Leg ulcers in patients with sickle cell disease [see comments]

Koshy, Matthew; Entsuah, Richard; Koranda, A.; Kraus, AP; Johnson, Robin; Bellvue, R; Flournoy-Gill, Zanet; Levy, Paul

doi:10.1182/blood.v74.4.1403.bloodjournal7441403

Cited by 73 publications

(146 citation statements)

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“…Even though Jamaican males are generally known to have poorer health seeking behaviours [17] associated with worse health outcomes, the SCD males had similar rates of annual health maintenance visits but higher rates of annual total visits when compared to SCD females in this study. Males had higher prevalence of leg ulcers (similar to what exists in previous literature [27,28]) and respiratory events. They also tended to have their first episode of significant pain crisis, respiratory event and leg ulcerations at lower ages than girls did.…”

Section: Annual Total Visits To Scusupporting

confidence: 87%

Socio-environmental exposures and health outcomes among persons with sickle cell disease

et al. 2017

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There is much variability in the expression of sickle cell disease (SCD) and recent works suggest that environmental and social factors may also influence this variability. This paper aims to use geographic information systems technology to examine the association between socio-environmental exposures and health outcomes in all persons who have attended or currently attend the Sickle Cell Unit in Jamaica. Rural patients presented for clinical care at older ages and had less annual visits to clinic. Persons travelled relatively long distances to seek SCD care and those travelling longer had less health maintenance visits. Urban patients had a higher prevalence of significant pain crises (69.4% vs. 55.8%, p value<0.001) and respiratory events (21.2% vs. 14%, p value<0.001). Prevalence of leg ulcers did not vary between rural and urban patients but was higher in males than in females. Females also had lower odds of having respiratory events but there was no sex difference in history of painful crises. Persons with more severe genotypes lived in higher poverty and travelled longer for healthcare services. Persons in areas with higher annual rainfall, higher mean temperatures and living farther from factories had less painful crises and respiratory events. The paper highlights a need for better access to healthcare services for Jamaicans with SCD especially in rural areas of the island. It also reports interesting associations between environmental climatic exposures and health outcomes.

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Section: Annual Total Visits To Scusupporting

confidence: 87%

Socio-environmental exposures and health outcomes among persons with sickle cell disease

et al. 2017

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“…In the literature large differences in prevalence of chronic leg ulcers can be found with prevalence rates as high as 30-70% in studies from Jamaica 30,31 whereas the Cooperative Study in the US found a prevalence rate of 5%. 13 In a recent report of Minniti et al a prevalence of 21.4% in HbSS patients and 9.6% in HbSC patients was observed 32 which equals the prevalence in our cohort.…”

Section: Ajh Ajh | E587supporting

confidence: 62%

Prospective evaluation of chronic organ damage in adult sickle cell patients: A seven‐year follow‐up study

et al. 2017

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Organ damage in sickle cell disease (SCD) is a crucial determinant for disease severity and prognosis. In a previous study, we analyzed the prevalence of SCD-related organ damage and complications in adult sickle cell patients. We now describe a seven-year follow-up of this cohort.All patients from the primary analysis in 2006 (n = 104), were included for follow-up. Patients were screened for SCD-related organ damage and complications (microalbuminuria, renal failure, elevated tricuspid regurgitation flow velocity (TRV) (≥2.5 m/seconds), retinopathy, iron overload, cholelithiasis, avascular osteonecrosis, leg ulcers, acute chest syndrome (ACS), stroke, priapism and admissions for vaso-occlusive crises (VOC) biannually. Upon 7 years of follow-up, progression in the prevalence of avascular osteonecrosis (from 12.5% to 20.4%), renal failure (from 6.7% to 23.4%), retinopathy (from 39.7% to 53.8%) was observed in the whole group. In HbSS/HbSβ -thal patients also progression in microalbuminuria (from 34% to 45%) and elevated TRV (from 40% to 48%) was observed while hardly any progression in the prevalence of cholelithiasis, priapism, stroke or chronic ulcers was seen. The proportion of patients with at least one episode of ACS increased in the group of HbSS/HbSβ -thal patients from 32% to 49.1%. In conclusion, 62% of the sickle cell patients in this prospective cohort study developed a new SCD-related complication in a comprehensive care setting within 7 years of follow-up. Although the hospital admission rate for VOC remained stable, multiple forms of organ damage increased substantially. These observations underline the need for continued screening for organ damage in all adult patients with SCD.

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“…Follow-up of patients with venous incompetence but without leg ulcers will document its importance in the genesis of new ulcers. These observations do not explain the striking difference in prevalence of ulcers between the 50-70% reported from Jamaica and the 5-10% reported from elsewhere (Koshy et al, 1989). Venous incompetence is unlikely to be specific to Jamaican patients and one must assume that other factors, probably environmental, contribute to the marked difference in prevalence of ulceration.…”

Section: Discussionmentioning

confidence: 66%

Chronic leg ulceration in homozygous sickle cell disease: the role of venous incompetence

et al. 2002

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Summary. Chronic leg ulceration is a common cause of morbidity in Jamaican patients with homozygous sickle cell (SS) disease. Ulcers heal more rapidly on bed rest and deteriorate on prolonged standing, suggesting a role of venous hypertension in their persistence. This hypothesis has been tested by Doppler detection of venous competence in SS patients and in matched controls with a normal haemoglobin (AA) genotype in the Jamaican Cohort Study. Venous incompetence was significantly more frequent in SS disease [137/183 (75%)] than in non‐pregnant AA controls [53/137 (39%)]. Past or present ulceration occurred in 78 (43%) SS patients, with a highly significant association between leg ulceration and venous incompetence in the same leg (P < 0·001). Prominence and/or varicosities of the veins and spontaneous leg ulcers were more common among patients with multiple sites of incompetence. The association of venous incompetence with chronic leg ulceration identifies a further pathological mechanism contributing to the morbidity of SS disease. The cause of venous incompetence is unknown but the sluggish circulation associated with dependency, turbidity and impaired linear flow at venous valves, hypoxia‐induced sickling, the rheological effects of high white cell counts, and activation of components of the coagulation system may all contribute. Venous hypertension in SS patients with leg ulceration suggests that firm elastic supportive dressings might promote healing of chronic leg ulcers.

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Leg ulcers in patients with sickle cell disease [see comments]

Cited by 73 publications

References 0 publications

Socio-environmental exposures and health outcomes among persons with sickle cell disease

Socio-environmental exposures and health outcomes among persons with sickle cell disease

Prospective evaluation of chronic organ damage in adult sickle cell patients: A seven‐year follow‐up study

Chronic leg ulceration in homozygous sickle cell disease: the role of venous incompetence

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