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https://www.readbyqxmd.com/read/29786866/characterization-of-efficacy-and-safety-of-pathogen-inactivated-and-quarantine-plasma-in-routine-use-for-treatment-of-acquired-immune-thrombotic-thrombocytopenic-purpura
#1
R Herbrecht, M Ojeda-Uribe, D Kientz, C Fohrer, A Bohbot, O Hinschberger, K-L Liu, E Remy, C Ernst, J-S Lin, L Corash, J P Cazenave
BACKGROUND: Auto-immune thrombotic thrombocytopenic purpura (TTP) is a morbid multi-organ disorder. Cardiac involvement not recognized in initial disease descriptions is a major cause of morbidity. Therapeutic plasma exchange (TPE) requires exposure to multiple plasma donors with risk of transfusion-transmitted infection (TTI). Pathogen inactivation (PI) with amotosalen-UVA, the INTERCEPT Blood System for Plasma (IBSP) is licensed to reduce TTI risk. METHODS: An open-label, retrospective study evaluated the efficacy of quarantine plasma (QP) and IBSP in TTP and defined treatment emergent cardiac abnormalities...
May 21, 2018: Vox Sanguinis
https://www.readbyqxmd.com/read/29782427/clinical-profile-of-levamisole-adulterated-cocaine-induced-vasculitis-vasculopathy-a-30-case-series
#2
Carlos Horacio Muñoz-Vahos, Sebastián Herrera-Uribe, Álvaro Arbeláez-Cortés, Daniel Jaramillo-Arroyave, Luis Alonso González-Naranjo, Gloria Vásquez-Duque, Mauricio Restrepo-Escobar, Luis Alfonso Correa-Londoño, Luis Fernando Arias-Restrepo, Adriana Lucía Vanegas-García
OBJECTIVES: The aims of this study were to describe clinical and laboratory manifestations of patients with levamisole-adulterated cocaine-induced vasculitis/vasculopathy and to propose a skin classification according to the distribution and severity of lesions. METHODS: We report the characteristics of 30 patients admitted with levamisole-adulterated cocaine-induced vasculitis/vasculopathy in 4 high-complexity institutions in Colombia, from December 2010 to May 2017...
May 19, 2018: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/29780064/systemic-lupus-erythematosus-and-sj%C3%A3-gren-s-syndrome-complicated-by-conversion-disorder-a-case-report
#3
Misa Nakamura, Seiji Tanaka, Tadashi Inoue, Yasuto Maeda, Kiyohito Okumiya, Takuya Esaki, G O Shimomura, Kenji Masunaga, Shinichiro Nagamitsu, Yushiro Yamashita
Conversion disorder (CD) is sometimes accompanied by motor and sensory impairments, such as muscle weakness, paralysis, sensory hypersensitivity, and sensory loss. Sjögren's syndrome (SS) complicates 5-10% of cases of systemic lupus erythematosus (SLE). Patients with SS or SLE present with various neurological symptoms and psychiatric manifestations. When neurological symptoms are present, it is important to distinguish whether the symptoms are caused by a neurological or a mental disorder because the former requires early intensive intervention, such as methylprednisolone pulse therapy (MPT), whereas psychotherapy or antidepressant drugs are recommended for mental disorders...
May 21, 2018: Kurume Medical Journal
https://www.readbyqxmd.com/read/29779928/the-diagnosis-and-clinical-management-of-the-catastrophic-antiphospholipid-syndrome-a-comprehensive-review
#4
REVIEW
Ricard Cervera, Ignasi Rodríguez-Pintó, Gerard Espinosa
The catastrophic antiphospholipid syndrome (CAPS) is a life-threating variant of the antiphospholipid syndrome characterized by the development of multiple thrombosis in a short period of time, usually ending up in the failure of function of several vital organs. Most CAPS episodes are related to a prothrombotic situation or precipitating factor such as infections, surgical procedures or malignant diseases. In patients with CAPS, the development of multiple thrombosis leads to an important cytokine release that worsens the already critical patient's situation...
May 17, 2018: Journal of Autoimmunity
https://www.readbyqxmd.com/read/29777164/mrna-treatment-produces-sustained-expression-of-enzymatically-active-human-adamts13-in-mice
#5
Susan Liu-Chen, Brendan Connolly, Lei Cheng, Romesh R Subramanian, Zhaozhong Han
Thrombotic thrombocytopenic purpura (TTP) is primarily caused by deficiency of ADAMTS13 within the blood stream due to either genetic defects or presence of inhibitory autoantibodies. Preclinical and clinical studies suggest that enzyme replacement therapy with recombinant human ADAMTS13 protein (rhADAMTS13) is effective and safe in treatment of TTP. However, frequent dosing would be required due to the relatively short half-life of rhADAMTS13 in circulation as well as the presence of inhibitory autoantibodies that collectively result in the poor pharmacological profile of rhADAMTS13...
May 18, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29776888/iga-vasculitis-as-a-presentation-of-human-immunodeficiency-virus-infection
#6
Anahy M Brandy-García, Jorge Santos-Juanes, Silvia Suarez, Luis Caminal-Montero
IgA vasculitis is a small-vessel vasculitis mediated by immune complexes. In clinical terms, it is characterized by palpable purpura in the lower limbs, joint involvement in the form of arthralgia or arthritis, and gastrointestinal and renal involvement (this will mark a poorer prognosis in adults). Infectious processes, mainly in the upper respiratory tract, are frequently found to be triggers. On the other hand, human immunodeficiency virus (HIV) causes immune dysfunction, which triggers hypergammaglobulinemia and can trigger autoimmune disorders...
May 15, 2018: Reumatología Clinica
https://www.readbyqxmd.com/read/29772615/-a-case-of-renal-pelvic-cancer-with-acquired-hemophilia-and-leukemoid-reaction
#7
Daiki Ikarashi, So Omori, Wataru Izumida, Yasuyuki Nakamura, Masakazu Abe, Takashi Ujiie, Wataru Obara
A 71-year-old man was referred to us with a right renal mass that was discovered by computed tomography (CT) examination for acquired hemophilia and leukemoid reaction. He presented with persistent low-grade fever and purpura on the lower legs caused by acquired hemophilia. Contrastenhanced CT scan showed a right renal tumor 6.0×7.4 cm in diameterwith inhomogeneous enhancement. The result of his urine cytology was negative. After improvement of his coagulation by treatment with immunosuppressants and steroids, he underwent open nephrectomy...
April 2018: Hinyokika Kiyo. Acta Urologica Japonica
https://www.readbyqxmd.com/read/29771863/upshaw-schulman-syndrome-with-c-2728c-t-mutation-in-adamts13-gene
#8
Shahzadi Resham, Zehra Fadoo, Bushra Moiz
Congenital thrombotic thrombocytopenic purpura is a rare autosomal recessive disorder presenting with hemolytic anemia, thrombocytopenia, micro vascular thrombosis, and end organ damage. Here, we present a case of a 7-year-old girl having recurrent neonatal hemolysis, developmental delay, frequent seizures, and thrombocytopenia. Characteristic clinical picture and gene sequencing of a disintegrin and metalloproteinase with thrombospondin motifs 13 confirmed the diagnosis of Upshaw-Schulman syndrome. She was treated successfully with plasma infusion...
May 16, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29770208/case-report-polyarteritis-nodosa-or-complicated-henoch-schonlein-purpura-a-rare-case
#9
Sajad Hasanzadeh, Seyedeh Maryam Alavi, Elahe Masnavi, Maryam Rohani, Saeid Jokar
Background: Polyarteritis nodosa (PAN) is a vasculitis that affects medium sized arteries. PAN is a rare disease and requires a high vilgilance for diagnosis. For instance, PAN and Henoch-Schonlein purpura (HSP) have narrowing differential diagnosis. Here, we report a case of PAN. Case presentation : Our patient was a 65 year old woman that came to hospital due to abdominal pain and skin lesion on the right upper and right lower extremities. All rheumatologic tests were negative. A biopsy of the skin lesion was reported as mild hyperkeratosis, slight spongiosis with intact basal layer...
2018: F1000Research
https://www.readbyqxmd.com/read/29765791/cutaneous-leukocytoclastic-vasculitis-following-influenza-vaccination-in-older-adults-report-of-bullous-purpura-in-an-octogenarian-after-influenza-vaccine-administration
#10
Stella X Chen, Philip R Cohen
The influenza vaccination is recommended annually for protection against influenza infection. Adults over 65 years of age are especially vulnerable to complications from influenza infection; in addition, they constitute the largest group of influenza vaccination recipients each year. Cutaneous leukocytoclastic vasculitis involves inflammation of small vessel walls by neutrophils. An 88-year-old man with a history of idiopathic pulmonary fibrosis who developed bullous cutaneous leukocytoclastic vasculitis 14 days after receiving the influenza vaccine is described and the characteristics of influenza-associated cutaneous leukocytoclastic vasculitis in older individuals are reviewed...
March 14, 2018: Curēus
https://www.readbyqxmd.com/read/29764574/-clinical-effect-and-mechanism-of-hemoperfusion-in-treatment-of-children-with-severe-abdominal-henoch-sch%C3%A3-nlein-purpura
#11
Ying Zhu, Yang Dong, Da-Liang Xu, Jia-Yun Jiang, Lin Wu, Rui-Juan Ke, Shao-Han Fang, Yin Peng
OBJECTIVE: To study the clinical effect and mechanism of hemoperfusion (HP) in the treatment of children with severe abdominal Henoch-Schönlein purpura (HSP). METHODS: A total of 24 children with severe abdominal HSP were divided into two groups: conventional treatment and HP (n=12 each). Ten healthy children who underwent physical examination were enrolled as the control group. Before and after treatment, chemiluminescence was used to measure the serum levels of interleukin-6 (IL-6) and tumor necrosis factor-α (TNF-α); thiobarbituric acid colorimetry was used to measure the plasma level of malondialdehyde (MDA); the hydroxylamine method was used to measure the plasma level of superoxide dismutase (SOD); chemical colorimetry was used to measure the plasma level of total anti-oxidant capability (T-AOC)...
May 2018: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/29763513/child-onset-thrombotic-thrombocytopenic-purpura-caused-by-p-r498c-and-p-g259pfsx133-mutations-in-adamts13
#12
An-Sofie Schelpe, Christelle Orlando, Bogaç Ercig, Chloë Geeroms, Inge Pareyn, Nele Vandeputte, Leydi Carolina Velásquez Pereira, Elien Roose, Karel Fostier, Gerry A F Nicolaes, Hans Deckmyn, Simon F De Meyer, Karen Vanhoorelbeke, Kristin Jochmans
INTRODUCTION: Patients suffering from congenital thrombotic thrombocytopenic purpura (cTTP) have a deficiency in ADAMTS13 due to mutations in their ADAMTS13 gene. OBJECTIVE: The aim of this study was to determine ADAMTS13 parameters (activity, antigen and mutations), to investigate if the propositus suffered from child-onset cTTP and to study the in vitro effect of the ADAMTS13 mutations. METHODS: ADAMTS13 activity and antigen were determined using the FRETS VWF73 assay and ELISA and ADAMTS13 mutations via sequencing of the exons...
May 15, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29761111/myocardial-infarction-as-an-early-presentation-in-thrombotic-thrombocytopenic-purpura-a-rare-case-series
#13
Sumit Dahal, Dipesh K C Ghimire, Saroj Sapkota, Suyash Dahal, Paritosh Kafle, Manjul Bhandari
Renal and neurological involvements are frequently seen in thrombotic thrombocytopenic purpura (TTP). Cardiac involvement, however, has been rarely reported. In this article, we present 2 cases of myocardial infarction in patients with TTP. In the first case, a young man presented with non-ST-segment elevation myocardial infarction that resolved promptly with plasmapheresis. The second patient developed ST-segment elevation myocardial infarction early in the course of the disease and died before plasmapheresis could be initiated...
January 2018: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/29758989/the-double-hazard-of-bleeding-and-thrombosis-in-hemostasis-from-a-clinical-point-of-view-a-global-assessment-by-rotational-thromboelastometry-rotem
#14
Olga Meltem Akay
Hemostasis is a complex dynamic process involving bleeding and thrombosis as two end-points. Conventional coagulation tests which are measured in plasma examine only isolated portions of the coagulation cascade, thereby giving no information on important interactions essential to the clinical evaluation of hemostatic function. Thromboelastography (TEG), originally described in 1948 has improved over the decades and become a valuable tool of coagulation testing because of the limitations of standard coagulation tests...
January 1, 2018: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/29756710/mefv-gene-mutations-and-clinical-course-in-pediatric-patients-with-henoch-sch%C3%A3-nlein-purpura
#15
Emrah Can, Zubeyde Kılınç Yaprak, Şahin Hamilçıkan, Meltem Erol, Özlem Bostan Gayret Y Özgül Yiğit
OBJECTIVE: To determine the frequency of the MEFV gene mutations in pediatric patients diagnosed with HSP and to assess the effect of the MEFV gene mutations on their prognosis. Material and Methods. Ccross-sectional study; pediatric patients between 2-11 years diagnosed with HSP were included. These cases were investigated for 6 MEFV gene mutations (M694V, M680I, A744S, R202Q, K695R, E148Q). RESULTS: Eighty cases were included in the study of which 55% were male (n= 44)...
June 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29755528/hypogammaglobulinemia-observed-one-year-after-rituximab-treatment-for-idiopathic-thrombocytopenic-purpura
#16
Bilal Ahmad Shoukat, Osama Ali, Dileep Kumar, Muhammad Bilal Gilani, Adeela Zahid, Shaheer Aslam Joiya, Maqsood Anwar Malik
We present the case of a 19-year-old female with severe hypogammaglobulinemia after having had treatment with rituximab for idiopathic thrombocytopenic purpura requiring intravenous immunoglobulins. She was admitted with the diagnosis of left-sided pneumonia with parapneumonic effusion. The patient was treated with piperacillin/tazobactam after having a poor response to co-amoxiclav. The patient had been tested for immunoglobulin levels, and the levels were very low. She has a history of ITP for which she received steroids...
2018: Case Reports in Medicine
https://www.readbyqxmd.com/read/29753419/severe-renal-failure-and-thrombotic-microangiopathy-induced-by-malignant-hypertension-successfully-treated-with-spironolactone
#17
H M Villafuerte Ledesma, J M Peña Porta, P Iñigo Gil, P Martin Azara, J E Ruiz Lalglesia, A Tomás LaTorre, A Martínez Burillo, P Vernet Perna, R Álvarez Lipe
Malignant hypertension can cause thrombotic microangiopathy (TMA) characterized by hemolytic anemia and thrombocytopenia. On the other hand, severe hypertension is sometimes associated with hemolytic uremic syndrome (HUS) or thrombotic thrombocytopenic purpura (TTP). Distinguishing these entities is important because of therapeutic implications. Plasmapheresis should be initiated as soon as possible if we are dealing with TTP. We describe the case of a 30-year-old man referred to our hospital with malignant hypertension, severe renal failure and TMA: haemoglobin=9g/dL, total bilirubin=0...
May 9, 2018: Annales de Cardiologie et D'angéiologie
https://www.readbyqxmd.com/read/29751935/down-regulation-of-mir-218-5p-promotes-apoptosis-of-human-umbilical-vein-endothelial-cells-through-regulating-high-mobility-group-box-1-in-henoch-schonlein-purpura
#18
Shao-Fei Yu, Wan-Yu Feng, Shao-Qing Chai, Xiao-Bo Meng, Zhong-Xia Dou, Hua Zhu
BACKGROUND: Apoptosis of human umbilical vein endothelial cells (HUVECs) plays an important role in the progression of Henoch-Schonlein purpura (HSP). In the present study, we explored the function of miR-218-5p in HUVEC apoptosis and HSP development. MATERIALS AND METHODS: HSP rat model was established and peripheral blood mononuclear cells (PBMC) were isolated. The expression of miR-218-5p and high-mobility group box-1 (HMGB1) protein in HUVECs was determined by quantitative real-time polymerase chain reaction and western blot, respectively...
April 5, 2018: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/29751784/henoch-sch%C3%A3-nlein-purpura-with-acute-pancreatitis-analysis-of-13-cases
#19
Qin Zhang, Qi Guo, Ming Gui, Zhenhua Ren, Bo Hu, Ling Lu, Fang Deng
BACKGROUND: Henoch-Schönlein purpura is a common small vessel vasculitis in children. Acute pancreatitis rarely presents as a complication of Henoch-Schönlein purpura and has not been well characterized. METHODS: We retrospectively reviewed 13 cases of Henoch-Schönlein purpura with acute pancreatitis among 3212 patients who attended our hospital between January 2003 and June 2016 and analyzed their clinical characteristics, laboratory findings, imaging findings, treatment and overall prognosis...
May 11, 2018: BMC Pediatrics
https://www.readbyqxmd.com/read/29751499/survival-and-prognostic-factors-in-mixed-cryoglobulinemia-data-from-246-cases
#20
Cesare Mazzaro, Luigino Dal Maso, Endri Mauro, Valter Gattei, Michela Ghersetti, Pietro Bulian, Giulia Moratelli, Gabriele Grassi, Francesca Zorat, Gabriele Pozzato
INTRODUCTION: The clinical and therapeutic management of mixed cryoglobulinemia (MC) remains a subject of controversy. In addition, most studies have not recorded the long-term follow-up and the outcome of these cases. MATERIAL AND METHODS: We enrolled 246 patients affected by MC who were consecutively admitted to our Department from January 1993 to February 2013. Clinical and biological data had been recorded until June 2014. RESULTS: The median age (at diagnosis) was 60 years (range 26⁻83)...
May 3, 2018: Diseases (Basel)
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