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Treating sickle cell anemia.

Tisdale JF, Thein SL, Eaton WA.

Science. 2020 Mar 13;367(6483):1198-1199. doi: 10.1126/science.aba3827. No abstract available.


Cardiovascular complications of sickle cell disease.

Sachdev V, Rosing DR, Thein SL.

Trends Cardiovasc Med. 2020 Feb 11. pii: S1050-1738(20)30020-7. doi: 10.1016/j.tcm.2020.02.002. [Epub ahead of print] Review.


Hemolytic transfusion reactions in sickle cell disease: underappreciated and potentially fatal.

Thein SL, Pirenne F, Fasano RM, Habibi A, Bartolucci P, Chonat S, Hendrickson JE, Stowell SR.

Haematologica. 2020 Mar;105(3):539-544. doi: 10.3324/haematol.2019.224709. Epub 2020 Feb 6. No abstract available.


Whole genome sequence-based haplotypes reveal a single origin of the 1393 bp HBB deletion.

Wang X, Xu JZ, Conrey A, Mendelsohn L, Shriner D, Pirooznia M, Thein SL.

J Med Genet. 2020 Jan 30. pii: jmedgenet-2019-106698. doi: 10.1136/jmedgenet-2019-106698. [Epub ahead of print]


American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support.

Chou ST, Alsawas M, Fasano RM, Field JJ, Hendrickson JE, Howard J, Kameka M, Kwiatkowski JL, Pirenne F, Shi PA, Stowell SR, Thein SL, Westhoff CM, Wong TE, Akl EA.

Blood Adv. 2020 Jan 28;4(2):327-355. doi: 10.1182/bloodadvances.2019001143.


Proteomic Studies for the Investigation of γ-Globin Induction by Decitabine in Human Primary Erythroid Progenitor Cultures.

Theodorou A, Phylactides M, Katsantoni E, Vougas K, Garbis SD, Fanis P, Sitarou M, Thein SL, Kleanthous M.

J Clin Med. 2020 Jan 3;9(1). pii: E134. doi: 10.3390/jcm9010134.


Neutrophils remain detrimentally active in hydroxyurea-treated patients with sickle cell disease.

Barbu EA, Dominical VM, Mendelsohn L, Thein SL.

PLoS One. 2019 Dec 23;14(12):e0226583. doi: 10.1371/journal.pone.0226583. eCollection 2019.


Optimal disease management and health monitoring in adults with sickle cell disease.

Howard J, Thein SL.

Hematology Am Soc Hematol Educ Program. 2019 Dec 6;2019(1):505-512. doi: 10.1182/hematology.2019000055.


Pro-inflammatory cytokines associate with NETosis during sickle cell vaso-occlusive crises.

Barbu EA, Mendelsohn L, Samsel L, Thein SL.

Cytokine. 2020 Mar;127:154933. doi: 10.1016/j.cyto.2019.154933. Epub 2019 Nov 25.


Identifying Clinical and Research Priorities in Sickle Cell Lung Disease. An Official American Thoracic Society Workshop Report.

Ruhl AP, Sadreameli SC, Allen JL, Bennett DP, Campbell AD, Coates TD, Diallo DA, Field JJ, Fiorino EK, Gladwin MT, Glassberg JA, Gordeuk VR, Graham LM, Greenough A, Howard J, Kato GJ, Knight-Madden J, Kopp BT, Koumbourlis AC, Lanzkron SM, Liem RI, Machado RF, Mehari A, Morris CR, Ogunlesi FO, Rosen CL, Smith-Whitley K, Tauber D, Terry N, Thein SL, Vichinsky E, Weir NA, Cohen RT, Klings ES.

Ann Am Thorac Soc. 2019 Sep;16(9):e17-e32. doi: 10.1513/AnnalsATS.201906-433ST.


A Growing Population of Older Adults with Sickle Cell Disease.

Shet AS, Thein SL.

Clin Geriatr Med. 2019 Aug;35(3):349-367. doi: 10.1016/j.cger.2019.03.006. Epub 2019 May 9. Review.


The carrier state for sickle cell disease is not completely harmless.

Xu JZ, Thein SL.

Haematologica. 2019 Jun;104(6):1106-1111. doi: 10.3324/haematol.2018.206060. Epub 2019 May 16. No abstract available.


Interactions of an Anti-Sickling Drug with Hemoglobin in Red Blood Cells from a Patient with Sickle Cell Anemia.

Strader MB, Liang H, Meng F, Harper J, Ostrowski DA, Henry ER, Shet AS, Eaton WA, Thein SL, Alayash AI.

Bioconjug Chem. 2019 Mar 20;30(3):568-571. doi: 10.1021/acs.bioconjchem.9b00130. Epub 2019 Feb 28.


Voxelotor treatment of a patient with sickle cell disease and very severe anemia.

Shet AS, Mendelsohn L, Harper J, Ostrowski D, Henry ER, Gwaabe E, Nichols J, Alayash AI, Eaton WA, Thein SL.

Am J Hematol. 2019 Apr;94(4):E88-E90. doi: 10.1002/ajh.25389. Epub 2019 Jan 8. No abstract available.


Genetic Modifiers of Fetal Haemoglobin in Sickle Cell Disease.

Menzel S, Thein SL.

Mol Diagn Ther. 2019 Apr;23(2):235-244. doi: 10.1007/s40291-018-0370-8. Review.


A ten year review of the sickle cell program in Muhimbili National Hospital, Tanzania.

Makani J, Tluway F, Makubi A, Soka D, Nkya S, Sangeda R, Mgaya J, Rwezaula S, Kirkham FJ, Kindole C, Osati E, Meda E, Snow RW, Newton CR, Roberts D, Aboud M, Thein SL, Cox SE, Luzzatto L, Mmbando BP.

BMC Hematol. 2018 Nov 14;18:33. doi: 10.1186/s12878-018-0125-0. eCollection 2018.


The platelet NLRP3 inflammasome is upregulated in sickle cell disease via HMGB1/TLR4 and Bruton tyrosine kinase.

Vogel S, Arora T, Wang X, Mendelsohn L, Nichols J, Allen D, Shet AS, Combs CA, Quezado ZMN, Thein SL.

Blood Adv. 2018 Oct 23;2(20):2672-2680. doi: 10.1182/bloodadvances.2018021709.


How I treat the older adult with sickle cell disease.

Thein SL, Howard J.

Blood. 2018 Oct 25;132(17):1750-1760. doi: 10.1182/blood-2018-03-818161. Epub 2018 Sep 11. Review.


GlycA is not a useful biomarker of inflammation in sickle cell disease.

Weisman JK, Meeks D, Mendelsohn L, Remaley AT, Sampson M, Allen DT, Nichols J, Shet AS, Thein SL.

Int J Lab Hematol. 2018 Dec;40(6):704-709. doi: 10.1111/ijlh.12907. Epub 2018 Aug 27.


Sickle cell disease-Unanswered questions and future directions in therapy.

Thein SL, Tisdale J.

Semin Hematol. 2018 Apr;55(2):51-52. doi: 10.1053/j.seminhematol.2018.05.010. Epub 2018 May 26. No abstract available.


Abnormal Ventilation-Perfusion Scan Is Associated with Pulmonary Hypertension in Sickle Cell Adults.

Mehari A, Igbineweka N, Allen D, Nichols J, Thein SL, Weir NA.

J Nucl Med. 2019 Jan;60(1):86-92. doi: 10.2967/jnumed.118.211466. Epub 2018 Jun 7.


A survey of genetic fetal-haemoglobin modifiers in Nigerian patients with sickle cell anaemia.

Adeyemo TA, Ojewunmi OO, Oyetunji IA, Rooks H, Rees DC, Akinsulie AO, Akanmu AS, Thein SL, Menzel S.

PLoS One. 2018 Jun 7;13(6):e0197927. doi: 10.1371/journal.pone.0197927. eCollection 2018.


MYB - A regulatory factor in hematopoiesis.

Wang X, Angelis N, Thein SL.

Gene. 2018 Jul 30;665:6-17. doi: 10.1016/j.gene.2018.04.065. Epub 2018 Apr 25. Review.


Sickle Cell Anemia and Its Phenotypes.

Williams TN, Thein SL.

Annu Rev Genomics Hum Genet. 2018 Aug 31;19:113-147. doi: 10.1146/annurev-genom-083117-021320. Epub 2018 Apr 11. Review.


Switching from fetal to adult hemoglobin.

Wang X, Thein SL.

Nat Genet. 2018 Apr;50(4):478-480. doi: 10.1038/s41588-018-0094-z. No abstract available.


Proteomic analysis of plasma from children with sickle cell anemia and silent cerebral infarction.

Tewari S, Renney G, Brewin J, Gardner K, Kirkham F, Inusa B, Barrett JE, Menzel S, Thein SL, Ward M, Rees DC.

Haematologica. 2018 Jul;103(7):1136-1142. doi: 10.3324/haematol.2018.187815. Epub 2018 Mar 15.


Pain and opioid use after reversal of sickle cell disease following HLA-matched sibling haematopoietic stem cell transplant.

Darbari DS, Liljencrantz J, Ikechi A, Martin S, Roderick MC, Fitzhugh CD, Tisdale JF, Thein SL, Hsieh M.

Br J Haematol. 2019 Feb;184(4):690-693. doi: 10.1111/bjh.15169. Epub 2018 Mar 12. No abstract available.


g(HbF): a genetic model of fetal hemoglobin in sickle cell disease.

Gardner K, Fulford T, Silver N, Rooks H, Angelis N, Allman M, Nkya S, Makani J, Howard J, Kesse-Adu R, Rees DC, Stuart-Smith S, Yeghen T, Awogbade M, Sangeda RZ, Mgaya J, Patel H, Newhouse S, Menzel S, Thein SL.

Blood Adv. 2018 Feb 13;2(3):235-239. doi: 10.1182/bloodadvances.2017009811.


Platelets at the crossroads of thrombosis, inflammation and haemolysis.

Vogel S, Thein SL.

Br J Haematol. 2018 Mar;180(5):761-767. doi: 10.1111/bjh.15117. Epub 2018 Jan 30.


Overnight auto-adjusting continuous airway pressure + standard care compared with standard care alone in the prevention of morbidity in sickle cell disease phase II (POMS2b): study protocol for a randomised controlled trial.

Howard J, Slee AE, Skene S, Inusa B, Kawadler J, Downes M, Gavlak J, Koelbel M, Stotesbury H, Chorozoglou M, Tebbs S, Chakravorty S, Awogbade M, Rees DC, Gupta A, Murphy PB, Hart N, Sahota S, Nwosu C, Gwam M, Saunders D, Muthurangu V, Barber N, Ako E, Thein SL, Marshall M, Reading IC, Cheng MYE, Kirkham FJ, Liossi C.

Trials. 2018 Jan 22;19(1):55. doi: 10.1186/s13063-017-2419-0.


Heterogeneity of respiratory disease in children and young adults with sickle cell disease.

Lunt A, Mortimer L, Rees D, Height S, Thein SL, Greenough A.

Thorax. 2018 Jun;73(6):575-577. doi: 10.1136/thoraxjnl-2017-210206. Epub 2017 Nov 29.


Catheter-related right atrial thrombus in sickle cell disease.

Zhao C, Sathya B, Nadal Rios R, Arai AE, Brofferio A, Thein SL, Ruhl AP.

Clin Case Rep. 2017 Sep 20;5(11):1898-1900. doi: 10.1002/ccr3.1187. eCollection 2017 Nov.


Genetic Basis and Genetic Modifiers of β-Thalassemia and Sickle Cell Disease.

Thein SL.

Adv Exp Med Biol. 2017;1013:27-57. doi: 10.1007/978-1-4939-7299-9_2. Review.


Therapeutic advances in sickle cell disease in the last decade.

Shet AS, Thein SL.

Indian J Med Res. 2017 Jun;145(6):708-712. doi: 10.4103/ijmr.IJMR_1153_17. No abstract available.


Increased prevalence of renal cysts in patients with sickle cell disease.

Meeks D, Navaratnarajah A, Drasar E, Jaffer O, Wilkins CJ, Thein SL, Sharpe CC.

BMC Nephrol. 2017 Sep 21;18(1):298. doi: 10.1186/s12882-017-0714-3.


A Plea for the Newborn Diagnosis of Hb S-Hereditary Persistence of Fetal Hemoglobin.

Serjeant GR, Serjeant BE, Hambleton IR, Oakley M, Thein SL, Clark B.

Hemoglobin. 2017 May;41(3):216-217. doi: 10.1080/03630269.2017.1360336. Epub 2017 Sep 5.


Fetal Hemoglobin is Associated with Peripheral Oxygen Saturation in Sickle Cell Disease in Tanzania.

Nkya S, Mgaya J, Urio F, Makubi A, Thein SL, Menzel S, Cox SE, Newton CR, Kirkham FJ, Mmbando BP, Makani J.

EBioMedicine. 2017 Sep;23:146-149. doi: 10.1016/j.ebiom.2017.08.006. Epub 2017 Aug 8.


Molecular basis of β thalassemia and potential therapeutic targets.

Thein SL.

Blood Cells Mol Dis. 2018 May;70:54-65. doi: 10.1016/j.bcmd.2017.06.001. Epub 2017 Jun 20. Review.


Detection of Hb Rothschild HBB: c.[112T>A or 112T>C], Through High Index of Suspicion on Abnormal Pulse Oximetry.

Alli NA, Wessels P, Rampersad N, Clark BE, Thein SL.

Hemoglobin. 2017 Mar;41(2):137-139. doi: 10.1080/03630269.2017.1324796. Epub 2017 Jun 16.


Association of plasma CD40L with acute chest syndrome in sickle cell anemia.

Garrido VT, Sonzogni L, Mtatiro SN, Costa FF, Conran N, Thein SL.

Cytokine. 2017 Sep;97:104-107. doi: 10.1016/j.cyto.2017.05.017. Epub 2017 Jun 10.


Next-generation sequencing as a tool for breakpoint analysis in rearrangements of the globin gene clusters.

Clark BE, Shooter C, Smith F, Brawand D, Thein SL.

Int J Lab Hematol. 2017 May;39 Suppl 1:111-120. doi: 10.1111/ijlh.12680. Review.


Genetic control of erythropoiesis.

Tumburu L, Thein SL.

Curr Opin Hematol. 2017 May;24(3):173-182. doi: 10.1097/MOH.0000000000000333. Review.


Interim assessment of liver damage in patients with sickle cell disease using new non-invasive techniques.

Drasar E, Fitzpatrick E, Gardner K, Awogbade M, Dhawan A, Bomford A, Suddle A, Thein SL.

Br J Haematol. 2017 Feb;176(4):643-650. doi: 10.1111/bjh.14462. Epub 2016 Dec 16.


Sickle cell disease in the older adult.

Thein MS, Igbineweka NE, Thein SL.

Pathology. 2017 Jan;49(1):1-9. doi: 10.1016/j.pathol.2016.10.002. Epub 2016 Nov 30. Review.


Associations between environmental factors and hospital admissions for sickle cell disease.

Piel FB, Tewari S, Brousse V, Analitis A, Font A, Menzel S, Chakravorty S, Thein SL, Inusa B, Telfer P, de Montalembert M, Fuller GW, Katsouyanni K, Rees DC.

Haematologica. 2017 Apr;102(4):666-675. doi: 10.3324/haematol.2016.154245. Epub 2016 Dec 1.


ASH1L (a histone methyltransferase protein) is a novel candidate globin gene regulator revealed by genetic study of an English family with beta-thalassaemia unlinked to the beta-globin locus.

Breton A, Theodorou A, Aktuna S, Sonzogni L, Darling D, Chan L, Menzel S, van der Spek PJ, Swagemakers SM, Grosveld F, Philipsen S, Thein SL.

Br J Haematol. 2016 Nov;175(3):525-530. doi: 10.1111/bjh.14256. Epub 2016 Jul 19.


World Sickle Cell Day 2016 : A time for appraisal.

Thein MS, Thein SL.

Indian J Med Res. 2016 Jun;143(6):678-681. doi: 10.4103/0971-5916.191917. No abstract available.


Reversible binding of hemoglobin to band 3 constitutes the molecular switch that mediates O2 regulation of erythrocyte properties.

Chu H, McKenna MM, Krump NA, Zheng S, Mendelsohn L, Thein SL, Garrett LJ, Bodine DM, Low PS.

Blood. 2016 Dec 8;128(23):2708-2716. Epub 2016 Sep 29.


Loss of Major DNase I Hypersensitive Sites in Duplicated β-globin Gene Cluster Incompletely Silences HBB Gene Expression.

Reading NS, Shooter C, Song J, Miller R, Agarwal A, Lanikova L, Clark B, Thein SL, Divoky V, Prchal JT.

Hum Mutat. 2016 Nov;37(11):1153-1156. doi: 10.1002/humu.23061. Epub 2016 Aug 25.


Beta thalassaemia intermedia due to co-inheritance of three unique alpha globin cluster duplications characterised by next generation sequencing analysis.

Clark B, Shooter C, Smith F, Brawand D, Steedman L, Oakley M, Rushton P, Rooks H, Wang X, Drousiotou A, Kyrri A, Hadjigavriel M, Will A, Fisher C, Higgs DR, Phylipsen M, Harteveld C, Kleanthous M, Thein SL.

Br J Haematol. 2018 Jan;180(1):160-164. doi: 10.1111/bjh.14294. Epub 2016 Jul 29. No abstract available.


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