Complication Of Sickle Cell Trait Research Articles

Are pregnant patients with sickle cell trait more likely to experience anemia and associated complications?

There is a growing body of literature investigating the complications of sickle cell trait (SCT) in non-pregnant individuals; however there is little information regarding the impact of SCT in pregnancy. Our objective is to determine whether pregnant patients with SCT are more likely to have anemia and associated adverse complications.

Clinical Outcomes Associated With Sickle Cell Trait: A Systematic Review.

Although sickle cell trait (SCT) is largely a benign carrier state, it may increase risk for certain clinical outcomes. To evaluate associations between SCT and clinical outcomes in children and adults. English-language searches of PubMed, CINAHL, the Cochrane Library, Current Contents Connect, Scopus, and Embase (1 January 1970 to 30 June 2018) and bibliographies of review articles. Observational controlled studies (published in English) in children or adults that examined an association between SCT and any of 24 clinical outcomes specified a priori in the following 6 categories: exertion-related injury; renal, vascular, pediatric, and surgery- or trauma-related outcomes; and overall mortality. A single reviewer extracted study data, which was checked by another; 2 reviewers independently assessed study quality; and strength of evidence was assessed by consensus. Of 7083 screened studies, 41 met inclusion criteria. High-strength evidence supported a positive association between SCT and risk for pulmonary embolism, proteinuria, and chronic kidney disease. Moderate-strength evidence supported a positive association between SCT and exertional rhabdomyolysis and a null association between SCT and deep venous thrombosis, heart failure or cardiomyopathy, stroke, and pediatric height or weight. Absolute risks for thromboembolism and rhabdomyolysis were small. For the remaining 15 clinical outcomes, data were insufficient or strength of evidence was low. Publication bias was possible, and high-quality evidence was scant. Sickle cell trait is a risk factor for a few adverse health outcomes, such as pulmonary embolism, kidney disease, and exertional rhabdomyolysis, but does not seem to be associated with such complications as heart failure and stroke. Insufficient data or low-strength evidence exists for most speculated complications of SCT. National Human Genome Research Institute.

A Traveler to Holland with a Benign Killer : A Case Report of a Sickle Cell Trait Male with Splenic Infarction

Sickle cell trait is highly prevalent in Saudi Arabia. However, most of the patient are asymptomatic, only few present with symptoms of the complications of the disease. We report a case of 24-year-old male who presented to the hospital with history of dull left upper quadrant, nausea and vomiting while he was on the airplane. Investigation showed a picture of sickle cell trait with hemoglobin S of 40%. Peripheral blood smear was within normal. CT scan of the abdomen confirmed the presence of splenic infraction. This case emphasized on the need to increase the level of threshold to recognize the rare complications of sickle cell trait and how to manage them appropriately.

The Risk of Potential Thromboembolic, Renal and Cardiac Complications of Sickle Cell Trait

Many complications of sickle cell trait have been well-established, but associations with additional disease states remain controversial. We conducted a retrospective cohort study to examine the frequency of receiving a diagnosis of thromboembolism, pulmonary embolism (PE), ischemic stroke, renal disease (acute, chronic), coronary artery disease (CAD) and congestive heart failure (CHF) in patients with sickle cell trait. A total of 13 964 adult African Americans registered in the Kaiser Permanente Northern California (KPNC) health system (Oakland, CA, USA), were included based on laboratory and diagnostic code data for the years 1995–2008: 2642 with sickle cell trait, 11 183 with normal hemoglobin (Hb) and 139 with sickle cell disease. Disease outcomes were obtained from coded diagnoses. The adjusted relative risk of PE and chronic kidney disease in sickle cell trait patients compared to patients with normal Hb were 1.37 [95% confidence interval (CI) 1.07–1.75] and 1.13 (95% CI 1.03–1.23), respectively. There were no other significant differences in the outcomes for sickle cell trait patients compared to patients with normal Hb.

Acute Splenic Syndrome in an African-American Male with Sickle Cell Trait on a Commercial Airplane Flight

BackgroundPatients with sickle cell trait (SCT) generally suffer few effects of sickle cell disease. Acute splenic syndrome is a rare but well-documented complication of SCT that can present in the setting of low oxygen tension that occurs with major changes in altitude, either by unpressurized air flight or ground travel such as mountain climbing. ObjectiveOur objective was to increase emergency physician awareness of the rare diagnosis of altitude-induced splenic infarction in patients with SCT by presenting and reviewing recorded literature. Case ReportThis is an unusual case of a man with SCT who suffered acute splenic syndrome while on a pressurized airplane flight subsequently requiring splenectomy. ConclusionsAlthough it is a rare phenomenon, patients with SCT who are exposed to high altitudes can be subject to splenic syndrome. There are many patients who are unaware that they have SCT, which makes the diagnosis challenging for emergency physicians. In addition, because patients with SCT generally do not have complications related to their disease, diagnosing splenic syndrome might be delayed, potentially resulting in inadequate hydration, pain management, and other supportive treatment, which ultimately leads to worsening splenic syndrome and splenectomy.

Epinephrine Modulates BCAM/Lu and ICAM-4 Expression on the Sickle Cell Trait Red Blood Cell Membrane

Collapse and sudden death in physical training are the most serious complications of sickle cell trait (SCT). There is evidence that erythrocytes in SCT patients aggregate during strenuous exercise, likely because of adhesive interactions with the extracellular matrix (ECM) and endothelial cells, and because of their irregular viscoelastic properties. This results in inflammation, blood flow impairment, and vaso-occlusive events. However, the exact role of stress conditions and how they lead to these complications is virtually unknown. Using single-molecule atomic force microscopy experiments, we found that epinephrine, a hormone that is secreted under stressful conditions, increases both the frequency and strength of adhesion events between basal cell adhesion molecule (BCAM/Lu) and ECM laminin, and between intercellular adhesion molecule-4 (ICAM-4) and endothelial αvβ3, compared with nonstimulated SCT erythrocytes. Increases in adhesion frequency provide significant evidence of the role of epinephrine in BCAM/Lu-laminin and ICAM-4-αvβ3 bonding, and suggest mechanisms of vaso-occlusion during physical exertion in SCT.

A Policy Impact Analysis of the Mandatory NCAA Sickle Cell Trait Screening Program

To estimate the impact of the mandatory National Collegiate Athletic Association (NCAA) sickle cell trait (SCT) screening policy on the identification of sickle cell carriers and prevention of sudden death. We used NCAA reports, population-based SCT prevalence estimates, and published risks for exercise-related sudden death attributable to SCT. We estimated the number of sickle cell carriers identified and the number of potentially preventable sudden deaths with mandatory SCT screening of NCAA Division I athletes. We calculated the number of student-athletes with SCT using a conditional probability based upon SCT prevalence data and self-identified race/ethnicity status. We estimated sudden deaths over 10years based on published attributable risk of exercise-related sudden death due to SCT. We estimate that over 2,000 NCAA Division I student-athletes with SCT will be identified under this screening policy and that, without intervention, about seven NCAA Division I student-athletes would die suddenly as a complication of SCT over a 10-year period. Universal sickle cell screening of NCAA Division I student-athletes will identify a substantial number of sickle cell carriers. A successful intervention could prevent about seven deaths over a decade.

A Survey of Sickle Cell Trait Screening in NCAA and NAIA Athletic Programs

Screening for sickle cell trait (SCT) in athletes has been recommended by several professional organizations; however, it is not known how many colleges and universities have implemented screening of athletes. PURPOSE: To survey factors related to policies and procedures of SCT screening at colleges and universities, including all divisions of the NCAA and NAIA athletic programs. METHODS: An on-line survey regarding SCT testing policies was sent to the head athletic trainer or director of sports medicine at 1384 NCAA and NAIA institutions. RESULTS: A total of 375 schools responded (NCAA D-I = 130, D-II = 74, D-III = 122, NAIA = 49) for a response rate of 27%. Our results show that 21.8% of the institutions surveyed screened all of their athletes for SCT, with Division I institutions having the highest rate of screening at 44.6%. A total of 30.1% of the institutions indicated they screened some of their athletes, with 66.9% of Division I institutions having the highest incidence. Over 26% of institutions did not know if any of their athletes had SCT. Overall, 57% of the institutions counsel athletes about complications of SCT, and 40% of the institutions modify workouts for the athletes. Division I institutions had the highest incidences with 87.7% providing counseling and 66.1% modifying workouts. The most common criteria for screening are race/ethnicity and family history of SCT. When asked reasons for not screening all athletes, 71.6% of all institutions surveyed indicated cost was the primary reason. CONCLUSIONS: Screening for SCT remains a relatively uncommon practice in colleges and universities, particularly in NCAA Divisions II, III and NAIA institutions. Institutions reported the cost of testing was overwhelmingly the primary reason for not screening all athletes for SCT.

Survey of Sickle Cell Trait Screening in NCAA and NAIA Institutions

Background: Screening for sickle cell trait (SCT) in athletes has been recommended by several professional organizations; however, it is not known how many colleges and universities have implemented such screening in athletes. Aims: This study examines factors related to policies and procedures of SCT screening at colleges and universities, including all divisions of the National Collegiate Athletic Association (NCAA) and the National Association of Intercollegiate Athletics (NAIA) programs. Methods: A total of 375 head athletic trainers or directors of sports medicine from NCAA and NAIA schools responded to an online survey. Results: 21.8% of the respondent institutions screened all of their athletes for SCT, with Division I institutions having the highest rate of screening at 44.6%. A total of 30.1% of the respondent institutions indicated that they screened some of their athletes, with 66.9% of Division I institutions having the highest incidence. Over 26% of respondent institutions did not know if any of their athletes had SCT. Overall, 57% of the institutions counsel athletes about complications of SCT, and 40% of the institutions modify workouts for the athletes. Division I institutions had the highest incidences, with 87.7% providing counseling and 66.1% modifying workouts. The most common criteria for screening are race/ethnicity and family history of SCT. When asked to provide reasons for not screening all athletes, 71.6% of all respondent institutions indicated cost was the primary reason. Conclusion: Screening for SCT remains a relatively uncommon practice in colleges and universities, particularly in NCAA Divisions II and III, and NAIA institutions. From the institutions' perspective, the cost of testing is overwhelmingly the primary reason for not screening all athletes for SCT.

Survey of Sickle Cell Trait Screening in NCAA and NAIA Institutions

Background: Screening for sickle cell trait (SCT) in athletes has been recommended by several professional organizations; however, it is not known how many colleges and universities have implemented such screening in athletes. Aims: This study examines factors related to policies and procedures of SCT screening at colleges and universities, including all divisions of the National Collegiate Athletic Association (NCAA) and the National Association of Intercollegiate Athletics (NAIA) programs. Methods: A total of 375 head athletic trainers or directors of sports medicine from NCAA and NAIA schools responded to an online survey. Results: 21.8% of the respondent institutions screened all of their athletes for SCT, with Division I institutions having the highest rate of screening at 44.6%. A total of 30.1% of the respondent institutions indicated that they screened some of their athletes, with 66.9% of Division I institutions having the highest incidence. Over 26% of respondent institutions did not know if any of their athletes had SCT. Overall, 57% of the institutions counsel athletes about complications of SCT, and 40% of the institutions modify workouts for the athletes. Division I institutions had the highest incidences, with 87.7% providing counseling and 66.1% modifying workouts. The most common criteria for screening are race/ethnicity and family history of SCT. When asked to provide reasons for not screening all athletes, 71.6% of all respondent institutions indicated cost was the primary reason. Conclusion: Screening for SCT remains a relatively uncommon practice in colleges and universities, particularly in NCAA Divisions II and III, and NAIA institutions. From the institutions' perspective, the cost of testing is overwhelmingly the primary reason for not screening all athletes for SCT.

Sickle-cell trait: novel clinical significance.

There is a long-standing controversy in the literature as to whether sickle-cell trait (SCT) should be viewed as a benign carrier state or as an intermediate disease phenotype. Because SCT is routinely detected by neonatal screening for sickle-cell disease, it becomes imperative that consensus on this issue be achieved in order to provide the best medical advice to affected individuals. The issue of selective screening in the post-neonatal period was thrust into the limelight recently by the National Collegiate Athletic Association's recommendation that its member colleges and universities test student-athletes to confirm their carrier status if not already known. The stated goal of this recommendation was to prevent exercise-related sudden death in athletes with SCT. We review some of the reported complications of SCT for which new information has emerged, focusing particularly on venous thromboembolism and renal manifestations.

An unusual complication of sickle cell trait: intraureter thrombus

A 37-year-old African American woman with sickle cell trait presented with 2 days history of right flank pain and macroscopic hematuria. She had a past history of non-Hodgkin lymphoma and splenectomy for splenic infarct. Physical examination showed right costovertebral angle tenderness. Laboratory evaluation was notable for a hemoglobin level of 12.7 g/dl, a creatinine level of 1.1 mg/dl, and red blood cells on urine microscopy. Contrast-enhanced computed tomography of the abdomen showed a filling defect involving the right collecting system, right ureter, and bladder. No stone or mass was identified. Urine culture and urine cytology were negative. Her symptoms resolved after 48 h of conservative treatment (intravenous fluid, bed rest and pain control). She underwent a cystoscopy with ureteroscopy which showed renal papillary necrosis and intraureter thrombus. Hemoglobin electrophoresis demonstrated 60% hemoglobin A and 40% hemoglobin S, consistent with sickle cell trait. A repeated computed tomography after 10 days showed complete resolution of the intraureter thrombus.

LEFT ARM PAIN AND SWELLING IN A COLLEGE FOOTBALL PLAYER

HISTORY An 18 year old African American male presented with left arm swelling after starting summer football camp. He had been weightlifting the day prior to developing swelling, stiffness and arm pain confined to his left forearm and mid-biceps. He noted that the intensity of the weightlifting, which was primarily bicep and shoulder work, was greater than normal for him. He denied any previous episodes of arm pain and had been working out alone during the summer. He denied any dizziness, numbness or tingling or recent viral illness. He denied chest pain, SOB, nausea or vomiting, fever, chills or rash. He denied any hematuria but his urine may have been a bit darker than normal. He denied using any supplements, illegal drugs or alcohol. PMHX: exertional heat cramps in the past; NKDA, MEDS: none, FHX: significant for sickle cell trait in his father and mother. His review of systems was negative. PHYSICAL EXAMINATION general: WDWN African American male in NAD, height 68 inches, weight 167 lbs. vitals: stable, BP 110/60, afebrile. HEENT: unremarkable. neck: FROM, no nodules or LAN chest: CTA. heart: RRR, normal S1 and S2, no MRG. abdomen: benign. extremities: Left arm; ecchymosis or erythema, mid bicep circumference was 36 cm, right mid bicep circumference was 34 cm, tenderness to palpation over left biceps and brachialis muscles. Limited left elbow extension to 150 degrees, flexion WNL neuro: Left bicep strength 4+/5 limited by pain, otherwise was 5/5 in all major muscles of upper and lower extremities; no sensory deficit was noted; 2+ radial and brachial pulses bilaterally, no cyanosis, cap refill < 2 seconds. DIFFERENTIAL DIAGNOSIS Musculoskeletal: delayed-onset muscle soreness (DOMS), compartment syndrome, exertional rhabdomyolysis. Infection: cellulitis. Vascular: venous or arterial thrombosis. Bone: fracture, tumor. TEST AND RESULTS WBC 12.8, Hgb 39.1, MCV 79.9, CPK 63,880 U/L, BUN 15.0, creatinine 1.5, Urinalysis: negative, no blood or RBCs. Hgb A 59%, Hgb F 0%, Hgb S 38%, HgC 0%, HgA2 3%, postive for sickle cell trait; repeat testing 1 week later confirmed sickle cell trait and CPK had decreased to 2001 U/L. FINAL/WORKING DIAGNOSIS Exertional in the presence of sickle cell trait. TREATMENT AND OUTCOMES Pain and swelling subsided with rest and aggressive hydration. He was placed on relative rest and restricted to activities of daily living. Two weeks after presentation, he had no swelling, pain or stiffness in his left arm. His CPK returned to normal. He was permitted to return to football but limited to sprints less than 400 yards. He was advised to modify his exercise regimen to avoid excessive muscle injury. The genetics and potential complications of sickle cell trait were discussed with him.

Sudden death in police pursuit

A rare complication of sickle cell trait is sudden death and collapse. Military recruits, pilots, and subjects exposed to hypoxic stress such as high altitude and experiencing sudden cardiorespiratory collapse as a result of sickle cell trait have been well described. This is a case of a 13-year-old black male who collapsed after a police pursuit and was found to have sickle cell trait and microscopic evidence of asthma.

Sudden Death in Police Pursuit

A rare complication of sickle cell trait is sudden death and collapse. Military recruits, pilots, and subjects exposed to hypoxic stress such as high altitude and experiencing sudden cardiorespiratory collapse as a result of sickle cell trait have been well described. This is a case of a 13-year-old black male who collapsed after a police pursuit and was found to have sickle cell trait and microscopic evidence of asthma.

Exertional Collapse and Sudden Death Associated with Sickle Cell Trait

The most serious complication of sickle cell trait (SCT) is sudden death during exertion. SCT often remains unrecognized in the 2.5 million African Americans affected. Exertional collapse and sudden death associated with SCT is characterized by rhabdomyolysis, heat stroke, and cardiac arrhythmia. There is a 40-fold increased risk of sudden death in affected soldiers during military basic training and there are many cases reported in athletes during preseason training. There have been no cases reported in soldiers beyond basic training. In the case presented, a soldier with 3 years of military service succumbed to SCT-associated sudden death during physical fitness testing.

Unusual Cause of Acute Abdomen

Splenic infarction under hypoxic conditions is a well-known complication of sickle cell trait (Hgb AS). However, this case illustrates that it may occur without any clinical conditions of circumstances associated with decreased Pao2. In addition, sickle cell trait should be included in the differential diagnoses of splenic infarction even if the patient is not of African ancestry.