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PubMed1996

Exertional collapse and sudden death associated with sickle cell trait.

Although rare, exertional collapse and sudden death are the most serious potential complications of sickle cell trait. Studies suggest that this condition may occur in susceptible persons when poor physical conditioning, dehydration, heat stress or hypoxic states precipitate sickling of the abnormal erythrocytes. Sickling leads to endothelial damage, which can cause vasoconstriction, disseminated intravascular coagulation and local tissue damage. Cardiac effects include acute ischemia and arrhythmias. Muscle damage results in acute compartment syndromes and release of myoglobin into the circulation. Acute renal failure is possible. Diagnosis is based on a high index of suspicion, and characteristic presentation and laboratory findings, including myoglobinuria, hyperkalemia, hypocalcemia, hyperphosphatemia and elevated creatine kinase levels. The differential diagnosis includes pulmonary embolism, acute cardiac events, anaphylaxis and heat stroke. Management is based on stabilization, rehydration, and the treatment and prevention of complications.

Death, Sudden↗
PubMed1987

Sickle-cell trait as a risk factor for sudden death in physical training.

Case reports of sudden death during exertion have not established an association between the sickle-cell trait (hemoglobin AS) and exercise-related death. To test this association, all deaths occurring among 2 million enlisted recruits during basic training in the U.S. Armed Forces in 1977 to 1981 were classified from autopsy and clinical records as non-sudden deaths or as sudden deaths explained or unexplained by preexisting disease. On the basis of known numbers of entering recruits (according to race, age, and sex) and published prevalence rates for hemoglobin AS (8 percent for black and 0.08 percent for nonblack recruits), death rates (per 100,000) were 32.2 for sudden unexplained deaths, 2.7 for sudden explained deaths, and 0 for non-sudden deaths among black recruits with hemoglobin AS, as compared with 1.2, 1.2, and 0.7 among black recruits without hemoglobin S and 0.7, 0.5, and 1.1 among nonblack recruits without hemoglobin S. Among black recruits the relative risk of sudden unexplained death (hemoglobin AS vs. non-hemoglobin S) was 27.6 (95 percent confidence interval, 9 to 100; P less than 0.001), whereas among all recruits this risk was 39.8 (95 percent confidence interval, 17 to 90; P less than 0.001). The relative risk of sudden unexplained death among all recruits increased with age (P less than 0.04), from 13 (ages 17 to 18) to 95 (ages 26 to 30). We conclude that recruits in basic training with the sickle-cell trait have a substantially increased, age-dependent risk of exercise-related sudden death unexplained by any known preexisting cause.

Adolescent↗
PubMed1997

Effects of gamma irradiation on red cells from donors with sickle cell trait.

BACKGROUND: Transfusion-associated graft-versus-host disease can be prevented by gamma irradiation of blood components. Red cells (RBCs) from sickle cell disease patients may exhibit oxidative changes of RBC membranes due to the instability of hemoglobin (Hb) S. Persons with sickle cell trait are eligible to donate blood, and 35 to 45 percent of their total Hb is Hb S. The effect of gamma irradiation on RBCs from such persons is of interest. STUDY DESIGN AND METHODS: RBCs from 12 donors with sickle cell trait (Hb AS) and from 12 with normal Hb (Hb AA) were studied. Each of the 24 RBC units was divided equally into two transfer bags via a sterile connecting device. One bag from each RBC unit received a 2500-cGy dose of gamma irradiation at its mid-plane and was stored at 4 degrees C; the second set of bags was stored without irradiation. For RBCs from 6 donors with Hb AS and 6 donors with Hb AA, units were irradiated on Day 7 and studied on Day 35 of storage (Group 1). For the RBCs from the other 6 donors with Hb AS and the other 6 donors with Hb AA, units were irradiated on Day 28 and studied on Day 42 of storage (Group 2). RESULTS: For Group 1 and Group 2, plasma potassium and plasma Hb concentrations were significantly higher and RBC ATP concentrations were slightly lower in the irradiated units than in the nonirradiated units. In Group 1 and Group 2, there were no significant differences in the plasma potassium or RBC ATP concentrations in either the irradiated or the nonirradiated units of RBCs from donors with Hb AS and donors with Hb AA. Plasma Hb concentrations were consistently lower in the units from donors with Hb AS, whether or not they were irradiated. However, in both groups, proportionally similar changes in plasma Hb concentration were detected when the irradiated Hb AS and Hb AA units were compared to nonirradiated Hb AS and Hb AA units. CONCLUSION: Gamma irradiation of RBCs from donors with Hb AS or with Hb AA resulted in comparable changes in plasma potassium, RBC ATP, and plasma Hb concentrations, although donors with Hb AS had lower plasma Hb. RBCs from donors with Hb AS subjected to 2500 cGy of gamma irradiation did not evidence a storage lesion greater than that seen in RBCs from donors with Hb AA.

Adenosine Triphosphate↗
PubMed1981

Sickle cell trait counseling-evaluation of counselors and counselees.

In this study, information about both counselee and counselor performance was obtained from taped recordings of 193 structured counseling sessions with persons diagnosed as having sickle cell trait. The data provide evidence that: (1) lay persons can understand essential sickle cell information; (2) trained lay persons using a structured format can transmit successfully sickle cell information; (3) only education and age, among counselee characteristics studies, were related to successful learning; (4) the evaluation of information transfer in counseling programs cannot be limited to counselees' comprehension but must also consider other variables such as counselor performance and curriculum content; (5) a reduction in negative feelings associated with a diagnosis of sickle cell trait is an immediate effect of counseling; and (6) audio-taping of counseling sessions is client acceptable and useful for evaluation, quality control, and counselor training.

Adolescent↗
PubMed1991

Lymphoproliferative responses to Plasmodium falciparum antigens in children with and without the sickle cell trait.

Blood mononuclear cells (BMNC) were isolated from sickle cell trait (HbAS) healthy donors and normal haemoglobin (HbAA) healthy donors resident in a P. falciparum endemic area of eastern Sudan. Blood samples were collected during the malaria season. BMNC were tested for their proliferative responses to phytohaemagglutinin (PHA), purified protein derivative of tuberculin (PPD) and to soluble P. falciparum antigens (SPAg). Higher responses to SPAg and PPD were observed in the HbAS children compared with the HbAA children, whereas no differences were observed among adults of the two phenotypes. Proliferative responses to PHA were comparable in all individuals tested. The significance of these findings in relation to haemoglobin phenotype, age and the possible immunoregulatory mechanisms operating in HbAS and HbAA children during the malaria season is discussed.

Animals↗
PubMed1997

Sickle cell trait as a risk factor for secondary hemorrhage in children with traumatic hyphema.

PURPOSE: To determine risk factors for secondary hemorrhage and poor visual outcome in children with traumatic hyphemas. METHODS: We reviewed 99 eyes of 97 children younger than 18 years who had been hospitalized for hyphema within 48 hours of blunt eye trauma. Inpatient records were examined for race, age, sickle cell trait status, size of hyphema and intraocular pressure at admission, secondary hemorrhage (rebleed of hyphema), and medications while hospitalized. Fifty-five eyes of 53 children had at least 1 month of follow-up or attained best-corrected visual acuity of 20/50 or better at their last outpatient visit. RESULTS: Among 99 eyes of 97 children with traumatic hyphema, secondary hemorrhage occurred in nine eyes (9%). Among 72 eyes of 70 African-American children, secondary hemorrhage occurred in nine eyes (14%), whereas in 27 eyes of 27 white children, there were no secondary hemorrhages. However, when the 14 eyes of 13 sickle cell trait-positive children were excluded from the African-American group, the 57 eyes of sickle cell trait-negative African-American and white children did not have any secondary hemorrhages. The sickle cell trait-positive group had secondary hemorrhages in nine of 14 eyes (64%), significantly (P < .005) different from the 0% rate in the 57 eyes of African-American sickle cell trait-negative and white children. The sickle cell trait-positive group also had higher intraocular pressure and permanent visual impairment. CONCLUSION: Sickle cell trait is a significant risk factor for secondary hemorrhage, increased intraocular pressure, and permanent visual impairment in children who have traumatic hyphemas following blunt trauma.

Administration, Topical↗
PubMed1989

Prevalence of the sickle cell trait among students in a physical education college in Côte-d'Ivoire.

The prevalence of sickle cell trait (SCT), was studied in 263 students enrolled in a physical education college in Côte-d'Ivoire. Most of the students were athletes of average training level, but some competed at an international level. The prevalence of SCT (hemoglobin AS), observed in the student population (13.7%) was not significantly different from that of the general population (12%) of Côte-d'Ivoire and did not differ between sexes. The percentage of international level athletes was similar in SCT (25%) and control (hemoglobin AA) groups (25.7%). Particular activities practised by SCT international level athletes were individual track events (sprint race, 400 m race and long and high jump) as well as team sports (football and handball), indicating the excellent aptitude for aerobic and anaerobic metabolic activities. As none of the international athletes engaged in endurance activities, no conclusions can be drawn regarding either the aptitude of SCT subjects for intense and prolonged exertion or a possible limitation of oxygen transport produced by their hemoglobinopathy.

Anemia, Sickle Cell↗
PubMed2005

The sickle cell trait is associated with enhanced immunoglobulin G antibody responses to Plasmodium falciparum variant surface antigens.

The sickle cell trait (HbAS) protects against severe Plasmodium falciparum malaria in young African children. We investigated the extent of the association between HbAS and antibodies directed to parasite-derived variant surface antigens (VSAs) on the membrane of infected erythrocytes. We measured immunoglobulin G (IgG) responses with specificity for VSAs of 2 heterologous parasite isolates in 458 Gabonese children aged between 6 months and 11 years. Logistic regression analyses showed a highly significant independent association (P<.001) between carriage of HbAS and the presence of IgG anti-VSA responses; this association was related specifically to IgG1 and IgG4 subclasses in the anti-VSA profile. IgG2 and IgG3 anti-VSA responses were both independently associated with older age, consistent with the pattern observed in semi-immune adults. The results imply that enhanced levels of cross-reactive anti-VSA responses in children with HbAS may be intimately associated with the protection they have against malaria.

Animals↗
PubMed1998

Renal medullary carcinoma in patients with sickle cell trait.

Renal medullary carcinoma has recently been described as an aggressive neoplasm affecting young African Americans with sickle cell disease or sickle cell trait. We report the presentation, treatment, and outcome in 3 patients with renal medullary carcinoma along with a description of the unsuccessful treatment attempts. A brief discussion and review of the literature is included.

Adult↗
PubMed2002

Pregnancy outcome in women with sickle cell trait.

OBJECTIVE: The aim of the study is to assess the pregnancy outcome among Omani women with sickle cell trait (SCT), and to compare it with a control group of Omani women with normal hemoglobin. METHODS: Relevant data of both groups was obtained from labor room records, patient files and computerized records at the Nizwa Hospital, Dakhliya Region, Nizwa, Oman from January 1999 to July 2000. Available data was analyzed. RESULTS: Three thousand, five hundred and one pregnant Omani women were included in this study, 319 had SCT (9.1%), the average age was 27 years in both groups, and 51% were primigravida. There was an increase in the incidence of anemia in the SCT women. The incidence of abortion and neonatal death in previous pregnancies was significantly increased among SCT women. CONCLUSION: Pregnant women with SCT need special care and attention during pregnancy, labor, puerperium and surgery. They should be identified early to prevent complications such as anemia, infection and fetal wastage.

Adult↗
PubMedDate not supplied

Massive ocular hemorrhage resulting in blindness in a patient with the sickle cell trait who developed leptospirosis. Case report.

This case report describes the findings of a 18 year-old black male from Bahia, a Northeastern state in Brazil, with the sickle cell trait, who developed bilateral hyphema and vitreous hemorrhage with blindness in the course of leptospirosis. The patient started to complain of blurred vision four days after the start of fever and muscular pain and approximately twelve hours after the introduction of penicillin. The severity of the leptospirosis in conjunction with sickle cell trait was considered to be the most likely explanation for this ocular complication.

Adolescent↗
PubMed1996

Exercise-induced death in sickle cell trait: role of aging, training, and deconditioning.

The pathophysiological process of exercise-induced death in subjects with sickle cell trait (SCT) remains unclear. Concerning the cause of death, authors have suggested stressful environmental conditions such as altitude, heat and humidity, or abnormal patient conditions such as deconditioning, fatigue, and disease. These conditions are thought to lead to hypoxemia, hyperlactatemia, acidosis, dehydration, hyperthermia, or exercise-induced rhabdomyolysis, all of which may initiate sickle cell crisis, disseminated intravascular coagulation, myoglobinuria, and renal failure. We report the case of a 41-yr-old, healthy, and apparently well-conditioned subject with SCT who died during a cross-country race under normal environmental conditions in good weather (in terms of temperature and humidity). The medical and athletic history of the subject were unremarkable. We refer to an epidemiological study that reported a relation between age and exercise-induced sudden death in subjects with SCT. We then review the pathophysiological effects of aging in association with deconditioning and high-level training reported in the literature, particularly the decrease in aerobic metabolism in deconditioned subjects, and the exercise-induced hypoxemia in highly trained subjects. We discuss the consequences of deconditioning and high-level training in subjects with SCT during exercise, and conclude that these factors may be involved in the age-dependent risk of exercise-related sudden death in subjects with SCT.

Adult↗
PubMed1981

Multifocal Best's disease and sickle cell trait.

Ophthalmoscopy performed during a routine eye checkup on a 22-year-old black man revealed multiple intraretinal, cyst-like lesions scattered throughout the macular and extramacular areas in both eyes. Based on clinical findings, morphologic appearance, and an abnormal electro--oculogram, the diagnosis of multifocal Best's macular dystrophy was made. Hemoglobin electrophoresis showed an AS pattern confirming the presence of sickle cell trait. This is the first case of the coexistence of these two genetically transmitted diseases, Best's disease and sickle cell trait.

Adult↗