Abdullah Kutlar

Summary

Affiliation: Medical College of Georgia
Country: USA

Publications

  1. ncbi The methylene tetrahydrofolate reductase (C677T) mutation as a potential risk factor for avascular necrosis in sickle cell disease
    A Kutlar
    Sickle Cell Center, Department of Medicine, Medical College of Georgia, Augusta 30912, USA
    Hemoglobin 25:213-7. 2001
  2. ncbi Sickle cell disease: a multigenic perspective of a single gene disorder
    Abdullah Kutlar
    Sickle Cell Center, Medical College of Georgia, Augusta, Georgia 30912 3680, USA
    Hemoglobin 31:209-24. 2007
  3. ncbi Sickle cell disease: a multigenic perspective of a single-gene disorder
    Abdullah Kutlar
    Sickle Cell Center, Medical College of Georgia, Augusta, GA 30912, USA
    Med Princ Pract 14:15-9. 2005
  4. pmc Genome-wide association study of cardiac structure and systolic function in African Americans: the Candidate Gene Association Resource (CARe) study
    Ervin R Fox
    Department of Medicine, University of Mississippi Medical Center, 2500 North State St, Jackson, MS 39216, USA
    Circ Cardiovasc Genet 6:37-46. 2013
  5. doi A dose-escalation phase IIa study of 2,2-dimethylbutyrate (HQK-1001), an oral fetal globin inducer, in sickle cell disease
    Abdullah Kutlar
    Adult Sickle Cell Center, Georgia Regents University Medical Center, Augusta, GA, USA
    Am J Hematol 88:E255-60. 2013
  6. doi Neonatal cyanosis due to a novel fetal hemoglobin: Hb F-Circleville [Ggamma63(E7)His-->Leu, CAT>CTT]
    Erin Dainer
    Department of Medicine, Medical College of Georgia, Augusta, GA 30912, USA
    Hemoglobin 32:596-600. 2008
  7. ncbi Regular transfusion lowers plasma free hemoglobin in children with sickle-cell disease at risk for stroke
    Nelson E Lezcano
    Department of Neurology, School of Medicine, Medical College of Georgia, Augusta, GA 30912, USA
    Stroke 37:1424-6. 2006
  8. doi Hb Fulton-Georgia [α20(B1)His→Pro; HBA1: c.62A>C]: a new α-globin variant coinherited with α-thalassemia-2 (3.7 kb deletion) and Hb SC disease
    Lina Zhuang
    Department of Medicine, Georgia Health Sciences University Medical College of Georgia, Augusta, Georgia, USA
    Hemoglobin 37:481-5. 2013
  9. pmc The proinflammatory cytokine GM-CSF downregulates fetal hemoglobin expression by attenuating the cAMP-dependent pathway in sickle cell disease
    Tohru Ikuta
    Department of Anesthesiology and Perioperative Medicine, Medical College of Georgia, Georgia Health Sciences University, Augusta, USA
    Blood Cells Mol Dis 47:235-42. 2011
  10. doi Two new hemoglobin variants: Hb Tallahassee [α3(A1)Ser→Tyr; HBA2: c.11C>A] and Hb madison-NC [β119(GH2)Gly→Ser; HBB: c.358G>A]
    Ferdane Kutlar
    Department of Medicine, Medical College of Georgia Georgia Regents University, Augusta, Georgia, USA
    Hemoglobin 38:207-10. 2014

Detail Information

Publications37

  1. ncbi The methylene tetrahydrofolate reductase (C677T) mutation as a potential risk factor for avascular necrosis in sickle cell disease
    A Kutlar
    Sickle Cell Center, Department of Medicine, Medical College of Georgia, Augusta 30912, USA
    Hemoglobin 25:213-7. 2001
    ..6% in patients with AVN and 12.9% in those without AVN (p = 0.006). These data suggest that the thermolabile MTHFR variant may be a contributing risk factor for AVN in some populations with sickle cell disease...
  2. ncbi Sickle cell disease: a multigenic perspective of a single gene disorder
    Abdullah Kutlar
    Sickle Cell Center, Medical College of Georgia, Augusta, Georgia 30912 3680, USA
    Hemoglobin 31:209-24. 2007
    ..This brief review focuses on the recent advances in our understanding of the impact of non globin genetic modifiers on the phenotypic diversity of the disease...
  3. ncbi Sickle cell disease: a multigenic perspective of a single-gene disorder
    Abdullah Kutlar
    Sickle Cell Center, Medical College of Georgia, Augusta, GA 30912, USA
    Med Princ Pract 14:15-9. 2005
    ..Mention is made of genetic polymorphisms that might predispose to stroke. The application of gene expression profiling to the study of SCD is very promising and some preliminary data are provided...
  4. pmc Genome-wide association study of cardiac structure and systolic function in African Americans: the Candidate Gene Association Resource (CARe) study
    Ervin R Fox
    Department of Medicine, University of Mississippi Medical Center, 2500 North State St, Jackson, MS 39216, USA
    Circ Cardiovasc Genet 6:37-46. 2013
    ..Using data from 4 community-based cohorts of African Americans, we tested the association between genome-wide markers (single-nucleotide polymorphisms) and cardiac phenotypes in the Candidate-gene Association Resource study...
  5. doi A dose-escalation phase IIa study of 2,2-dimethylbutyrate (HQK-1001), an oral fetal globin inducer, in sickle cell disease
    Abdullah Kutlar
    Adult Sickle Cell Center, Georgia Regents University Medical Center, Augusta, GA, USA
    Am J Hematol 88:E255-60. 2013
    ..Total hemoglobin increased by a mean of 0.65 g/dL (95% CI, 0.5-1.0 g/dL), and 13 subjects (25%) had increases ≥1 g/dL. Future studies are warranted to evaluate the therapeutic potential of HQK-1001 in sickle cell disease. ...
  6. doi Neonatal cyanosis due to a novel fetal hemoglobin: Hb F-Circleville [Ggamma63(E7)His-->Leu, CAT>CTT]
    Erin Dainer
    Department of Medicine, Medical College of Georgia, Augusta, GA 30912, USA
    Hemoglobin 32:596-600. 2008
    ..Here we describe a novel fetal Hb variant, Hb F-Circleville [Ggamma63(E7)His-->Leu], associated with methemoglobinemia and cyanosis in the newborn. The patient's sister also had neonatal cyanosis at birth...
  7. ncbi Regular transfusion lowers plasma free hemoglobin in children with sickle-cell disease at risk for stroke
    Nelson E Lezcano
    Department of Neurology, School of Medicine, Medical College of Georgia, Augusta, GA 30912, USA
    Stroke 37:1424-6. 2006
    ..PFH has been associated with harmful endothelial actions including scavenging nitric oxide (NO). Whether PFH plays a role in stroke in SCD has not been examined...
  8. doi Hb Fulton-Georgia [α20(B1)His→Pro; HBA1: c.62A>C]: a new α-globin variant coinherited with α-thalassemia-2 (3.7 kb deletion) and Hb SC disease
    Lina Zhuang
    Department of Medicine, Georgia Health Sciences University Medical College of Georgia, Augusta, Georgia, USA
    Hemoglobin 37:481-5. 2013
    ..Coinheritance of Hb Fulton-Georgia, heterozygous α-thal-2 and Hb SC disease was associated with a mild phenotype, consisting of microcytosis and anisocytosis, but no anemia or other hematological abnormality. ..
  9. pmc The proinflammatory cytokine GM-CSF downregulates fetal hemoglobin expression by attenuating the cAMP-dependent pathway in sickle cell disease
    Tohru Ikuta
    Department of Anesthesiology and Perioperative Medicine, Medical College of Georgia, Georgia Health Sciences University, Augusta, USA
    Blood Cells Mol Dis 47:235-42. 2011
    ..Reduction in GM-CSF levels upon hydroxyurea therapy may be critical for efficient HbF induction. The results showing the involvement of GM-CSF in HbF expression may suggest possible mechanisms for hydroxyurea resistance in SCD...
  10. doi Two new hemoglobin variants: Hb Tallahassee [α3(A1)Ser→Tyr; HBA2: c.11C>A] and Hb madison-NC [β119(GH2)Gly→Ser; HBB: c.358G>A]
    Ferdane Kutlar
    Department of Medicine, Medical College of Georgia Georgia Regents University, Augusta, Georgia, USA
    Hemoglobin 38:207-10. 2014
    ..11C > A] named Hb Tallahassee and the other is a β-globin chain variant located at codon 119 [β119(GH2)Gly→Ser; HBB: c.358G > A] called Hb Madison-NC...
  11. pmc Pomalidomide augments fetal hemoglobin production without the myelosuppressive effects of hydroxyurea in transgenic sickle cell mice
    Steffen E Meiler
    Department of Anesthesiology and Perioperative Medicine, Georgia Health Sciences University, Augusta, GA, USA
    Blood 118:1109-12. 2011
    ..Surprisingly, combinatory therapy with both drugs failed to mitigate hydroxyurea's myelotoxic effects and caused loss of HbF induction. These findings support further evaluation of pomalidomide as a novel therapy for SCD...
  12. doi Two new hemoglobin variants: Hb Sinai-Greenspring [beta34(B16)Val-->Ile, GTC > ATC] and Hb Sinai-Bel Air [beta53(D4)Ala-->Asp, GCT > GAT]
    Erin Dainer
    Sickle Cell Center, Department of Medicine, Medical College of Georgia, Augusta, GA 30912, USA
    Hemoglobin 32:588-91. 2008
    ..The variants were characterized by cation exchange high performance liquid chromatography (HPLC), reversed phase HPLC, and sequencing of amplified beta-globin genes. Functional studies could not be performed at this time...
  13. ncbi A novel approach to rapid determination of betaS-globin haplotypes: sequencing of the Agamma-IVS-II region
    Amy E Vinson
    Sickle Cell Center, Department of Medicine Medical College of Georgia, Augusta, Georgia, USA
    Hemoglobin 28:317-23. 2004
    ..We provide an algorithm that allows rapid assignment of the four African haplotypes carrying the sickle mutation...
  14. doi Acute splenic complications and implications of splenectomy in hemoglobin SC disease
    Karthi Subbannan
    Department of Medicine, Section of Hematology Oncology, Sickle Cell Center, Medical College of Georgia, Augusta, Georgia 30912, USA
    Eur J Haematol 83:258-60. 2009
    ..Although it appears to be safe regarding short-term complications of surgery, long-term adverse effects such as infections have to be evaluated cautiously...
  15. ncbi Vasoactive factors in sickle cell disease: in vitro evidence for endothelin-1-mediated vasoconstriction
    Sitki Ergul
    Division of Hematology and Oncology, Medical University of South Carolina, Charleston, SC, USA
    Am J Hematol 76:245-51. 2004
    ....
  16. doi A new Aγ-globin chain variant: Hb F-Sykesville MD [Aγ113(G15)Val → Ile; HBG1: c.340G>A] detected in a Caucasian baby
    Niren Patel
    Department of Medicine, Georgia Regent s University Augusta, Augusta, Georgia, USA
    Hemoglobin 39:52-4. 2015
    ..In this report we discuss the molecular characteristics and diagnostic criteria of a new (A)γ chain variant that was detected during newborn screening and named Hb F-Sykesville MD [(A)γ113(G15)Val → Ile; HBG1: c.340G>A]...
  17. pmc A phase 1/2 trial of HQK-1001, an oral fetal globin inducer, in sickle cell disease
    Abdullah Kutlar
    Department of Medicine, Sickle Cell Center, Medical College of Georgia, Augusta, GA, USA
    Am J Hematol 87:1017-21. 2012
    ..These findings of favorable PK profiles, tolerability, early rises in HbF, and total Hgb indicate that trials of longer duration appear warranted to more definitively evaluate the therapeutic potential of HQK-1001 in sickle cell disease...
  18. pmc The risks and benefits of long-term use of hydroxyurea in sickle cell anemia: A 17.5 year follow-up
    Martin H Steinberg
    Department of Medicine, Boston University School of Medicine, Boston Medical Center, Massachusetts, USA
    Am J Hematol 85:403-8. 2010
    ..Our results, while no longer the product of a randomized study because of the ethical concerns of withholding an efficacious treatment, suggest that long-term use of hydroxyurea is safe and might decrease mortality...
  19. doi Rare occurrence of Hb Lepore-Baltimore in African Americans: molecular characteristics and variations of Hb Lepores
    Sean M McKeown
    Department of Medicine, Medical College of Georgia, Augusta, GA, USA
    Ann Hematol 88:545-8. 2009
    ..We have used state-of-the-art technology, combining protein- and DNA-based methods, in the analysis of the hybrid hemoglobin and discuss its molecular characteristics...
  20. ncbi Sickle cell disease: a multigenic perspective of a single gene disorder
    Abdullah Kutlar
    Medical College of Georgia, Sickle Cell Center, Augusta, GA, USA
    Hematology 10:92-9. 2005
  21. pmc Coagulation activation in sickle cell trait: an exploratory study
    Chirag Amin
    Department of Medicine, University of North Carolina School of Medicine, Chapel Hill, NC, USA
    Br J Haematol 171:638-46. 2015
    ..Further studies in larger cohorts of subjects with sickle cell trait are needed to confirm the results of this preliminary investigation. ..
  22. doi Two new γ chain variants: Hb F-Augusta GA [(G)γ59(E3)Lys → Arg; HBG2: c.179A > G] and Hb F-Port Royal-II [(A)γ125(H3)Glu → Ala; HBG1: c.377A > C]
    Ferdane Kutlar
    Department of Medicine, Georgia Regents University at Augusta, Augusta, Georgia, USA and
    Hemoglobin 38:376-80. 2014
    ..One is a new (G)γ-globin chain variant, Hb F-Augusta GA [(G)γ59(E3)Lys → Arg; HBG2: c.179A > G] and the other one is Hb F-Port Royal-II [(A)γ125(H3)Glu → Ala; HBG1: c.377A > C]...
  23. ncbi Erythrocyte sickling during exercise and thermal stress
    Michael F Bergeron
    Department of Physical Therapy, Medical College of Georgia, Augusta, GA 30912 0800, USA
    Clin J Sport Med 14:354-6. 2004
    ..To examine effects of exercise in the heat and fluid intake on erythrocyte sickling and neutrophil activation in carriers of sickle cell trait (HbAS)...
  24. ncbi Mortality in sickle cell patients on hydroxyurea therapy
    Sule M Bakanay
    Sickle Cell Center, Department of Medicine, Office of Biostatistics and Bioinformatics, Medical College of Georgia, Augusta 30912, USA
    Blood 105:545-7. 2005
    ..Sickle cell patients who die while on HU therapy may represent a subgroup of older patients, possibly with more severe disease and more severe organ damage. Such patients need early identification and prompt HU institution...
  25. pmc Genetic risk factors for cerebrovascular disease in children with sickle cell disease: design of a case-control association study and genomewide screen
    Gaye T Adams
    Sickle Cell Center, Department of Medicine, Medical College of Georgia, Augusta, GA, USA
    BMC Med Genet 4:6. 2003
    ..The genetic factors leading to the development of a high TCD velocity (i.e. cerebrovascular disease) and ultimately to stroke are not well characterized...
  26. pmc Association of genetic variation with systolic and diastolic blood pressure among African Americans: the Candidate Gene Association Resource study
    Ervin R Fox
    Department of Medicine, University of Mississippi Medical Center, Jackson, MS, USA
    Hum Mol Genet 20:2273-84. 2011
    ..More broadly, this work supports that notion that blood pressure among AAs is a trait with genetic underpinnings but also with significant complexity...
  27. pmc Endothelin-1 contributes to the progression of renal injury in sickle cell disease via reactive oxygen species
    J Brett Heimlich
    1epartment of Physiology, Augusta University, Augusta, GA 30912, USA
    Br J Pharmacol 173:386-95. 2016
    ..The current study was designed to determine whether ET-1 acting via the ETA receptor contributes to renal injury in a mouse model of sickle cell disease...
  28. doi Cellular adhesion and the endothelium: P-selectin
    Abdullah Kutlar
    Sickle Cell Center, Department of Medicine, Georgia Regents University, 1120 15th Street, Augusta, GA 30912, USA
    Hematol Oncol Clin North Am 28:323-39. 2014
    ....
  29. ncbi Interaction of sickle cell trait with hereditary spherocytosis: splenic infarcts and sequestration
    Celatettin Ustun
    Department of Medicine, Section of Hematology Oncology, Medical College of Georgia, Augusta, GA 30912, USA
    Acta Haematol 109:46-9. 2003
    ..The interaction of HS and SCT leads to RBC dehydration with increased MCHC and intracellular Hb S concentration presumably favoring intrasplenic sickling and resultant splenic infarcts and sequestration as seen in this case...
  30. ncbi Concordant fetal hemoglobin response to hydroxyurea in siblings with sickle cell disease
    Martin H Steinberg
    Boston University School of Medicine, Room 211, 88 E Newton Street, Boston, MA 02118, USA
    Am J Hematol 72:121-6. 2003
    ..Our results provide additional evidence that some elements that regulate HbF expression are linked to the beta-globin gene cluster...
  31. ncbi Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment
    Martin H Steinberg
    Boston University School of Medicine, Center of Excellence in Sickle Cell Disease, Boston Medical Center, Boston, Mass 02118, USA
    JAMA 289:1645-51. 2003
    ..Hydroxyurea increases levels of fetal hemoglobin (HbF) and decreases morbidity from vaso-occlusive complications in patients with sickle cell anemia (SCA). High HbF levels reduce morbidity and mortality...
  32. ncbi Alpha Thalassemia is associated with decreased risk of abnormal transcranial Doppler ultrasonography in children with sickle cell anemia
    Lewis L Hsu
    Emory University School of Medicine and Georgia Comprehensive Sickle Cell Center, Atlanta, Georgia 30303, USA
    J Pediatr Hematol Oncol 25:622-8. 2003
    ..The authors hypothesized that children with abnormal TCD are less likely to have alpha thalassemia-2, and an increased hemoglobin level accounts for this protective effect...
  33. ncbi Hb Setif [alpha94(G1)Asp --> Tyr (alpha2)] detected in a Turkish family
    Guncag Dincol
    Division of Hematology, Department of Internal Medicine, Istanbul Medical School, University of Istanbul, Capa, Istanbul, Turkey
    Hemoglobin 27:249-52. 2003
  34. pmc Hemoglobin binding to A beta and HBG2 SNP association suggest a role in Alzheimer's disease
    Rodney T Perry
    Department of Epidemiology, University of Alabama at Birmingham, Birmingham, AL 35294 0022, USA
    Neurobiol Aging 29:185-93. 2008
    ..010) and >65 years (p=0.013), and families not homozygous for the APOE4 allele (p=0.041). We hypothesize that Hb F may be less toxic than adult Hb in its interaction with A beta and may protect against the development of AD...
  35. ncbi Molecular characterization of Hb D-Ibadan [beta87(F3)Thr-->Lys] in combination with Hb S [beta6(A3)Glu-->Val] and with beta+-Thalassemia: report of two cases
    Rupa Redding-Lallinger
    Department of Pediatrics and Internal Medicine, University of North Carolina, Chapel Hill 27599 7220, USA
    Hemoglobin 26:129-34. 2002
    ....
  36. ncbi Longitudinal changes in ferritin during chronic transfusion: a report from the Stroke Prevention Trial in Sickle Cell Anemia (STOP)
    Beatrice Files
    Children s Healthcare of Atlanta Scottish Rite, Atlanta, Georgia 30342, USA
    J Pediatr Hematol Oncol 24:284-90. 2002
    ..Iron overload, the inevitable result of chronic transfusion, is commonly monitored with serum ferritin concentration...
  37. doi Efficacy and safety of the Gardos channel blocker, senicapoc (ICA-17043), in patients with sickle cell anemia
    Kenneth I Ataga
    Division of Hematology Oncology, University of North Carolina at Chapel Hill, CB no 7305, 3009 Old Clinic Bldg, Chapel Hill, NC 27599 7305, USA
    Blood 111:3991-7. 2008
    ..This study is registered at http://clinicaltrials.gov as NCT00040677...