Acute painful crises may be causes of sudden deaths in sickle cell diseases (2025)

An exaggerated capillary endothelial edema may be the cause of sudden deaths in sickle cell diseases

World Family Medicine Journal /Middle East Journal of Family Medicine, 2023

Background: Sickle cell diseases (SCDs) are inborn and severe inflammatory processes on vascular endothelium, particularly at the capillaries which are the actual distributors of the sickled or just hardened red blood cells (RBCs) into the tissues. Methods: All patients of the SCDs were included. Results: We studied 222 males and 212 females with similar ages (30.8 vs 30.3 years, p>0.05, respectively). Disseminated teeth losses (5.4% vs 1.4%, p<0.001), ileus (7.2% vs 1.4%, p<0.001), cirrhosis (8.1% vs 1.8%, p<0.001), leg ulcers (19.8% vs 7.0%, p<0.001), digital clubbing (14.8% vs 6.6%, p<0.001), coronary heart disease (18.0% vs 13.2%, p<0.05), chronic renal disease (9.9% vs 6.1%, p<0.05), chronic obstructive pulmonary disease (25.2% vs 7.0%, p<0.001), and stroke (12.1% vs 7.5%, p<0.05) were higher in males but not acute chest syndrome (2.7% vs 3.7%), pulmonary hypertension (12.6% vs 11.7), deep venous thrombosis and/or varices and/or telangiectasias (9.0% vs 6.6%), or mean age of mortality (30.2 vs 33.3 years) (p>0.05 for all). The sickled or just hardened RBCsinduced capillary endothelial damage, inflammation, edema, and fibrosis are initiated at birth, and terminate with disseminated tissue hypoxia, multiorgan failures, and sudden deaths even at childhood. Although RBCs suspensions and corticosteroids in acute and aspirin plus hydroxyurea in acute and chronic phases decrease severity of the destructive process, survivals are still shortened in both genders, dramatically. Infections, medical or surgical emergencies, or emotional stresses-induced increased basal metabolic rate aggravates the sickling and capillary endothelial edema, and may terminate with multiorgan failures-induced sudden deaths in the SCDs.

August 2021 - Volume 15, Issue 3 AUTOSPLENECTOMY MAY NOT HAVE AN ATHEROSCLEROTIC BACKGROUND IN SICKLE CELL DISEASES

Middle East Journal of Nursing, 2021

Background: We tried to understand whether or not there is a significant relationship between autosplenectomy and atherosclerosis in sickle cell diseases (SCD). Methods: All patients with the SCD were included. Results: The study included 434 patients (222 males and 212 females) with similar mean ages in male and female genders (30.8 versus 30.3 years, respectively, p&gt;0.05). Smoking (23.8% versus 6.1%, p&lt;0.001) and alcohol (4.9% versus 0.4%, p&lt;0.001) were higher in males, significantly. Transfused units of red blood cells (RBC) in their lives (48.1 versus 28.5, p=0.000) were also higher in males, significantly. Similarly, disseminated teeth losses (&lt;20 teeth present) (5.4% versus 1.4%, p&lt;0.001), chronic obstructive pulmonary disease (COPD) (25.2% versus 7.0%, p&lt;0.001), ileus (7.2% versus 1.4%, p&lt;0.001), cirrhosis (8.1% versus 1.8%, p&lt;0.001), leg ulcers (19.8% versus 7.0%, p&lt;0.001), digital clubbing (14.8% versus 6.6%, p&lt;0.001), coronary heart disease (C...

The impact of Hydroxyurea on the rates of Vaso–occlusive crises in patients with sickle cell disease in Saudi Arabia: a single–center study

BMC Emergency Medicine, 2022

Background: Vaso-occlusive crises (VOCs) are acute and common painful complication of sickle cell disease (SCD), and are the main reason behind the frequent emergency department visits among SCD patients. Hydroxyurea (HU) is an old and commonly used medication that demonstrated its effectiveness in reducing the risk of VOCs and the incidence of hospitalization. Although multiple studies have examined the impact of HU on the rates of VOCs, few have explored its effectiveness among SCD patients in Saudi Arabia. This was a single-center retrospective cohort study in which the electronic medical records of patients with SCD who have not had any previous exposure to HU prior to the initiation of HU treatment for ≥12 months were recruited. Paired t-test was conducted to examine the difference in the rates of VOCs, and levels of hemoglobin (Hgb), hematocrit (HCT), and platelet counts (PLT Ct) prior to the initiation of HU therapy and 12 months later. Multiple linear regression was conducted to examine whether age, gender, use of opioid analgesics, Hgb, HCT, and PLT Ct levels predict higher or lower rates of VOCs. Results: One hundred and fifty-six patients met the inclusion criteria and were included in the analysis. About 51% of the patients were males, and their mean age was 12.69 years. The mean HU dosage was 16.52 mg/kg/day, and the mean reduction in the rate of VOCs was 1.36 events per patient per year (95% CI [1.03-1.70], p < 0.0001) after the initiation of HU. Females were more likely to have greater reduction in the rates of VOCs in comparison to their male counterparts (β-estimate = 12.85, 95% ], p = 0.0374). The use of HU results in a significant reduction in the rates of VOCs and emergency department visits. Future studies with robust research designs should be conducted to further examine the impact of HU on VOCs, hospitalization, and length of stay as well as compare HU to other newly approved medications for SCD, such as crizanlizumab.

Pathophysiology of pulmonary hypertension in sickle cell diseases

2018

Background: Pulmonary hypertension (PHT) is common in sickle cell diseases (SCDs). Methods: All patients with SCDs were included. Results: The study included 434 patients (212 females) with similar mean ages in males and females (30.8 versus 30.3 years, respectively, p&gt;0.05). Smoking (23.8% versus 6.1%) and alcohol (4.9% versus 0.4%) were higher in males (p&lt;0.001 for both). Disseminated teeth losses (&lt;20 teeth present) (5.4% versus 1.4%, p&lt;0.001), transfused units of red blood cell (RBC) (48.1 versus 28.5, p=0.000), chronic obstructive pulmonary disease (COPD) (25.2% versus 7.0%, p&lt;0.001), ileus (7.2% versus 1.4%, p&lt;0.001), cirrhosis (8.1% versus 1.8%, p&lt;0.001), leg ulcers (19.8% versus 7.0%, p&lt;0.001), digital clubbing (14.8% versus 6.6%, p&lt;0.001), coronary heart disease (CHD) (18.0% versus 13.2%, p&lt;0.05), chronic renal disease (CRD) (9.9% versus 6.1%, p&lt;0.05), and stroke (12.1% versus 7.5%, p&lt;0.05) were also higher in males. There were 31 mortali...

Atherosclerotic Background of Digital Clubbing in Sickle Cell Diseases

Annals of Clinical and Medical Case Reports, 2023

We studied 222 males and 212 females with similar mean ages (30.8 vs 30.3 years, p>0.05, respectively). Beside digital clubbing (14.8% vs 6.6%, p<0.001), smoking (23.8% vs 6.1%, p<0.001), alcohol (4.9% vs 0.4%, p<0.001), transfused red blood cells (RBCs) in their lives (48.1 vs 28.5 units, p=0.000), disseminated teeth losses (5.4% vs 1.4%, p<0.001), chronic obstructive pulmonary disease (COPD) (25.2% vs 7.0%, p<0.001), ileus (7.2% vs 1.4%, p<0.001), cirrhosis (8.1% vs 1.8%, p<0.001), leg ulcers (19.8% vs 7.0%, p<0.001), coronary heart disease (CHD) (18.0% vs 13.2%, p<0.05), chronic renal disease (CRD) (9.9% vs 6.1%, p<0.05), and stroke (12.1% vs 7.5%, p<0.05) were all higher, and autosplenectomy (50.4% vs 53.3%, p<0.05) and mean age of mortality were lower in males, significantly (30.2 vs 33.3 years, p<0.05)

Microvascular oxygen consumption during sickle cell pain crisis

Blood, 2014

Ticagrelor does not impact patient-reported pain in young adults with sickle cell disease: a multicentre, randomised phase IIb study

British Journal of Haematology

Ticagrelor is an antiplatelet agent for adults with coronary artery disease. The inhibition of platelet activation may decrease the frequency of vaso-occlusion crisis (VOC) in sickle cell disease (SCD). The HESTIA2 study (NCT02482298) randomised 87 adults with SCD (aged 18-30 years) 1:1:1 to twicedaily ticagrelor 10, 45 mg or placebo for 12 weeks. Numerical decreases from baseline in mean proportion of days with patient-reported pain (primary endpoint) were seen in all three groups, as well as in pain intensity and analgesic use, with no significant differences between placebo and ticagrelor treatment groups. Plasma ticagrelor concentrations and platelet inhibition increased with dose. Adverse events were distributed evenly across groups and two non-major bleeding events occurred per group. Ticagrelor was well tolerated with a low bleeding risk, but no effect on diary-reported pain was detected. Potential effects on frequency of VOCs will need to be evaluated in a larger and longer study.

Kaya MN, İlhan G. Evaluation of the Effect of Mortality, Life Expectancy, and Treatment Modalities of Sickle Cell Patients on Mortality. Erciyes Med J 2021; 43(3): 273–7.

Erciyes Medical Journal, 2021

Objective: We aimed to contribute to the literature by investigating the causes of mortality, average life expectancies, and the clinical features that occur in this process, in relation to sickle cell disease (SCD), and by comparing the results obtained with other similar clinical studies. Materials and Methods: This study was designed as a monocentric, cross-sectional, and retrospective study. The patient files were reviewed in terms of the age, use of hydroxyurea, use of chelators, exchange transfusion history, surgical operation history, the annual frequency of painful crises, the annual hospitalization frequency, and the annual frequency of follow-up visits which the patients have attended, the complications experienced by the patients, and the causes of mortality. Results: Acute chest syndrome was the most prevalent cause of death of the patients included in our study. No significant difference was found between the premature death and late death groups, that is, the groups that we have determined on the basis of the SCD patients ages of death, in terms of use of hydroxyurea, use of iron chelator, and use of exchange transfusion depending on the disease. Conclusion: Based on our findings, acute chest syndrome was the primary cause of death in SCD patients we have studied, followed by pulmonary embolism. Use of hydroxyurea, use of iron chelator, exchange transfusion history, and surgical operation history due to SCD were not found to be significantly effective when the mean age of death reported in the literature was taken as the base value.

Circumstances of death in adult sickle cell disease patients

American Journal of Hematology, 2006

The purpose of the study was to analyze clinical and/or autopsy findings at the time of death among adults with sickle cell disease (SCD) at Howard University in Washington, DC over a 25-year period. A single physician recorded circumstances of death among 141 adult SCD patients he treated and knew well from 1976 to 2001. These findings were determined by autopsy report and/or clinical assessment. In a subset of 31 patients, autopsy records were reviewed for reports of iron deposition in liver and heart and of organ pathology. One hundred and fourteen (80.9%) of the patients had SS phenotype and 66 (46.8%) were female. The mean ± SD age at death was 36 ± 11 years. Leading circumstances of death included pulmonary hypertension (PHT) (26.2%), sudden death (23.4%), renal failure (22.6%), infection (18.4%), thromboembolism (14.9%), cardiac diagnoses (12.0%), cirrhosis (11.3%), pneumonia or acute chest syndrome (9.9%), bleeding (7.8%), and iron overload (7.0%). When circumstances of deaths that occurred after 1991 (n = 69) were compared to those that occurred in 1991 or earlier (n = 72), PHT (36.2% vs. 16.6%; P < 0.01) was significantly more common in 1992 or later. Significant associations were found between PHT and thromboembolism and between cirrhosis and iron overload. In this proportional mortality study of adults with SCD, PHT was the leading finding at the time of death. Thromboembolism was associated with PHT, and iron overload was associated with cirrhosis. Am. J. Hematol., 2006. © 2006 Wiley-Liss, Inc.

Avascular Necrosis May Not Have an Atherosclerotic Background in Sickle Cell Diseases

Middle East Journal of Nursing, 2021

Background: We tried to understand whether or not there is a significant association between avascular necrosis (AVN) and atherosclerosis in sickle cell diseases (SCD). Methods: All patients with the SCD were included. Results: The study included 434 patients (212 females) with similar mean ages in male and female genders (30.8 versus 30.3 years, respectively, p&gt;0.05). Smoking (23.8% versus 6.1%, p&lt;0.001) and alcohol (4.9% versus 0.4%, p&lt;0.001) were higher in male gender, significantly. Transfused units of red blood cells (RBC) in their lives (48.1 versus 28.5, p=0.000) were also higher in male gender, significantly. Similarly, disseminated teeth losses (&lt;20 teeth present) (5.4% versus 1.4%, p&lt;0.001), chronic obstructive pulmonary disease (COPD) (25.2% versus 7.0%, p&lt;0.001), ileus (7.2% versus 1.4%, p&lt;0.001), cirrhosis (8.1% versus 1.8%, p&lt;0.001), leg ulcers (19.8% versus 7.0%, p&lt;0.001), digital clubbing (14.8% versus 6.6%, p&lt;0.001), coronary heart dise...