scholarly journals Enhanced Hypercoagulability in Sickle Cell Anaemia Patients with Chronic Leg Ulcers

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.

scholarly journals Topical sodium nitrite for chronic leg ulcers in patients with sickle cell anaemia: a phase 1 dose-finding safety and tolerability trial

2014 ◽  
Vol 1 (3) ◽  
pp. e95-e103 ◽  
Author(s):  
Caterina P Minniti ◽  
Alexander M Gorbach ◽  
Dihua Xu ◽  
Yuen Yi Hon ◽  
Kara-Marie Delaney ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Sodium Nitrite ◽  
Sickle Cell Anaemia ◽  
Phase 1 ◽  
Dose Finding ◽  
Leg Ulcers ◽  
Chronic Leg Ulcers

scholarly journals Evaluation of Acute Painful Episodes with Foetal Hemoglobin Level and Other Haematological Parameters in Sickle Cell Patients in Abuja Nigeria

2019 ◽  
pp. 1-5
Author(s):  
O. Chinwe Okeke ◽  
O. Ernest Ukaejiofo ◽  
E. Nnodu Obiageli ◽  
D. Ezigbo Eyiuche ◽  
C. Okeke Chinedu
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Haematological Parameters ◽  
Severe Illness ◽  
Control Group ◽  
The Mean ◽  
Hb Concentration ◽  
The Relationship ◽  
Painful Episode ◽  
Apparently Healthy

Introduction: Heterogeneity in sickle cell anaemia manifestations ranges from near asymptomatic cases to severe illness.  Objective: This study determined the relationship between foetal haemoglobin F level, other haematological parameters and acute painful episode score of sickle cell disease patients in FCT Abuja Nigeria. Methods: 60 Sickle cell patients were selected for the study. 20 severe crises, 20 non-severe crisis SS were enrolled in the study. Control group comprised 20 apparently healthy haemoglobin AA individuals. Data were analysed descriptively. Results: Hb F level increased significantly in non-severe crisis sickle cell anaemia (7.12%± 3.6) and severe crisis (5.30%±2.3) groups, compared to the control group (0.32±1.8). This trend was also observed in RDW, MCHC and MCV. The mean Hb concentration and haematocrit (Hct) were significantly lower for both non- severe crisis and severe crisis SCA groups. There was no significant correlation between HbF and any of the haematological parameters in both non severe crisis and severe crisis groups. Patients with SCA had higher levels of HbF than matched controls. HbF had no correlation with any of the haematological parameters in both severe and non-severe SCA groups studied. Conclusion: Further studies should focus on environmental factors contributing to this variability.

scholarly journals PSS58 A SCORING SYSTEM FOR QUALITY OF CARE EVALUATION. A COMMUNITY-BASED STUDY OF CHRONIC LEG ULCERS IN NORTH GERMANY

2008 ◽  
Vol 11 (6) ◽  
pp. A627
Author(s):  
M Augustin ◽  
L Grams ◽  
K Herberger ◽  
N Franzke ◽  
S Debus ◽  
...  
Keyword(s):  
Quality Of Care ◽  
Scoring System ◽  
Leg Ulcers ◽  
Community Based ◽  
North Germany ◽  
Care Evaluation ◽  
Chronic Leg Ulcers

scholarly journals Haematological indices of sickle cell patients with chronic leg ulcers on compression therapy

2020 ◽  
Vol 9 (1) ◽  
Author(s):  
Oluwatoyin A. Babalola ◽  
Ayodele Ogunkeyede ◽  
Abayomi B. Odetunde ◽  
Foluke Fasola ◽  
Anthony A. Oni ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Compression Therapy ◽  
Leg Ulcers ◽  
Haematological Indices ◽  
Chronic Leg Ulcers

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

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.

scholarly journals The Iron Status of Sickle Cell Anaemia Patients in Ilorin, North Central Nigeria

2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Musa A. Sani ◽  
James O. Adewuyi ◽  
Abiola S. Babatunde ◽  
Hannah O. Olawumi ◽  
Rasaki O. Shittu
Keyword(s):  
Sickle Cell ◽  
Serum Ferritin ◽  
Transferrin Receptor ◽  
Sickle Cell Anaemia ◽  
Iron Status ◽  
Serum Transferrin ◽  
Cross Sectional ◽  
Serum Transferrin Receptor ◽  
Iron Deficient ◽  
The Mean

Objectives. Sickle cell anaemia (SCA) is one of the commonest genetic disorders in the world. It is characterized by anaemia, periodic attacks of thrombotic pain, and chronic systemic organ damage. Recent studies have suggested that individuals with SCA especially from developing countries are more likely to be iron deficient rather than have iron overload. The study aims to determine the iron status of SCA patients in Ilorin, Nigeria.Methods. A cross-sectional study of 45 SCA patients in steady state and 45 non-SCA controls was undertaken. FBC, blood film, sFC, sTfR, and sTfR/log sFC index were done on all subjects.Results. The mean patients’ serum ferritin (589.33 ± 427.61 ng/mL) was significantly higher than the mean serum ferritin of the controls (184.53 ± 119.74 ng/mL). The mean serum transferrin receptor of the patients (4.24 ± 0.17 μg/mL) was higher than that of the controls (3.96 ± 0.17 μg/mL) (p=0.290). The mean serum transferrin receptor (sTfR)/log serum ferritin index of the patients (1.65 ± 0.27 μg/mL) was significantly lower than that of the control (1.82 ± 0.18 μg/mL) (p=0.031).Conclusion. Iron deficiency is uncommon in SCA patients and periodic monitoring of the haematological, biochemical, and clinical features for iron status in SCA patients is advised.

Transfusional Iron Overload In An Adult Sickle Cell Disease Population: Epidemiology, Prevalence, and Management

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 1005-1005 ◽  
Author(s):  
James Son ◽  
Hongyan Xu ◽  
Nadine J Barrett ◽  
Leigh G Wells ◽  
Latanya Bowman ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Iron Overload ◽  
Sickle Cell ◽  
Serum Ferritin ◽  
Chelation Therapy ◽  
Organ Damage ◽  
Morbidity And Mortality ◽  
Genotype Distribution ◽  
Cell Disease ◽  
The Mean

Abstract Transfusional iron (Fe) overload remains a significant problem among patients with chronic, transfusion dependent anemias, especially in transfusion dependent ß-thalassemia (Thal) syndromes. If not treated vigorously with chelation, Fe overload in Thal is associated with significant organ damage, especially with chronic liver disease and cardiac abnormalities which can contribute to morbidity and mortality. In recent decades, the significance of Fe overload in sickle cell disease (SCD) has also been recognized especially among pediatric patients on chronic transfusion regimens predominantly for primary and secondary prevention of stroke. The prevalence and significance of this problem among adult SCD patients is less clear, although it is widely believed that episodic, mostly unnecessary transfusion practices play a more prominent role in this patient population. There have been reports of an association between iron overload and increased morbidity and mortality among adult SCD patients; it has also been speculated that the chronic inflammatory state that exists in SCD affords some degree of protection against severe organ damage through upregulation of hepcidin and sequestration of Fe in these patients. We performed a retrospective review of 635 adult SCD patients followed at our Center to define and ascertain the epidemiology, prevalence, etiology, and clinical correlates of transfusional Fe overload. Fe overload was defined as two consecutive serum ferritin values of > 1000 ng/ml. 80 patients (12.6%) met this criterion. Of these, 38 were male and 42 were female. Genotype distribution was: 73 SS, 3 S-β+ thal, 2 S-β0 thal and 2 SC. The mean age was 35.9 (range 18-69). Out of the 80 patients with transfusional Fe overload, 24 (30%) were/had been on a chronic transfusion regimen (23 for secondary or primary stroke prevention and one for childhood cardiomyopathy). Seventy percent of the patients (n=56) developed Fe overload from episodic transfusions predominantly performed at outlying community hospitals. The mean highest ferritin value was 4991 ng/ml (range 1,052-16,500). There was no correlation between ferritin levels and the number of hospitalizations or painful episodes (p=0.9). Thirty seven patients (46.2%) had a history of chelation therapy (with desferoxamine, deferasirox, or both). In 25 patients who have been on deferasirox for a period of 6 months or more, serum ferritin levels decreased from 4452.3 to 3876.6 ng/ml (p=0.3239). Our retrospective study shows that transfusional Fe overload is not rare among adults with SCD and develops predominantly as a result of episodic blood transfusions. This underscores the importance of the development and dissemination of evidence based guidelines, especially for episodic transfusions in SCD. A careful study of the extent and degree of organ damage associated with transfusional Fe overload in SCD and why less than half (46.2%) of patients are exposed to chelation therapy needs to be done. These studies should include liver iron concentration (LIC), cardiac iron and liver histology, when indicated, in parallel with serum hepcidin levels. The fact that the reduction in serum ferritin levels with deferasirox did not reach statistical significance in this cohort can be explained by the relatively small number of patients as well as by the short period (6 months) of exposure to chelation therapy. Disclosures: No relevant conflicts of interest to declare.

Topical Sodium Nitrite Is Effective In Reducing Leg Ulcer-Associated Pain In Patients With Sickle Cell Disease

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 2236-2236
Author(s):  
Caterina P. Minniti ◽  
Caroline Cantilena ◽  
Dihua Xu ◽  
Anna K. Conrey ◽  
Marlene Peters-Lawrence ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Sodium Nitrite ◽  
Opioid Analgesic ◽  
Leg Ulcers ◽  
Cell Disease ◽  
Before And After ◽  
Vas Scores ◽  
Dose Dependent ◽  
Chronic Leg Ulcers ◽  
Analgesic Use

Abstract Background Chronic leg ulcers are a debilitating complication that affects individuals with SCD 10 times more frequently and at a much younger age than the general population. The underlying mechanism is not completely understood, and current therapies are not very effective. Severe pain at the ulcer site is often a greater concern to the patients than the status of the ulcer itself and a limiting factor in performing daily tasks and caring for the ulcer. We have previously reported the safety and tolerability of a Phase 1 study of topical sodium nitrite in patients with sickle cell disease and chronic leg ulcer in which escalating concentrations of sodium nitrite cream were applied twice a week to one ulcer per patient, for four weeks. We investigated pain and physical functioning in adults enrolled in this study. We hypothesized that there would be a significant reduction in the amount of pain at the ulcer site during the course of treatment sodium nitrite. In the subgroup of patients with more than one ulcer, we hypothesized that pain would be unchanged in the ulcers not treated with sodium nitrite. Patients and Methods Adult patients with sickle cell anemia consented to an IRB-approved FDA investigational New Drug protocol at the NIH Clinical Center, and completed a Brief Pain Inventory (BPI) before and after completion of therapy. Patients were asked to identify in detail the areas that were contributing to their overall pain status. Patients completed a 7-day opioid diary in which they were to record all medications taken, and frequency of consumption the week before and the week after completing the trial. In addition a Visual Analog pain Scale (VAS) score was obtained prospectively at each of seven patient encounters during the study for the study ulcer and each of the non study ulcers. A complete opioid usage history was collected before and after the four weeks of therapy to identify changes in opoid analgesic usage. Ulcer size was measured each time. Student T tests and one way ANOVA with Bonferroni correction were used for data analysis. Results Eighteen subjects (8 men and 10 women, ages 20 to 59 years); with active chronic leg ulcers were enrolled. Chronic daily opioid use occurred in 89% (16/18) and was attributed to localized pain at the ulcer site in 15 of them. Ulcers had been present for a median of 10 months (range 2 to 300 months); half of the patients had more than one ulcer. Baseline BPI pain severity and interference scores correlated with C reactive protein (CRP)(p=0.01), but not with ulcer age size, hydroxyurea use or transfusions, and % S. BPI scores and VAS scores of the treated ulcer improved significantly after use of topical sodium nitrite(p<0.005 and <001 respectively). In the subgroup that had more than one ulcer, VAS scores for non treated ulcers did not change significantly. Opioid analgesic use trended toward a decrease over time, but was not statistically significant for the entire group (392 mg before vs. 110 mg morphine equivalents after). Opioid analgesic use declined in 9 of 16 subjects, 3/16 increased (one had a new ulcer the last week of study, which was the source of the pain), six did not change. Ulcer size decreased after application of sodium nitrite, in a dose dependent manner, p=0.001. Changes in pain correlated with changes in size, and were dose dependent. Conclusions Patients with SCD and chronic ulcer experience significant pain at the ulcer site, which requires systemic narcotic therapy in most of them. Inflammation seems to be the main determinant of pain, not size or age of the ulcer or use of transfusions or hydroxyurea. Therapies that target ulcers, such as sodium nitrite, may reduce systemic opioid analgesic use and facilitate ulcer manipulation and care. Sodium nitrite may affect pain via mechanisms and at a dose that is independent from its healing properties. Disclosures: No relevant conflicts of interest to declare.

Effects of Autologous Keratinocyte Transplantation on the Quality of Life of Patients with Severe Chronic Leg Ulcers

2001 ◽  
Vol 2 (2) ◽  
pp. 73-76 ◽  
Author(s):  
M. Augustin ◽  
T. Ermut ◽  
S. Johnsen ◽  
E. Schöpf ◽  
W. Vanscheidt ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Leg Ulcers ◽  
Chronic Leg Ulcers
Sign in / Sign up

Export Citation Format

Share Document