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https://www.readbyqxmd.com/read/28328098/plasma-exchange-successfully-treated-macrophage-activation-syndrome-in-rheumatoid-factor-positive-polyarticular-juvenile-idiopathic-arthritis-with-combined-pneumonia
#1
Lianjie Shi, Fanlei Hu, Chuanhui Xu, Huaqun Zhu, Di Qie, Chuanjie Yuan, Yuhong Tao, Hanmin Liu
Macrophage activation syndrome (MAS) is one of the serious complications associated with rheumatic diseases, especially systemic juvenile idiopathic arthritis (sJIA). Here we describe a 9-year-old girl with rheumatoid factor (RF)-positive polyarticular JIA, not sJIA, combined with pneumonia who was successfully treated by plasma exchange. She was diagnosed with RF-positive polyarticular JIA based on positive RF and multiple joint swelling and tenderness 3 years ago. She was admitted in our hospital with myalgia for 2 days and a high fever for half a day...
March 22, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28325251/interferon-induced-thrombotic-microangiopathy-tma-analysis-and-concise-review
#2
REVIEW
Ajay Kundra, Jen Chin Wang
Interferon (IFN) has been associated with development of thrombotic microangiopathy including thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). We reviewed literature from the earliest reported association in 1993, to July 2016 and found 68 cases. Analysis of this data shows: (1) Mean age at diagnosis was 47 years (95% CI, 44-50). (2) Majority of cases were seen where IFN was used for the treatment of chronic myelogenous leukemia (CML), multiple sclerosis (MS), chronic hepatitis C virus infection (HCV) and one case each for hairy cell leukemia (HCL) and Sezary syndrome...
April 2017: Critical Reviews in Oncology/hematology
https://www.readbyqxmd.com/read/28321086/acquired-thrombotic-thrombocytopenic-purpura-with-a-smoldering-clinical-course
#3
Chiaki Naito, Yoshiyuki Ogawa, Kunio Yanagisawa, Takuma Ishizaki, Masahiro Mihara, Masaki Hayakawa, Masanori Matsumoto, Yoshihisa Nojima, Hiroshi Handa
Acquired thrombotic thrombocytopenic purpura (aTTP) is caused by a deficiency of ADAMTS13 activity due to neutralizing auto-autoantibodies (inhibitors) against ADAMTS13. Patients with aTTP show a rapid and fatal clinical course, unless an effective therapeutic intervention such as plasma exchange therapy (PEX) is performed. There is, however, a small population of patients who show a smoldering clinical course, for which no effective treatment strategy has yet been established. We herein report a 77-year-old man, who had repeated episodes of cerebral infarction and persistent thrombocytopenia over several months, but was not correctly diagnosed as having aTTP at a local hospital...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28321080/the-improvement-of-the-outcome-of-osmotic-demyelination-syndrome-by-plasma-exchange
#4
Saeko Kumon, Ryosuke Usui, Shinzo Kuzuhara, Kosaku Nitta, Minako Koike
A 71-year-old Japanese woman presented with progressive fatigue, lethargy, dysarthria and a gait disorder. Her laboratory data revealed hyponatremia (Na 101 mEq/L), and we started correcting her serum sodium level. Within a few days, she became comatose, bedridden, and was intubated. We diagnosed osmotic demyelination syndrome (ODS) and started performing plasma exchange (PE) on the 39th day of hospitalization. She fully recovered after starting PE, and was discharged on foot unassisted. PE can be a beneficial treatment in patients with chronic ODS...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28319287/iatrogenic-thyrotoxicosis-and-the-role-of-therapeutic-plasma-exchange
#5
Kabeer K Shah, Michael M Mbughuni, Edwin A Burgstaler, Darci R Block, Jeffrey L Winters
Thyroid storm or severe thyrotoxicosis results from extreme thyroid hormone elevation. Therapy includes medical management to prevent hormone production, release, recycling, and peripheral conversion while stabilizing adrenergic tone. Thyroid dysfunction is the usual cause but it can be due to excessive thyroid hormone ingestion. Therapeutic plasma exchange (TPE) has been used to rapidly remove protein-bound thyroid hormone. American Society for Apheresis guidelines make a weak recommendation to perform TPE in selected patients in the treatment of thyrotoxicosis based on low quality evidence...
March 20, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/28316443/low-density-lipoprotein-apheresis-in-a-pediatric-patient-of-familial-hypercholesterolemia-primi-experientia-from-a-tertiary-care-center-in-north-india
#6
Kanchan Dogra, Alpesh Goyal, Rajesh Khadgawat, Yashdeep Gupta, Diptiranjan Rout, Parag Prabhakar Fulzele, Rahul Chaurasia, Poonam Coshic, Kabita Chatterjee
Familial hypercholesterolemia (FH) is an autosomal dominant disorder due to mutation of apolipoprotein-B receptor gene causing severe dyslipidemia. Lifestyle modification and medical treatment attenuate the disease progression, but as these fail to control the blood cholesterol levels, low-density lipoprotein (LDL) apheresis comes forth as a treatment option. To the best of our knowledge, the following is the very first case of pediatric FH being treated by LDL-apheresis to be reported from India. A severely malnourished female child presented with yellowish skin lesions over different parts of the body, viz...
January 2017: Asian Journal of Transfusion Science
https://www.readbyqxmd.com/read/28316440/abo-incompatible-renal-transplant-transfusion-medicine-perspective
#7
Raj Nath Makroo, Sweta Nayak, Mohit Chowdhry, Sanjiv Jasuja, Gaurav Sagar, N L Rosamma, Uday Kumar Thakur
INTRODUCTION: Our study presents an analysis of the trends of ABO antibody titers and the TPE (Therapeutic Plasma Exchange) procedures required pre and post ABO incompatible renal transplant. MATERIALS AND METHODS: Twenty nine patients underwent ABO incompatible renal transplant during the study period. The ABO antibody titers were done using the tube technique and titer reported was the dilution at which 1+ reaction was observed. The baseline titers of anti-A and anti-B antibodies were determined...
January 2017: Asian Journal of Transfusion Science
https://www.readbyqxmd.com/read/28304106/therapeutic-plasma-exchange-versus-double-plasma-molecular-absorption-system-in-hepatitis-b-virus-infected-acute-on-chronic-liver-failure-treated-by-entercavir-a-prospective-study
#8
Yue-Meng Wan, Yu-Hua Li, Zhi-Yuan Xu, Jing Yang, Li-Hong Yang, Ying Xu, Jin-Hui Yang
BACKGROUND: Therapeutic plasma exchange (TPE) and double plasma molecular absorption system (DPMAS) were two extracorporeal liver support systems. Few studies compared their efficacy profile. OBJECTIVE: This study was to compare the efficacy of TPE and DPMAS on acute-on-chronic liver failure (ACLF) caused by hepatitis B virus (HBV-ACLF). METHODS: 60 HBV-ACLF patients were enrolled and prospectively studied. All patients received entecavir therapy, and were assigned to TPE group (n = 33) and DPMAS group (n = 27)...
March 17, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/28302661/severe-co-trimoxazole-induced-hypoglycaemia-in-a-patient-with-microscopic-polyangiitis
#9
Thomas Edward Conley, Atif Mohiuddin, Noshaba Naz
A 69-year-old man presented to the emergency department with lower respiratory tract infection and febrile neutropaenia. He was recently discharged following a 50-day hospital stay with newly diagnosed microscopic polyangiitis, complicated by pulmonary haemorrhage and severe renal dysfunction requiring renal replacement therapy, plasma exchange and immunosuppression (cyclophosphamide and methylprednisolone). High risk of pneumocystis pneumonia (PCP) led to an escalation in treatment from prophylactic to therapeutic oral co-trimoxazole, alongside broad-spectrum antibiotics...
March 16, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28295573/therapeutic-plasma-exchange-for-perioperative-management-of-patients-with-congenital-factor-xi-deficiency
#10
Mohamed S Alsammak, Aneel A Ashrani, Jeffrey L Winters, Rajiv K Pruthi
BACKGROUND: Factor XI (FXI) deficiency (hemophilia C [HEM-C]) is a bleeding disorder with unpredictable severity that correlates poorly with FXI coagulation activity (FXI:C). It poses a perioperative hemostatic management challenge. For US patients with severe disease, fresh frozen plasma (FFP) or, in current use, thawed plasma is the most readily available option but comes with risk of volume overload. We report our experience of using therapeutic plasma exchange (TPE) as an alternative perioperative management strategy...
March 15, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/28279164/erratum-to-a-new-prescription-model-for-regional-citrate-anticoagulation-in-therapeutic-plasma-exchanges
#11
Sébastien Kissling, Cécile Legallais, Menno Pruijm, Daniel Teta, Bruno Vogt, Michel Burnier, Eric Rondeau, Christophe Ridel
No abstract text is available yet for this article.
March 9, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28271646/two-treatment-cases-of-severe-fever-and-thrombocytopenia-syndrome-with-oral-ribavirin-and-plasma-exchange
#12
In Park, Hye In Kim, Ki Tae Kwon
Severe fever with thrombocytopenia syndrome (SFTS) is an emerging tick-borne disease. The primary symptoms associated with SFTS are fever, thrombocytopenia, leukopenia, nausea, and vomiting. Disease progression shows high mortality rate accompanied with multiple organ failure, bleeding tendency, and altered mentality. However, only supportive care has been the basis for the treatment of SFTS. We are reporting two patients who showed central nervous system manifestation, but cured them with ribavirin together with plasma exchange in an early state...
January 19, 2017: Infection & Chemotherapy
https://www.readbyqxmd.com/read/28266045/outcomes-in-necrotizing-soft-tissue-infections-treated-with-therapeutic-plasma-exchange
#13
Nabiha Huq Saifee, Heather L Evans, Amalia S Magaret, John R Hess, Meghan Delaney, Grant E O'Keefe, Tam N Pham, Hugh Foy, Eileen Bulger, Monica B Pagano
BACKGROUND: Treatment of necrotizing soft tissue infections (NSTIs) includes prompt surgical debridement and antibiotics, but despite standard care, the morbidity and mortality remain high. Since therapeutic plasma exchange (TPE) has been considered for treatment of severe sepsis, this study evaluates the efficacy of TPE for patients with NSTI. STUDY DESIGN AND METHODS: This is a retrospective study of patients with diagnosis of NSTI who received treatment with and without TPE over an 11-year period...
March 7, 2017: Transfusion
https://www.readbyqxmd.com/read/28265581/impact-of-desensitization-on-antiviral-immunity-in-hla-sensitized-kidney-transplant-recipients
#14
Mieko Toyoda, Bong-Ha Shin, Shili Ge, James Mirocha, David Thomas, Maggie Chu, Edgar Rodriguez, Christine Chao, Anna Petrosyan, Odette A Galera, Ashley Vo, Jua Choi, Alice Peng, Joseph Kahwaji, Stanley C Jordan
Viral infections represent significant morbidity and mortality factors in kidney transplant recipients, with CMV, EBV, and BKV infections being most common. Desensitization (DES) with IVIg and rituximab with/without plasma exchange followed by kidney transplantation with alemtuzumab induction increased successful transplant rates in HLA-sensitized patients but may represent an increased risk for viral infections due to severe lymphocyte depletion. Here, we report on the posttransplant viral infection status in 372 DES versus 538 non-DES patients...
2017: Journal of Immunology Research
https://www.readbyqxmd.com/read/28265287/pernicious-anemia-associated-cobalamin-deficiency-and-thrombotic-microangiopathy-case-report-and-review-of-the-literature
#15
Farhanah Yousaf, Bruce Spinowitz, Chaim Charytan, Marilyn Galler
A 43-year-old Hispanic male without significant previous medical history was brought to emergency department for syncope following a blood draw to investigate a 40 lbs weight loss during the past 6 months associated with decreased appetite and progressive fatigue. The patient also reported a 1-month history of jaundice. On examination, he was hemodynamically stable and afebrile with pallor and diffuse jaundice but without skin rash or palpable purpura. Normal sensations and power in all extremities were evident on neurological exam...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28260457/early-versus-late-acute-antibody-mediated-rejection-among-renal-transplant-recipients-in-terms-of-response-to-rituximab-therapy-a-single-center-experience
#16
Osama Gheith, Torki Al-Otaibi, Medhat A Halim, Tarek Mahmoud, Prasad Nair, Mohamed Abdel Monem, Salah Al-Waheeb, Rashad Hassan, Narayanam Nampoory
OBJECTIVES: There are no comparable trials concerning the use of rituximab among renal transplant recipients with acute antibody-mediated rejection. Here, we compared early and late acute antibody-mediated rejection in renal transplant recipients in terms of response to rituximab therapy. MATERIALS AND METHODS: Of 1230 kidney transplants performed at Hamed Al-Essa Organ Transplant Center (Kuwait) over the past 10 years, 103 recipients developed acute antibody-mediated rejections and were subcategorized into 4 groups according to the onset of rejection and rituximab treatment...
February 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28259863/-review-of-the-psychiatric-aspects-of-anti-nmda-n-methyl-d-aspartic-acid-receptor-encephalitis-case-report-and-our-plans-for-a-future-study
#17
Levente Herman, Ildiko Reka Zsigmond, Laszlo Peter, Janos M Rethelyi
Anti-NMDAR (N-methyl-D-aspartic acid receptor) encephalitis, first described in 2007, is a rare, autoimmune limbic encephalitis. In half of the cases anti-NMDAR antibodies are paraneoplastic manifestations of an underlying tumor (mostly ovarian teratoma). In the early stage of the disease psychiatric symptoms are prominent, therefore 60-70% of the patients are first treated in a psychiatric department. In most of the cases, typical neurological symptoms appear later. Besides the clinical picture and typical symptoms, verifying presence of IgG antibodies in the serum or CSF is necessary to set up the diagnosis...
December 2016: Neuropsychopharmacologia Hungarica
https://www.readbyqxmd.com/read/28259520/derivation-and-external-validation-of-the-plasmic-score-for-rapid-assessment-of-adults-with-thrombotic-microangiopathies-a-cohort-study
#18
Pavan K Bendapudi, Shelley Hurwitz, Ashley Fry, Marisa B Marques, Stephen W Waldo, Ang Li, Lova Sun, Vivek Upadhyay, Ayad Hamdan, Andrew M Brunner, John M Gansner, Srinivas Viswanathan, Richard M Kaufman, Lynne Uhl, Christopher P Stowell, Walter H Dzik, Robert S Makar
BACKGROUND: Among the syndromes characterised by thrombotic microangiopathy, thrombotic thrombocytopenic purpura is distinguished by a severe deficiency in the ADAMTS13 enzyme. Patients with this disorder need urgent treatment with plasma exchange. Because ADAMTS13 activity testing typically requires prolonged turnaround times and might be unavailable in resource-poor settings, a method to rapidly assess the likelihood of severe ADAMTS13 deficiency is needed. METHODS: All consecutive adult patients presenting to three large academic medical centres in Boston, MA, USA, with thrombotic microangiopathy and a possible diagnosis of thrombotic thrombocytopenic purpura between Jan 8, 2004, and Dec 6, 2015, were included in an ongoing multi-institutional registry (the Harvard TMA Research Collaborative)...
March 1, 2017: Lancet Haematology
https://www.readbyqxmd.com/read/28259232/guillain-barr%C3%A3-syndrome
#19
REVIEW
Eelco F M Wijdicks, Christopher J Klein
Guillain-Barré syndrome is an acute inflammatory immune-mediated polyradiculoneuropathy presenting typically with tingling, progressive weakness, and pain. Variants and formes frustes may complicate recognition. The best known variant is the sensory ataxic form of Miller Fisher syndrome, which also affects the oculomotor nerves and the brain stem. Divergent pathologic mechanisms lead to demyelinating, axonal, or mixed demyelinating-axonal damage. In the demyelinating form, yet to be identified antigens are inferred by complement activation, myelin destruction, and macrophage-activated cleanup...
March 2017: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/28250608/complements-spurned-our-experience-with-atypical-hemolytic-uremic-syndrome
#20
Vidya S Nagar, Rudrarpan Chaterjee, Ankita Sood, Basavaraj Sajjan, Aniruddha Kaushik, Sameer V Vyahalkar
Atypical hemolytic uremic syndrome (aHUS) is a rare disorder resulting from a dysregulated activation of the alternative pathway of the complement system. It results in significant morbidity and mortality if not diagnosed and treated promptly. It lends itself to myriad renal and extrarenal manifestations, all potentially disabling. Eculizumab, a monoclonal antibody to complement C5 is now the widely accepted norm for treatment. However, in resource-limited settings, plasma exchange if instituted early may be as beneficial...
February 2017: Indian Journal of Critical Care Medicine
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