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Philippe Connes - One of the best experts on this subject based on the ideXlab platform.
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Sickle Cell Trait exertional rhabdomyolysis and compartment syndrome
The Lancet, 2015Co-Authors: Philippe ConnesAbstract:1948 www.thelancet.com Vol 385 May 16, 2015 exertional rhabdomyolysis. The authors proposed that the patient’s Sickle Cell Trait might have precipitated the occurrence and the worsening of this complication. Metabolic changes occurring during exercise were suggested to promote the polymerisation of haemoglobin S and red blood Cell sickling. The resulting microvascular obstruction would cause muscle ischaemia, leading to rhabdomyolysis. Unfortunately, oxygen therapy did not improve the condition of the patient, who developed compartment syndrome. However, whether red blood Cell sickling is truly the cause of this complication is unknown. The presence of Sickled red blood Cells has rarely been observed in peripheral blood from exercising Sickle Cell Trait carriers. Moreover, muscle biopsies in this population show the presence of wider capillaries than in individuals without Sickle Cell Trait, suggesting that a small loss of red blood Cell deformability caused by the polymerisation of less than 40–45% of haemoglobin S could not be suffi cient to occlude these large capillaries. Exertional rhabdomyolysis affects one in 10 000 people per year, and its incidence in the military population has more than tripled between 2006 and 2011. Whereas Sickle Cell Trait could increase the risks of exertional rhabdomyolysis in people of African descent, other inherited metabolic muscle disorders could be involved too. Patricia Deuster and colleagues reported that the GG genotype of the muscle-specific creatine kinase isoform (CKMM) Nco1 polymorphism was associated with an increased risk for exertional rhabdomyolysis and was more frequently found in African Americans (28·1%) than in white people (14·2%). They also reported that individuals with the myosin light chain kinase 2 (MYLK2) A allele—an allele also more frequently encountered in African Americans than in white people—were more likely to have had a clinical episode of exertional rhabdomyolysis. Moreover, the presence of another genetic defect could also transform the silent Sickle Cell Trait into a syndrome resembling Sickle Cell disease with vaso-occlusion. For instance, Gretchen Kimmick and John Owen described the case of a black man with both Sickle Cell Trait and glucose-6-phosphate dehydrogenase (G6PD) deficiency who developed exertional rhabdomyolysis at diff erent occasions. Since G6PD defi ciency and Sickle Cell Trait can be expected to occur simultaneously in up to 1% of men of African descent, physicians should be encouraged to screen for the former defect too. Indeed, an alternative hypothesis to that proposed by Ridha and colleagues is that haemoglobin S could be a surrogate for or act in combination with other gene variants to precipitate severe exertional rhabdomyolysis. Screening for other genetic variants might help to clarify the pathophysiological mechanisms underlying exertional rhabdomyolysis in people of African ethnic origin.
Barry J. Maron - One of the best experts on this subject based on the ideXlab platform.
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Sickle Cell Trait and Sudden Death in Athletes-Reply
JAMA: The Journal of the American Medical Association, 1996Co-Authors: Barry J. MaronAbstract:In Reply. —Drs Kerle and Runkle have made a reasonable point regarding the potential role that Sickle Cell Trait may have played in the sudden deaths of young African-American athletes with cardiovascular disease in our study population. As Kerle and Runkle point out, Sickle Cell Trait has been incriminated as a sole determinant of sudden cardiac death with exertion,1,2and indeed, 1 of our athletes without a detectable cardiac abnormality had only the finding of Sickle Trait to explain sudden death. Among the 134 competitive athletes in our study, we found hypertrophic cardiomyopathy to be the most common cause of sudden death. Of the 48 athletes assigned this diagnosis at autopsy, the majority were black. Unfortunately, our study design did not include a systematic analysis for Sickle Cell Trait in these individuals. Also, we encountered 14 other athletes who showed an increase in left ventricular mass, but no other features of
David E. Soper - One of the best experts on this subject based on the ideXlab platform.
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Bacteriuria in pregnant women with Sickle Cell Trait
American journal of obstetrics and gynecology, 2006Co-Authors: Andrea Ries Thurman, Lisa L. Steed, Thomas C. Hulsey, David E. SoperAbstract:Objective The purpose of this study was to compare the following outcome variables in pregnant patients with Sickle Cell Trait and matched pregnant control patients: asymptomatic bacteriuria, acute cystitis, urinary pathogens that were present, and pyelonephritis. Study design This was a retrospective cohort study that was conducted at a university clinic. Pregnant patients with Sickle Cell Trait (n = 455) were matched with control patients (n = 448) for race, age, gestational age at entry into prenatal care, and number of prenatal visits. Results Women with Sickle Cell Trait received urine testing significantly more often. There was no difference in the incidence of positive urine cultures, urinary pathogens, or asymptomatic bacteriuria among the comparison groups. Sickle Cell Trait carriers had significantly higher rates of pyelonephritis, but many affected patients had risk factors, such as previous pyelonephritis or noncompliance with therapy. Conclusion Sickle Cell Trait carriers were no more susceptible to acute cystitis and asymptomatic bacteriuria than were the control patients. On the basis of these data, we outline recommendations for urinary screening and pyelonephritis prevention in pregnant patients with Sickle Cell Trait.
George A. Macones - One of the best experts on this subject based on the ideXlab platform.
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Pregnant women with the Sickle Cell Trait are not at increased risk for developing preeclampsia.
American journal of perinatology, 2003Co-Authors: David M. Stamilio, Harish M. Sehdev, George A. MaconesAbstract:The primary objective of this study was to determine whether having the Sickle Cell Trait is independently associated with preeclampsia. We performed a retrospective cohort study of 1998 pregnant patients who either did or did not have the Sickle Cell Trait. All patients were screened for the Sickle Trait using the "Sickledex" test. Data on neonatal and maternal outcome, including preeclampsia, and potential confounding variables were abstracted from medical records. Unadjusted, stratified, and multiple logistic regression analyses were used to identify interactions, and confounding between multiple variables and the association between Sickle Cell Trait and preeclampsia. With an anticipated 6.5% rate of preeclampsia, and alpha = 0.05, this cohort study has 80% power to detect a relative risk (RR) of 2.3 for preeclampsia. Univariate analysis revealed that the two cohorts were similar with regard to primiparity, maternal age, chronic diseases, birth weight, and gestational age at delivery, but the Sickle Cell Trait cohort was more likely to have gestational diabetes and had a higher mean body mass index (BMI). In the univariate analysis, the Sickle Cell Trait cohort was not at increased risk for preeclampsia [unadjusted RR = 0.5, 95% CI (0.2-1.6)]. After controlling for potential confounding variables with logistic regression analysis, Sickle Trait was not independently associated with preeclampsia [adjusted RR = 0.5, 95% CI (0.2- 1.6)]. In contrast to prior work, these data suggest that the Sickle Cell Trait is not an independent risk factor for preeclampsia or postpartum complications. In fact, the data are more consistent with the Sickle Trait being protective for developing preeclampsia.
Manju Monga - One of the best experts on this subject based on the ideXlab platform.
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Women with Sickle Cell Trait are at increased risk for preeclampsia
American journal of obstetrics and gynecology, 1997Co-Authors: Kelly D. Larrabee, Manju MongaAbstract:Abstract Objective: Our purpose was to determine the rate of preeclampsia in women who are positive for Sickle Cell Trait. Study design: All African-American women were tested for Sickle Cell Trait with the "Sickledex" screen at the first prenatal visit and prospectively enrolled in this study from March 1994 to June 1995. "Sickledex" screens were confirmed with hemoglobin electrophoresis. Demographic data were collected at the time of enrollment. Outcome data, including preeclampsia (as defined by The American College of Obstetricians and Gynecologists criteria), gestational age at delivery, birth weight, and postpartum endometritis were collected immediately post partum. Assuming a 10% rate of positive Sickle Cell Trait, 1100 patients were required to demonstrate a doubling in the rate of preeclampsia with 80% power and p t test, the Mann-Whitney U test, χ 2 analysis, and Fisher's exact tests were used for statistical analysis. Results: Of 1584 women enrolled in the study, 162 were positive for Sickle Cell Trait. Sickle Cell Trait–positive women were older than the Sickle Cell Trait–negative women (24.4 ± 4.6 vs 23.0 ± 4.4 years, p p p p p p Conclusion: This is the first prospective study to demonstrate that Sickle Cell Trait–positive women are at significantly higher risk for development of perinatal complications that have traditionally been associated with Sickle disease. (Am J Obstet Gynecol 1997;177:425-8.)