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Primary amyloidosis

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https://www.readbyqxmd.com/read/28742994/unusual-case-of-a-massive-macroglossia-secondary-to-myxedema-a-case-report-and-literature-review
#1
James C Melville, Kelsey D Menegotto, Timothy C Woernley, Blake D Maida, Ibrahim Alava
Macroglossia is classified as true macroglossia, which exhibits abnormal histology with clinical findings, and relative macroglossia, in which normal histology does not correlate with pathologic enlargement. This report describes an atypical case of morbidity with massive macroglossia secondary to myxedema; the macroglossia enlarged over a 3-month period before being presented to the Department of Oral and Maxillofacial Surgery, University of Texas Health Sciences Center at Houston (Houston, TX). Substantial enlargement of the tongue (16 cm long × 10 cm wide) was first attributed to angioedema, which was refractory to the discontinuation of lisinopril and a C1 esterase inhibitor...
June 30, 2017: Journal of Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/28721361/role-of-endoplasmic-reticulum-stress-in-learning-and-memory-impairment-and-alzheimer-s-disease-like-neuropathology-in-the-ps19-and-app-swe-mouse-models-of-tauopathy-and-amyloidosis
#2
Denise Isabelle Briggs, Erwin Defensor, Pooneh Memar Ardestani, Bitna Yi, Michelle Halpain, Guy Seabrook, Mehrdad Shamloo
Emerging evidence suggests that endoplasmic reticulum (ER) stress may be involved in the pathogenesis of Alzheimer's disease (AD). Recently, pharmacological modulation of the eukaryotic translation initiation factor-2 (eIF2α) pathway was achieved using an integrated stress response inhibitor (ISRIB). While members of this signaling cascade have been suggested as potential therapeutic targets for neurodegeneration, the biological significance of this pathway has not been comprehensively assessed in animal models of AD...
July 2017: ENeuro
https://www.readbyqxmd.com/read/28689003/primary-amyloidosis-with-renal-involvement-outcomes-in-77-consecutive-patients-at-a-single-center
#3
Sandy W Wong, Denis Toskic, Melissa Warner, Cindy Varga, Alejandro Moreno-Koehler, Daniel Fein, Teresa Fogaren, Lisa Lee, Colin M Oliver, Spencer D Guthrie, Raymond L Comenzo
BACKGROUND: Outcomes in primary amyloid renal patients are of interest as the era of monoclonal antibody therapies begins. PATIENTS AND METHODS: We studied 77 consecutive primary amyloid renal patients (58% men) for renal progression (end stage renal disease [ESRD]), renal response (RR), and overall survival (OS). RESULTS: At diagnosis median age was 63 (range, 35-81) years, estimated glomerular filtration rate 70 mL/min (range, 5-114), difference between involved and uninvolved free light chains 127 mg/L (range, 1-9957), ESRD 4%, renal stage 2 and 3 78%, and cardiac stage 2 and 3 56%...
June 17, 2017: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/28687586/a-rash-with-a-heavy-heart
#4
Sergio Barros-Gomes, Niyada Naksuk, Dragan Jevremovic, Hector R Villarraga
Cardiac amyloidosis (CA) is relatively rare and frequently misdiagnosed. Other disorders presenting with increased left ventricular (LV) mass can mimic its diagnosis. This case illustrates unique findings of primary light chain (AL) amyloidosis in a patient with remarkable signs of CA. Here, we report a 49-year-old man with prior diagnosis of hypertrophy cardiomyopathy (HCM) based on an echocardiogram performed 1 year earlier that presented with 8 weeks of periorbital rash. He had numbness in the past 3 years...
July 7, 2017: Echo Research and Practice
https://www.readbyqxmd.com/read/28646945/primary-amyloidosis-of-the-bladder-a-mimicker-of-bladder-cancer
#5
Ryan Fitzpatrick, Nicholas R Paterson, Eric C Belanger, Arleigh McCurdy, James Watterson
Amyloidosis is a protein folding disorder characterized by the deposition of fibrillar proteins into solid organs or tissues. Primary localized amyloidosis of the bladder is very rare and can mimic bladder cancer in its presentation with hematuria, lower urinary tract symptoms or a mass on imaging. A case of localized amyloidosis of the bladder in a 48-year-old man with painless gross hematuria and evidence of bladder mass on ultrasound is presented. Amyloidosis is a rare but important non-malignant process of the bladder...
June 2017: Canadian Journal of Urology
https://www.readbyqxmd.com/read/28622303/addition-of-cyclophosphamide-and-higher-doses-of-dexamethasone-do-not-improve-outcomes-of-patients-with-al-amyloidosis-treated-with-bortezomib
#6
E Kastritis, M Gavriatopoulou, M Roussou, D Fotiou, D C Ziogas, M Migkou, E Eleutherakis-Papaiakovou, I Panagiotidis, N Kanellias, E Psimenou, E Papadopoulou, C Pamboucas, E Manios, H Gakiopoulou, A Ntalianis, A Tasidou, S Giannouli, E Terpos, M A Dimopoulos
Bortezomib, in combination with dexamethasone (VD) or with the addition of cyclophosphamide (VCD), is highly effective in patients with amyloid light-chain (AL) amyloidosis. Currently, VCD is considered as a primary regimen for patients with AL, but it is not clear whether the addition of cyclophosphamide to VD further and significantly improves efficacy, given the substantial activity of bortezomib itself. We retrospectively compared the outcomes of 101 patients with AL amyloidosis who received VD (n=59) or VCD (n=42) in two consecutive periods...
June 16, 2017: Blood Cancer Journal
https://www.readbyqxmd.com/read/28583160/differential-expression-of-cathepsin-e-in-transthyretin-amyloidosis-from-neuropathology-to-the-immune-system
#7
Nádia Pereira Gonçalves, João Moreira, Diana Martins, Paulo Vieira, Laura Obici, Giampaolo Merlini, Margarida Saraiva, Maria João Saraiva
BACKGROUND: Increasing evidence supports a key role for inflammation in the neurodegenerative process of familial amyloidotic polyneuropathy (FAP). While there seems to be an overactivation of the neuronal interleukin-1 signaling pathway, the immune response is apparently compromised in FAP. Accordingly, little immune cell infiltration is observed around pre-fibrillar or fibrillar amyloid deposits, with the underlying mechanism for this phenomenon remaining poorly understood. Cathepsin E (CtsE) is an important intermediate for antigen presentation and chemotaxis, but its role in the pathogenesis of FAP disease remains unknown...
June 6, 2017: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/28583087/incomplete-ileus-and-hemafecia-as-the-presenting-features-of-multi-organ-involved-primary-systemic-al-amyloidosis-a-rare-case-report
#8
Li Tian, Anliu Tang, Xian Zhang, Zhen Mei, Fen Liu, Jingbo Li, Xiayu Li, Feiyan Ai, Xiaoyan Wang, Shourong Shen
BACKGROUND: AL Amyloidosis is known to be a systemic disease affecting multiple organs and tissue while it's rare that patients present with gastrointestinal symptoms at first and later develop multiple-organ dysfuction. Clinical signs are not specific and the diagnosis is rarely given before performing immunofixation and endoscopy with multiple biopsies. We would like to emphasize the value of precise diagnostic process of AL amyloidosis. CASE PRESENTATION: In this case report, we describe a 56-year-old man who presented with recurrent periumbilical pain for 4 months and gradually worsened over a month...
June 5, 2017: BMC Gastroenterology
https://www.readbyqxmd.com/read/28579588/a-case-of-primary-amyloidosis-with-spontaneous-hepatic-rupture
#9
Tsuyoshi Suda, Hikaru Oguri
A 79-year-old man was diagnosed to have primary amyloid light-chain (AL) amyloidosis with associated liver damage and prominent hepatomegaly. He was followed up without any treatment. One year after the diagnosis, he was taken to the hospital with a sudden onset of features of shock. Computed tomography revealed hepatic rupture, and he was treated by emergent transcutaneous arterial embolization. However, the procedure was unable to save his life. AL amyloidosis with prominent hepatomegaly is considered to be a risk factor for spontaneous liver rupture and intra-abdominal hemorrhage...
2017: Nihon Shokakibyo Gakkai Zasshi, the Japanese Journal of Gastro-enterology
https://www.readbyqxmd.com/read/28557715/amyloidosis-cutis-dyschromica-a-rare-cause-of-hyperpigmentation-a-new-case-and-literature-review
#10
Oya Kuseyri, Dorothea Haas, Nina Lang, Knut Schäkel, Markus Bettendorf
Amyloidosis cutis dyschromica is a rare form of primary cutaneous amyloidosis without systemic involvement and characterized by asymptomatic, progressive hyper- and hypopigmentation. We present the first case of a patient with amyloidosis cutis dyschromica diagnosed previously elsewhere as having Addison disease with generalized hyperpigmentation of the skin. This case suggests that in patients presenting with asymptomatic cutaneous dyschromia a skin biopsy for histopathological examination should be considered...
May 2017: Pediatrics
https://www.readbyqxmd.com/read/28544119/common-fibril-structures-imply-systemically-conserved-protein-misfolding-pathways-in%C3%A2-vivo
#11
Karthikeyan Annamalai, Falk Liberta, Marie-Theres Vielberg, William Close, Hauke Lilie, Karl-Heinz Gührs, Angelika Schierhorn, Rolf Koehler, Andreas Schmidt, Christian Haupt, Ute Hegenbart, Stefan Schönland, Matthias Schmidt, Michael Groll, Marcus Fändrich
Systemic amyloidosis is caused by the misfolding of a circulating amyloid precursor protein and the deposition of amyloid fibrils in multiple organs. Chemical and biophysical analysis of amyloid fibrils from human AL and murine AA amyloidosis reveal the same fibril morphologies in different tissues or organs of one patient or diseased animal. The observed structural similarities concerned the fibril morphology, the fibril protein primary and secondary structures, the presence of post-translational modifications and, in case of the AL fibrils, the partially folded characteristics of the polypeptide chain within the fibril...
May 23, 2017: Angewandte Chemie
https://www.readbyqxmd.com/read/28540895/pattern-of-acute-glomerulonephritis-in-adult-population-in-dubai-a-single-center-experience
#12
Amna K Alhadari, Fakhriya J Alalawi, Ayman Aly Seddik, Kaneez Zahra, Dileep Kumar, Hussain Yousif, Hind Alnour, Michael Jansen, Mohammad J Railey
Epidemiological data of renal diseases have great geographic variability throughout the world. Due to the lack of a national renal data registry system, there is no information on the prevalence rate, clinical and pathological features of various glomerulonephritis (GN) in the United Arab Emirates (UAE). In a retrospective cross-sectional study, we analyzed 158 renal biopsies done in Dubai Hospital, UAE, between the years of 2005 and September 2014, with an aim to determine the prevalence rate and frequency of different pathological patterns of GN in adult patients who presented with proteinuria ± hematuria...
May 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28538749/complications-of-hidradenitis-suppurativa
#13
Joyce T Yuan, Haley B Naik
Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease characterized by recurrent painful nodules and abscesses involving intertriginous areas. Repeated episodes of profound inflammation in HS can lead to a number of complications, causing significant morbidity and decreasing quality of life. Complications of HS may affect the skin alone or may have systemic impact. Cutaneous complications of HS include sinus tracts, fistulae, scarring and contractures, squamous cell carcinoma, and lymphedema...
June 2017: Seminars in Cutaneous Medicine and Surgery
https://www.readbyqxmd.com/read/28537855/multifocal-primary-cutaneous-nodular-amyloidosis
#14
Martha Emily Matsumoto, Mary-Katharine Collins, Anastasios Raptis, Jaroslaw Jedrych, Timothy Patton
Nodular cutaneous amyloidosis (NCA), the least common form of primary cutaneous amyloidosis, is characterized clinically by waxy, purpuric plaques and nodules and histologically by amyloid deposits in the dermis and subcutaneous tissue. We present a patient who developed multiple, non-contiguous NCA lesions over a three year period without evidence of systemic disease. We reviewed the literature and found few other cases of this unusual presentation.
May 15, 2017: Dermatology Online Journal
https://www.readbyqxmd.com/read/28535110/successful-treatment-of-lichen-amyloidosis-accompanied-by-atopic-dermatitis-by-fractional-co2-laser
#15
Howard Chu, Jung U Shin, Jungsoo Lee, Chang Ook Park, Kwang Hoon Lee
Lichen amyloidosis (LA) is a subtype of primary cutaneous amyloidosis that is presented as persistent, multiple-grouped hyperkeratotic papules usually located on the shins, back, forearms or thighs. The treatment of LA has been considered to be difficult, and of the various methods available, recent studies have reported the efficacy of fractional CO2 lasers. LA may be accompanied by atopic dermatitis (AD) in which the treatment options may be more limited. Herein, we report three cases of LA accompanied by AD treated by a fractional CO2 laser...
May 23, 2017: Journal of Cosmetic and Laser Therapy: Official Publication of the European Society for Laser Dermatology
https://www.readbyqxmd.com/read/28505007/al-amyloidoma-of-the-skin-subcutis-cutaneous-amyloidosis-plasma-cell-dyscrasia-or-a-manifestation-of-primary-cutaneous-marginal-zone-lymphoma
#16
Noreen M Walsh, Ian Marie Lano, Peter Green, Christopher Gallant, Sylvia Pasternak, Thai Yen Ly, Luis Requena, Heinz Kutzner, Andreas Chott, Lorenzo Cerroni
It is unclear whether AL amyloidoma of the skin/subcutis represents a distinct entity, an indolent precursor of systemic amyloidosis, or a manifestation of cutaneous marginal zone lymphoma (cMZL). We collected 10 cases of cutaneous AL amyloidoma in order to better characterize the clinicopathologic features of this elusive entity (M:F=4:6; median age: 62.5 y, range: 31 to 82 y). Nine patients had a solitary nodule or plaque on the lower extremity (n=7), upper extremity (n=1), or chin (n=1). One patient had an AL amyloidoma on the right thigh and a second lesion on the right arm showing histopathologic features of cMZL without amyloid deposits...
August 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28482636/intrahippocampal-pathways-involved-in-learning-memory-mechanisms-are-affected-by-intracerebral-infusions-of-amyloid-%C3%AE-25-35-peptide-and-hydrated-fullerene-c60-in-rats
#17
Rita Gordon, Igor Podolski, Ekaterina Makarova, Alexander Deev, Ekaterina Mugantseva, Sergey Khutsyan, Frank Sengpiel, Arkady Murashev, Vasily Vorobyov
Primary memory impairments associated with increased level of amyloid-β (Aβ) in the brain have been shown to be linked, partially, with early pathological changes in the entorhinal cortex (EC) which spread on the whole limbic system. While the hippocampus is known to play a key role in learning and memory mechanisms, it is as yet unclear how its structures are involved in the EC pathology. In this study, changes in memory and neuronal morphology in male Wistar rats intrahippocampally injected with Aβ25-35 were correlated on days 14 and 45 after the injection to reveal specific cognitive-structural associations...
2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28454452/flow-cytometry-based-immunophenotypic-analysis-of-primary-systemic-light-chain-amyloidosis
#18
Xiangwen Diao, Juan Li, Juan Ouyang, Junru Liu, Beihui Huang, Meilan Chen, Jingli Gu
Using flow cytometry, the present study aimed to investigate the immunophenotypic characteristics of malignant plasma cells (PCs) in the bone marrow of patients with primary systemic light chain amyloidosis (AL) compared with the characteristics of patients with multiple myeloma (MM). Flow cytometric results of 51 patients with AL and 150 patients with MM were reviewed. The proportion of total bone marrow PCs in the patients with AL was significantly lower than that in the patients with MM, 1.35% (0.3-9.5%) vs...
April 2017: Oncology Letters
https://www.readbyqxmd.com/read/28434267/amyloid-in-bone-marrow-smears-in-systemic-light-chain-amyloidosis
#19
Christoph Kimmich, Stefan Schönland, Sandra Kräker, Mindaugas Andrulis, Anthony D Ho, Gudrun Mayer, Tobias Dittrich, Michael Hundemer, Ute Hegenbart
We performed a prospective sensitivity analysis to detect amyloid in bone marrow (BM) smears stained with Congo red (CR) and according to Pappenheim of patients with systemic light-chain (AL) amyloidosis. Results were directly compared to routine BM histology and fat aspiration. We analysed 198 BM smears from patients with the diagnosis or suspicion of systemic AL amyloidosis. Ultimately, the diagnosis could be established for 168 patients. Amyloid was detected on BM smears with CR in 33% (56/168). All patients suspicious for amyloid on Pappenheim staining (n = 39) showed substantial amyloid infiltration on CR...
April 23, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28426270/the-successful-treatment-of-primary-cutaneous-localized-amyloidosis-with-a-combined-therapy-of-ablative-fractionated-co2-laser%C3%AF-topical-retinoid-corticosteroids-and-levovitamin-c-five-cases-with-a-two-year-follow-up
#20
Yao-Jia Luo, Xing-Hua Gao, Hong-Duo Chen, Yuan-Hong Li
No abstract text is available yet for this article.
April 20, 2017: Journal of Cosmetic and Laser Therapy: Official Publication of the European Society for Laser Dermatology
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