Access the full text.
Sign up today, get DeepDyve free for 14 days.
M. Steinberg, A. Adewoye (2006)
Modifier genes and sickle cell anemiaCurrent Opinion in Hematology, 13
(1998)
A Syllabus of Hemoglobin Variants, ed 2. Augusta
M. Hynes, H. Lehmann (1956)
Haemoglobin D in a Persian Girl: Presumably the First Case of Haemoglobin-D—ThalassaemiaBritish Medical Journal, 2
A. Bahadir, Aylin Köseler, A. Atalay, H. Koyuncu, E. Akar, N. Akar, E. Atalay (2009)
Hb D-Los Angeles [beta121(GH4)Glu>Gln] and Hb Beograd [beta121(GH4)Glu>Val]: Implications for their laboratory diagnosis and genetic origins.Turkish journal of haematology : official journal of Turkish Society of Haematology, 26 1
K. Adachi, J. Kim, S. Ballas, S. Surrey, T. Asakura (1988)
Facilitation of Hb S polymerization by the substitution of Glu for Gln at beta 121.The Journal of biological chemistry, 263 12
KD Lanclos, C. Oner, AJ Dimovski, Y.-C. Gu, TH Huisman (1991)
Sequence variations in the 5' flanking and IVS-II regions of the G gamma- and A gamma-globin genes of beta S chromosomes with five different haplotypesBlood, 77
S. Charache, S. Charache, C. Conley, C. Conley (1964)
RATE OF SICKLING OF RED CELLS DURING DEOXYGENATION OF BLOOD FROM PERSONS WITH VARIOUS SICKLING DISORDERS.Blood, 24
E. Bissé, H. Wieland (1988)
High-performance liquid chromatographic separation of human haemoglobins. Simultaneous quantitation of foetal and glycated haemoglobins.Journal of chromatography, 434 1
M. Mukherjee, R. Surve, R. Gangakhedkar, D. Mohanty, R. Colah (2005)
Hemoglobin sickle D Punjab—a case reportIndian Journal of Human Genetics, 11
R. Schneider, S. Ueda, J. Alperin, W. Levin, R. Jones, B. Brimhall (1968)
Hemoglobin D Los Angeles in two Caucasian families: hemoglobin SD disease and hemoglobin D thalassemia.Blood, 32 2
R. Bookchin, R. Nagel, T. Balazs (1975)
Role of hybrid tetramer formation in gelation of haemoglobin SNature, 256
E. Atalay, A. Atalay, Emre Üstel, Sanem Yildiz, O. Öztürk, Aylin Köseler, A. Bahadir (2007)
Genetic Origin of Hb D-Los Angeles [β121(GH4)Glu→Gln, GAA→CAA] According to the β-Globin Gene Cluster HaplotypesHemoglobin, 31
S. Charache, W. Zinkham, J. Dickerman, B. Brimhall, G. Dover (1977)
Hemoglobin SC, SS/GPhiladelphia and SOArab diseases diagnostic importance of an integrative analysis of clinical, hematologic and electrophoretic findings.The American journal of medicine, 62 3
A. Adekile, M. Al-Kandari, M. Haider, Marouf Rajaa, M. D'souza, Jalaja Sukumaran (2007)
Hemoglobin F Concentration as a Function of Age in Kuwaiti Sickle Cell Disease PatientsMedical Principles and Practice, 16
P. Milner, C. Miller, R. Grey, M. Seakins, W. Dejong, L. Went (1970)
Hemoglobin O arab in four negro families and its interaction with hemoglobin S and hemoglobin C.The New England journal of medicine, 283 26
A. Adekile, M. Haider (1996)
Morbidity, βs Haplotype and α-Globin Gene Patterns among Sickle Cell Anemia Patients in KuwaitActa Haematologica, 96
A. Adekile, T. Huisman (1993)
Hb F in sickle cell anemiaExperientia, 49
A. Adekile, A. Owunwanne, K. al-Za'abi, M. Haider, M. Tuli, S. Al-Mohannadi (2002)
Temporal sequence of splenic dysfunction in sickle cell diseaseAmerican Journal of Hematology, 69
P. Sturgeon, H. Itano, W. Bergren (1955)
Clinical manifestations of inherited abnormal hemoglobins. I. The interaction of hemoglobin-S with hemoglobin-D.Blood, 10 5
Harvey Itano (1951)
A Third Abnormal Hemoglobin Associated with Hereditary Hemolytic Anemia.Proceedings of the National Academy of Sciences of the United States of America, 37 12
A. Adekile, Renu Gupta, F. Yacoub, T. Sinan, M. Al-Bloushi, M. Haider (2001)
Avascular Necrosis of the Hip in Children with Sickle Cell Disease and High Hb F: Magnetic Resonance Imaging Findings and Influence of α-Thalassemia TraitActa Haematologica, 105
J. Kelleher, J. Park, H. Kim, W. Schroeder (1984)
Life-threatening complications in a child with hemoglobin SD-Los Angeles disease.Hemoglobin, 8 3
(1991)
␥ -globin genes of the  S chromosomes with five different haplotypes
(2010)
Reproduced with the permission
(1991)
Sickle cell anaemia: S -globin gene cluster haplotypes as prognostic indicators of vital organ damage
(1964)
Hemo - globin D Los Angeles : 2 2 121 GluNH 2
(2002)
Silent brain infarcts are rare in Kuwaiti children with sickle cell disease and elevated
Hemoglobin (Hb) SD-Los Angeles compound heterozygotes usually have a severe clinical course although the effect of an elevated Hb F on the clinical phenotype has not been reported previously. We describe 5 Kuwaiti children with Hb SD associated with Hb F levels >20%. They all presented with sickling-related symptoms by ≤2 years of age and have been followed for 3–15 years. All had severe clinical courses marked by varying degrees of splenic sequestration crises, acute chest syndrome, vaso-occlusive crises, osteomyelitis and avascular necrosis of the femoral head. This pattern is in contrast with the usually mild presentation in Kuwaiti Hb SS patients with elevated Hb F. It therefore appears that Hb F does not ameliorate the clinical phenotype in Hb SD-Los Angeles. The reasons for this are not quite clear.
Acta Haematologica – Karger
Published: Jan 1, 2010
Keywords: Avascular necrosis; Hb F; Hb SD-Los Angeles; Hemoglobin; Sickle cell disease; Vaso-occlusive crises
Read and print from thousands of top scholarly journals.
Already have an account? Log in
Bookmark this article. You can see your Bookmarks on your DeepDyve Library.
To save an article, log in first, or sign up for a DeepDyve account if you don’t already have one.
Copy and paste the desired citation format or use the link below to download a file formatted for EndNote
Access the full text.
Sign up today, get DeepDyve free for 14 days.
All DeepDyve websites use cookies to improve your online experience. They were placed on your computer when you launched this website. You can change your cookie settings through your browser.