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Bladder Amyloidosis

Segundo Bujan-Rivas, Maria Basagaña, Francisca Sena, Maria Méndez, Maria Teresa Dordal, Eva Gonzalez-Roca, Estibaliz Ruiz-Ortiz, Anna Mensa-Vilaró, Susana Plaza, Consuelo Modesto, Josep Ordi-Ros, Jordi Yagüe, Ferrán Martínez-Valle, Juan Ignacio Aróstegui
OBJECTIVES: Cryopyrin-associated periodic syndromes (CAPS) usually start during infancy as an urticarial-like rash and a marked acute phase response, with additional manifestations appearing during its evolution. The aim of this study was to expand the clinical diversity of CAPS by the description of novel atypical features. METHODS: Clinical data were collected from patients' medical charts. Sanger sequencing analyzed NLRP3. Response to anti-IL-1 blockade was evaluated by clinical assessments and by measurements of laboratory parameters...
January 9, 2017: Clinical and Experimental Rheumatology
Sotonye Tolofari, Asif Ansari, Ross J Knight
Primary and localized amyloidosis of the urinary tract is considered to be a rare clinical entity with approximately 160 cases documented in the literature. Here we present a case of a 77 year old gentleman with painless visible hematuria. Flexible cystoscopy revealed abnormal and polypoidal bladder mucosa. Transurethral resection of these lesions revealed histology with congo-red staining and positive green-apple birefringence, these findings are consistent with amyloidosis. There has so far been no sign of recurrence in this gentleman, with routine cystoscopic surveillance...
January 2017: Urology Case Reports
Sentaro Imamura, Shintaro Narita, Ryuta Nishikomori, Hiroshi Tsuruta, Kazuyuki Numakura, Atsushi Maeno, Mitsuru Saito, Takamitsu Inoue, Norihiko Tsuchiya, Hiroshi Nanjo, Toshio Heike, Shigeru Satoh, Tomonori Habuchi
BACKGROUND: Secondary bladder amyloidosis is an extremely rare disease, resulting from a chronic systematic inflammatory disorder associated with amyloid deposits. Although uncommon in Japan, familial Mediterranean fever (FMF) is a hereditary autoinflammatory disease characterized by recurrent episodes of fever of short duration and serositis and is frequently associated with systemic amyloidosis. Here, we present a case of a Japanese patient complaining of fever and macroscopic hematuria after a living donor renal transplantation...
October 19, 2016: BMC Research Notes
S C Karan, Amit Kumar Shah, Anand Srivastava, Reena Bhardwaj
No abstract text is available yet for this article.
January 2016: Medical Journal, Armed Forces India
Shameem Mahmood, Frank Bridoux, Christopher P Venner, Sajitha Sachchithanantham, Janet A Gilbertson, Dorota Rowczenio, Thomas Wagner, Rabya Sayed, Ketna Patel, Marianna Fontana, Carol J Whelan, Helen J Lachmann, Philip N Hawkins, Julian D Gillmore, Ashutosh D Wechalekar
BACKGROUND: Localised immunoglobulin light-chain amyloidosis, involving one type of tissue, is rare. Little systematic data exists regarding clinical presentations, course or outcomes, or risk of progression to systemic amyloidosis. We aimed to report clinical features and outcomes of a large series of patients with localised light-chain amyloidosis. METHODS: We examined data for all patients with localised amyloidosis who were diagnosed, assessed, and followed at the UK National Amyloidosis Centre (NAC) between Jan 2, 1980, and Dec 15, 2011, from the NAC database and written records...
June 2015: Lancet Haematology
Rashad S Barsoum
UNLABELLED: In this review, the clinical manifestations of urinary schistosomiasis are displayed from a pathogenetic perspective. According to the prevailing host's immune response profile, urinary schistosomiasis may be broadly categorized into cell-mediated and immune-complex-mediated disorders. The former, usually due to Schistosoma haematobium infection, are attributed to the formation of granulomata along the entire urinary tract. As they heal with excessive fibrosis, they may lead to strictures, calcifications and urodynamic abnormalities...
September 2013: Journal of Advanced Research
Rashad S Barsoum, Gamal Esmat, Tamer El-Baz
The clinical manifestations of schistosomiasis pass by acute, sub acute and chronic stages that mirror the immune response to infection. The later includes in succession innate, TH1 and TH2 adaptive stages, with an ultimate establishment of concomitant immunity. Some patients may also develop late complications, or suffer the sequelae of co-infection with other parasites, bacteria or viruses. Acute manifestations are species-independent; occur during the early stages of invasion and migration, where infection-naivety and the host's racial and genetic setting play a major role...
September 2013: Journal of Advanced Research
Christopher J Dru, Tom S Feng, Howard H Kim
Amyloidosis is a disorder of protein folding characterized by extracellular aggregation and deposition of amyloid protein fibrils. Light-chain amyloidosis, also known as primary systemic amyloidosis, is the most common form of the disease. We present a case of an 84-year-old male with a history of systemic primary amyloidosis causing genitourinary, cardiac, and autonomic dysfunction who presented with hematuria and hypotension secondary to bladder perforation. He underwent open repair of a large extraperitoneal bladder defect...
2014: Case Reports in Urology
Fang Zhou, Peng Lee, Ming Zhou, Jonathan Melamed, Fang-Ming Deng
Localized urinary tract amyloidosis (UTA) is a rare disease that mimics neoplasia clinically, cystoscopically, and radiologically. We report eleven cases of isolated UTA from the urinary bladder (n=7) and upper urinary tract including the ureter (n=2) and renal pelvis (n=2). All cases clinically presented as mass lesions prior to histologic examination and clinically suggested a neoplastic process. The amyloid composition in most cases was mixed Kappa and Lambda light chains. All cases were cured after surgical excision except one case which was diagnosed as plasmacytosis/plasmacytoma six months later...
2014: American Journal of Clinical and Experimental Urology
Takaaki Kobayashi, John Roberts, Jeffrey Levine, Justin Degrado
Primary bladder amyloidosis is a rare disease, with only 200 cases reported worldwide. This condition is clinically important since it masquerades as a malignancy. We herein present a case of primary bladder amyloidosis presenting as painless hematuria and dysuria. Computed tomography and ultrasound showed thickening of the posterior bladder wall. Cystoscopy revealed abnormal bladder tissue suspicious of malignancy. However, transurethral resection of a bladder tumor showed no evidence of malignant cells. Amyloid was identified on Congo red staining...
2014: Internal Medicine
Nelson Arturo Diez Calzadilla, José Antonio March Villalba, Adriana Canosa Fernández, Pilar Soriano Sarrió
OBJECTIVE: To present the therapeutic management of intractable hematuria secondary to systemic amyloidosis with bladder involvement. METHODS: We describe the clinical case, the medical management, the endo-urological technique used, and the results supported by relevant published literature. RESULTS: A 50-year-old woman with a 20-year history of rheumatoid arthritis in chronic treatment with corticosteroids and non-steroidal anti-inflammatory drugs in addition to chronic renal insufficiency not requiring hemodialysis...
September 2014: Archivos Españoles de Urología
Tori Ida, Kazunao Hayashi, Toru Ida, Satoko Hirano, Fumie Nakamura, Hirohito Sone, Masahiro Fujiwara
We present a systemic primary AL amyloidosis patient with severe gastrointestinal bleeding for whom treatment with bortezomib and dexamethasone was very effective. An 83-year-old woman was admitted to our hospital suffering from multiple gastric ulcers with bleeding. She had monoclonal protein (IgG, λ) in her serum. Systemic primary AL amyloidosis was diagnosed. She was also suspected of having amyloid deposition in cardiac muscle, as well as her bladder and intestinal mucosa. However, we were unable to carry out other invasive examinations due to her bleeding tendency...
July 2014: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
F M Klenke, C Wirtz, Y Banz, M J B Keel, N D Klass, U Novak, L M Benneker
Study Design Case report. Objectives With only two previously reported cases, localized amyloidosis of the sacrum is extremely rare. Here we report a 64-year-old woman with a large osteolytic lesion accompanied by weakness and paresthesia of the right leg and difficulties in bladder control. Methods Fine needle biopsy and standard staging procedures revealed a primary solitary amyloidoma that was treated with intralesional resection, lumbopelvic stabilization, and consolidation radiotherapy. Results Clinical follow-up revealed the diagnosis of multiple myeloma 9 months after initial treatment...
June 2014: Global Spine Journal
Jose David Jimenez-Parra, Diego Garcia-Gracia, Rosa Maria Guarch Troyas, Lorena Torres-Varas, Amaia Sotil Arrieta, Manuel Montesino Semper
OBJECTIVE: Amyloidosis is a disease characterised by deposition of eosinophilic hyaline material in different tissues. Urinary bladder involvement is uncommon with less than 200 cases of the primary form published in the literature. We present a new case of primary AA type amyloidosis of the urinary bladder (typical of secondary forms). METHODS: A 66-year-old male was seen in the outpatient urology consultation with several-weeks history intermittent haematuria with decreased voiding urinary calibre...
May 2014: Archivos Españoles de Urología
Katrine Skydsgaard Schou-Jensen, Claus Dahl, Anette Pedersen Pilt, Nessn Htum Azawi
Amyloidosis refers to a number of diseases characterized by extracellular deposition of misfolded proteins, called amyloid fibrils, in the tissues and organs of the body. Amyloidosis in the bladder is a generally localized, rare condition, with approximately 200 cases reported in the literature. This report presents three cases of amyloidosis in the bladder, two of which had coexisting transitional cell carcinoma. Evaluation for systemic disease is recommended in patients with newly discovered amyloidosis, even if first recognized in an area with the localized form, as in the bladder...
October 2014: Scandinavian Journal of Urology
E Gizard, A C Ford, J-P Bronowicki, L Peyrin-Biroulet
BACKGROUND: Extraintestinal manifestations are frequent in inflammatory bowel diseases (IBD). Most studies published so far focused on viral hepatitis and liver toxicity of IBD-related drugs. AIM: To conduct a systematic review of hepatobiliary manifestations associated with IBD. We excluded viral hepatitis and liver toxicity of IBD-related drugs. METHODS: Studies were identified through the electronic database of MEDLINE, EMBASE and the annual meetings of Digestive Disease Week, the American College of Gastroenterology, the United European Gastroenterology Week and the European Crohn's and Colitis Organization...
July 2014: Alimentary Pharmacology & Therapeutics
Chau Yee Ng, Wen-Hung Huang, Hsin-Chieh Huang, Li-Jen Wang, Shen-Yang Lee
No abstract text is available yet for this article.
May 2014: Kidney International
Kapeel Raja, Ejaz Ahmed, Muhammed Mubarak, Tanveer Iqbal, Syed Mujahid Hassan
Amyloidosis is a disorder of protein metabolism characterized by extracellular deposition of abnormal protein fibrils. It may either be localized to any organ or systematically distributed throughout the body. The biochemical nature of proteins varies but the physical and tinctorial properties are shared by all the amyloidogenic proteins. In the West, it is mainly composed of amyloid light (AL) type immunoglobulin (Ig) light chains. Amyloidosis of the genitourinary tract is rare except for the kidney and isolated primary amyloidosis of the urinary bladder is even rarer...
November 2013: Nephro-urology Monthly
Yijuan Sun, Amarpreet Sandhu, Darlene Gabaldon, Jonathan Danaraj, Karen S Servilla, Antonios H Tzamaloukas
AL amyloidosis complicating monoclonal gammopathy of undetermined significance (MGUS) has usually a predominant glomerular deposition of lambda light chain. Heavy proteinuria is one of its cardinal manifestations. A 78-year-old man with a 9-year history of IgG kappa light-chain-MGUS and normal urine protein excretion developed severe renal failure. Serum levels of kappa light chain and serum IgG had been stable while proteinuria was absent throughout the nine-year period. For the first eight years, he had stable stage III chronic kidney disease attributed to bladder outlet obstruction secondary to prostatic malignancy...
2012: Case Reports in Nephrology
Oluwatoyin F Bamgbola
Schistosomiasis is the second most common socio-economically devastating parasitic disease after malaria, affecting about 240 million residents of developing countries. In Africa, it predominantly manifests as urogenital disease, and the main infective agent is Schistosoma hematobium. Endemicity is propagated by poor socio-economic status and environmental degradation due to rapid urbanization. Recreational swimming is a potent medium for the spread of disease in children and adolescents. Most affected individuals are asymptomatic...
November 2014: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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