Sickle cell trait as a contributory cause of death in natural disease. 2015

Varsha Podduturi, and Joseph M Guileyardo
Department of Pathology, Baylor University Medical Center, Dallas, TX, 75246.

Sickle cell trait (SCT) affects 300 million people globally, and awareness is growing that SCT is not an entirely benign condition; however, most reported cases have been non-natural deaths. Autopsy records from the Baylor University Medical Center (BUMC) in Dallas, Texas, contained seven natural deaths from January 2007 to October 2013 in which micro-occlusive sickling was identified at autopsy and SCT confirmed by postmortem hemoglobin fractionation. Sickle crisis was never diagnosed clinically. These cases illustrate the importance of red cell morphology in autopsy material. When sickling is suspected, hemoglobin fractionation should be performed. If confirmed, SCT should be listed as an autopsy finding and the severity and distribution of sickling documented. Extensive micro-occlusive sickling should be considered contributory to death; however, its relative importance depends on all facts of the case. Accurate reporting should facilitate further research and the development of evidence-based preventative and supportive strategies for these patients.

UI MeSH Term Description Entries
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D002423 Cause of Death Factors which produce cessation of all vital bodily functions. They can be analyzed from an epidemiologic viewpoint. Causes of Death,Death Cause,Death Causes
D002908 Chronic Disease Diseases which have one or more of the following characteristics: they are permanent, leave residual disability, are caused by nonreversible pathological alteration, require special training of the patient for rehabilitation, or may be expected to require a long period of supervision, observation, or care (Dictionary of Health Services Management, 2d ed). For epidemiological studies chronic disease often includes HEART DISEASES; STROKE; CANCER; and diabetes (DIABETES MELLITUS, TYPE 2). Chronic Condition,Chronic Illness,Chronically Ill,Chronic Conditions,Chronic Diseases,Chronic Illnesses,Condition, Chronic,Disease, Chronic,Illness, Chronic
D005260 Female Females
D006451 Hemoglobin, Sickle An abnormal hemoglobin resulting from the substitution of valine for glutamic acid at position 6 of the beta chain of the globin moiety. The heterozygous state results in sickle cell trait, the homozygous in sickle cell anemia. Hemoglobin S,Deoxygenated Sickle Hemoglobin,Deoxyhemoglobin S,Hemoglobin SS,Hemoglobin, Deoxygenated Sickle,SS, Hemoglobin,Sickle Hemoglobin,Sickle Hemoglobin, Deoxygenated
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D000368 Aged A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available. Elderly
D012805 Sickle Cell Trait The condition of being heterozygous for hemoglobin S. Cell Trait, Sickle,Cell Traits, Sickle,Sickle Cell Traits,Trait, Sickle Cell,Traits, Sickle Cell

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