Association Of Sickle Cell Trait Research Articles

Association of Sickle Cell Trait With Chronic Kidney Disease and Albuminuria in African Americans

Association of Sickle Cell Trait With Chronic Kidney Disease and Albuminuria in African Americans

Sickle cell trait and priapism: a case report and review of the literature

BackgroundA 32 year-old African-American man presented to our institution after attempting suicide via ingestion with quetiapine. He had reported a history of several days of substance abuse with alcohol, cocaine and marijuana related to a partying binge. Following this, his partner removed him from his residence resulting in a suicide attempt. During hospitalization the patient developed priapism, a condition he had not experienced before.Case presentationGiven this was his first time with priapism, an extensive work-up revealed the patient had previously undiagnosed sickle cell trait, which we postulate to have been a significant factor in his development of acute priapism. Sickle cell trait is considered to be a generally benign condition except for a few rare complications under more demanding physical conditions. However, upon reviewing the literature on the association of sickle cell trait with priapism, we believe this may not be the case. Case reports and small series that appeared in the 1960s and 1970s indicated an association between priapism and sickle trait. Little has been reported recently, and the general teaching regarding sickle cell trait does not include this information. However, one case was reported with the use of phosphodiesterase-5 (PDE-5) inhibitors and the development of priapism in a patient with sickle cell trait. These medications are now first line treatment in erectile dysfunction. They act by enhancing nitric oxide (NO) production leading to relaxation of smooth muscle in the corpora cavernosa and penile arteries.ConclusionPriapism was not reported in the initial studies of these medications. Further review of the literature indicates this may be a complex relationship. Interestingly, PDE5 inhibitors also have been postulated to be protective in sickle cell disease and perhaps also sickle cell trait because priapism might be caused by reduced NO availability. In this article, we examine the evidence linking sickle cell trait to priapism, explore the implications of PDE5 use, particularly in the setting of sickle cell trait, and propose that teaching about sickle cell trait include a discussion of priapism risk.

Sickle cell trait and stroke in the young adult

Two young patients with sickle cell trait (AS haemaglobinopathy) and ischaemic stroke are reported. The stroke involved the internal carotid artery territory in one and the brainstem in the other. A review of the literature is presented to suggest that the association of sickle cell trait and cerebral infarction is more than coincidental. Haemoglobin electrophoresis should be undertaken routinely in young subjects with ischaemic stroke.

A case-control study of the association of sickle cell trait and chronic open-angle glaucoma.

Chronic open-angle glaucoma is reported to be more common in blacks than in whites. The reasons for a higher prevalence of this disease in blacks are unclear. Two recent small case series from clinical practice have been published in which secondary open-angle glaucoma was diagnosed in blacks with sickle cell trait who had experienced either traumatic or nontraumatic hyphema. The association of sickle cell trait and chronic open-angle glaucoma has been tested only in a small case-control study of uncontrolled chronic open-angle glaucoma patients undergoing surgery. No significant association was found. However, the higher prevalence of sickle cell trait in blacks compared with whites raises the question of whether the presence of sickle hemoglobin is a risk factor for chronic open-angle glaucoma in the black population. To test for a possible association between this disease and sickle cell trait, we conducted a case-control study in black ophthalmic patients at the University of Pennsylvania and affiliated hospitals between December 1981 and July 1982. Sickle cell trait, as measured by the sickle cell preparation test, was no more prevalent in patients with chronic open-angle glaucoma than in age- and sex-matched ophthalmic clinic controls. It is unlikely that hemoglobin SA is a risk factor for chronic open-angle glaucoma.