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https://www.readbyqxmd.com/read/29684562/bmt-following-non-myeloablative-treosulfan-conditioning-is-curative-in-a-murine-model-of-sickle-cell-disease
#1
Divya Devadasan, Chiao-Wang Sun, Erik R Westin, Li-Chen Wu, Kevin M Pawlik, Tim M Townes, Frederick D Goldman
Allogeneic hematopoietic stem cell transplantation (HSCT) can be curative for patients with sickle cell disease (SCD). However, morbidity associated with myeloablative conditioning and graft versus host disease has limited its utility. To this end, autologous HSCT for SCD using lentiviral gene-modified bone marrow (BM) or peripheral blood stem cells has been undertaken, though toxicities of fully ablative conditioning with busulfan and incomplete engraftment have been encountered. Treosulfan, a busulfan analog with a low extra-medullary toxicity profile, has been employed successfully as part of a myeloablative conditioning regimen in the allogeneic setting in SCD...
April 20, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29683954/a-retrospective-analysis-of-sociodemographic-and-hematologic-characteristics-associated-with-achieving-optimal-hydroxyurea-therapy-in-children-with-sickle-cell-disease
#2
Paul E George, Juan C Bazo-Alvarez, Vivien A Sheehan
Hydroxyurea (HU) has proven hematologic and clinical benefits, especially when escalated to the maximum tolerated dose (MTD). We reviewed clinical data from patients with sickle cell disease (January 2011 to 2016) to determine baseline sociodemographic and laboratory parameters associated with reaching HU MTD without significant delays. In total, 210 patients (mean HU start age, 6.6 y) were included. Initial Kaplan-Meier event analysis showed 1 year to be an inflection point for reaching MTD. In total, 116 patients (55%) reached MTD in <1 year, with 56 (27%) taking >1 year to reach MTD and 38 (18%) patients not successfully reaching MTD during follow-up...
April 20, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29682347/prevalence-of-microalbuminuria-in-adult-patients-with-sickle-cell-disease-in-eastern-saudi-arabia
#3
Ahmed M Alkhunaizi, Adil A Al-Khatti, Mansour A Alkhunaizi
Background: Proteinuria is a common feature of sickle cell nephropathy (SCN) that can progress to renal insufficiency and end stage renal disease. Microalbuminuria (MA) is the earliest manifestation of SCN and precedes the development of overt proteinuria. In addition to the renal consequences, MA is linked to cardiovascular complications. Periodic screening and early detection of MA allow early intervention that may reduce the risk of progression to advanced renal failure and cardiovascular diseases...
2018: International Journal of Nephrology
https://www.readbyqxmd.com/read/29679812/protective-role-of-nimbolide-against-chemotherapeutic-drug-hydroxyurea-induced-genetic-and-oxidative-damage-in-an-animal-model
#4
Md Fahim Ahmad, Mohd Owais Ansari, Sana Jameel, Ab Latif Wani, Nuzhat Parveen, Hifzur R Siddique, G G H A Shadab
Nimbolide is known to be an antioxidant found in neem plant. Hydroxyurea is a medication frequently used in sickle-cell disease, different cancers and HIV infection. The present study aimed to evaluate the adverse effect of HU and possible amelioration by nimbolide in Wistar rats. To test our hypothesis, we performed genotoxicity tests, biochemical assays, and histopathological studies. We observed that HU caused higher levels of genotoxicity in the treated animals. The observed genetic and oxidative damage might be due to the presence of reactive species as HU increased the level of the malondialdehyde-a biomarker of oxidative damage...
April 12, 2018: Environmental Toxicology and Pharmacology
https://www.readbyqxmd.com/read/29677351/retinal-oxygen-delivery-and-metabolism-in-healthy-and-sickle-cell-retinopathy-subjects
#5
Mahnaz Shahidi, Anthony E Felder, Ou Tan, Norman P Blair, David Huang
Purpose: Reduction in inner retinal oxygen delivery (DO2) can cause retinal hypoxia and impair inner retinal oxygen metabolism (MO2), leading to vision loss. The purpose of the current study was to establish measurements of DO2 and MO2 in healthy subjects and test the hypothesis that DO2 and MO2 are reduced in sickle cell retinopathy (SCR) subjects. Methods: Dual wavelength retinal oximetry and Doppler optical coherence tomography were performed in 12 healthy control and 12 SCR subjects...
April 1, 2018: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/29676678/spontaneous-epidural-hematoma-secondary-to-bone-infarction-in-sickle-cell-anemia-case-report
#6
Rohini Komarla, Bruno P Soares, Joshua J Chern, Sarah S Milla
Spontaneous epidural hematoma (EDH) is a rare occurrence in patients with sickle cell disease, with a small number of cases reported. Appropriate diagnosis is critical, because rapid neurosurgical intervention may be required. This unique case illustrates clinical and MRI features of an 18-year-old woman presenting with a headache and subsequent progression to severe focal neurological symptoms. Imaging demonstrated a large EDH of mixed signal characteristics and underlying calvarial infarction, requiring emergency decompression and evacuation...
April 20, 2018: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/29676452/cardiac-manifestations-in-sickle-cell-disease-varies-with-patient-genotype
#7
Paul Guedeney, François Lionnet, Alexandre Ceccaldi, Katia Stankovic Stojanovic, Ariel Cohen, Sarah Mattioni, Gilles Montalescot, Claude Bachmeyer, Richard Isnard, Jean-Philippe Haymann, Nadjib Hammoudi
Cardiac involvement is well characterized in sickle cell anaemia (SCA) but cardiac features associated with Haemoglobin SC (HbSC) disease are mostly unknown. We compared 60 patients with HbSC disease (median age 31 years, 25 men) to 60 SCA patients and 60 controls matched for age and gender. Left ventricular ejection fraction (LVEF), left ventricle (LV) mass index (LVMi), cardiac index and peak tricuspid regurgitation velocity (TRV) were measured using echocardiography. LV filling pressures were assessed using the ratio of early diastolic transmitral velocity to tissue velocity (E/e' ratio)...
April 20, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29676405/reply-to-comment-on-ocular-manifestations-of-sickle-cell-disease-and-genetic-susceptibility-for-refractive-errors
#8
COMMENT
Palak Shukla, Henu Verma, Santosh Patel, P K Patra, L V K S Bhaskar
No abstract text is available yet for this article.
January 2018: Taiwan Journal of Ophthalmology
https://www.readbyqxmd.com/read/29675950/risk-factors-for-red-blood-cell-alloimmunization-in-the-recipient-epidemiology-and-donor-evaluation-study-reds-iii-database
#9
Matthew S Karafin, Matt Westlake, Ronald G Hauser, Christopher A Tormey, Philip J Norris, Nareg H Roubinian, Yanyun Wu, Darrell J Triulzi, Steve Kleinman, Jeanne E Hendrickson
Despite the significance of red blood cell (RBC) alloimmunization, the lack of standardized registries in the US has prevented the completion of large studies. Data from 3·5 years of the Recipient Epidemiology and Donor Evaluation Study-III (REDS-III) recipient database, containing information from 12 hospitals, were studied. A RBC alloantibody responder had an antibody identified at any point during the study, and a non-responder had a negative antibody screen at least 15 days post-RBC transfusion. Demographics, blood type, ICD9/10 codes, and other potential correlates were evaluated...
April 19, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29675595/involvement-of-cytosolic-and-mitochondrial-iron-in-iron-overload-cardiomyopathy-an-update
#10
REVIEW
Richard Gordan, Suwakon Wongjaikam, Judith K Gwathmey, Nipon Chattipakorn, Siriporn C Chattipakorn, Lai-Hua Xie
Iron overload cardiomyopathy (IOC) is a major cause of death in patients with diseases associated with chronic anemia such as thalassemia or sickle cell disease after chronic blood transfusions. Associated with iron overload conditions, there is excess free iron that enters cardiomyocytes through both L- and T-type calcium channels thereby resulting in increased reactive oxygen species being generated via Haber-Weiss and Fenton reactions. It is thought that an increase in reactive oxygen species contributes to high morbidity and mortality rates...
April 19, 2018: Heart Failure Reviews
https://www.readbyqxmd.com/read/29675352/ocular-manifestations-of-sickle-cell-disease
#11
Beuy Joob, Viroj Wiwanitkit
No abstract text is available yet for this article.
January 2018: Taiwan Journal of Ophthalmology
https://www.readbyqxmd.com/read/29673144/serum-hepcidin-concentration-in-individuals-with-sickle-cell-anemia-basis-for-the-dietary-recommendation-of-iron
#12
Juliana Omena, Cláudia Dos Santos Cople-Rodrigues, Jessyca Dias do Amaral Cardoso, Andrea Ribeiro Soares, Marcos Kneip Fleury, Flávia Dos Santos Barbosa Brito, Josely Correa Koury, Marta Citelli
Dietary iron requirements in patients with sickle cell disease (SCD) remain unclear. SCD is a neglected hemoglobinopathy characterized by intense erythropoietic activity and anemia. Hepcidin is the hormone mainly responsible for iron homeostasis and intestinal absorption. Intense erythropoietic activity and anemia may reduce hepcidin transcription. By contrast, iron overload and inflammation may induce it. Studies on SCD have not evaluated the role of hepcidin in the presence and absence of iron overload. We aimed to compare serum hepcidin concentrations among individuals with sickle cell anemia, with or without iron overload, and those without the disease...
April 17, 2018: Nutrients
https://www.readbyqxmd.com/read/29668544/splenectomy-to-optimize-hemoglobin-s-control-in-children-with-sickle-cell-anemia-on-chronic-transfusion-therapy-for-stroke-prevention
#13
L Vandy Black, Jolie S Ramesar, Catherine H Boston
Chronic transfusion therapy with the goal of maintaining a hemoglobin (Hb) S <30% is the primary recommended treatment for children with sickle cell anemia and a history of overt stroke or abnormal transcranial Doppler examination. We report chronic hypersplenism as a cause of poor HbS% control in 3 children on chronic transfusion therapy for stroke prevention. Splenectomy resulted in a 39.77% (95% confidence interval, 34.3-45.3, P<0.0001) mean reduction in HbS% with no perioperative or infectious complications suggesting the need for additional research into splenectomy as a therapeutic option for select high-risk children to optimize transfusion therapy for stroke prevention...
April 17, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29668535/response-to-long-term-vitamin-d-therapy-for-bone-disease-in-children-with-sickle-cell-disease
#14
Kristen M Williams, Margaret T Lee, Maureen Licursi, Gary M Brittenham, Ilene Fennoy
Patients with sickle cell disease (SCD) are at risk for bone fragility from multiple factors including vitamin D deficiency. To date, no studies have evaluated the efficacy and safety of long-term vitamin D therapy for bone disease in children with SCD. We report a cohort of 4 children with SCD found to have severe vitamin D deficiency, secondary hyperparathyroidism, and abnormal bone mineral density treated with monthly high-dose oral cholecalciferol over 2 years. All patients exhibited a positive response to therapy without hypervitaminosis D or hypercalcemia...
April 17, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29668080/control-of-the-ethylene-signaling-pathway-prevents-plant-defenses-during-intracellular-accommodation-of-the-rhizobia
#15
Fathi Berrabah, Thierry Balliau, El Hosseyn Aït-Salem, Jeoffrey George, Michel Zivy, Pascal Ratet, Benjamin Gourion
Massive intracellular populations of symbiotic bacteria, referred to as rhizobia, are housed in legume root nodules. Little is known about the mechanisms preventing the development of defense in these organs although genes such as symCRK and DNF2 of the model legume Medicago truncatula are required for this control after rhizobial internalization in host nodule cells. Here we investigated the molecular basis of the symbiotic control of immunity. Proteomic analysis was performed to compare functional (wild-type) and defending nodules (symCRK)...
April 18, 2018: New Phytologist
https://www.readbyqxmd.com/read/29667775/views-of-parents-of-children-with-sickle-cell-disease-on-pre-implantation-genetic-diagnosis
#16
Isha Darbari, Jeanne E O'Brien, Steven J Hardy, Barbara Speller-Brown, Lisa Thaniel, Brenda Martin, Deepika S Darbari, Robert S Nickel
Pre-implantation genetic diagnosis (PGD) is an option for parents who have a child with sickle cell disease (SCD) to have another child without SCD. We conducted a survey of 19 parents with at least one child with SCD to investigate views on PGD. Before education, 44% of parents were aware of PGD. All parents rated PGD education as important. All parents considering another child also reported interest in using PGD if insurance covered its costs. Parents who have a child with SCD appear to be interested in PGD and educational tools informing this group about PGD should be developed...
April 18, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29667731/hemoglobin-s-monitoring-on-tosoh-g8-in-hemoglobin-a1c-mode-in-case-of-urgent-red-blood-cell-exchange
#17
Sophie Van Aelst, Helena Claerhout, Elke Nackers, Koen Desmet, Davy Kieffer
BACKGROUND: Pre- and post-transfusion hemoglobin S (HbS) levels are used to document the efficacy of red blood cell exchange (RCE) in patients with sickle cell disease (SCD). In case of urgent RCE a 24/7 short turn-around time (STAT) analysis, with the ability to identify and quantify HbS, is warranted. The use of TOSOH G8 (Tosoh Europe) is evaluated for this purpose, using the variant HbA1c mode. METHODS: Analytical performance of the HbS analysis on TOSOH G8 in variant HbA1c mode was evaluated, including assessment of imprecision and linearity for HbS...
April 18, 2018: Journal of Clinical Laboratory Analysis
https://www.readbyqxmd.com/read/29667721/diverse-manifestations-of-acute-sickle-cell-hepatopathy-in-pediatric-patients-with-sickle-cell-disease-a-case-series
#18
Lydia H Pecker, Nidhi Patel, Susan Creary, Anil Darbari, Emily Riehm Meier, Deepika S Darbari, Ross M Fasano
The hepatic complications of sickle cell disease (SCD) are associated with increased morbidity and mortality in adults; children usually survive but may suffer significant sequelae. Few diagnostic tools differentiate the various hepatic manifestations of SCD. Why patients exhibit one hepatic pathology versus another is unclear. We report four pediatric patients with hemoglobin SS disease with diverse manifestations of acute hepatic involvement including acute sickle hepatic crisis, hepatic sequestration, sickle cell intrahepatic cholestasis, and a non-SCD cause of hepatopathy in a patient with viral hepatitis...
April 18, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29666710/acute-chest-syndrome-progressing-to-ards-in-a-patient-of-25-week-gestation
#19
Jefferson Chambers, Nichole Smith, Matthew Sehring, Subramanyam Chittivelu
Acute chest syndrome is a complication of sickle cell disease and represents the highest cause of mortality in those afflicted with the disorder. Pregnancy represents an increased risk for complications of sickle cell disease in both the mother and fetus. We present a case of a 20-year-old patient with known sickle cell disease who was at 25-week gestation and developed acute chest syndrome refractory to conventional therapies and requiring emergency cesarean section. Following delivery, the patient developed acute respiratory distress syndrome (ARDS) requiring extracorporeal membrane oxygenation (ECMO)...
2018: Case Reports in Critical Care
https://www.readbyqxmd.com/read/29666677/should-premarital-screening-for-blood-disorders-be-an-obligatory-measure-in-oman
#20
Amal A Al-Balushi, Budoor Al-Hinai
Due to the high rate of consanguineous marriages in Oman, there is a correspondingly high prevalence of hereditary blood disorders, particularly sickle cell disease and β-thalassaemia. This article proposes the possibility of implementing mandatory premarital carrier screening for blood disorders in Oman, while giving due consideration to potential social and cultural obstacles. Although the implementation of such legislation would require collaboration between different sectors and may negatively affect the autonomy of certain individuals, mandatory premarital screening would help to alleviate the burden of hereditary blood disorders on the national healthcare system, as well as reduce avoidable suffering among carriers and their families...
February 2018: Sultan Qaboos University Medical Journal
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