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https://www.readbyqxmd.com/read/28101432/atypical-hemolytic-uremic-syndrome-in-first-trimester-pregnancy-successfully-treated-with-eculizumab
#1
Gabriela Andries, Michael Karass, Srikanth Yandrapalli, Katherine Linder, Delong Liu, John Nelson, Rahul Pawar, Savneek Chugh
BACKGROUND: Atypical hemolytic uremic syndrome is a rare disorder which is known to cause acute thrombotic microangiopathy during pregnancy with poor maternal and fetal outcomes. Atypical hemolytic uremic syndrome is caused mostly by dysregulation of alternative complement pathway secondary to genetic mutations. Most of the cases reported have been in the post-partum period. We report a rare case of a patient who presents with thrombotic microangiopathy in the first trimester of her eleventh pregnancy and was successfully treated with eculizumab...
2017: Experimental Hematology & Oncology
https://www.readbyqxmd.com/read/28098879/cytokines-inducers-and-inhibitors-modulate-mmp-2-and-mmp%C3%A2-9-secretion-by-human-fanconi-anemia-immortalized-fibroblasts
#2
M W Roomi, T Kalinovsky, M Rath, A Niedzwiecki
Acute myeloid leukemia and head and neck squamous cell carcinomas are the major causes of mortality and morbidity in Fanconi anemia (FA) patients. Matrix metalloproteinases (MMPs), particularly MMP-2 and MMP-9, have been implicated in tumor invasion and metastasis. Various cytokines, mitogens, growth factors, inducers and inhibitors control MMP activities. We investigated the roles of these in the regulation of MMP-2 and MMP-9 in human immortalized fibroblasts from FA. Human FA immortalized fibroblast cell lines FA-A:PD220 and FA-D2:PD20 were grown in minimum essential medium (MEM) supplemented with 15% fetal bovine serum (FBS) and antibiotics in 24-well tissue culture plates...
January 16, 2017: Oncology Reports
https://www.readbyqxmd.com/read/28095881/renal-function-in-patients-with-non-dialysis-chronic-kidney-disease-receiving-intravenous-ferric-carboxymaltose-an-analysis-of-the-randomized-find-ckd-trial
#3
Iain C Macdougall, Andreas H Bock, Fernando Carrera, Kai-Uwe Eckardt, Carlo Gaillard, David Van Wyck, Yvonne Meier, Sylvain Larroque, Simon D Roger
BACKGROUND: Preclinical studies demonstrate renal proximal tubular injury after administration of some intravenous iron preparations but clinical data on renal effects of intravenous iron are sparse. METHODS: FIND-CKD was a 56-week, randomized, open-label, multicenter study in which patients with non-dialysis dependent chronic kidney disease (ND-CKD), anemia and iron deficiency without erythropoiesis-stimulating agent therapy received intravenous ferric carboxymaltose (FCM), targeting either higher (400-600 μg/L) or lower (100-200 μg/L) ferritin values, or oral iron...
January 17, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28090490/iron-deficiency-anemia-in-infants-and-toddlers
#4
Eun Young Joo, Keun Young Kim, Dong Hyun Kim, Ji-Eun Lee, Soon Ki Kim
BACKGROUND: In Korea, the prevalence of anemia and iron deficiency anemia (IDA) among older infants and young children remains high. To detect IDA early and to reduce its adverse impact, we assessed the characteristics of infants and young children who had IDA or were at risk of developing IDA, or who exhibited characteristics associated with severe anemia. METHODS: Among the 1,782 IDA-affected children aged 6 months to 18 years who visited the hospital, we retrospectively analyzed the medical records and laboratory data of 1,330 IDA-affected children aged 6-23 months who were diagnosed between 1996 and 2013...
December 2016: Blood Research
https://www.readbyqxmd.com/read/28090473/three-year-old-male-with-multiple-dieulafoy-lesions-treated-with-epinephrine-injections-via-therapeutic-endoscopy
#5
Christina L Baldwin, Michael Wilsey
Dieulafoy lesions, vascular anomalies typically found along the gastrointestinal tract, have been viewed as rare and obscure causes of sudden intestinal bleeding, especially in pediatric patients. Since their discovery in the late 19th century, the reported incidence has increased. This is due to an increased awareness of, and knowledge about, their presentation and to advanced endoscopic diagnosis and therapy. Our patient was a three-year-old male, without a complex medical history. He presented to the emergency department with acute hematemesis with blood clots and acute anemia requiring blood transfusion...
December 2016: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/28089985/giant-cell-arteritis-related-stroke-a-retrospective-multicenter-case-control-study
#6
Hubert de Boysson, Eric Liozon, Delphine Larivière, Maxime Samson, Jean-Jacques Parienti, Jonathan Boutemy, Gwénola Maigné, Nicolas Martin Silva, Kim Ly, Emmanuel Touzé, Bernard Bonnotte, Achille Aouba, Karim Sacré, Boris Bienvenu
OBJECTIVE: Our aim was to describe patients with giant cell arteritis (GCA)-related stroke and to compare them with a control group of GCA patients without stroke. METHODS: We created a retrospective multicenter cohort of patients with (1) GCA diagnosed according to the American College of Rheumatology criteria between 1995 and 2015, and (2) stroke occurring at the time of GCA diagnosis or occurring within 4 weeks of starting GCA therapy. The control group consisted of GCA patients without stroke...
January 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28089922/x-linked-elliptocytosis-with-impaired-growth-is-related-to-mutated-ammecr1
#7
Lina Basel-Vanagaite, Nir Pillar, Ofer Isakov, Pola Smirin-Yosef, Irina Lagovsky, Naama Orenstein, Mali Salmon-Divon, Hannah Tamary, Tami Zaft, Lily Bazak, Joseph Meyerovitch, Tal Pelli, Shay Botchan, Luba Farberov, Daphna Weissglas, Noam Shomron
In this study, we report a family with X-linked recessive syndrome caused by mutated AMMECR1 and characterized by elliptocytosis with or without anemia, midface hypoplasia, proportionate short stature and hearing loss. Recently, mutations in AMMECR1 were reported in two maternal half-brothers, presenting with nephrocalcinosis, midface hypoplasia and, in one of the siblings, deafness and elliptocytosis. AMMECR1 gene is localized in the critical region of contiguous deletion syndrome on Xq22.3 implicated in Alport syndrome, mental retardation, midface hypoplasia, and elliptocytosis (AMME complex)...
January 9, 2017: Gene
https://www.readbyqxmd.com/read/28088971/-eltrombopag-for-refractory-thrombocytopenia-in-patients-with-allogeneic-hematopoietic-stem-cell-transplantation
#8
Y R Ma, X J Huang, X D Mo, W Han, C H Yan, Y Chen, Y Ji, Y Y Chen, Y Wang, X H Zhang, K Y Liu, L P Xu
Objective: To evaluate the efficacy and safety of eltrombopag in post-HSCT thrombocytopenia. Methods: A total of 10 patients who underwent post-HSCT thrombocytopenia at Peking University center, who had been treated with eltrombopag, were retrospectively evaluated. Results: Of the 10 cases, 5 males and 5 females with a median of 34 years old (range, 17-54 years), 5 patients were acute myeloid leukemia, 3 with acute lymphoid leukemia and 2 with severe aplastic anemia. Nine patients had undergone haplo-identical donor transplantation, and one patient was a matched related recipient...
December 14, 2016: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/28078211/a-case-series-of-the-safety-and-efficacy-of-testosterone-replacement-therapy-in-renal-failure-and-kidney-transplant-patients
#9
Ahmad Majzoub, Daniel A Shoskes
BACKGROUND: Hypogonadism is common in patients with renal dysfunction and does not always correct following transplantation. Recent studies show increased mortality for dialysis and transplant patients with low testosterone (T). These patients are often not treated due to concerns over efficacy and complications (both real and imagined). There is surprisingly scant literature supporting the use of T therapy in these patients. We wished to examine the results of T therapy in our patients with renal failure or following transplant...
December 2016: Translational Andrology and Urology
https://www.readbyqxmd.com/read/28067886/dose-adapted-post-transplant-cyclophosphamide-for-hla-haploidentical-transplantation-in-fanconi-anemia
#10
M S Thakar, C Bonfim, M C Walters, R Storb, R Pasquini, L Burroughs, B M Sandmaier, A Woolfrey, H-P Kiem
We developed a haploidentical transplantation protocol with post-transplant cyclophosphamide (CY) for in vivo T-cell depletion (TCD) using a novel adapted-dosing schedule (25 mg/kg on days +3 and +4) for Fanconi anemia (FA). With median follow-up of 3 years (range, 37 days to 6.2 years), all six patients engrafted. Two patients with multiple pre-transplant comorbidities died, one from sepsis and one from sepsis with associated chronic GVHD. Four patients without preexisting comorbidities and early transplant referrals are alive with 100% donor chimerism and excellent performance status...
January 9, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28066855/spectrum-of-small-bowel-mucosal-abnormalities-identified-by-capsule-endoscopy-in-patients-with-portal-hypertension-of-varied-etiology
#11
T S Chandrasekar, Gokul Bollu Janakan, Viveksandeep Thoguluva Chandrasekar, Raja Yogesh Kalamegam, Sathiamoorthy Suriyanarayanan, Prasad Menta Sanjeevaraya
BACKGROUND: Bleeding from small intestinal ectopic varices and persistent anemia caused by portal hypertensive enteropathy (PHE) can be very challenging. Capsule endoscopy (CE) is one of the best noninvasive modalities in identifying such lesions. AIM: The aims of this study are to study the prevalence of small-bowel changes related to portal hypertension (PHT) and to correlate them with the observations related to the effects of portal hypertension in the esophagus, stomach, and colon...
January 9, 2017: Indian Journal of Gastroenterology: Official Journal of the Indian Society of Gastroenterology
https://www.readbyqxmd.com/read/28065528/a-comprehensive-evaluation-of-the-gastrointestinal-tract-in-iron-deficiency-anemia-with-predefined-hemoglobin-below-9mg-dl-a-prospective-cohort-study
#12
Xavier Bosch, Elisabet Montori, Mar Guerra-García, Jaime Costa-Rodríguez, Mariano H Quintanilla, Paula E Tolosa-Chapasian, Pedro Moreno, Neus Guasch, Alfons López-Soto
BACKGROUND: Anemia is defined as hemoglobin below the cutoff of normal in studies examining the gastrointestinal (GI) tract in iron-deficiency anemia (IDA). Although the risk of GI cancer (GIC) increases as hemoglobin decreases, guidelines do not usually recommend hemoglobin thresholds for IDA investigation. METHODS: To elucidate whether underlying GI disorders explain the different hemoglobin values and clinical outcomes observed initially in IDA patients referred for GI workup, we prospectively investigated the diagnostic yield of a thorough GI examination in consecutive IDA adults with predefined hemoglobin <9g/dL and no extraintestinal bleeding...
December 21, 2016: Digestive and Liver Disease
https://www.readbyqxmd.com/read/28054454/epoetin-beta-pegol-ameliorates-flow-mediated-dilation-with-improving-enos-coupling-state-in-non-obese-diabetic-rats
#13
Kenichi Serizawa, Kenji Yogo, Yoshihito Tashiro, Ryohei Kawasaki, Koichi Endo, Yasushi Shimonaka, Michinori Hirata
BACKGROUND/AIMS: Patients with diabetic nephropathy have a high cardiovascular mortality. Epoetin beta pegol (continuous erythropoietin receptor activator, C.E.R.A.) is a drug for the treatment of renal anemia. In this study, we investigated the effect of C.E.R.A. on vascular endothelial function as evaluated by flow-mediated dilation (FMD) and the relationship between hematopoiesis and FMD in diabetic nephropathy rats. METHODS: Male Spontaneously Diabetic Torii rats (SDT, 22 weeks old) were used...
January 5, 2017: Cardiovascular Therapeutics
https://www.readbyqxmd.com/read/28052565/cerebral-hemodynamics-and-pseudo-continuous-arterial-spin-labeling-considerations-in-adults-with-sickle-cell-anemia
#14
Meher R Juttukonda, Lori C Jordan, Melissa C Gindville, Larry T Davis, Jennifer M Watchmaker, Sumit Pruthi, Manus J Donahue
Sickle cell anemia (SCA) is a genetic disorder resulting in reduced oxygen carrying capacity and elevated stroke risk. Pseudo-continuous arterial spin labeling (pCASL) measures of cerebral blood flow (CBF) may have relevance for stroke risk assessment; however, the effects of elevated flow velocity and reduced bolus arrival time (BAT) on CBF quantification in SCA patients have not been thoroughly characterized, and pCASL model parameters used in healthy adults are often applied to patients with SCA. Here, cervical arterial flow velocities and pCASL labeling efficiencies were computed in adults with SCA (n = 19) and age- and race-matched controls without sickle trait (n = 7) using pCASL in sequence with phase contrast MR angiography (MRA)...
January 4, 2017: NMR in Biomedicine
https://www.readbyqxmd.com/read/28042550/pure-red-cell-aplasia-due-to-antibody-against-erythropoietin-in-hemodialysis-patients
#15
Maryam Rahbar, Zahra Chitsazian, Firoozeh Abdoli, Seyed-Masoud Moeini Taba, Hosein Akbari
BACKGROUND: Anemia is a common complication of chronic renal failure due to reduce erythropoietin production by kidneys. Anemia treated with recombinant human erythropoietin (rHu-EPO). Pure red cell aplasia (PRCA) due to antibody productionagainst rHu-EPO is a rare but major complication of this drug. OBJECTIVES: The aim of this study was to determine the prevalence of PRCA due to antibodies in dialysis patients with resistant anemia who received erythropoietin...
January 2017: Journal of Nephropathology
https://www.readbyqxmd.com/read/28040382/assessment-of-urinary-dipstick-in-patients-admitted-to-an-emergency-department-for-blunt-abdominal-trauma
#16
F Moustafa, C Loze, B Pereira, M A Vaz, L Caumon, C Perrier, J Schmidt
INTRODUCTION: Clinicians still face significant challenge in predicting intra-abdominal injuries in patients admitted to an emergency department for blunt abdominal trauma. This study was thus designed to investigate the value of dipstick urinalysis in patients with blunt abdominal trauma. METHODS: We performed a retrospective, multicenter, cohort study involving patients admitted to the emergency department for abdominal traumas, examined by means of urinary dipstick and abdominal CT scan...
December 22, 2016: American Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28040087/femoral-non-union-with-malalignment-reconstruction-and-biological-stimulation-with-the-chipping-technique
#17
Yoshinobu Watanabe, Takashi Matsushita
INTRODUCTION: The purpose of this study was to investigate the effectiveness of the chipping technique for the treatment of femoral non-unions associated with malalignment. PATIENTS AND METHODS: A total of 21 femoral non-unions were managed with the chipping procedure and re-fixation. 15 of them had malalignment (angular or rotational deformity or limb length discrepancy). The deformities were simultaneously corrected by a combination of chipping and temporal external fixator...
December 2016: Injury
https://www.readbyqxmd.com/read/28039098/efficacy-and-tolerability-of-interferon-free-antiviral-therapy-in-kidney-transplant-recipients-with-chronic-hepatitis-c
#18
Inmaculada Fernández, Raquel Muñoz-Gómez, Juan M Pascasio, Carme Baliellas, Natalia Polanco, Nuria Esforzado, Ana Arias, Martín Prieto, Lluis Castells, Valentín Cuervas-Mons, Olga Hernández, Javier Crespo, José L Calleja, Xavier Forns, María-Carlota Londoño
BACKGROUND AND AIMS: The development of direct-acting antivirals (DAAs) is a major step forward in the treatment of hepatitis C (HCV). The aims of the study were to evaluate the efficacy and tolerability of DAAs in kidney transplant (KT) recipients. METHODS: Hepa-C is a Spanish registry of patients treated with DAAs in which clinical, virological and analytical data were prospectively included. RESULTS: We report on the data from 103 KT recipients who received DAAs...
December 27, 2016: Journal of Hepatology
https://www.readbyqxmd.com/read/28032666/capsule-retention-in-a-meckel-s-diverticulum-for-7-5-years
#19
Chun-Run Ling, Mo-Jin Wang, Wen Zhuang
A 32-year-old man underwent wireless capsule endoscopy (WCE) (OMOM Capsule Endoscopy, Jinshan Science & Technology Company, Chongqing, China) for detecting obscure gastrointestinal bleeding 7.5 years ago. The result of WCE did not reveal any hemorrhagic focus, diverticulum or other abnormal changes of the gastrointestinal tract until its battery exhausted. The man then moved out from the area and lost to follow up without a report of capsule excretion. For confirming the diagnosis of recurrent melena and iron-deficiency anemia, the patient came to our clinical center...
December 29, 2016: Digestive Endoscopy: Official Journal of the Japan Gastroenterological Endoscopy Society
https://www.readbyqxmd.com/read/28024505/-comparison-of-therapeutic-efficacy-for-neonatal-abo-hemolytic-disease-treated-with-intravenous-immunoglobin-g-by-different-modes-of-administration
#20
Yun-Feng Liu, Chao-Chun Zou, Hua-Qin Yang, Li-Jiang Lou
OBJECTIVE: To compare the therapeutic efficacy of patients with neonatal ABO hemolytic disease treated with introvenous immunoglobin G (IVIG) by different modes of administration. METHODS: Ninety-three in patients with neonatal ABO hemolytic disease treated in our hospital were divided into group A (31 cases), B(31 cases) and C (31 cases). Based on basic treatment, the patients in group A were treated by a single high dose of IVIG (1 g/kg), patients in group B were treated by multiple low-dose of IVIG (0...
December 2016: Zhongguo Shi Yan Xue Ye Xue za Zhi
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