Clinical and laboratory features of sickle cell disease s/d punjab: impact of hbf and hydroxyurea
Conclusion: SD Punjab is the third most common SCD genotype in Oman and was associated with recurrent VOC, ACS, AVN, and gall stones comparable to other SCD genotypes. Patients with> 3 VOC/year had significantly increased incidence of Stroke, AVN, and gallstones. However, HU was not associated with improved prognosis and better survival in this cohort of patients.Keywords: Sickle Cell Disease; Haemoglobin SD Punjab; vaso-occlusive crises; acute chest syndrome; Avascular Necrosis; HbF; hydroxyurea. (Source: Mediterranean Journal of Hematology and Infectious Diseases)
Source: Mediterranean Journal of Hematology and Infectious Diseases - April 30, 2024 Category: Hematology Authors: r. Salam Al-Kindi, Isra Al-Busaidi, Anil Pathare Source Type: research
Prognostic value of exercise longitudinal right ventricular free wall strain in patients with sickle cell disease
AbstractLongitudinal right ventricular free wall strain (RVFWS) has been identified as an independent prognostic marker in patients with pulmonary hypertension. Little is known however about the prognostic value of RVFWS in patients with sickle cell (SC) disease, particularly during exercise. We therefore examined the prognostic significance of RVFWS both at rest and with exercise in patients with SC disease and normal resting systolic pulmonary artery pressure (SPAP). Consecutive patients with SC disease referred for bicycle ergometer stress echocardiography (SE) were enrolled ftom July 2019 to January 2021. All patients ...
Source: The International Journal of Cardiovascular Imaging - April 30, 2024 Category: Radiology Source Type: research
Hemoglobin scavenger receptor CD163 as a potential biomarker of hemolysis induced hepatobiliary injury in sickle cell disease
Am J Physiol Cell Physiol. 2024 Apr 29. doi: 10.1152/ajpcell.00386.2023. Online ahead of print.ABSTRACTSickle cell disease (SCD) associated chronic hemolysis promotes oxidative stress, inflammation and thrombosis leading to organ damage, including liver damage. Hemoglobin scavenger receptor CD163 plays a protective role in SCD by scavenging both hemoglobin-haptoglobin complexes and cell free hemoglobin. A limited number of studies in the past have shown a positive correlation of CD163 expression with poor disease outcomes in patients with SCD. However, the role and regulation of CD163 in SCD related hepatobiliary injury ha...
Source: American Journal of Physiology. Cell Physiology - April 29, 2024 Category: Cytology Authors: Tomasz W Kaminski Ayynar Sivanantham Anna Mozhenkova Ashley Smith Ramakrishna Ungalara Rikesh Dubey Bibhav Shrestha Corinne Hanway Omika Katoch Jes ús Tejero Prithu Sundd Enrico M Novelli Gregory J Kato Tirthadipa Pradhan-Sundd Source Type: research
Hematopoietic Stem Cell Transplantation in Sickle Cell Disease: A Multidimentional Review
Cell Transplant. 2024 Jan-Dec;33:9636897241246351. doi: 10.1177/09636897241246351.ABSTRACTWhile exagamglogene autotemcel (Casgevy) and lovotibeglogene autotemcel (Lyfgenia) have been approved by the US Food and Drug Administration (FDA) as the first cell-based gene therapies for the treatment of patients 12 years of age and older with sickle cell disease (SCD), this treatment is not universally accessible. Allogeneic hematopoietic stem cell transplant (HSCT) has the potential to eradicate the symptoms of patients with SCD, but a significant obstacle in HSCT for SCD is the availability of suitable donors, particularly human...
Source: Cell Transplantation - April 29, 2024 Category: Cytology Authors: Tahereh Rostami Soroush Rad Mohammad Reza Rostami Seied Amirhossein Mirhosseini Hediyeh Alemi Naghmeh Khavandgar Ghasem Janbabai Azadeh Kiumarsi Amir Kasaeian Seied Asadollah Mousavi Source Type: research
Hemoglobin scavenger receptor CD163 as a potential biomarker of hemolysis induced hepatobiliary injury in sickle cell disease
Am J Physiol Cell Physiol. 2024 Apr 29. doi: 10.1152/ajpcell.00386.2023. Online ahead of print.ABSTRACTSickle cell disease (SCD) associated chronic hemolysis promotes oxidative stress, inflammation and thrombosis leading to organ damage, including liver damage. Hemoglobin scavenger receptor CD163 plays a protective role in SCD by scavenging both hemoglobin-haptoglobin complexes and cell free hemoglobin. A limited number of studies in the past have shown a positive correlation of CD163 expression with poor disease outcomes in patients with SCD. However, the role and regulation of CD163 in SCD related hepatobiliary injury ha...
Source: American Journal of Physiology. Cell Physiology - April 29, 2024 Category: Cytology Authors: Tomasz W Kaminski Ayynar Sivanantham Anna Mozhenkova Ashley Smith Ramakrishna Ungalara Rikesh Dubey Bibhav Shrestha Corinne Hanway Omika Katoch Jes ús Tejero Prithu Sundd Enrico M Novelli Gregory J Kato Tirthadipa Pradhan-Sundd Source Type: research
Hemoglobin scavenger receptor CD163 as a potential biomarker of hemolysis induced hepatobiliary injury in sickle cell disease
Am J Physiol Cell Physiol. 2024 Apr 29. doi: 10.1152/ajpcell.00386.2023. Online ahead of print.ABSTRACTSickle cell disease (SCD) associated chronic hemolysis promotes oxidative stress, inflammation and thrombosis leading to organ damage, including liver damage. Hemoglobin scavenger receptor CD163 plays a protective role in SCD by scavenging both hemoglobin-haptoglobin complexes and cell free hemoglobin. A limited number of studies in the past have shown a positive correlation of CD163 expression with poor disease outcomes in patients with SCD. However, the role and regulation of CD163 in SCD related hepatobiliary injury ha...
Source: Am J Physiol Cell Ph... - April 29, 2024 Category: Cytology Authors: Tomasz W Kaminski Ayynar Sivanantham Anna Mozhenkova Ashley Smith Ramakrishna Ungalara Rikesh Dubey Bibhav Shrestha Corinne Hanway Omika Katoch Jes ús Tejero Prithu Sundd Enrico M Novelli Gregory J Kato Tirthadipa Pradhan-Sundd Source Type: research
Hematopoietic Stem Cell Transplantation in Sickle Cell Disease: A Multidimentional Review
Cell Transplant. 2024 Jan-Dec;33:9636897241246351. doi: 10.1177/09636897241246351.ABSTRACTWhile exagamglogene autotemcel (Casgevy) and lovotibeglogene autotemcel (Lyfgenia) have been approved by the US Food and Drug Administration (FDA) as the first cell-based gene therapies for the treatment of patients 12 years of age and older with sickle cell disease (SCD), this treatment is not universally accessible. Allogeneic hematopoietic stem cell transplant (HSCT) has the potential to eradicate the symptoms of patients with SCD, but a significant obstacle in HSCT for SCD is the availability of suitable donors, particularly human...
Source: Cell Transplantation - April 29, 2024 Category: Cytology Authors: Tahereh Rostami Soroush Rad Mohammad Reza Rostami Seied Amirhossein Mirhosseini Hediyeh Alemi Naghmeh Khavandgar Ghasem Janbabai Azadeh Kiumarsi Amir Kasaeian Seied Asadollah Mousavi Source Type: research
Hemoglobin scavenger receptor CD163 as a potential biomarker of hemolysis induced hepatobiliary injury in sickle cell disease
Am J Physiol Cell Physiol. 2024 Apr 29. doi: 10.1152/ajpcell.00386.2023. Online ahead of print.ABSTRACTSickle cell disease (SCD) associated chronic hemolysis promotes oxidative stress, inflammation and thrombosis leading to organ damage, including liver damage. Hemoglobin scavenger receptor CD163 plays a protective role in SCD by scavenging both hemoglobin-haptoglobin complexes and cell free hemoglobin. A limited number of studies in the past have shown a positive correlation of CD163 expression with poor disease outcomes in patients with SCD. However, the role and regulation of CD163 in SCD related hepatobiliary injury ha...
Source: Am J Physiol Cell Ph... - April 29, 2024 Category: Cytology Authors: Tomasz W Kaminski Ayynar Sivanantham Anna Mozhenkova Ashley Smith Ramakrishna Ungalara Rikesh Dubey Bibhav Shrestha Corinne Hanway Omika Katoch Jes ús Tejero Prithu Sundd Enrico M Novelli Gregory J Kato Tirthadipa Pradhan-Sundd Source Type: research
Fludarabine-Based Reduced-Intensity Conditioning Regimen for Hematopoietic Stem Cell Transplantation in a Pediatric Patient with Sickle Cell Disease: A Case Report
Int J Hematol Oncol Stem Cell Res. 2024 Jan 1;18(1):104-109. doi: 10.18502/ijhoscr.v18i1.14750.ABSTRACTReduced-intensity conditioning (RIC) regimens have the potential to decrease toxicities related to hematopoietic stem cell transplantation (HCT) in patients with sickle cell disease (SCD). While initial results may have been acceptable in adults and young adults, there are no well-established strategies in children with SCD. Here, it is described the clinical course of two children with symptomatic SCD who have successfully undergone HSCT using Fludarabin-based conditioning.PMID:38680715 | PMC:PMC11055425 | DOI:10.18502/i...
Source: Cell Research - April 29, 2024 Category: Cytology Authors: Natalia Builes Source Type: research
Hemoglobin scavenger receptor CD163 as a potential biomarker of hemolysis induced hepatobiliary injury in sickle cell disease
Am J Physiol Cell Physiol. 2024 Apr 29. doi: 10.1152/ajpcell.00386.2023. Online ahead of print.ABSTRACTSickle cell disease (SCD) associated chronic hemolysis promotes oxidative stress, inflammation and thrombosis leading to organ damage, including liver damage. Hemoglobin scavenger receptor CD163 plays a protective role in SCD by scavenging both hemoglobin-haptoglobin complexes and cell free hemoglobin. A limited number of studies in the past have shown a positive correlation of CD163 expression with poor disease outcomes in patients with SCD. However, the role and regulation of CD163 in SCD related hepatobiliary injury ha...
Source: American Journal of Physiology. Cell Physiology - April 29, 2024 Category: Cytology Authors: Tomasz W Kaminski Ayynar Sivanantham Anna Mozhenkova Ashley Smith Ramakrishna Ungalara Rikesh Dubey Bibhav Shrestha Corinne Hanway Omika Katoch Jes ús Tejero Prithu Sundd Enrico M Novelli Gregory J Kato Tirthadipa Pradhan-Sundd Source Type: research
Hemoglobin scavenger receptor CD163 as a potential biomarker of hemolysis induced hepatobiliary injury in sickle cell disease
Am J Physiol Cell Physiol. 2024 Apr 29. doi: 10.1152/ajpcell.00386.2023. Online ahead of print.ABSTRACTSickle cell disease (SCD) associated chronic hemolysis promotes oxidative stress, inflammation and thrombosis leading to organ damage, including liver damage. Hemoglobin scavenger receptor CD163 plays a protective role in SCD by scavenging both hemoglobin-haptoglobin complexes and cell free hemoglobin. A limited number of studies in the past have shown a positive correlation of CD163 expression with poor disease outcomes in patients with SCD. However, the role and regulation of CD163 in SCD related hepatobiliary injury ha...
Source: Am J Physiol Cell Ph... - April 29, 2024 Category: Cytology Authors: Tomasz W Kaminski Ayynar Sivanantham Anna Mozhenkova Ashley Smith Ramakrishna Ungalara Rikesh Dubey Bibhav Shrestha Corinne Hanway Omika Katoch Jes ús Tejero Prithu Sundd Enrico M Novelli Gregory J Kato Tirthadipa Pradhan-Sundd Source Type: research
Hematopoietic Stem Cell Transplantation in Sickle Cell Disease: A Multidimentional Review
Cell Transplant. 2024 Jan-Dec;33:9636897241246351. doi: 10.1177/09636897241246351.ABSTRACTWhile exagamglogene autotemcel (Casgevy) and lovotibeglogene autotemcel (Lyfgenia) have been approved by the US Food and Drug Administration (FDA) as the first cell-based gene therapies for the treatment of patients 12 years of age and older with sickle cell disease (SCD), this treatment is not universally accessible. Allogeneic hematopoietic stem cell transplant (HSCT) has the potential to eradicate the symptoms of patients with SCD, but a significant obstacle in HSCT for SCD is the availability of suitable donors, particularly human...
Source: Cell Transplantation - April 29, 2024 Category: Cytology Authors: Tahereh Rostami Soroush Rad Mohammad Reza Rostami Seied Amirhossein Mirhosseini Hediyeh Alemi Naghmeh Khavandgar Ghasem Janbabai Azadeh Kiumarsi Amir Kasaeian Seied Asadollah Mousavi Source Type: research
Cost-Effectiveness of Lovotibeglogene Autotemcel (Lovo-Cel) Gene Therapy for Patients with Sickle Cell Disease and Recurrent Vaso-Occlusive Events in the United States
ConclusionsOur analysis of lovo-cel gene therapy compared with common care for patients in the US with SCD with recurrent VOEs estimated meaningful improvements in survival, quality of life, and other clinical outcomes accompanied by increased overall costs for the health care system and for broader society. The predicted economic value of lovo-cel gene therapy was influenced by uncertainty in long-term clinical effects and by positive spillover effects on patient productivity and caregiver burden. (Source: PharmacoEconomics)
Source: PharmacoEconomics - April 29, 2024 Category: Health Management Source Type: research
Blood count changes in malaria patients according to blood groups (ABO/Rh) and sickle cell trait
Introduction: Malaria continues to be the leading cause of hospitalization and death in Angola, a country in sub- Saharan Africa. In 2023, in the first quarter, 2,744,682 cases were registered, and of these 2,... (Source: Malaria Journal)
Source: Malaria Journal - April 29, 2024 Category: Infectious Diseases Authors: Euclides N. M. Sacomboio, Santo D. Zua, Adelino T. Tchivango, Ant ónio D. Pululu, Adilson C. D. Caumba, Adelina B. M. Paciência, Danilson V. Sati, Sabina G. Agostinho, Yolanda S. Agostinho, Fernando G. Mazanga, Neusa B. Ntambo, Cruz S. Sebastião, Joana Tags: Research Source Type: research
Epigenetic Aging Associations With Psychoneurological Symptoms and Social Functioning in Adults With Sickle Cell Disease
Conclusion: Epigenetic age acceleration was associated with worse symptom experiences, suggesting the potential for epigenetic age acceleration as a biomarker to aid in risk stratification or targets for intervention to mitigate symptom burden in SCD.PMID:38679469 | DOI:10.1177/10998004241250322 (Source: Cell Research)
Source: Cell Research - April 28, 2024 Category: Cytology Authors: Mitchell R Knisely Rita V Masese Joacy G Mathias Qing Yang Daniel Hatch Brandon M L ê Faith Luyster Melanie E Garrett Paula J Tanabe Nirmish R Shah Allison Ashley-Koch Source Type: research
