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https://www.readbyqxmd.com/read/28637108/-henoch-sch%C3%A3-nlein-purpura-presenting-as-intussusception
#1
Keun Young Kim
Henoch-Schönlein purpura (HSP) is systemic vasculitis disease with various clinical manifestations. Gastrointestinal symptoms in patients with HSP are usually common, with an incidence rate of 62-90%. Most of these gastrointestinal symptoms occur after typical skin purpura, which is a very important clinical evidence for making a diagnosis of HSP. It is difficult to diagnose HSP without skin rash. About 25% of patients may experience gastrointestinal symptoms as their first symptoms. Herein, we report a case of ileo-colic intussusception associated with HSP in a 5-years-old girl presented with diffuse abdominal distension...
June 25, 2017: Korean Journal of Gastroenterology, Taehan Sohwagi Hakhoe Chi
https://www.readbyqxmd.com/read/28629237/improved-pregnancy-outcome-following-earlier-splenectomy-in-women-with-immune-thrombocytopenia-a-5-year-observational-study
#2
Mohamed Rezk, Alaa Masood, Ragab Dawood, Mahmoud Emara, Hanan El-Sayed
OBJECTIVE: to assess prospectively the maternal and fetal outcome in women with immune thrombocytopenic purpura (ITP) who undergone earlier splenectomy compared to women on medical therapy. METHODS: a 5-year observational study included pregnant women in the first trimester previously diagnosed with primary ITP with 74 patients underwent splenectomy before pregnancy and 86 patients on medical therapy. Patients were followed throughout pregnancy and labor to record their obstetric outcome...
June 19, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28628467/juvenile-and-adult-onset-systemic-lupus-erythematosus-a-comparative-study-in-a-large-cohort-from-the-spanish-society-of-rheumatology-lupus-registry-relesser
#3
Vicenç Torrente-Segarra, Tarek Carlos Salman Monte, Iñigo Rúa-Figueroa, Fernando Sánchez-Alonso, Francisco Javier López-Longo, María Galindo-Izquierdo, Jaime Calvo-Alén, Alejandro Olivé-Marqués, Jesús Ibañez-Ruán, Loreto Horcada, Ana Sánchez-Atrio, Carlos Montilla, Rafael Benito Melero González, Elvira Díez-Álvarez, Victor Martinez-Taboada, José Luis Andreu, Olaia Fernández-Berrizbeitia, José Ángel Hernández-Beriain, Marian Gantes, Blanca Hernández-Cruz, Ángela Pecondón-Español, Carlos Marras, Gema Bonilla, José M Pego-Reigosa
OBJECTIVES: We aimed to describe juvenile-onset systemic lupus erythematosus (jSLE) features and to establish its differences compared to adult-onset SLE (aSLE) from a large national database. METHODS: Data from patients (≥4 ACR criteria) included in Spanish Society of Rheumatology Lupus Registry (RELESSER) were analysed. Sociodemographic, clinical, serological, activity, treatment, cumulative damage, comorbidities and severity data were collected. Patients with disease onset <18 years were described and compared to those with disease onset ≥18 years...
June 12, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28620827/asthma-foot-drop-and-palpable-purpura-in-a-young-woman
#4
Gabriela Kuftinec, Maha Sami, Paul Aronowitz
No abstract text is available yet for this article.
June 15, 2017: Journal of General Internal Medicine
https://www.readbyqxmd.com/read/28620491/pseudo-thrombotic-thrombocytopenic-purpura-presenting-as-multi-organ-dysfunction-syndrome-a-rare-complication-of-pernicious-anemia
#5
Saroj Kandel, Nibash Budhathoki, Shanta Pandey, Bikash Bhattarai, Aam Baqui, Ramesh Pandey, Divya Salhan, Danilo Enriquez, Joseph Quist, Frances M Schmidt
OBJECTIVE: We present a rare case of pernicious anemia presented as multi-organ dysfunction syndrome, later found to have pseudo-thrombotic thrombocytopenic purpura. METHODS: An 86-year-old female presented with respiratory distress, altered mental status, acute renal failure and was intubated in emergency room. She was found to have severe anemia, thrombocytopenia, high lactate, high lactate dehydrogenase and low haptoglobin. Peripheral smear revealed multilobulated neutrophils with schistocytes, poikilocytes and anisocytes...
2017: SAGE open medical case reports
https://www.readbyqxmd.com/read/28619707/purpura-in-a-5-year-old-girl
#6
Chi-Hone Lien, Ming-Dar Lee, Chien-Yu Lin
No abstract text is available yet for this article.
June 15, 2017: BMJ: British Medical Journal
https://www.readbyqxmd.com/read/28612584/purpura-fulminans-as-the-presenting-manifestation-in-a-patient-with-homozygous-methylenetetrahydrofolate-reductase-gene-mutation
#7
Ibrahim Piskin, Makbule Ercan, Nazmiye Yüksek, Ertug Toroslu, Zuhal Ornek
No abstract text is available yet for this article.
August 2017: Minerva Pediatrica
https://www.readbyqxmd.com/read/28612332/cocaine-levamisole-induced-vasculitis-vasculopathy-syndrome
#8
REVIEW
Javier Marquez, Lina Aguirre, Carolina Muñoz, Andres Echeverri, Mauricio Restrepo, Luis F Pinto
PURPOSE OF REVIEW: To understand the clinical spectrum of cocaine-levamisole-induced vasculitis. Worldwide recreational drug consumption is high among the adult population from various social strata. The use of cocaine with levamisole, a frequently added antiparasitic diluent, favors the manifestations of vasculitic lesions, especially in the skin. RECENT FINDINGS: New insights into immunological mechanisms involved in the pathogenesis of the disease. There are still many unknown aspects in the pathogenesis of this disease, such as the immune system interaction with p-ANCAs and the release of inflammatory NETs (neutrophil extracellular traps), which are the origin of auto-antigens and tissue damage, manifesting as vasculitic purpura on the skin...
June 2017: Current Rheumatology Reports
https://www.readbyqxmd.com/read/28609345/histopathologic-findings-of-cutaneous-hyperpigmentation-in-addison-disease-and-immunostain-of-the-melanocytic-population
#9
Angel Fernandez-Flores, David S Cassarino
The histopathological features of cutaneous hyperpigmentation in Addison disease have very occasionally been reported, and they include acanthosis, hyperkeratosis, focal parakeratosis, spongiosis, superficial perivascular lymphocytic infiltrate, basal melanin hyperpigmentation, and superficial dermal melanophages. We present a study on 2 biopsies from the arm and the thigh in a 77-year-old woman with a long clinical history of Addison disease as well as senile purpura and alopecia of female pattern. The patient presented diffuse hyperpigmentation of the skin, more pronounced on her face, and left upper forehead...
May 31, 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28607779/thrombotic-microangiopathy-secondary-to-intravenous-abuse-of-opana%C3%A2-er
#10
Kamia Thakur, Vaibhav Agrawal, Ashley Kass, Lauren M Dimarino, R Patrick Dorion, Joseph Vadakara
Opana ER (oxymorphone) is an opioid drug available throughout the United States, and intravenous abuse of the crushed oral formulation has been associated with drug-induced thrombotic microangiopathy. In this abstract, we describe two young patients who lived together and used Opana ER intravenously. Both presented with microangiopathic hemolytic anemia that mimicked thrombotic thrombocytopenic purpura (TTP). Treating this condition poses a clinical challenge, as it is difficult to distinguish it from TTP. The role for plasma exchange is not clear but can be used while awaiting the results of the ADAMTS-13 activity, but ultimately supportive care with drug discontinuation is the recommended therapy of choice...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28605168/characteristics-and-management-of-iga-vasculitis-henoch-sch%C3%A3-nlein-purpura-in-adults-data-from-the-260-patients-included-in-the-igavas-survey
#11
Alexandra Audemard-Verger, Benjamin Terrier, Agnès Dechartres, Johan Chanal, Zahir Amoura, Noémie Le Gouellec, Patrice Cacoub, Noémie Jourde-Chiche, Geoffrey Urbanski, Jean-François Augusto, Guillaume Moulis, Loic Raffray, Alban Deroux, Aurélie Hummel, Bertrand Lioger, Mélanie Catroux, Stanislas Faguer, Julie Goutte, Nihal Martis, François Maurier, Etienne Rivière, Sébastien Sanges, Aurélie Baldolli, Nathalie Costedoat-Chalumeau, Mélanie Roriz, Xavier Puéchal, Marc André, Christian Lavigne, Boris Bienvenu, Arsène Mekinian, Elie Zagdoun, Charlotte Girard, Alice Bérezné, Loïc Guillevin, Eric Thervet, Evangéline Pillebout
OBJECTIVES: Data on adult IgA vasculitis (IgAV) are scarce. This survey was designed to better define clinical spectrum and efficacy of treatments in this population. METHODS: We analyzed data from 260 patients with IgAV included in a French multicenter retrospective survey. RESULTS: Mean age at diagnosis was 50.1±18 years, and 63% were male. Baseline manifestations included purpura (100%), arthralgia (62%), glomerulonephritis (70%) or gastro-intestinal involvement (53%)...
June 12, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28598133/-therapeutic-efficacy-of-qingre-jiedu-and-huoxue-huayu-recipe-in-prednisone-dependant-patients-with-chronic-primary-immune-thrombocytopenic-purpura
#12
Yi-Ming Yang, Xue-Qiang Wu, Jian Li, Kai-Ji Zhang
OBJECTIVES: To observe the clinical efficacy of Qingre Jiedu and Huoxue Huayu Recipe on the prednisone-dependant patients with chronic primary immune thrombocytopenic purpura (CPITP). METHODS: Fifty prednisone-dependant CPITP patients were treated with Qingre Jiedu and Huoxue Huayu Recipe orally one dose a day,the dosage of prednisone for these patients was tapered according to the monitoring result of blood platelet count (BPC).The therapeutic efficacy in these patients was evaluated before and after Chinese medicine treatment over 4 weeks...
November 2016: Sichuan da Xue Xue Bao. Yi Xue Ban, Journal of Sichuan University. Medical Science Edition
https://www.readbyqxmd.com/read/28597906/platelet-receptors-as-therapeutic-targets-past-present-and-future
#13
Janina Jamasbi, Keng Ayabe, Shinya Goto, Bernhard Nieswandt, Karlheinz Peter, Wolfgang Siess
Anti-platelet drugs reduce arterial thrombosis after plaque rupture and erosion, prevent stent thrombosis and are used to prevent and treat myocardial infarction and ischaemic stroke. Some of them may also be helpful in treating less frequent diseases such as thrombotic thrombocytopenic purpura. The present concise review aims to cover current and future developments of anti-platelet drugs interfering with the interaction of von Willebrand factor (VWF) with glycoprotein (GP) Ibα, and directed against GPVI, GPIIb/IIIa (integrin αIIbβ3), the thrombin receptor PAR-1, and the ADP receptor P2Y12...
June 8, 2017: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/28596653/neonatal-outcomes-of-pregnancy-with-immune-thrombocytopenia
#14
Nuriye Aslı Melekoğlu, Ali Bay, Elif H Aktekin, Mehmet Yilmaz, Ercan Sivasli
Neonates born to mothers with immune thrombocytopenia (ITP) have an increased risk for neonatal thrombocytopenia and hemorrhagic complications. The aim of this study was to determine the maternal and neonatal outcomes of pregnancies with ITP and also to identify risk factors that predicts neonatal thrombocytopenia. We performed a retrospective analysis of 40 pregnancies with ITP and their 40 neonates. Among the 40 neonates, thrombocytopenia (platelet count of less than 150 × 10(9)/L) was detected in 15 neonates (37...
June 2017: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/28596255/thrombotic-thrombocytopenic-purpura-associated-with-pegylated-interferon-alfa-2a-use-in-a-patient-with-polycythemia-vera
#15
Radhika Gangaraju, Soo J Kim, Jing-Fei Dong, Sabina Swierczek, Josef T Prchal
Pegylated interferon alfa-2a (pegIFNa) is being increasingly used for treatment of myeloproliferative neoplasms; however, its side effects, including autoimmune complications, are not unusual. We report on a 47-year-old woman with polycythemia vera (PV) treated with pegIFNa and in complete hematologic remission who developed thrombotic thrombocytopenic purpura (TTP). To our knowledge, thrombotic microangiopathy has been reported as a side effect of interferon (IFN) use in patients with hepatitis and chronic myeloid leukemia, but not in those with PV...
June 2017: Journal of the National Comprehensive Cancer Network: JNCCN
https://www.readbyqxmd.com/read/28596204/malignant-hypertension-as-a-rare-cause-of-thrombotic-microangiopathy
#16
Guramrinder Thind, Karthik Kailasam
Malignant hypertension can occasionally be associated with microangiopathic haemolytic anaemia. A 38-year-old male presented with nausea, vomiting, loss of appetite and oliguria for 2 weeks. He was diagnosed with hypertensive emergency with cardiac and renal dysfunction. Interestingly, further workup was diagnostic for the presence of thrombotic microangiopathy (TMA): haemoglobin =12.7 g/dL, indirect bilirubin =2.0 mg/dL, haptoglobin ≤6 mg/dL, platelet count =121 000/μL and schistocytes on peripheral smear...
June 8, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28593405/inducible-nitric-oxide-synthase-gene-polymorphisms-are-associated-with-a-risk-of-nephritis-in-henoch-sch%C3%A3-nlein-purpura-children
#17
Jue Jiang, Wuqiong Duan, Xu Shang, Hua Wang, Ya Gao, Peijun Tian, Qi Zhou
Henoch-Schönlein purpura (HSP) is the most common form of systemic small-vessel vasculitis in children, and HSP nephritis (HSPN) is a major complication of HSP and is the primary cause of morbidity and mortality. Previous studies have suggested that inducible nitric oxide synthase (iNOS) may play an important role in the pathogenesis of HSP. In this study, we performed a detailed analysis to investigate the potential association between iNOS polymorphisms and the risk of HSP and the tendency for children with HSP to develop HSPN in a Chinese Han population...
June 8, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28592769/thrombotic-complications-after-hematopoietic-stem-cell-transplantation
#18
Shosaku Nomura
Hematopoietic stem cell transplantation (HSCT) involves some serious transplant-associated complications (TACs) or vascular disorders, such as veno-occlusive disease (VOD), thrombotic microangiopathy (TMA), and graft-versus-host disease (GVHD). VOD is related to a clinical syndrome characterized by tender hepatomegaly, jaundice, fluid retention, and unexplained weight gain. When TMA is described in patients who have undergone HSCT, it is often implied that the clinical diagnosis of TMA is similar to that of thrombotic thrombocytopenic purpura...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28591945/-effect-of-ifn-%C3%AE-2b-on-cox-2-and-angiogenesis-in-jak2v617f-mutation-myeloproliferative-neoplasms
#19
Ya-Ling Zhao, Li-Jun Zhang, Jian-Zhu Fu, Qian Xu, Gui-Min Liu, Xu-Lei Xie, Wen-Tong Liang, Zhi-Yon Cheng
OBJECTIVES: To investigate the influence of interferon-alpha-2b (IFN-α2b) with JAK2 kinase, COX-2 and microvessel density in patients of MPN and the relation of JAK2V617F and COX-2 in human erythroleukemia cell line (HEL) cells. METHODS: Forty-two cases of MPN patients with JAK2V617F mutation of initial treatment were collected from the Frist hospital of Baoding, including the IFN-α2b treatment group with 17 cases and untreated group with 25 cases. 10 cases of idiopathic immune thrombocytopenic purpura (ITP) patients synchronization were enrolled as controls...
July 2016: Sichuan da Xue Xue Bao. Yi Xue Ban, Journal of Sichuan University. Medical Science Edition
https://www.readbyqxmd.com/read/28591051/association-between-red-blood-cell-distribution-width-and-henoch-schonlein-purpura-nephritis
#20
Hui Xu, Wei Li, Jian-Hua Mao, Yan-Xiang Pan
To investigate whether red blood cell distribution width (RDW) is a marker of the risk of Henoch-Schonlein purpura (HSP) nephritis (HSPN), a total of 669 HSP patients and 168 healthy controls were included in this retrospective study. Two hundred fifty-six (38.3%) of the patients had kidney involvement. Compared with the HSP group, RDW was significantly higher in the HSPN group (P < .001). Binary logistic regression identified that HSPN was independently associated with age, RDW, platelet, and total cholesterol (odds ratio = 1...
June 2017: Medicine (Baltimore)
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