Leg ulceration and abnormalities of calf blood flow in sickle‐cell anemia

Saad, Sara T.O.; Zago, M A

doi:10.1111/j.1600-0609.1991.tb01275.x

Cited by 7 publications

(4 citation statements)

References 12 publications

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“…Leg ulcers among SCA patients are thought to be vascular in nature. Vaso-occlusive crises and thrombotic events lead to ischaemia along the extremity which is followed by poor healing and further ischemia 22 . Local trauma may initially caused skin breakdown, due to poor perfusion and healing difficulties leading to persistent leg ulcers.…”

Section: Spine Osteonecrosismentioning

confidence: 99%

The prevalence and factors associated with musculoskeletal disorders, in patients with sickle cell anaemia, at Mulago National Referral Hospital, Uganda

Akaro¹,

Madewo²,

Orwotho³

et al. 2016

East. Cent. Afr. J. Surg.

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Section: Spine Osteonecrosismentioning

confidence: 99%

The prevalence and factors associated with musculoskeletal disorders, in patients with sickle cell anaemia, at Mulago National Referral Hospital, Uganda

Akaro¹,

Madewo²,

Orwotho³

et al. 2016

East. Cent. Afr. J. Surg.

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“…5 Venous hyperpressure has been evidenced by Saad and Zago, who measured the clearance (% decrease/min) of 99m Tc, in SCD patients with leg ulcers to demonstrate that their calf muscle blood flow was impaired. 107 Additionally, Billet et al and Mohan et al observed rapid refill times using venous pulse recordings, venous plethysmography and laser Doppler flowmetry in SCD patients with leg ulcers compared to those without. 32,108 To better understand the origin of venous hyperpressure, venous incompetency has been studied.…”

Section: Venous Insufficiencymentioning

confidence: 99%

“…Early observations indicated that bed rest promotes ulcer healing in SCD patients, suggesting that venous hyperpressure may contribute to ulcer healing delay by causing oedema 5 . Venous hyperpressure has been evidenced by Saad and Zago, who measured the clearance (% decrease/min) of 99m Tc, in SCD patients with leg ulcers to demonstrate that their calf muscle blood flow was impaired 107 . Additionally, Billet et al.…”

Section: Delayed Healing Of Scd Ulcers Mainly Venous Ulcersmentioning

confidence: 99%

Controversies in the pathophysiology of leg ulcers in sickle cell disease

Catella,

Guillot,

Nader

et al. 2024

Br J Haematol

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SummaryPatients with sickle cell disease (SCD) often experience painful vaso‐occlusive crises and chronic haemolytic anaemia, as well as various acute and chronic complications, such as leg ulcers. Leg ulcers are characterized by their unpredictability, debilitating pain and prolonged healing process. The pathophysiology of SCD leg ulcers is not well defined. Known risk factors include male gender, poor social conditions, malnutrition and a lack of compression therapy when oedema occurs. Leg ulcers typically start with spontaneous pain, followed by induration, hyperpigmentation, blister formation and destruction of the epidermis. SCD is characterized by chronic haemolysis, increased oxidative stress and decreased nitric oxide bioavailability, which promote ischaemia and inflammation and consequently impair vascular function in the skin. This cutaneous vasculopathy, coupled with venostasis around the ankle, creates an ideal environment for local vaso‐occlusive crises, which can result in the development of leg ulcers that resemble arterial ulcers. Following the development of the ulcer, healing is hindered as a result of factors commonly observed in venous ulceration, including venous insufficiency, oedema and impaired angiogenesis. All of these factors are modulated by genetic factors. However, our current understanding of these genetic factors remains limited and does not yet enable us to accurately predict ulceration susceptibility.

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“…However, edema and pain often precede ulceration in these patients, and numerous studies since then have linked venous stasis with sickle cell leg ulcers [7]. Venous stasis in the calf muscles was suggested by the delayed clearance of 99m Tc [22] and by magnetic resonance spectroscopy studies [23] in SS patients with leg ulcers as compared with those without. Mohan et al described reduced venous refilling time and cutaneous red blood cell flux recovery time after exercise in patients with SS disease with leg ulcers compared to SS and AA patients without ulcers.…”

Section: Venous Incompetencementioning

confidence: 99%

Leg Ulceration in Sickle Cell Disease: An Early and Visible Sign of End‐Organ Disease

Singh¹,

Minniti²

2016

Sickle Cell Disease - Pain and Common Chronic Complications

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Introduction: Leg ulcers are a frequent and debilitating complication of sickle cell disease (SCD), particularly of the SS genotype. The prevalence of leg ulcers in patients with sickle cell disease (SCD) varies geographically ranging widely from 75% in Jamaica to as low as 1% in Saudi Arabia. The prevalence of leg ulcers in the Cooperative Study of Sickle Cell Disease (CSSCD) in the United States was 5% in SS genotype with the incidence increasing with age. As patients with SCD have increasingly improved survival, the prevalence of leg ulcers is likely to be higher. These ulcers are slow to heal, have a high rate of recurrence, and are associated with severe unremitting pain and depression, thus leading to high healthcare costs. Despite being a well-recognized complication of SCD, there are no specifically designed evidence-based guidelines to help clinicians manage these patients.Methods: To prepare this manuscript, we searched PubMed using the search terms "sickle cell," "ulcer," "sickle cell," and "wound." We also appraised the references mentioned in the identified articles. Inclusion criteria included case reports, case series, retrospective reviews, clinical trials, randomized controlled trials, systematic reviews, and meta-analyses from 1945 to 2016. We present our extensive personal observations and expert opinion, whenever there is a lack of reliable data. Conclusion:Our understanding of the pathophysiology of leg ulceration in sickle cell disease is improved, though still limited since the first described case in the English literature over 100 years ago. Moreover, there remains a paucity of good quality randomized clinical trials to test new and effective therapies. No evidence-based guidelines for the management of these patients are available. Currently, a holistic multidisciplinary approach is recommended with adequate systemic control of SCD as well as aggressive local therapy, with a focus on targeting pathways involved in potentiating healing of these ulcers including novel approaches like topical nitric oxide donors. SCD patients with leg ulcers represent a cohort of patients who are at an increased risk of developing other vasculopathic complications that have a potentially common mechanism including pulmonary hypertension, renal and retinal disease, and

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Leg ulceration and abnormalities of calf blood flow in sickle‐cell anemia

Cited by 7 publications

References 12 publications

The prevalence and factors associated with musculoskeletal disorders, in patients with sickle cell anaemia, at Mulago National Referral Hospital, Uganda

The prevalence and factors associated with musculoskeletal disorders, in patients with sickle cell anaemia, at Mulago National Referral Hospital, Uganda

Controversies in the pathophysiology of leg ulcers in sickle cell disease

Leg Ulceration in Sickle Cell Disease: An Early and Visible Sign of End‐Organ Disease

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