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https://www.readbyqxmd.com/read/28516871/diagnosis-of-catastrophic-anti-phospholipid-syndrome-in-a-patient-tested-negative-for-conventional-tests
#1
Fabrizio Conti, Roberta Priori, Cristiano Alessandri, Roberta Misasi, Antonella Capozzi, Monica Pendolino, Simona Truglia, Silvia Frisenda, Maurizio Sorice, Guido Valesini
Catastrophic antiphospholipid syndrome (CAPS) is a severe variant of APS, characterised by clinical evidence of multiple organ involvement developing over a very short period of time, histopathological evidence of multiple small vessel occlusions and laboratory confirmation of the presence of aPL (lupus anticoagulant and/or anticardiolipin antibodies and/or anti-Beta2-glcyoprotein I antibodies). Here we report a case of a 39-year-old woman patient who developed a CAPS which was negative to the conventional aPL but positive for aPL in thin layer chromatography immunostaining and vimentin/cardiolipin antibodies by ELISA test...
May 8, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28515608/thrombotic-thrombocytopenic-purpura-in-a-case-of-dengue-fever-a-rare-presentation
#2
Abhijit S Gavali, Jayant Shelgaonkar, Sandip Bartakke
Here, we present an unusual occurrence of thrombotic thrombocytopenic purpura (TTP) in a case of dengue fever. Both the conditions are fatal and can result in significant mortality and morbidity if left untreated. In this case, as soon as, we diagnosed the patient as having TTP, we treated her with plasma exchange therapy, steroids, and monoclonal antibodies such as rituximab. The patient responded very well to the treatment and completely recovered from neurological symptoms and laboratory parameters also normalized...
April 2017: Indian Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/28515156/anti-glomerular-basement-membrane-disease
#3
Stephen P McAdoo, Charles D Pusey
Anti-glomerular basement membrane (anti-GBM) disease is a rare small vessel vasculitis that affects the capillary beds of the kidneys and lungs. It is an archetypic autoimmune disease, caused by the development of directly pathogenic autoantibodies targeting a well characterized autoantigen expressed in the basement membranes of these organs, although the inciting events that induce the autoimmune response are not fully understood. The recent confirmation of spatial and temporal clustering of cases suggests that environmental factors, including infection, may trigger disease in genetically susceptible individuals...
May 17, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/28514704/guillain-barr%C3%A3-syndrome-variants-forms-fruste-reclassification-with-new-criteria
#4
Fu Liong Hiew, Rahmansah Ramlan, Shanthi Viswanathan, Santhi Puvanarajah
OBJECTIVES: This study aimed to evaluate the clinical and electrophysiological characteristics of various distinctive classical and localised Guillain-Barré syndrome (GBS) subtypes. PATIENTS AND METHODS: Clinical characteristics and electrophysiological data of sixty-one consecutive patients admitted between 2012 and 2015 were systematically analysed and reclassified according to the new GBS clinical classification. Neurophysiology was evaluated with Hadden et al...
May 4, 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28512098/a-rare-case-of-thrombotic-microangiopathy-triggered-by-acute-pancreatitis
#5
Kevin Singh, Ahmed Jamal Nadeem, Behzad Doratotaj
Thrombotic microangiopathy (TMA) occurring after acute pancreatitis is rarely described. Without prompt intervention, TMA can be, and often is, lethal, so prompt recognition is important. Here, we present a case of a 61-year-old woman with a history of alcohol misuse who presented with epigastric pain, nausea and vomiting after binge drinking. Elevated serum lipase and imaging were suggestive of acute-on-chronic pancreatitis. Although the patient's symptoms of acute pancreatitis subsided, her anaemia, thrombocytopenia and acute kidney injury worsened...
May 15, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28509135/renal-limited-vasculitis-with-elevated-levels-of-multiple-antibodies
#6
Noriaki Sato, Hideki Yokoi, Hirotaka Imamaki, Eiichiro Uchino, Kaoru Sakai, Takeshi Matsubara, Tatsuo Tsukamoto, Sachiko Minamiguchi, Motoko Yanagita
Renal-limited vasculitis (RLV) is a type of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis that presents with crescentic glomerulonephritis with no other organ involvement. Although several studies reported patients with crescentic glomerulonephritis who were dual positive for proteinase 3 (PR3)-ANCA and myeloperoxidase (MPO)-ANCA or ANCA and anti-glomerular basement membrane (GBM) antibody, patients positive for all three antibodies, i.e., triple-positive patients, were rarely reported. We herein report the case of a male with pauci-immune type crescentic glomerulonephritis positive for MPO-ANCA, PR3-ANCA, and anti-GBM antibody...
March 3, 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28509118/anti-glomerular-basement-membrane-disease-accompanied-by-systemic-lupus-erythematosus-presenting-central-nervous-system-involvement
#7
Hirohito Sugawara, Hideki Takizawa, Yoshinosuke Shimamura, Norihito Moniwa, Koichi Hasegawa, Yayoi Ogawa
We report a case of rapidly progressive glomerulonephritis caused by anti-glomerular basement membrane (GBM) disease accompanied by systemic lupus erythematosus (SLE) presenting central nervous system involvement in a 32-year-old Japanese male. He was admitted to our hospital because of a 3-week history of fever and rapidly failing renal function requiring hemodialysis (HD). Laboratory tests showed anti-GBM antibody elevation with a value of 16,385 units/ml. On day 85, he had generalized tonic-clonic seizure...
August 27, 2016: CEN Case Reports
https://www.readbyqxmd.com/read/28508984/successful-treatment-of-myeloma-cast-nephropathy-using-bortezomib-based-chemotherapy-plus-selective-plasma-exchange
#8
Mayuko Kawabe, Izumi Yamamoto, Ai Katsuma, Naomi Hayashi, Yo Komatsuzaki, Yasuyuki Nakada, Akihiro Shimizu, Yudo Tanno, Ichiro Ohkido, Nobuo Tsuboi, Kazuhito Suzuki, Takaki Shimada, Yoji Ogasawara, Katsuki Sugiyama, Keisuke Aiba, Takashi Yokoo
Myeloma cast nephropathy is a major complication of multiple myeloma. Recent evidence has demonstrated that the earlier induction of bortezomib-based chemotherapy with plasma exchange (PE) provides better results for kidney function and patient survival. Due to its non-selectivity, PE with albumin replacement carries the risk of fibrinogen loss, leading to bleeding. We herein report a case of successful treatment of myeloma cast nephropathy using bortezomib-based chemotherapy and selective PE. A 61-year-old woman who had a 20-year history of type II diabetes mellitus was admitted to our hospital for the evaluation of hypercalcemia, severe kidney dysfunction, and anemia...
November 2016: CEN Case Reports
https://www.readbyqxmd.com/read/28508980/an-adult-case-of-severe-steroid-resistant-henoch-sch%C3%A3-nlein-purpura-nephritis-treated-with-intravenous-cyclophosphamide-and-tonsillectomy
#9
Emi Sasaki, Maki Shibata, Asami Kato, Naoto Hamano, Takashi Katsuki, Manami Tada, Fumihiko Hinoshita
A previously healthy 37-year-old Canadian man living in Japan visited a hospital in Thailand while traveling because of edematous legs, purpura, arthralgia, bloody stool, and fever after an insect bite. Henoch-Schönlein purpura (HSP) was suspected. His creatinine level was 5.2 mg/dL. He was treated with oral prednisolone (PSL) and oral cyclophosphamide (CPA); after treatment, his creatinine level improved to 2.4 mg/dL. Upon returning to Japan, he was admitted to the National Center for Global Health and Medicine Hospital in Tokyo...
November 2016: CEN Case Reports
https://www.readbyqxmd.com/read/28508977/thrombotic-microangiopathy-caused-by-interferon-%C3%AE-1b-for-multiple-sclerosis-a-case-report
#10
Haruomi Nishio, Tatsuo Tsukamoto, Takeshi Matsubara, Yoichiro Okada, Ryosuke Takahashi, Motoko Yanagita
A 41-year-old man with a history of multiple sclerosis (MS) developed thrombotic microangiopathy after taking interferon β-1b for 10 years. Although the relapse of his MS was well controlled under normal blood pressure, he had persistent nausea, anorexia, gait disturbance and visual disorder 1 month before admission. He showed lethargy and high blood pressure (180/102 mmHg). Laboratory test results revealed anemia and thrombocytopenia, elevated LDH and renal dysfunction. Urinary dipstick showed a 2+ result for proteinuria and 3+ for hematuria...
November 2016: CEN Case Reports
https://www.readbyqxmd.com/read/28502324/favorable-long-term-outcomes-of-isolated-liver%C3%A2-transplantation-in-a-child-with-atypical-hemolytic-uremic-syndrome-caused-by-a%C3%A2-novel%C3%A2-complement-factor-h-mutation%C3%A2
#11
Heeyeon Cho, Yeonhee Lee
Atypical hemolytic uremic syndrome (aHUS) is a rare syndrome characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury that is usually caused by complement dysregulation. Complement factor H (CFH) is a regulator of the complement system produced in the liver, and CFH gene mutations are the most frequent causes of aHUS. To date, the therapeutic options for aHUS with CFH mutations have consisted of plasma infusions, plasma exchange, kidney transplantation, isolated liver transplantation, or combined liver and kidney transplantation...
May 15, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28500622/implementation-of-a-rapid-assay-of-adamts13-activity-was-associated-with-improved-30-day-survival-rate-in-patients-with-acquired-primary-thrombotic-thrombocytopenic-purpura-who-received-platelet-transfusions
#12
Yumi Yoshii, Yoshihiro Fujimura, Charles L Bennett, Ayami Isonishi, Norio Kurumatani, Masanori Matsumoto
BACKGROUND: Platelet (PLT) transfusions are probably harmful in patients with acquired idiopathic thrombotic thrombocytopenic purpura (aTTP). Introduction of a rapid assay for ADAMTS13 activity should reduce the time to definite diagnosis of aTTP, reduce the amount of inappropriately transfused PLT concentrates, and improve mortality and morbidity. STUDY DESIGN AND METHODS: We selected 265 aTTP patients with severe ADAMTS13 deficiency. Of these, 91 patients were diagnosed by March 2005 (Period 1), when ADAMTS13 activity was measured by von Willebrand factor multimer assay, which took 4 to 7 days until the result was reported...
May 12, 2017: Transfusion
https://www.readbyqxmd.com/read/28499586/-gemcitabine-induced-thrombotic-microangiopathy-can-we%C3%A2-improve-screening-and%C3%A2-treatment
#13
Xavier Charmetant, Anne Jolivot, Thomas Fournier, Jean-Charles Puthet, Philippe Cassier, Sandrine Lemoine, Laurent Juillard
Thrombotic microangiopathy is a rare but severe complication of treatment with gemcitabine. Its prevalence increases because gemcitabine's indications are growing. We report four cases, which presented with common clinical and biological manifestations, i.e. high blood pressure, proteinuria and increasing plasmatic creatinine level. However, severity was not similar, hemodialysis was inconstant. There is no consensus on treatment for this condition. Stopping gemcitabine is essential. Treatment was dispensed considering the severity of the presentation: plasma exchange therapy of variable outcome, and eculizumab, which was efficient when used...
May 9, 2017: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/28495143/central-and-peripheral-nervous-system-immune-mediated-demyelinating-disease-after-allogeneic-hematopoietic-stem-cell-transplantation
#14
M I Stefanou, F Bischof
OBJECTIVE: We aimed to evaluate clinical and diagnostic features of central and peripheral immune-mediated demyelinating disease (CPID) in allogeneic hematopoietic stem cell transplantation (aHSCT) recipients. BACKGROUND: CPID refers to the late-onset, immune-mediated neurological complications following aHSCT, when other frequent differential diagnoses have been ruled out, and when symptoms and signs of systemic GvHD manifestations are absent. METHODS: Case records at the University of Tuebingen, between 2001 and 2015, were screened to identify patients with CPID after aHSCT...
June 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28495137/early-predictors-of-rapidly-evolving-multiple-sclerosis-a-case-report
#15
Kryshani T M Fernando, Michael James
Objective The aim of this case report is to highlight some important features of rapidly evolving Multiple Sclerosis. BACKGROUND: In a small proportion of patients, Multiple Sclerosis (MS) can present as a fulminant disease characterised by severe and frequent relapses. This form of rapidly evolving MS is associated with significant morbidity and mortality. It is therefore important to identify these patients as early as possible, so that they can be managed effectively. However, due to the rarity of fulminant forms of MS, there is limited data on the natural history and management of this condition...
June 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28488841/catastrophic-antiphospholipid-syndrome-an-update
#16
Gerard Espinosa, Ignasi Rodriguez-Pinto, Ricard Cervera
Catastrophic antiphospholipid syndrome (CAPS) is a rare variant that accounts for 1% of patients with APS. Despite its low frequency, the mortality-related is very high ranging from 50% of patients in the first series to 37% in the most recent data. The current knowledge of this potential devastating entity comes from the International Registry of patients with CAPS, named CAPS Registry. Small vessel thrombosis, laboratory features of microangiopathic haemolytic anemia, and development of multisystem involvement in a very short period of time are the main characteristics of this syndrome...
May 8, 2017: Panminerva Medica
https://www.readbyqxmd.com/read/28488614/safety-and-efficacy-of-therapeutic-membrane-plasmapheresis-in-the-treatment-of-guillain-barr%C3%A3-syndrome-a-study-from-a-tertiary-care-hospital-from-india
#17
Sanjay Vikrant, Surinder Thakur, Ashok Sharma, Dalip Gupta, Sudhir Sharma
BACKGROUND: Reports on therapeutic plasma exchange (TPE) with the standard hemodialysis equipment are scarce, particularly from developing countries. MATERIALS AND METHODS: A retrospective analysis of safety and efficacy of membrane-based TPE with a standard hemodialysis equipment for the treatment of severe Guillain-Barré syndrome (GBS) was conducted. RESULTS: A total of 120 TPE sessions were performed in 31 GBS patients over a period of 5½ years...
May 2017: Neurology India
https://www.readbyqxmd.com/read/28485075/a-prospective-study-comparing-tryptophan-immunoadsorption-with-therapeutic-plasma-exchange-for-the-treatment-of-chronic-inflammatory-demyelinating-polyneuropathy
#18
Ina Lieker, Torsten Slowinski, Lutz Harms, Katrin Hahn, Juliane Klehmet
BACKGROUND: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare paralyzing inflammatory neuropathy with probably autoimmune origin. While plasma exchange (PE) constitutes a first-line treatment option for CIDP, there is only little known about the efficacy and safety of immunoadsorption (IA), a more selective apheresis procedure with assumed better tolerability. METHODS: In this prospective-randomized pilot trial, patients were randomly assigned to receive 6 sessions of PE (n = 10) or IA (n = 10) treating equal plasma volumes...
May 9, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/28485072/therapeutic-plasma-exchange-in-children-one-center-s-experience
#19
Gerard Cortina, Violeta Ojinaga, Thomas Giner, Magdalena Riedl, Siegfried Waldegger, Alejandra Rosales, Raphaela Trojer, Johannes Hofer
BACKGROUND: Therapeutic plasma exchange (TPE) has evolved to an accepted therapy for selected indications. However, it is technically challenging in children. Moreover, data on safety and efficacy are mainly derived from adult series. The aim of this study was to review the procedure in the context of clinical indications, effectiveness, and safety. STUDY DESIGN AND METHODS: All TPE procedures performed at a tertiary care hospital during a 12-year period (2005-2016) were retrospectively evaluated...
May 9, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/28485027/membrane-therapeutic-plasma-exchange-with-and-without-heparin-anticoagulation
#20
Bruna Brunetta Gavranić, Nikolina Bašić-Jukić, Vedran Premužić, Petar Kes
BACKGROUND: Administration of an anticoagulant during therapeutic plasma exchange (TPE) is necessary to avoid circuit clotting and impaired treatment effectiveness. Citrate is the preferred anticoagulant for apheresis worldwide, and unfractionated heparin (UH) is the second most preferred, yet there are only a few published studies regarding the use of UH during TPE. There are even fewer studies regarding the use of low-molecular-weight heparin (LMWH) and TPE performed without anticoagulation...
May 9, 2017: Journal of Clinical Apheresis
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