Tags

Type your tag names separated by a space and hit enter

Monozygotic twins with sickle cell anemia and discordant clinical courses: clinical and laboratory studies.

Abstract

We describe a rare set of monozygotic twins with coexistent sickle cell anemia and alpha-/alpha alpha thalassemia who have asynchronous painful crises of different frequency and severity. Studies include measurements of cell deformability and other hemorheologic tests, cell density distribution, the percentage of irreversibly sickled cells, adherence of red cells to endothelial cells, membrane heme and membrane free iron, calcium containing internal vesicles and serum antioxidants. Results of these studies, including estimates of organ damage (bone, spleen, retina), were similar except for an increase in red cell membrane free iron in the patient with more frequent and severe painful crises. The study supports the concept that non-inherited factors are important contributors to the frequency and severity of painful crises in sickle cell anemia.

Links

Authors+Show Affiliations

,

Department of Medicine, Mount Sinai Hospital, University of Health Sciences/The Chicago Medical School, IL 60608.

, , , , , , ,

Source

Hemoglobin 15:4 1991 pg 247-56

MeSH

Adult
Anemia, Sickle Cell
Diseases in Twins
Erythrocyte Deformability
Fetal Hemoglobin
Humans
Male
Pain
Thalassemia
Twins, Monozygotic

Pub Type(s)

Case Reports
Comparative Study
Journal Article
Research Support, Non-U.S. Gov't
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

1723971

Citation

Amin, B R., et al. "Monozygotic Twins With Sickle Cell Anemia and Discordant Clinical Courses: Clinical and Laboratory Studies." Hemoglobin, vol. 15, no. 4, 1991, pp. 247-56.
Amin BR, Bauersachs RM, Meiselman HJ, et al. Monozygotic twins with sickle cell anemia and discordant clinical courses: clinical and laboratory studies. Hemoglobin. 1991;15(4):247-56.
Amin, B. R., Bauersachs, R. M., Meiselman, H. J., Mohandas, N., Hebbel, R. P., Bowen, P. E., ... Westerman, M. P. (1991). Monozygotic twins with sickle cell anemia and discordant clinical courses: clinical and laboratory studies. Hemoglobin, 15(4), pp. 247-56.
Amin BR, et al. Monozygotic Twins With Sickle Cell Anemia and Discordant Clinical Courses: Clinical and Laboratory Studies. Hemoglobin. 1991;15(4):247-56. PubMed PMID: 1723971.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Monozygotic twins with sickle cell anemia and discordant clinical courses: clinical and laboratory studies. AU - Amin,B R, AU - Bauersachs,R M, AU - Meiselman,H J, AU - Mohandas,N, AU - Hebbel,R P, AU - Bowen,P E, AU - Schlegel,R A, AU - Williamson,P, AU - Westerman,M P, PY - 1991/1/1/pubmed PY - 1991/1/1/medline PY - 1991/1/1/entrez SP - 247 EP - 56 JF - Hemoglobin JO - Hemoglobin VL - 15 IS - 4 N2 - We describe a rare set of monozygotic twins with coexistent sickle cell anemia and alpha-/alpha alpha thalassemia who have asynchronous painful crises of different frequency and severity. Studies include measurements of cell deformability and other hemorheologic tests, cell density distribution, the percentage of irreversibly sickled cells, adherence of red cells to endothelial cells, membrane heme and membrane free iron, calcium containing internal vesicles and serum antioxidants. Results of these studies, including estimates of organ damage (bone, spleen, retina), were similar except for an increase in red cell membrane free iron in the patient with more frequent and severe painful crises. The study supports the concept that non-inherited factors are important contributors to the frequency and severity of painful crises in sickle cell anemia. SN - 0363-0269 UR - https://www.unboundmedicine.com/medline/citation/1723971/Monozygotic_twins_with_sickle_cell_anemia_and_discordant_clinical_courses:_clinical_and_laboratory_studies_ L2 - http://babysfirsttest.org/newborn-screening/conditions/sickle-cell-anemia DB - PRIME DP - Unbound Medicine ER -