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hemolytic anemias

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https://www.readbyqxmd.com/read/28212921/towards-developing-criteria-for-scleroderma-renal-crisis-a-scoping-review
#1
REVIEW
Sabrina Hoa, Edward P Stern, Christopher P Denton, Marie Hudson
OBJECTIVE: The absence of a gold standard for scleroderma renal crisis (SRC) has hindered our understanding of this problem. The objective of this scoping review was to identify the criteria used to define SRC in order to guide the development of a consensus definition for SRC. METHODS: We conducted a search in three databases: Medline, Embase and non-Ovid Pubmed. Papers were eligible for inclusion if they were full-length articles in English whose main topic was SRC or scleroderma renal disease...
February 14, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28211224/splenectomy-resolves-hemolytic-anemia-caused-by-adenylate-kinase-deficiency
#2
Hidetaka Niizuma, Hitoshi Kanno, Atsushi Sato, Hiromi Ogura, Masue Imaizumi
No abstract text is available yet for this article.
February 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28210931/serotonin-syndrome-from-5-hydroxytryptophan-supplement-ingestion-in-a-9-month-old-labrador-retriever
#3
Hopkins Jennifer, Pardo Mariana, Bischoff Karyn
INTRODUCTION: 5-Hydroxytryptophan (5-HTP) supplements are available over the counter and labeled as sleeping aids and anxiolytics for human use. 5-HTP is a serotonin precursor and overdose can lead to serotonin syndrome. CASE REPORT: A 9-month-old female Labrador retriever was evaluated after ingestion of a 5-HTP supplement. Signs of agitation developed within 1 h of ingestion, and emesis was attempted by the owner with  3% hydrogen peroxide (H2O2) orally. On presentation, the dog was obtunded, bilaterally mydriatic and salivating...
February 16, 2017: Journal of Medical Toxicology: Official Journal of the American College of Medical Toxicology
https://www.readbyqxmd.com/read/28210839/thrombotic-microangiopathy-caused-by-methionine-synthase-deficiency-diagnosis-and-treatment-pitfalls
#4
Maria Helena Vaisbich, Andressa Braga, Maria Gabrielle, Clarissa Bueno, Flávia Piazzon, Fernando Kok
BACKGROUND: Inborn errors of cobalamin (Cbl) metabolism form a large group of rare diseases. One of these, Cbl deficiency type C (CblC), is a well-known cause of thrombotic microangiopathy (TMA), especially in infants. However, there has only been a single published case of TMA associated to Cbl deficiency type G (CblG), also known as methionine synthase deficiency (MSD). CASE DIAGNOSIS/TREATMENT: A 21-month-old boy presented with pallor and oral ulcers during episodes of upper respiratory infection (URI)...
February 16, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28202022/hematologic-manifestations-of-babesiosis
#5
REVIEW
Tamer Akel, Neville Mobarakai
BACKGROUND: Babesiosis, a zoonotic parasitic infection transmitted by the Ixodes tick, has become an emerging health problem in humans that is attracting attention worldwide. Most cases of human babesiosis are reported in the United States and Europe. The disease is caused by the protozoa of the genus Babesia, which invade human erythrocytes and lyse them causing a febrile hemolytic anemia. The infection is usually asymptomatic or self-limited in the immunocompetent host, or follows a persistent, relapsing, and/or life threatening course with multi-organ failure, mainly in the splenectomized or immunosuppressed patients...
February 15, 2017: Annals of Clinical Microbiology and Antimicrobials
https://www.readbyqxmd.com/read/28198349/impact-of-itpa-gene-polymorphisms-on-the-risk-of-ribavirin-induced-haemolytic-anaemia-using-interferon-free-antivirals-for-chronic-hepatitis-c
#6
Isabella Esposito, Laura Benítez-Gutiérrez, Ana Treviño, Ana Arias, Maria Jesus Citores, Silvia Requena, Vicente Soriano, Valentín Cuervas-Mons, Carmen de Mendoza
BACKGROUND: Single nucleotide polymorphisms (SNPs) at the ITPA gene are associated with hemolytic anemia in chronic hepatitis C patients treated with peginterferon-ribavirin (RBV). Information in patients treated with interferon-free, direct-acting antivirals (DAA) is scarce. METHODS: Median hemoglobin (Hb) levels were compared at baseline and at week 4, when ribavirin concentration achieves steady state, in all consecutive chronic hepatitis C patients treated with oral DAA plus RBV at our clinic...
February 15, 2017: Antiviral Therapy
https://www.readbyqxmd.com/read/28197963/a-concise-review-of-autoimmune-cytopenias-in-chronic-lymphocytic-leukemia
#7
REVIEW
Mazie Tsang, Sameer A Parikh
Chronic lymphocytic leukemia (CLL) is frequently associated with autoimmune complications such as autoimmune hemolytic anemia, immune thrombocytopenia, pure red cell aplasia, and autoimmune granulocytopenia. It is critical to diagnose cytopenias from these secondary complications of CLL accurately, since prognosis and therapy are substantially different from patients who have cytopenias due to extensive bone marrow infiltration by CLL. The pathogenesis of autoimmune cytopenias in CLL is complex; and it involves antigen presentation by CLL cells to polyclonal B cells resulting in production of autoantibody, and alteration of the T cell milieu tilting the balance in favor of an autoimmune response...
February 14, 2017: Current Hematologic Malignancy Reports
https://www.readbyqxmd.com/read/28193740/development-of-delayed-hemolytic-anemia-after-treatment-with-oral-artemether-lumefantrine-in-two-patients-with-severe-falciparum-malaria
#8
Yasuhiro Tsuchido, Fukumi Nakamura-Uchiyama, Kasumi Toyoda, Moritoshi Iwagami, Kentaro Tochitani, Koh Shinohara, Naokuni Hishiya, Taku Ogawa, Kenji Uno, Kei Kasahara, Yukiteru Ouji, Shigeyuki Kano, Keiichi Mikasa, Tsunehiro Shimizu, Masahide Yoshikawa, Haruhiko Maruyama
Recently, reports of delayed hemolytic anemia after treatment with artemisinin and its derivatives have emerged. Here we report two cases of delayed hemolytic anemia in a patient with severe falciparum malaria after treatment with oral artemether-lumefantrine (AL). The first patient, a 20-year-old Japanese male student, was diagnosed with falciparum malaria and was administered AL. As having a high parasitemia rate (20.6%) was the only severe malaria criterion met in this case and his general condition was stable, we continued with AL treatment...
February 13, 2017: American Journal of Tropical Medicine and Hygiene
https://www.readbyqxmd.com/read/28182327/microangiopathic-hemolytic-anemia-due-to-adamts-13-loss-in-idiopathic-systemic-capillary-leak-syndrome-reply
#9
Daniel C Moreira, Jorge Di Paola
We sincerely appreciate Dr Thachil's letter, which provides further insight into the case we recently published, in which ADAMTS-13 was likely lost in third-space fluid in a patient with idiopathic systemic capillary leak syndrome (SCLS) and that this loss was proposed as a novel mechanism for microangiopathic hemolytic anemia (MAHA) [1]. As mentioned in the original publication, identifying high levels of ADAMTS-13 in the transudate fluid would have proven the proposed mechanism, but unfortunately a sample of the transudate fluid was not available...
February 9, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28182045/thrombotic-microangiopathy-an-unusual-cause-of-renal-failure-in-rheumatoid-arthritis
#10
R Sakthirajan, J Dhanapriya, T Dineshkumar, N Gopalakrishnan, S Murugan, T Balasubramaniyan
Rheumatoid arthritis (RA) is one of the commonest rheumatological diseases. Renal involvement is not common but can occur as a result of chronic inflammation as part of disease process or drug toxicity. Thrombotic microangiopathy (TMA) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ failure of variable severity. Only a few cases of TMA in patients with RA were reported to date. We describe a 45-year-old female patient with RA who presented with oliguria and edema. Renal biopsy showed TMA with patchy cortical necrosis...
January 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/28143953/hemolytic-anemia-repressed-hepcidin-level-without-hepatocyte-iron-overload-lesson-from-g%C3%A3-nther-disease-model
#11
Sarah Millot, Constance Delaby, Boualem Moulouel, Thibaud Lefebvre, Nathalie Pilard, Nicolas Ducrot, Cécile Ged, Philippe Lettéron, Lucia de Franceschi, Jean Charles Deybach, Carole Beaumont, Laurent Gouya, Hubert De Verneuil, Saïd Lyoumi, Hervé Puy, Zoubida Karim
Hemolysis occurring in hematologic diseases is often associated with an iron loading anemia. This iron overload is the result of a massive outflow of hemoglobin into the bloodstream, but the mechanism of hemoglobin handling has not been fully elucidated. Here, in a congenital erythropoietic porphyria mouse model, we evaluate the impact of hemolysis and regenerative anemia on hepcidin synthesis and iron metabolism. Hemolysis was confirmed by a complete drop in haptoglobin, hemopexin and increased plasma lactate dehydrogenase, an increased red blood cell distribution width and osmotic fragility, a reduced half-life of red blood cells, and increased expression of heme oxygenase 1...
February 2017: Haematologica
https://www.readbyqxmd.com/read/28134038/outcomes-of-847-childhood-onset-systemic-lupus-erythematosus-patients-in-three-age-groups
#12
S R M Lopes, N W S Gormezano, R C Gomes, N E Aikawa, R M R Pereira, M T Terreri, C S Magalhães, J C Ferreira, E M Okuda, A P Sakamoto, A M E Sallum, S Appenzeller, V P L Ferriani, C M Barbosa, S Lotufo, A A Jesus, L E C Andrade, L M A Campos, E Bonfá, C A Silva
Objective The objective of this study was to assess outcomes of childhood systemic lupus erythematosus (cSLE) in three different age groups evaluated at last visit: group A early-onset disease (<6 years), group B school age (≥6 and <12 years) and group C adolescent (≥12 and <18 years). Methods An observational cohort study was performed in ten pediatric rheumatology centers, including 847 cSLE patients. Results Group A had 39 (4%), B 395 (47%) and C 413 (49%). Median disease duration was significantly higher in group A compared to groups B and C (8...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28133914/molecular-basis-of-pyruvate-kinase-deficiency-among-tunisians-description-of-new-mutations-affecting-coding-and-noncoding-regions-in-the-pklr-gene
#13
M Jaouani, L Manco, M Kalai, L Chaouch, K Douzi, A Silva, S Macedo, I Darragi, I Boudriga, D Chaouachi, Z Fitouri, R Van Wijk, M L Ribeiro, S Abbes
INTRODUCTION: Pyruvate kinase (PK) deficiency is one of the most common hereditary nonspherocytic hemolytic anemias worldwide with clinical manifestations ranging from mild to severe hemolysis. However, investigation of this enzymopathy is lacking in Tunisia. We report here a pioneer investigation of PK deficiency among Tunisian cases referred to our laboratory for biological analysis of unknown cause of hemolytic anemia. METHODS: Two hundred and fifty-three patients with unknown cause of hemolytic anemia have been addressed to our laboratory in order to investigate for red blood cells genetic disorders...
January 30, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28133771/thrombotic-thrombocytopenic-purpura-misdiagnosed-as-autoimmune-cytopenia-causes-of-diagnostic-errors-and-consequence-on-outcome-experience-of-the-french-thrombotic-microangiopathies-reference-centre
#14
Maximilien Grall, Elie Azoulay, Lionel Galicier, François Provôt, Alain Wynckel, Pascale Poullin, Steven Grange, Jean-Michel Halimi, Alexandre Lautrette, Yahsou Delmas, Claire Presne, Mohamed Hamidou, Stéphane Girault, Frédéric Pène, Pierre Perez, Tarik Kanouni, Amélie Seguin, Christiane Mousson, Dominique Chauveau, Mario Ojeda-Uribe, Virginie Barbay, Agnès Veyradier, Paul Coppo, Ygal Benhamou
Thrombotic thrombocytopenic purpura (TTP) has a devastating prognosis without adapted management. Sources of misdiagnosis need to be identified to avoid delayed treatment. We studied 84 patients with a final diagnosis of severe (<10%) acquired ADAMTS13 deficiency-associated TTP from our National database that included 423 patients, who had an initial misdiagnosis (20% of all TTP). Main diagnostic errors were attributed to autoimmune thrombocytopenia, associated (51%) or not (37%) with autoimmune hemolytic anemia...
January 30, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28132948/added-value-of-hepcidin-quantification-for-the-diagnosis-and-follow-up-of-anemia-related-diseases
#15
Thibaud Lefebvre, Sigismond Lasocki, Martine Fénéant-Thibault, Pierre-Jean Lamy, Séverine Cunat, Madeleine Ropert-Bouchet, Patricia Aguilar-Martinez, Sylvain Lehmann, Constance Delaby
Iron homeostasis is based on a strict control of both intestinal iron absorption and iron recycling through reticulo-endothelial system. Hepcidin controls the iron fluxes in order to maintain sufficient iron levels for erythropoietic activities, hemoproteins synthesis or enzymes function, but also to limit its toxic accumulation throughout the body. Hepcidin expression is regulated by various stimuli: inflammation and iron stimulate the production of the peptide, while anemia, erythropoiesis and hypoxia repress its production...
February 1, 2017: Annales de Biologie Clinique
https://www.readbyqxmd.com/read/28129870/50-years-ago-in-the-journal-of-pediatrics-vitamin-e-deficiency-a-previously-unrecognized-cause-of-hemolytic-anemia-in-the-premature-infant
#16
Alexandra N Carey, Christopher Duggan
No abstract text is available yet for this article.
February 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28128521/itpa-gene-variation-and-ribavirin-induced-anemia-in-patients-with-genotype-2-chronic-hepatitis-c-treated-with-sofosbuvir-plus-ribavirin
#17
Miyako Murakawa, Yasuhiro Asahina, Hiroko Nagata, Mina Nakagawa, Sei Kakinuma, Sayuri Nitta, Fukiko Kawai-Kitahata, Satoshi Otani, Shun Kaneko, Masato Miyoshi, Tomoyuki Tsunoda, Yu Asano, Ayako Sato, Yasuhiro Itsui, Seishin Azuma, Toshihiko Nouchi, Yohei Furumoto, Tooru Asano, Yoshimichi Chuganji, Shuji Tohda, Mamoru Watanabe
AIM: Sofosbuvir (SOF) and ribavirin (RBV) combination therapy produces a sustained response in many patients with genotype 2 chronic hepatitis C. However, RBV-induced anemia is a troublesome side effect that may limit this treatment. Genetic variation leading to inosine triphosphatase (ITPA) deficiency is known to protect against RBV-induced hemolytic anemia. This study aimed to evaluate the relationships between the efficacy and safety of SOF/RBV treatment and ITPA gene variants. METHODS: Ninety patients with genotype 2 chronic hepatitis C treated with SOF/RBV were studied...
January 27, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/28123330/the-plethora-clinical-manifestations-and-treatment-options-of-autoimmunity-in-patients-with-primary-immunodeficiency
#18
Hatice Ezgi Barış, Ayça Kıykım, Ercan Nain, Ahmet Oğuzhan Özen, Elif Karakoç-Aydıner, Safa Barış
AIM: Although the association between primary immunodeficiency and autoimmunity is already well-known, it has once again become a topic of debate with the discovery of newly-defined immunodeficiencies. Thus, investigation of the mechanisms of development of autoimmunity in primary immunodefficiency and new target-specific therapeutic options has come to the fore. In this study, we aimed to examine the clinical findings of autoimmunity, autoimmunity varieties, and treatment responses in patients who were genetically diagnosed as having primary immunodeficiency...
December 2016: Türk Pediatri Arşivi
https://www.readbyqxmd.com/read/28122158/microangiopathic-hemolytic-anemia-due-to-adamts-13-loss-in-idiopathic-systemic-capillary-leak-syndrome-comment
#19
Jecko Thachil
Moreira et al describe a fascinating case of a five-year-old who developed microangiopathic hemolytic anemia (MAHA) a week after presentation with idiopathic systemic capillary leak syndrome (SCLS) and propose ADAMTS-13 loss in the third-space fluid as the cause of MAHA [1]. It would however be useful if the authors could provide the values of vascular endothelial growth factor (VEGF) and von willebrand factor (VWF) levels in this patient during the illness episode before this conclusion is made. Endothelial dysfunction is well-known to cause very high levels of both VEGF and VWF, to the extent in many experimental studies; these markers are used as a marker for endothelial activation and altered function [2, 3]...
January 25, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28121924/autoimmune-diseases-and-hiv-infection-a-cross-sectional-study
#20
Emilie Virot, Antoine Duclos, Leopold Adelaide, Patrick Miailhes, Arnaud Hot, Tristan Ferry, Pascal Seve
To describe the clinical manifestations, treatments, prognosis, and prevalence of autoimmune diseases (ADs) in human immunodeficiency virus (HIV)-infected patients.All HIV-infected patients managed in the Infectious Diseases Department of the Lyon University Hospitals, France, between January 2003 and December 2013 and presenting an AD were retrospectively included.Thirty-six ADs were found among 5186 HIV-infected patients which represents a prevalence of 0.69% including immune thrombocytopenic purpura (n = 15), inflammatory myositis (IM) (n = 4), sarcoidosis (n = 4), Guillain-Barré syndrome (GBS) (n = 4), myasthenia gravis (n = 2), Graves' disease (n = 2), and 1 case of each following conditions: systemic lupus erythematosus, rheumatoid arthritis, autoimmune hepatitis, Hashimoto thyroiditis and autoimmune hemolytic anemia...
January 2017: Medicine (Baltimore)
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