart, liver, respiratory and gastrointestinal tracts, and the nervous system. It still has a bad prognosis nowadays, despite chemotherapy and the new biotherapies. Its physiopathology corresponds to an irreversible, extracellular accumulation of fibrillous proteins in the tissues. Notwithstanding the fact that a clear diagnosis can be made with histology (of solid injured organs or a subcutaneous biopsy), magnetic resonance imaging (MRI) can show various advantages, especially to prove cardiac involvement, with great specificity and sensibility as well...
March 15, 2018: Curēus
https://www.readbyqxmd.com/read/29729178/-primary-renal-amyloidosis-feautures-of-disease-course-and-the-possibilities-of-in-time-diagnosis-clinical-case-report
#4
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/29697879/drawing-attention-to-a-neglected-injecting-related-harm-a-systematic-review-of-aa-amyloidosis-among-people-who-inject-drugs
#5
REVIEW
Magdalena Harris, Rachel Brathwaite, Jenny Scott, Gail Gilchrist, Dan Ciccarone, Vivian Hope, Catherine R McGowan
BACKGROUND AND AIMS: Chronic skin and soft tissue infections (SSTI) among people who inject drugs (PWID) can lead to AA-amyloidosis: a serious, yet neglected, multi-organ disease. We aim to synthesise findings on the epidemiology, risk factors, clinical outcomes, screening recommendations, and challenges to treatment for AA-amyloidosis among PWID. METHODS: A systematic review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA)...
April 26, 2018: Addiction
https://www.readbyqxmd.com/read/29692592/revisiting-renal-amyloidosis-with-clinicopathological-characteristics-grading-and-scoring-a-single-institutional-experience
#6
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/29668344/phenotypic-plasticity-of-mesenchymal-stem-cells-is-crucial-for-mesangial-repair-in-a-model-of-immunoglobulin-light-chain-associated-mesangial-damage
#7
Guillermo A Herrera, Jiamin Teng, Chun Zeng, Hongzhi Xu, Man Liang, J Steven Alexander, Bing Liu, Chris Boyer, Elba A Turbat-Herrera
Mesangiopathies produced by glomerulopathic monoclonal immunoglobulin light chains (GLCs) acting on the glomerular mesangium produce two characteristic lesions: AL-amyloidosis (AL-Am) and light chain deposition disease (LCDD). In both cases, the pathology is centered in the mesangium, where initial and progressive damage occurs. In AL-Am the mesangial matrix is destroyed and replaced by amyloid fibrils and in LCDD, the mesangial matrix is increased and remodeled. The collagen IV rich matrix is replaced by tenascin...
April 18, 2018: Ultrastructural Pathology
https://www.readbyqxmd.com/read/29651545/extracardiac-18-f-florbetapir-imaging-in-patients-with-systemic-amyloidosis-more-than-hearts-and-minds
#8
T Wagner, J Page, M Burniston, A Skillen, J C Ross, R Manwani, D McCool, P N Hawkins, Ashutosh D Wechalekar
PURPOSE: 18 F-Florbetapir has been reported to show cardiac uptake in patients with systemic light-chain amyloidosis (AL). This study systematically assessed uptake of 18 F-florbetapir in patients with proven systemic amyloidosis at sites outside the heart. METHODS: Seventeen patients with proven cardiac amyloidosis underwent 18 F-florbetapir PET/CT imaging, 15 with AL and 2 with transthyretin amyloidosis (ATTR). Three patients had repeat scans. All patients had protocolized assessment at the UK National Amyloidosis Centre including imaging with 123 I-serum amyloid P component (SAP)...
April 12, 2018: European Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/29644070/intravenous-drug-users-who-require-dialysis-causes-of-renal-failure-and-outcomes
#9
Jemima K Scott, Dominic M Taylor, Chris R K Dudley
Background: Intravenous drug use is associated with progressive kidney disease of several aetiologies. It is associated with behavioural and lifestyle characteristics that make the provision of renal replacement therapies (RRTs) challenging. We observed that patients who use intravenous drugs [people who inject drugs (PWID)] present late to renal services and struggle to engage with treatment. We describe the experience of a UK centre providing renal services to a mixed city and rural population...
April 2018: Clinical Kidney Journal
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/29594138/changes-in-the-spectrum-of-kidney-diseases-an-analysis-of-40-759-biopsy-proven-cases-from-2003-to-2014-in-china
#11
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/29554974/renal-dialysis-and-long-term-treatment-of-a-dog-with-kidney-disease-associated-with-canine-leishmaniosis
#12
Gad Baneth, Gilad Segev, Michal Mazaki-Tovi, Hila Chen, Sharon Kuzi
BACKGROUND: Renal disease is considered the main cause of natural mortality in dogs with canine leishmaniosis. The pathological mechanisms associated with kidney injury in canine leishmaniosis include immune complex glomerulonephritis, tubulointerstitial nephritis and occasionally renal amyloidosis. Proteinuria is a frequent finding in canine leishmaniosis and its quantification by the urine protein-creatinine ratio (UPC) is an important parameter in the staging of canine lesihmaniosis as presented by the LeishVet group...
March 20, 2018: Parasites & Vectors
https://www.readbyqxmd.com/read/29464179/leukocyte-cell-derived-chemotaxin-2-associated-renal-amyloidosis-a-case-report
#13
Gagandeep Kaur, Babitha Bijin, Kamron Saleem, Benjamin Sarsah, Bijin Thajudeen
Amyloidosis is a disorder characterized by the deposition of abnormal protein fibrils in tissues. Leukocyte cell-derived chemotaxin 2-associated amyloidosis is a recently recognized entity and is characterized by a distinctive clinicopathologic type of amyloid deposition manifested in adults by varying degrees of impaired kidney function and proteinuria. There are only a limited number of cases reported in the literature. We present a 64-year-old Hispanic female with a history of hypertension who was referred for chronic kidney disease management...
May 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/29456220/importance-of-renal-biopsy-in-patients-aged-60-years-and-older-experience-from-a-tertiary-care-hospital
#14
Pallav Gupta, Devinder Singh Rana
As the life expectancy is increasing, there is a rise in elderly population and consequent increase in the patients with renal disease. There is an inconsistency between clinical and histopathological diagnosis in elderly, and so renal biopsy is important in these patients to decide appropriate clinical management and prognosis. This study outlines the importance of renal biopsy in elderly and describes the clinical and pathologic spectrum of renal diseases in patient ≥60 years. All patients (age ≥60 years) undergoing renal biopsies from January 2011 to December 2014 were included in this retrospective study...
January 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29446975/primary-adrenal-insufficiency-due-to-hereditary-apolipoprotein-ai-amyloidosis-endocrine-involvement-beyond-hypogonadism
#15
Adriana Pané, Sabina Ruiz, Aida Orois, Daniel Martínez, Mattia Squarcia, Lydia Sastre, Pablo Ruiz, Joan Caballería, Mireia Mora, Felicia A Hanzu, Irene Halperin
Several mutations in the gene encoding apolipoprotein AI (apoAI) have been described as a cause of familial amyloidosis. Individuals with apoAI-derived (AApoAI) amyloidosis frequently manifest with liver, kidney, laryngeal, skin and myocardial involvement. Although primary hypogonadism (PH) is considered almost pathognomonic of this disease, until now, primary adrenal insufficiency (PAI) has not been described as a common clinical feature. Here, we report the first kindred with AApoAI amyloidosis in which PAI is well-documented...
February 15, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29399587/oligosecretory-myeloma-with-amyloidosis-and-alopecia
#16
Anum Bilal, Paul Der Mesropian, Franklin Lam, Gulvahid Shaikh
Amyloidosis is a systemic illness characterized by the extracellular deposition of abnormal proteins in body tissues and organs. In addition to renal involvement, amyloidosis can also present with a variety of skin manifestations, though rarely with alopecia. Sixteen cases of alopecia secondary to systemic amyloidosis are reported. There is one reported case that presented with alopecia universalis. We report a case of a 68-year-old woman presenting with alopecia universalis, rapid decline in kidney function, and nephrotic syndrome who was found to have multiple myeloma-associated AL amyloidosis (immunoglobulin light chain)...
January 2018: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/29360446/a-mouse-model-of-a-novel-missense-mutation-gly83arg-in-a-chinese-kindred-manifesting-vitreous-amyloidosis-only
#17
Ling-Xia Ran, Zhi-Yong Zheng, Bing Xie, Xiao-Mei Nie, Xing-Wang Chen, Gang Su, Shan-Jun Cai
The purpose of this study is to establish a mouse model of transthyretin (TTR) Gly83Arg gene mutation by the technique of gene targeting for research on hereditary vitreous amyloidosis (HVA) and to confirm whether this point mutation is a genetic feature of HVA. A vector (pBR322-MK-TTR) was constructed to target ES cells. The successfully transfected ES cells were used for blastocyst injection, thus generating F0. F0 and Flp mice were mated to generate F1 (TTR+/- , Flp +/- ) mice that lacked the neo gene but carried the Flp gene...
April 2018: Experimental Eye Research
https://www.readbyqxmd.com/read/29314707/comparison-of-early-versus-late-onset-familial-mediterranean-fever
#18
Nazife Sule Yasar Bilge, Ismail Sari, Dilek Solmaz, Soner Senel, Hakan Emmungil, Levent Kilic, Sibel Yilmaz Oner, Fatih Yildiz, Sedat Yilmaz, Duygu Ersozlu Bozkirli, Muge Aydin Tufan, Sema Yilmaz, Veli Yazisiz, Yavuz Pehlivan, Cemal Bes, Gozde Yildirim Cetin, Sukran Erten, Emel Gonullu, Fezan Sahin, Servet Akar, Kenan Aksu, Umut Kalyoncu, Haner Direskeneli, Eren Erken, Mehmet Sayarlioglu, Muhammed Cınar, Timucin Kasifoglu
AIM: Familial Mediterranean fever (FMF) is the most common autoinflammatory disease. One of the common characteristics of this disease is its young age predominance. Nearly 90% of patients experience disease flares during early adult age periods. Currently there are limited data for the comparison of early versus late onset FMF and therefore the primary aim of this study was to investigate these two subsets with regard to their certain demographic, clinical and genetic differences. METHODS: Early (≤ 20 years, Group 1) and late (> 20 years, Group 2) onset FMF patients were identified from the national FMF registry that involves 2246 patients from 15 adult rheumatology clinics located in different geographical areas of Turkey...
April 2018: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/29298867/repeat-doses-of-antibody-to-serum-amyloid-p-component-clear-amyloid-deposits-in-patients-with-systemic-amyloidosis
#19
Duncan B Richards, Louise M Cookson, Sharon V Barton, Lia Liefaard, Thirusha Lane, David F Hutt, James M Ritter, Marianna Fontana, James C Moon, Julian D Gillmore, Ashutosh Wechalekar, Philip N Hawkins, Mark B Pepys
Systemic amyloidosis is a fatal disorder caused by pathological extracellular deposits of amyloid fibrils that are always coated with the normal plasma protein, serum amyloid P component (SAP). The small-molecule drug, miridesap, [(R)-1-[6-[(R)-2-carboxy-pyrrolidin-1-yl]-6-oxo-hexanoyl]pyrrolidine-2-carboxylic acid (CPHPC)] depletes circulating SAP but leaves some SAP in amyloid deposits. This residual SAP is a specific target for dezamizumab, a fully humanized monoclonal IgG1 anti-SAP antibody that triggers immunotherapeutic clearance of amyloid...
January 3, 2018: Science Translational Medicine
https://www.readbyqxmd.com/read/29250691/nephrotic-syndrome-secondary-to-amyloidosis-in-a-patient-with-monoclonal-gammopathy-with-renal-significance-mgrs
#20
Andrei Niculae, Ileana Peride, Vlad Vinereanu, Daniela Rădulescu, Ovidiu Gabriel Bratu, Bogdan Florin Geavlete, Ionel Alexandru ChecheriŢă
Monoclonal gammopathy with renal significance (MGRS) is a relative new-described entity, diagnosed especially in older patients and deriving from the group with monoclonal gammopathy of undetermined significance (MGUS). Various renal lesions may arise in MGRS, according to the ultrastructural characteristics of the monoclonal immunoglobulin deposition in the kidney, from proliferative glomerulopathies and amyloidosis to light chain proximal tubulopathy and crystal-storing histiocytosis. Although both are considered premalign or non-malignant hematological conditions, kidney involvement in MGRS aggravates the prognosis of the patients and need to be treated aggressively...
2017: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
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