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PubMed1985

Cerebral infarction in sickle cell trait.

Sickle cell disease is known to predispose patients to the risk of cerebral infarction. However, only scattered reports exist of the neurological sequelae of the sickle cell trait. Only 8 cases are reported in the English literature, in some of which the sickle cell trait was not documented by hemoglobin electrophoresis. This report describes 2 men, age 35 and 24 years, who developed acute cerebral infarction. Investigation revealed no apparent cause for the lesion other than the sickle cell trait.

Adult↗

PubMed2005

Compartment syndrome and fatal rhabdomyolysis in sickle cell trait.

Sickle cell trait is a relatively common condition in the African-American population. Individuals with this condition may have any of several complications under rare circumstances. We report a patient who presented with extensive compartment syndrome leading to death. A 31-year-old African-American male with known history of sickle cell trait developed extensive compartment syndrome followed by rhabdomyolysis, severe acidosis, acute renal failure, and coagulopathy. Although the patient underwent multiple fasciotomies for extensive compartment syndrome and received aggressive resuscitation with massive transfusions of blood, blood products and intravenous fluids, he died. Multiple compartment syndromes in patients with sickle cell trait represent a very unusual complication and can cause significant morbidity and mortality. Avoidance of strenuous exercise, especially in hot climates, early diagnosis, and aggressive therapy are very important to prevent this catastrophic complication.

Acidosis↗

PubMed2005

[Confidentiality, genetic counseling, and public health: a case study on sickle cell trait].

Sickle cell anemia is the most prevalent genetic disease in Brazil. Screening for sickle cell trait in blood donation centers has been recommended by the Brazilian government since 1976. This screening mechanism is one of most common forms of genetic screening for sickle cell disease in the country. This article is based on an ethnographic survey of individuals undergoing genetic counseling in a large blood donation center. The article contends that confidentiality should be a prerequisite for the broad dissemination of genetic information in public health. The ethical principle of confidentiality needs to be seen as a human right and public health issue.

Bioethical Issues↗

PubMed2002

Coagulation changes in individuals with sickle cell trait.

Sickle cell disorders, such as Hb SS and Hb SC, are associated with a hypercoagulable state that may contribute to the vaso-occlusive episodes observed in the disorders. To what extent increased coagulation activity occurs in individuals with sickle cell trait has had limited study. Because such information may help clarify clinical and pathologic findings that may occur in these individuals and may be useful in clarifying the hypercoagulable state in sickle cell disease, we have examined individuals with Hb AS to determine the extent that increased coagulation activity does occur. We measured d-dimers, thrombin-antithrombin (TAT) complexes, prothrombin fragment 1.2 (F1.2), absolute blood monocyte levels, proteins C and S, and isotypes of antiphospholipid antibodies in individuals with Hb AS and in matched controls (Hb AA). Results showed that d-dimers, TAT, and F1.2 were increased significantly above normal levels. Absolute blood monocyte levels were increased. The d-dimers, TAT, F1.2, and monocyte counts showed significant increasing trends through groups of increasing severity (Hb AA, Hb AS, Hb SC, and Hb SS). Our study shows that individuals with Hb AS have increased coagulation activity, with d-dimers, TAT, and F1.2 being consistent indicators. The measures of coagulation activity in Hb AS are lower than in patients with Hb SC and Hb SS disease. These results extend our previous observation that the degree of coagulation activation parallels the degree of disease severity among sickle cell genotypes. The findings suggest that monocytosis, with the possible expression of monocyte-derived tissue factor, and the associated hypercoagulable state are driven by disease severity.

Adult↗

PubMed1999

[Epidemiology of stroke in Guadeloupe and role of sickle cell trait].

Sickle cell disease (homozygotes SS) is known as a risk factor for both ischemic and hemorrhagic stroke, but heterozygotes AS seem to be spared. We carried out a retrospective study to assess the main risk factors and the influence of hemoglobin abnormalities on stroke in Guadeloupe. The percentages of AS, AC, and AA on 295 patients admitted for stroke were compared to the prevalence obtained on 72,000 newborn babies. Ischemic, hemorrhagic stroke and stroke complications represented respectively 83 p. 100, 10 p. 100 and 7 p. 100. Seventy one per 100 of patients had hypertension and 19 p. 100 had an association of diabetes and hypertension. The percentage of heterozygotes AS was significantly lower in the group with ischemic stroke (4 p. 100) in comparison with controls (8.5 p. 100), while AS were more represented in hemorrhagic stroke (16 p. 100). The risk of hemorrhagic stroke was 10 fold higher in AS patients admitted for stroke and the risk of ischemic stroke was reduced by 15 fold. These data suggest that the sickle cell trait could be associated to red cell and/or endothelial specificities which could prevent for ischemic stroke. The influence of AS heterozygote on the occurrence of stroke needs to be examined in a longitudinal, prospective study.

Adult↗

PubMed1985

Gross hematuria in a patient with sickle cell trait.

Sickle cell trait must be included in the differential diagnosis of hematuria in black patients. Therefore, diagnostic workup should include hemoglobin electrophoresis, urine culture, coagulation studies, intravenous pyelography, cystoscopy, renal ultrasonography, and renal arteriography. If the patient is found to have hemoglobin AS and no other abnormality, initial therapy consists of bed rest and intravenous fluids, with transfusion of red cells if needed. Intravenous epsilon-aminocaproic acid (EACA) has been used effectively to reverse hematuria in patients who do not respond to conservative management. In the case reported here, gross hematuria in a 24-year-old black woman resolved with use of EACA therapy.

Adult↗

PubMed2006

Sudden death in a patient newly diagnosed with diabetes having hyperosmolar non-ketotic acidosis with sickle cell trait.

Sickle cell trait (SCT) has a UK urban population rate estimated at 3.2%. The condition may remain unrecognised, but sudden death is a recognised feature. The sudden death of a 51-year-old man with SCT who presented with hyperosmolar non-ketotic acidosis (HONK) as his first presentation of diabetes is reported here. After admission to hospital, a rapid deterioration was seen in his condition, with loss of cardiac output, leading to death. He was found to have had acute-chest syndrome and hepatosplenic infarction on postmortem examination. The pathophysiology of sudden death in patients with SCT and the role of HONK in the patient's demise is discussed here.

Acidosis↗

PubMed1999

Morphological features of the human umbilical vein in normal, sickle cell trait, and sickle cell disease pregnancies.

Pathological changes often occur in the placenta of women with sickle cell disease (SCD). These alterations are caused by sickling of erythrocytes and vasoocclusion in the placental circulation, leading to regional hypoxia. However, the morphological status of the umbilical cord, which is in close physical association with the placenta, is not documented under such conditions. To explore this, the umbilical vein structure in healthy, sickle cell trait (the heterozygous state), and SCD pregnancies was studied using scanning (SEM) and transmission electron microscopy (TEM). Interestingly, the sickle cell trait umbilical vein architecture was morphologically similar to that in control veins, whereas numerous alterations were seen in the SCD umbilical vein wall. In SEM, the SCD umbilical vein endothelial cells showed atypical morphologies. TEM analysis of the tunica media showed (1) smooth muscle cell proliferation and increase in the thickness of the basement membrane underlying the cells; (2) areas of necrosis; (3) reduplication of the inner elastic lamina. Such features were often seen in sickle patients vasculature at autopsy. Our findings could have importance because tissue hypoxemia is an integral part of vasoocclusion. We conclude that the SCD umbilical vein may be an additional tool for studying vasoocclusion in sickle cell disease.

Adult↗

PubMed1955