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https://www.readbyqxmd.com/read/29062486/cardiac-tamponade-in-a-patient-with-autoimmune-polyglandular-syndrome-type-2
#1
Andromachi Vryonidou, Stavroula A Paschou, Fotini Dimitropoulou, Panagiotis Anagnostis, Vasiliki Tzavara, Apostolos Katsivas
We describe a case of a 40-year-old woman who was admitted to the intensive care unit with a rapid onset of dyspnea and orthopnea. She presented progressive weakness, weight loss and secondary amenorrhea during last year, while intermittent fever was present for the last two months. Initial biochemical evaluation showed anemia, hyponatremia and increased C-reactive protein levels. Clinical and echocardiographic evaluation revealed cardiac tamponade, which was treated with pericardiocentesis. Pleural fluid samples were negative for malignancy, tuberculosis or bacterial infection...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28905254/use-of-eculizumab-in-a-systemic-lupus-erythemathosus-patient-presenting-thrombotic-microangiopathy-and-heterozygous-deletion-in-cfhr1-cfhr3-a-case-report-and-systematic-review
#2
REVIEW
Maria Izabel de Holanda, Luis Cristóvão Pôrto, Teresa Wagner, Luis Fernando Christiani, Lilian M P Palma
The association of thrombotic microangiopathy (TMA) with systemic lupus erythematosus (SLE) has been described in 0.5 to 10% of cases, and patients present worse outcome. TMA is described as the association of microangiopathic hemolytic anemia, thrombocytopenia, and an organ injury, frequently the kidney. This study describes a successful case of use of eculizumab in a patient with SLE and TMA refractory to standard therapy, and provides a literature review. Case description and search in PubMed and MEDLINE using systemic lupus erythemathous and/or antiphospholipid syndrome (APS) and eculizumab retrieved 15 case reports...
December 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28638678/detailed-features-of-hematological-involvement-and-medication-induced-cytopenia-in-systemic-lupus-erythematosus-patients-single-center-results-of-221-patients
#3
Hava Üsküdar Teke, Döndü Üsküdar Cansu, Cengiz Korkmaz
OBJECTIVE: Systemic lupus erythematosus (SLE) may affect a number of systems, with the hematological system being one of the most common. Our aim is to determine the existence of cytopenia at diagnosis or during follow-up of our SLE patients as well as the associated factors. MATERIAL AND METHODS: A cohort of SLE patients that had been followed-up in the Department of Rheumotology from 1998 to 2015 was retrospectively assessed. Clinical and laboratory findings about the patients were recorded...
June 2017: European Journal of Rheumatology
https://www.readbyqxmd.com/read/28620416/antiphospholipid-syndrome-multiple-manifestations-in-a-single-patient-a-high-suspicion-is-still-needed
#4
Uroosa Ibrahim, Shiksha Kedia, Gwenalyn Garcia, Jean Paul Atallah
Antiphospholipid Syndrome (APS) is an autoimmune disorder with clinical and laboratory features of vascular thrombosis, pregnancy loss, and persistent antiphospholipid antibodies (aPLs). The pathophysiology is thought to involve the activation of endothelial cells, monocytes, platelets, and complement by aPLs. Disease can range from asymptomatic to rapidly fatal catastrophic APS. We present a case of a 34-year-old male referred for pancytopenia and splenomegaly. On examination, he had decreased sensation and 4/5 power in the left upper extremity...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28488841/catastrophic-antiphospholipid-syndrome-an-update
#5
Gerard Espinosa, Ignasi Rodríguez-Pintó, 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...
September 2017: Panminerva Medica
https://www.readbyqxmd.com/read/28355988/direct-oral-anticoagulants-an-alternative-treatment-for-thrombotic-antiphospholipid-syndrome
#6
A S Resseguier, B Pereira, V Rieu, G Le Guenno, V Grobost, M Ruivard
Background Direct oral anticoagulants (DOACs) demonstrate a lower risk-benefit ratio than vitamin K antagonists (VKAs) for secondary thromboprophylaxis of thrombotic events. But there are no data on the efficacy of DOACs for the prevention of thrombotic recurrence in patients with antiphospholipid syndrome (APS). In this study, we evaluated the efficacy of DOACs to prevent recurrences of thrombotic events in patients with APS. Methods This was a single-center pilot, using a multi-step Fleming design. If seven or fewer patients presented treatment failure with rivaroxaban, the study could conclude efficacy...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28302165/bilateral-adrenal-hemorrhage-in-the-background-of-escherichia-coli-sepsis-a-case-report
#7
Jahanzaib Khwaja
BACKGROUND: Sepsis is a syndrome of life-threatening organ dysfunction caused by a dysregulated host response to infection. It can have devastating consequences, including bilateral adrenal hemorrhage, particularly in patients at high thrombotic risk, such as those with antiphospholipid syndrome and those on long-term anticoagulation. CASE PRESENTATION: A 49-year-old white woman re-presented to hospital with a history suggestive of sepsis. She had a medical background of primary antiphospholipid syndrome on lifelong warfarin...
March 17, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28277850/diagnosis-and-management-of-catastrophic-antiphospholipid-syndrome
#8
REVIEW
Or Carmi, Maya Berla, Yehuda Shoenfeld, Yair Levy
Catastrophic antiphospholipid syndrome (CAPS) is a rare, life-threatening disease. In 1992, Asherson defined it as a widespread coagulopathy related to the antiphospholipid antibodies (aPL). CAPS requires rapid diagnosis and prompt initiation of treatment. Areas covered: This paper discusses all aspects of CAPS, including its pathophysiology, clinical manifestations, diagnostic approaches, differential diagnoses, management and treatment of relapsing CAPS, and its prognosis. To obtain the information used in this review, scientific databases were searched using the key words antiphospholipid antibodies, catastrophic antiphospholipid syndrome, hemolytic anemia, lupus anticoagulant, and thrombotic microangiopathic hemolytic anemia...
April 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28246490/cystic-adrenal-lesions-focus-on-pediatric-population-a-review
#9
REVIEW
Mara Carsote, Adina Ghemigian, Dana Terzea, Ancuta Augustina Gheorghisan-Galateanu, Ana Valea
BACKGROUND AND AIM: The cysts may potentially affect any organ; adrenals cysts are rare. This is a review of the literature regarding adrenal cysts, focusing on children and young adults. GENERAL DATA: Three major types have been described: pure cysts (endothelial, epithelial, and hemorrhagic or pseudocyst), parasitic (as hydatid) cysts and cystic part of a tumour (most frequent are neuroblastoma, ganglioneuroma, pheocromocytoma, and teratoma). The complications are: bleeding, local pressure effects; infection; rupture (including post-traumatic); arterial hypertension due to renal vessels compression...
2017: Clujul Medical (1957)
https://www.readbyqxmd.com/read/27898514/a-rare-combination-of-thrombotic-thrombocytopenic-purpura-and-antiphospholipid-syndrome
#10
Maya Viner, Irina Murakhovskaya
: Thrombocytopenia, in the setting of microangiopathic hemolytic anemia and thrombotic events, is characteristic of both thrombotic thrombocytopenic purpura and primary antiphospholipid syndrome. Clinically, it is difficult to distinguish between these two syndromes. We present a 41-year-old woman with chronic, relapsing thrombotic thrombocytopenic purpura in the presence of antiphospholipid antibodies. She had clinical manifestations of antiphospholipid syndrome without meeting laboratory criteria of the Sydney classification system...
July 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/27776934/the-antiphospholipid-syndrome-in-patients-with-systemic-lupus-erythematosus
#11
REVIEW
Guillermo J Pons-Estel, Laura Andreoli, Francesco Scanzi, Ricard Cervera, Angela Tincani
The antiphospholipid syndrome (APS) is an autoimmune disease characterized by the occurrence of venous and/or arterial thrombosis and pregnancy morbidity in the presence of pathogenic autoantibodies known as antiphospholipid antibodies (aPL). APS may be associated with other diseases, mainly systemic lupus erythematosus (SLE). The presence or absence of SLE might modify the clinical or serological expression of APS. Apart from the classical manifestations, APS patients with associated SLE more frequently display a clinical profile with arthralgias, arthritis, autoimmune hemolytic anemia, livedo reticularis, epilepsy, glomerular thrombosis, and myocardial infarction...
January 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/27708976/the-clinical-significance-of-antiphospholipid-antibodies-in-systemic-lupus-erythematosus
#12
REVIEW
Ozan Ünlü, Stephane Zuily, Doruk Erkan
Antiphospholipid syndrome (APS) is the association of thrombosis and/or pregnancy morbidity with antiphospholipid antibodies (aPL). Thirty to forty percent of systemic lupus erythematosus (SLE) patients are tested positive for aPL, which may have an impact on the SLE presentation, management, and prognosis. Compared with SLE patients without aPL, those with aPL have a higher prevalence of thrombosis, pregnancy morbidity, valve disease, pulmonary hypertension, livedo reticularis, thrombocytopenia, hemolytic anemia, acute/chronic renal vascular lesions, and moderate/severe cognitive impairment; worse quality of life; and higher risk of organ damage...
June 2016: European Journal of Rheumatology
https://www.readbyqxmd.com/read/27504588/aire-mutations-and-autoimmune-disease
#13
REVIEW
Øyvind Bruserud, Bergithe E Oftedal, Anette B Wolff, Eystein S Husebye
The gene causing the severe organ-specific autoimmune disease autoimmune polyendocrine syndrome type-1 (APS-1) was identified in 1997 and named autoimmune regulator (AIRE). AIRE plays a key role in shaping central immunological tolerance by facilitating negative selection of T cells in the thymus, building the thymic microarchitecture, and inducing a specific subset of regulatory T cells. So far, about 100 mutations have been identified. Recent advances suggest that certain mutations located in the SAND and PHD1 domains exert a dominant negative effect on wild type AIRE resulting in milder seemingly common forms of autoimmune diseases, including pernicious anemia, vitiligo and autoimmune thyroid disease...
December 2016: Current Opinion in Immunology
https://www.readbyqxmd.com/read/27442513/pregnancy-outcomes-in-chinese-patients-with-systemic-lupus-erythematosus-sle-a-retrospective-study-of-109-pregnancies
#14
Ming Ku, Shuiming Guo, Weifeng Shang, Qing Li, Rui Zeng, Min Han, Shuwang Ge, Gang Xu
Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease that primarily affects women during their reproductive years. The interaction between SLE and pregnancy remains debated. The objective of this study was to analyze the fetal and maternal outcomes of Chinese women with SLE. A total of 109 pregnancies in 83 SLE patients from June 2004 to June 2014 at a tertiary university hospital were reviewed retrospectively. Patients' characteristics, clinical and laboratory data during pregnancy were obtained from electronic medical records...
2016: PloS One
https://www.readbyqxmd.com/read/26950981/-renal-thrombotic-microangiopathy-and-antiphospholipid-syndrome-nephropathy-in-a-patient-with-lupus-nephritis
#15
Yusuke Sakamaki, Konosuke Konishi, Akinori Hashiguchi, Shigeki Tomita, Eiji Kubota, Hiroshi Itoh, Koichi Hayashi
The patient was a 48-year-old Japanese woman diagnosed as having systemic lupus erythematosus at the age of 21 years when she presented with fever and an erythematous skin rash on her face and extremities. Prednisolone was initiated at that time. Thirteen days before admission to our hospital, she was referred to us by her family physician. Upon admission, blood tests showed pancytopenia, hypocomplementemia, and renal dysfunction, as well as the presence of lupus anticoagulant. Urinalysis showed abundant proteinuria and heavy microscopic hematuria...
2016: Nihon Jinzo Gakkai Shi
https://www.readbyqxmd.com/read/26928404/association-of-autoimmune-thyroid-diseases-chronic-atrophic-gastritis-and-gastric-carcinoid-experience-from-a-single-institution
#16
C Castoro, R Le Moli, M L Arpi, M Tavarelli, G Sapuppo, L Frittitta, S Squatrito, G Pellegriti
PURPOSE: Autoimmune polyendocrine syndromes (APS) type III are characterized by the association of autoimmune thyroid disease (ATD) with other autoimmune diseases such as diabetes, alopecia, pernicious anemia, vitiligo and chronic atrophic gastritis. A strong association between ATD and atrophic gastritis (AG) has been demonstrated. Moreover 10 % of patients affected by AG have a predisposition to develop gastric carcinoid and adenocarcinoma as a result of chronic hypergastrinemia caused by achlorhydria and subsequent ELC cells neoplastic transformation...
July 2016: Journal of Endocrinological Investigation
https://www.readbyqxmd.com/read/26086363/immunotherapy-in-antiphospholipid-syndrome
#17
REVIEW
Ch Lopez-Pedrera, M A Aguirre, P Ruiz-Limon, C Pérez-Sánchez, Y Jimenez-Gomez, N Barbarroja, M J Cuadrado
Antiphospholipid syndrome (APS) is a disorder characterized by the association of arterial or venous thrombosis and/or pregnancy morbidity with the presence of antiphospholipid antibodies (anticardiolipin antibodies, lupus anticoagulant antibodies, and/or anti-β2-glycoprotein I antibodies). Thrombosis is the major manifestation in patients with aPLs, but the spectrum of symptoms and signs associated with aPLs has broadened considerably, and other manifestations, such as thrombocytopenia, non-thrombotic neurological syndromes, psychiatric manifestations, livedo reticularis, skin ulcers, hemolytic anemia, pulmonary hypertension, cardiac valve abnormality, and atherosclerosis, have also been related to the presence of those antibodies...
August 2015: International Immunopharmacology
https://www.readbyqxmd.com/read/25929633/childhood-lupus-with-microangiopathic-antiphospholipid-syndrome-and-pulmonary-hemorrhage
#18
Dharmendra Bhadauria, Praveen Etta, Anupma Kaul, Narayan Prasad
BACKGROUND: Concurrent thrombotic and hemorrhagic manifestations are uncommon in patients with Antiphospholipid Syndrome. CASE CHARACTERISTICS: A 10-year-old girl with fever, edema, rash and joint pains, who later developed deep venous thrombosis (DVT), stroke, thrombotic microangiopathic hemolytic anemia and pulmonary hemorrhage. Investigations confirmed Antiphospholipid syndrome associated with systemic lupus erythematosus. OUTCOME: She went into complete remission with intravenous immunoglobulins, plasmapheresis, immunosuppression and anticoagulation...
April 2015: Indian Pediatrics
https://www.readbyqxmd.com/read/25900788/antiphospholipid-syndrome-in-a-patient-suffering-from-congenital-dyserythropoietic-anemia-type-iii
#19
LETTER
Gonzalo Carreno-Tarragona, Francisco Trapiello-Valbuena, Judit Aranda-Salom, Joaquín Martínez-López, Carlos Lumbreras, Encarnación Oliveira-Ramírez, Carmen Díaz-Pedroche
No abstract text is available yet for this article.
August 2015: Annals of Hematology
https://www.readbyqxmd.com/read/25818822/prevalence-of-the-american-college-of-rheumatology-hematological-classification-criteria-and-associations-with-serological-and-clinical-variables-in-460-systemic-lupus-erythematosus-patients
#20
Thelma Skare, Renata Damin, Renata Hofius
OBJECTIVE: To study systemic lupus erythematosus in a Brazilian population using the American College of Rheumatology hematological classification criteria and report associations of the disease with serological and clinical profiles. METHODS: This is a retrospective study of 460 systemic lupus erythematosus patients followed in a single rheumatologic center during the last 10 years. Hematological manifestations considered for this study were hemolysis, leukopenia, lymphocytopenia and thrombocytopenia...
March 2015: Revista Brasileira de Hematologia e Hemoterapia
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