Priapism Associated with Hemoglobin C Trait

Gibson, Brent R.; Peterson, Andrew C.; Costabile, Raymond A.

doi:10.1016/s0022-5347(05)64314-5

Cited by 5 publications

(3 citation statements)

References 4 publications

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“…Precipitation of these chains occurs when the red blood cell is exposed to conditions of stress ultimately leading to increased rigidity of the cells. 5–9 During a hemodialysis session, red blood cells flow through an extracorporeal circuit that is exposed to environmental stressors (low temperature and low partial pressure of oxygen) potentially leading to increased destruction of red blood cells and a shortened half-life. Although we do not have measures of low-grade red-cell destruction in the current cohort, measurement of indices such as serum bilirubin, lactate dehydrogenase, and haptoglobin could be instructive in future studies.…”

Section: Discussionmentioning

confidence: 99%

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Variant hemoglobin phenotypes may account for differential erythropoiesis-stimulating agent dosing in African-American hemodialysis patients

Derebail

Nachman

Key

et al. 2011

Kidney International

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African-American patients with end-stage renal disease have historically lower hemoglobin concentrations and higher requirements of erythropoiesis-stimulating agent (ESA). While disparities in health-care access may partially explain these findings, the role of variant hemoglobin, such as sickle trait, has not been investigated. To clarify this, we evaluated 154 African-American patients receiving in-center hemodialysis with available hemoglobin phenotyping. The primary exposure was any abnormal hemoglobin variant and the primary outcome of higher-dose ESA was defined as a dose of 6500 or more units per treatment. Logistic regression assessed the association between variant hemoglobin and higher-dose ESA. Covariates included age, gender, diabetes, iron parameters, intravenous iron dose, parathyroid hormone, albumin, phosphorus, body mass index, vascular access type, hospitalization/missed treatments, smoking status, alcohol abuse, and gastrointestinal bleeding. Of 33 patients with variant hemoglobin, 24 had HbAS and 9 had HbAC. Univariate odds of higher-dose ESA among those with hemoglobin variants were twice that of those with the normal HbAA phenotype (odds ratio 2.05). In multivariate models, the likelihood of higher-dose ESA had an odds ratio of 3.31 and the nature of this relationship did not change in Poisson regression or sensitivity analyses. Hence, our findings may explain, in part, the difference in ESA dosing between Caucasians and African-Americans with end-stage renal disease but await further study.

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Section: Discussionmentioning

confidence: 99%

“…4 Although typically described as a benign carrier state, individuals with either HbAS or HbAC may experience clinical complications due to changes to red-cell rigidity under situations of stress 5–9 that could lead to low-grade sickling and hemolysis. Speculatively, such changes could occur during the hemodialysis procedure.…”

mentioning

confidence: 99%

Variant hemoglobin phenotypes may account for differential erythropoiesis-stimulating agent dosing in African-American hemodialysis patients

Derebail

Nachman

Key

et al. 2011

Kidney International

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“…[3] Although the exact pathogenesis of the priapism associated with Niemann–Pick disease is not clear, possible mechanism could be accumulation of foam cells causing sluggish blood flow, hypercoagulability and venous outlet obstruction of corpora cavernosa. [4] Since many cases of priapism have unidentifiable etiology, we consider that this could be one of the causes. This could be a coincidental and a random association.…”

Section: Discussionmentioning

confidence: 99%

Priapism associated with Niemann-Pick disease in a 15-year-old boy

Shetty¹,

Kumar²,

Sen³

2011

Indian J Urol

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A 15-year-old boy presented with priapism of 46 h duration. There was no known cause of priapism detected in him. During evaluation, biopsy of the lymph node and bone marrow aspiration detected patient to be having Niemann–Pick disease. He was managed with cavernous aspiration, saline irrigation, and intracavernosal phenylephrine injection. Although priapism is associated with many known diseases, about half of the cases are of idiopathic origin. We are reporting the first case of priapism associated with Niemann–Pick disease in the literature.

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Sickle Cell Trait, Hemoglobin C Trait, and Invasive Pneumococcal Disease

et al. 2010

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Background The cause of historically higher rates of invasive pneumococcal disease among blacks than whites has remained unknown. We tested the hypothesis that sickle cell trait or hemoglobin C trait is an independent risk factor for invasive pneumococcal disease. Methods Eligible children were born in Tennessee (1996–2003), had a newborn screen, enrolled in TennCare aged <1 year, and resided in a Tennessee county with laboratory-confirmed, pneumococcal surveillance. Race/ethnicity was ascertained from birth certificates. Children were followed through 2005 until loss of enrollment, pneumococcal disease episode, 5th birthday or death. We calculated incidence rates by race/ethnicity and hemoglobin type before and after pneumococcal conjugate vaccine (PCV7) introduction. Poisson regression analyses compared IPD rates among blacks with sickle cell trait or hemoglobin C trait to whites and blacks with normal hemoglobin, controlling for age, gender, time (pre-PCV7, transition year or post-PCV7) and high-risk conditions (i.e. heart disease). Results Over 10 years, 415 invasive pneumococcal disease episodes occurred during 451,594 observed child-years. Before PCV7 introduction, disease rates/100,000 child-years were 2941 for blacks with sickle cell disease, 258 for blacks with sickle cell trait or hemoglobin C trait and 188, 172, and 125 for blacks, whites, and Hispanics with normal hemoglobin. Post-PCV7, rates declined for all groups. Blacks with sickle cell trait or hemoglobin C trait had 77% (95% CI 22%–155%) and 42% (95% CI 1%–100%) higher rates than whites and blacks with normal hemoglobin. Conclusion Black children with sickle cell trait or hemoglobin C trait have an increased risk of invasive pneumococcal disease.

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Priapism Associated with Hemoglobin C Trait

Cited by 5 publications

References 4 publications

Variant hemoglobin phenotypes may account for differential erythropoiesis-stimulating agent dosing in African-American hemodialysis patients

Variant hemoglobin phenotypes may account for differential erythropoiesis-stimulating agent dosing in African-American hemodialysis patients

Priapism associated with Niemann-Pick disease in a 15-year-old boy

Sickle Cell Trait, Hemoglobin C Trait, and Invasive Pneumococcal Disease

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