Musculoskeletal Manifestations of Sickle Cell Anaemia: A Pictorial Review

Anemia ◽

10.1155/2011/794283 ◽

2011 ◽

Vol 2011 ◽

pp. 1-9 ◽

Cited By ~ 17

Author(s):

A. Ganguly ◽

W. Boswell ◽

H. Aniq

Keyword(s):

Sickle Cell ◽

Sickle Cell Anaemia ◽

Organ Damage ◽

Vessel Occlusion ◽

Tissue Ischemia ◽

Growth Defects ◽

Pictorial Review ◽

Abnormal Haemoglobin ◽

Musculoskeletal Manifestations ◽

Soft Tissue Changes

Sickle cell anaemia is an autosomal recessive genetic condition producing abnormal haemoglobin HbS molecules that result in stiff and sticky red blood cells leading to unpredictable episodes of microvascular occlusions. The clinical and radiological manifestations of sickle cell anaemia result from small vessel occlusion, leading to tissue ischemia/infarction and progressive end-organ damage. In this paper we discuss and illustrate the various musculoskeletal manifestations of sickle cell disease focusing primarily on marrow hyperplasia, osteomyelitis and septic arthritis, medullary and epiphyseal bone infarcts, growth defects, and soft tissue changes.

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Sickle Cell Disease: Role of Oxidative Stress and Antioxidant Therapy

Antioxidants ◽

10.3390/antiox10020296 ◽

2021 ◽

Vol 10 (2) ◽

pp. 296

Author(s):

Rosa Vona ◽

Nadia Maria Sposi ◽

Lorenza Mattia ◽

Lucrezia Gambardella ◽

Elisabetta Straface ◽

...

Keyword(s):

Oxidative Stress ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Globin Gene ◽

Organ Damage ◽

Cell Disease ◽

Vessel Occlusion ◽

Antioxidant Agents ◽

Oxidant Status

Sickle cell disease (SCD) is the most common hereditary disorder of hemoglobin (Hb), which affects approximately a million people worldwide. It is characterized by a single nucleotide substitution in the β-globin gene, leading to the production of abnormal sickle hemoglobin (HbS) with multi-system consequences. HbS polymerization is the primary event in SCD. Repeated polymerization and depolymerization of Hb causes oxidative stress that plays a key role in the pathophysiology of hemolysis, vessel occlusion and the following organ damage in sickle cell patients. For this reason, reactive oxidizing species and the (end)-products of their oxidative reactions have been proposed as markers of both tissue pro-oxidant status and disease severity. Although more studies are needed to clarify their role, antioxidant agents have been shown to be effective in reducing pathological consequences of the disease by preventing oxidative damage in SCD, i.e., by decreasing the oxidant formation or repairing the induced damage. An improved understanding of oxidative stress will lead to targeted antioxidant therapies that should prevent or delay the development of organ complications in this patient population.

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A double blind, placebo controlled randomized evaluation of the efficacy of a Polyherbal Preparation (FaradinR) in treating sickle cell anaemia in Nigerian children

Research Journal of Health Sciences ◽

10.4314/rejhs.v9i3.6 ◽

2021 ◽

Vol 9 (3) ◽

pp. 254-263

Author(s):

D.G. Gbadero ◽

T.A. Olutogun ◽

K.J. Olufemi-Aworinde ◽

L.P. Oluwadare ◽

A.T. Abolarin ◽

...

Keyword(s):

Sickle Cell ◽

Sickle Cell Anaemia ◽

White Cell Count ◽

Randomized Study ◽

Organ Damage ◽

Exclusion Criterion ◽

Double Blind ◽

Hemoglobin F ◽

Transfusion Requirements ◽

Alternative Medications

Introduction: The goal of management of sickle cell anaemia (SCA), for many years, has been to manage acute intermittent crises and slow down chronic end organ damage. In the past few decades, with increasing understanding of its pathophysiology, compounds primarily preventive in action are being investigated and used. Faradin® (a poly-herbal traditional supplement mixture) has been used aspreventive measure against painful episodes by SCA patients as an over the counter medication and anecdotal evidence suggests that it reduced the frequency and severity of painful crises as well as transfusion requirements. Alternative medications that are both affordable and available should be considered viable alternatives provided safety and efficacy are assured because of the high disease burden in Nigeria.Methods: This was a double controlled randomized study was carried out on twenty children. Each enrolled patient was randomized into either the herbal mixture or placebo after permission to participate in the study was obtain from the parents/guardian for children below 15 years or from both parents/guardian and the patients where the latter are older than 15 years. The main exclusion criterion was prior use or exposure to Faradin. Primary end points were pain alteration, death during study and blood transfusion frequency. Secondary endpoints were hemoglobin levels, neutrophil count, platelet count, hemoglobin F and A2 levels, serum bilirubin, nitric oxide concentration, drug toxicity and severe complications of sickle cell anemia reported during the study.Results: There was no severe adverse event, deaths or transfusion recorded in the two groups throughout the duration of the study. Mean hematocrit was increased in the Faradin group and reticulocyte count was increased by 12 %. Faradin reduced the total white cell count to half its baseline level and increased hemoglobin F levels by 10%. Weight and appetite were reported to increased and engenders a generalfeeling of wellbeing.Conclusion: Faradin appears to be an efficacious, nontoxic, available and affordable remedy for treating SCA patients in our setting.

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Sickle Cell Disease: Role of Oxidative Stress and Antioxidant Therapy

10.20944/preprints202012.0522.v1 ◽

2020 ◽

Author(s):

Rosa Vona ◽

Nadia Maria Sposi ◽

Lorenza Mattia ◽

Lucrezia Gambardella ◽

Elisabetta Straface ◽

...

Keyword(s):

Oxidative Stress ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Cell Metabolism ◽

Globin Gene ◽

Organ Damage ◽

Beta Globin ◽

Cell Disease ◽

Vessel Occlusion ◽

Antioxidant Agents

Sickle cell disease (SCD) is the most common hereditary disorder of hemoglobin (Hb) that affects approximately a millions people worldwide. It is characterized by a single nucleotide substitution on the β-globin gene, leading to the production of abnormal sickle hemoglobin with multi-system consequences. Mutated Hb leads to profound changes in: i) red blood cell metabolism and physiology; ii) endothelial signaling; and iii) immune response. Oxidative stress is an important hallmark of SCD. It plays a key role in the pathophysiology of hemolysis, vessel occlusion and the following organ damage in sickle cell patients. For this reason, reactive oxidizing species and the (end)-products of their oxidative reactions have been proposed as markers of both tissue pro-oxidant status and disease severity. Although more studies are needed to clarify their role, antioxidant agents have been shown to be effective in reducing pathological consequences of the disease by preventing oxidative damage in SCD, i.e. by decreasing the oxidant formation or repairing the induced damage. An improved understanding of oxidative stress will lead to targeted antioxidant therapies that should prevent or delay the development of organ complications in this patient population.

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Acute Pancreatitis as a Complication of Sickle Cell Anaemia

Reports ◽

10.3390/reports1030019 ◽

2018 ◽

Vol 1 (3) ◽

pp. 19

Author(s):

Parisa Moori ◽

Alexios Dosis ◽

Zoheb Ahmad ◽

Ambareen Kausar ◽

Diana Triantafyllopoulou

Keyword(s):

Acute Pancreatitis ◽

Sickle Cell ◽

Rare Case ◽

Autosomal Recessive ◽

Sickle Cell Anaemia ◽

Autosomal Recessive Disease ◽

Physiological Changes ◽

Common Cause ◽

Patient Morbidity ◽

Abnormal Haemoglobin

A common cause of abdominal pain is acute pancreatitis, with the majority of cases being attributed to gallstones and excess alcohol. Sickle cell anaemia (SCA) is an autosomal recessive disease causing the production of abnormal haemoglobin. Physiological changes can lead to vaso-occlusion in sickle cell anaemia. Cholelithiasis is frequently seen in patients with SCA and complications from this can increase patient morbidity. We present a rare case of acute pancreatitis inducing a vaso-occlusive crisis.

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Sickle cell disease

InnovAiT Education and inspiration for general practice ◽

10.1177/1755738015626186 ◽

2016 ◽

Vol 10 (2) ◽

pp. 73-81 ◽

Cited By ~ 1

Author(s):

Anietie Ekong

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Sickle Cell Anaemia ◽

Cell Disease ◽

Chronic Complications ◽

Health Maintenance ◽

Abnormal Haemoglobin ◽

The Uk ◽

The Tropics ◽

Cell Gene

The haemoglobinopathies are the most common monogenetic diseases in the world. They include the thalassaemias and sickle cell syndromes. The sickle cell syndromes encompass several abnormal haemoglobin variants, of which homozygosity for the sickle cell gene – that is, sickle cell anaemia is the most common and most severe. Originally characteristic of the tropics and subtropics, recent mobility and migratory trends have meant that the prevalence of sickle cell disease (SCD) has significantly increased in the UK. It is important that GPs have an understanding of this disease, in order to help their patients deal with complications of every-day life. This article will address three main aspects of SCD: diagnosis, health maintenance, and some acute and chronic complications of SCD.

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Pathophysiological Relevance of Renal Medullary Conditions on the Behaviour of Red Cells From Patients With Sickle Cell Anaemia

Frontiers in Physiology ◽

10.3389/fphys.2021.653545 ◽

2021 ◽

Vol 12 ◽

Author(s):

David C.-Y. Lu ◽

Rasiqh Wadud ◽

Anke Hannemann ◽

David C. Rees ◽

John N. Brewin ◽

...

Keyword(s):

Sickle Cell ◽

Sickle Cell Anaemia ◽

Renal Medulla ◽

Red Cells ◽

Red Cell ◽

Sickle Cells ◽

Hypoxic Conditions ◽

Intracellular Ca ◽

Concentrate Urine ◽

Abnormal Haemoglobin

Red cells from patients with sickle cell anaemia (SCA) contain the abnormal haemoglobin HbS. Under hypoxic conditions, HbS polymerises and causes red cell sickling, a rise in intracellular Ca2+ and exposure of phosphatidylserine (PS). These changes make sickle cells sticky and liable to lodge in the microvasculature, and so reduce their lifespan. The aim of the present work was to investigate how the peculiar conditions found in the renal medulla – hypoxia, acidosis, lactate, hypertonicity and high levels of urea – affect red cell behaviour. Results show that the first four conditions all increased sickling and PS exposure. The presence of urea at levels found in a healthy medulla during antidiuresis, however, markedly reduced sickling and PS exposure and would therefore protect against red cell adherence. Loss of the ability to concentrate urine, which occurs in sickle cell nephropathy would obviate this protective effect and may therefore contribute to pathogenesis.

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Enhanced Hypercoagulability in Sickle Cell Anaemia Patients with Chronic Leg Ulcers

Advances in Hematology ◽

10.1155/2020/5157031 ◽

2020 ◽

Vol 2020 ◽

pp. 1-6

Author(s):

David Sackey ◽

Yvonne Dei-Adomakoh ◽

Edeghonghon Olayemi

Keyword(s):

Sickle Cell ◽

Sickle Cell Anaemia ◽

Antiplatelet Agents ◽

Organ Damage ◽

Leg Ulcers ◽

Chronic Complications ◽

The Mean ◽

Hb Concentration ◽

Chronic Leg Ulcers

Sickle Cell Anaemia (SCA) is associated with a hypercoagulable state resulting in a predisposition to venous thromboembolism. With improvements in the quality of care, more patients with SCA survive into adulthood with an associated increase in the frequency of end-organ damage and chronic complications such as chronic leg ulcers (CLUs). These ulcers rarely occur in the first decade of life and are recurrent, painful, and slow-to-heal. This study tested the hypothesis that coagulation is enhanced in SCA patients with CLU. 145 participants (50 SCA with CLU, 50 SCA without CLU, and 45 with haemoglobin AA) were assessed to determine their coagulation profile using selected tests of coagulation. The SCA with the CLU group had the lowest mean haemoglobin (Hb) concentration. SCA patients with and without CLUs had elevated mean platelet counts, shorter mean aPTT, and marginally prolonged mean PT compared to HbAA patients. SCA with CLUs patients had a significantly shortened aPTT than those without CLUs ( p = 0.035) and HbAA ( p = 0.009). There were significant differences in the mean PT between SCA with CLUs patients and HbAA ( p = 0.017); SCA without CLU and HbAA ( p = 0.014). SCA with and without CLUs patients had higher mean D-dimer levels compared to HbAA. There was a negative correlation between Hb concentration and duration of CLU (r = -0.331, p = 0.021). In conclusion, our study demonstrates a heightened hypercoagulability in SCA patients with CLUs. We did not test for platelet activation, and it is not clear what role, if any, the enhanced hypercoagulability plays in the pathogenesis of CLUs in SCA. It will be useful to ascertain if antiplatelet agents or/and anticoagulants quicken the healing of CLUs in SCA patients.

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