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Acute and Chronic Sickle Cell Disease: Causes, Consequences, and Treatment:...
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Acute and Chronic Sickle Cell Disease: Causes, Consequences, and Treatment: Volume II

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Hematology".

Deadline for manuscript submissions: closed (31 October 2023) | Viewed by 5903

Special Issue Editors

Prof. Dr. Pablo Bartolucci

E-Mail Website
Guest Editor
Sickle Cell Referral Centre, Department of Internal Medicine, Mondor Hospital, AP-HP, 94000 Creteil, France
Interests: leukemia; sickle cell disease; hematology
Special Issues, Collections and Topics in MDPI journals
Dr. Kamal Kant Sahu

E-Mail Website
Guest Editor
Department of Medicine, Saint Vincent Hospital, Worcester, MA, USA
Interests: leukemia; lymphoma; myeloma; benign hematology
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Sickle cell disease (SCD) is an extremely common hematological disorder. It affects millions of people worldwide, with a specific predilection for sub-Saharan Africa. SCD is characterized by unpredictable, acute, life-threatening episodes and chronic complications such as hemolytic anemia and end-organ damage, significant morbidity, poor quality of life, and early mortality. Recently, there has been an increase in sickle cell disease research. In the last decade, the mortality of patients with SCD has significantly reduced, especially in high-income countries. This is due to early diagnosis and increased awareness amongst the general population and physicians around the world. As survival has improved, it is important to focus now on the morbidity associated with SCD due to long-term complications. A number of international organizations and research bodies are studying various aspects of newer and advanced treatments for SCD. For instance, the Centers for Disease Control and Prevention (United States) have launched a Sickle Cell Data Collection (SCDC) program that gathers health information from multiple sources. With an exponential interest in the field of SCD, there has been significant development in understanding the pathobiological complexities of SCD and potential therapeutic options. This Special Issue aims to encourage and sensitize researchers, scientists, and young physicians to share their experience, ideas, and innovations through their excellent submissions on various topics related to SCD. We look forward to your contributions.

Prof. Dr. Pablo Bartolucci
Dr. Kamal Kant Sahu
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Journal of Clinical Medicine is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • sickle cell disease
  • hemoglobinopathy
  • anemia
  • African American
  • racial disparity

Published Papers (4 papers)

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Research

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8 pages, 560 KiB  
Article
Early Administration of Intravenous Hydration and Opioid Analgesics Is Correlated with Decreased Admission Rates during Vaso-Occlusive Episodes in Sickle Cell Disease
by Bowon Joung, Ethan Miles, Farris Al-Manaseer, Won Jin Jeon, Darren Wijaya, Jin Hyun Moon, Philip Han, Jae Lee, Akhil Mehta, Alan Tseng, Kaylin Ngo, Huynh Cao and Esther G. Chong
J. Clin. Med. 2024, 13(7), 1858; https://doi.org/10.3390/jcm13071858 - 23 Mar 2024
Viewed by 945
Abstract
Background: Painful vaso-occlusive episodes (VOEs) are the hallmark of sickle cell disease (SCD) and account for frequent visits to the emergency department (ED) or urgent care (UC). Currently, the early administration of analgesics is recommended as initial management; however, there is a need [...] Read more.
Background: Painful vaso-occlusive episodes (VOEs) are the hallmark of sickle cell disease (SCD) and account for frequent visits to the emergency department (ED) or urgent care (UC). Currently, the early administration of analgesics is recommended as initial management; however, there is a need for further understanding of the effect of prompt analgesics and hydration during VOEs. The objective of this study is to analyze the factors associated with the rate of hospital admission in the setting of time to intravenous (IV) analgesics and hydration. Method: This retrospective single-institution study reviewed adult and pediatric patients with SCD who presented with VOEs from January 2018 to August 2023. Results: Of 303 patient encounters, the rates of admission for the overall group, the subgroup which received IV hydration within 60 min of arrival, and the subgroup which received both IV analgesics and hydration within 60 min were 51.8%, 25.6% (RR = 0.46), and 18.2% (RR = 0.33), respectively. Further, factors such as gender and the use of hydroxyurea were found to be significantly associated with the rate of admission. Conclusions: This signifies the importance of standardizing the management of VOEs through the timely administration of IV analgesics and hydration in both adult and pediatric ED/UC. Full article
(This article belongs to the Special Issue Acute and Chronic Sickle Cell Disease: Causes, Consequences, and Treatment: Volume II)
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11 pages, 557 KiB  
Article
Predisposing Factors and Incidence of Venous Thromboembolism among Hospitalized Patients with Sickle Cell Disease
by Mohammed S. Ziyadah, Eman M. Mansory, Hatem M. Alahwal, Salem M. Bahashwan, Abdullah T. Almohammadi, Osman O. Radhwi, Talal Alghamdi, Shahida A. Khan, Majed N. Almashjary and Ahmed S. Barefah
J. Clin. Med. 2023, 12(20), 6498; https://doi.org/10.3390/jcm12206498 - 12 Oct 2023
Viewed by 946
Abstract
Though patients with sickle cell disease (SCD) are at risk of developing venous thromboembolism (VTE), clear estimates of its incidence and predisposing factors in hospitalized SCD patients are not available. Therefore, this issue was addressed to facilitate an early diagnosis and initiate appropriate [...] Read more.
Though patients with sickle cell disease (SCD) are at risk of developing venous thromboembolism (VTE), clear estimates of its incidence and predisposing factors in hospitalized SCD patients are not available. Therefore, this issue was addressed to facilitate an early diagnosis and initiate appropriate prophylactic and treatment strategies. A retrospective observational study was conducted on patients with SCD who were admitted to an academic center in Saudi Arabia over a 10-year period. We identified 1054 admissions of 394 patients with SCD. Of the 3% of patients identified with VTE, 50% experienced pulmonary embolism (PE), 34.3% exhibited deep vein thrombosis (DVT), 6.3% exhibited cerebral vein thrombosis, and 9.4% showed other forms of VTE. In pregnant SCD patients, 6.4% developed a VTE event during their hospital admission. Of the risk factors, high white blood cell count, length of stay, and presence of any additional risk factor for VTE was associated significantly with higher risk of VTE. In our study, this risk seems to be much lower, which is likely attributed to the use of VTE prophylactic strategies implemented in our center. Nevertheless, further studies are needed to establish the ideal prophylactic strategy in patients with SCD. Full article
(This article belongs to the Special Issue Acute and Chronic Sickle Cell Disease: Causes, Consequences, and Treatment: Volume II)
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Review

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12 pages, 1338 KiB  
Review
Hope on the Horizon: New and Future Therapies for Sickle Cell Disease
by Fahd A. Kuriri
J. Clin. Med. 2023, 12(17), 5692; https://doi.org/10.3390/jcm12175692 - 01 Sep 2023
Cited by 1 | Viewed by 2466
Abstract
This article provides an overview of conventional, new, and future treatment options for sickle cell disease (SCD), a genetic disorder affecting the production of hemoglobin. Current treatments include hydroxyurea, a conventional SCD treatment that increases the levels of fetal hemoglobin, and new treatments [...] Read more.
This article provides an overview of conventional, new, and future treatment options for sickle cell disease (SCD), a genetic disorder affecting the production of hemoglobin. Current treatments include hydroxyurea, a conventional SCD treatment that increases the levels of fetal hemoglobin, and new treatments such as voxelotor, a recently approved SCD treatment that selectively binds hemoglobin, preventing formation of sickled red blood cells. In addition to discussing the mechanisms of action of current SCD treatments, potential side effects are also discussed, highlighting the need for new treatments that can address the limitations of current treatments and improve the quality of life for people with SCD. Future treatments, such as gene therapy, are also explored as promising treatment options for SCD patients. Full article
(This article belongs to the Special Issue Acute and Chronic Sickle Cell Disease: Causes, Consequences, and Treatment: Volume II)
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Other

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12 pages, 1577 KiB  
Systematic Review
Red Blood Cell Exchange as a Valid Therapeutic Approach for Pregnancy Management in Sickle Cell Disease: Three Explicative Cases and Systematic Review of Literature
by Caterina Giovanna Valentini, Claudio Pellegrino, Sara Ceglie, Vincenzo Arena, Francesca Di Landro, Patrizia Chiusolo and Luciana Teofili
J. Clin. Med. 2023, 12(22), 7123; https://doi.org/10.3390/jcm12227123 - 16 Nov 2023
Viewed by 1121
Abstract
Pregnancy in women with sickle cell disease (SCD) is a high-risk situation, especially during the third trimester of gestation and in the post-partum period, due to chronic hypoxia and vaso-occlusive phenomena occurring in the maternal–fetal microcirculation: as a result, unfavorable outcomes, such as [...] Read more.
Pregnancy in women with sickle cell disease (SCD) is a high-risk situation, especially during the third trimester of gestation and in the post-partum period, due to chronic hypoxia and vaso-occlusive phenomena occurring in the maternal–fetal microcirculation: as a result, unfavorable outcomes, such as intra-uterine growth restriction, prematurity or fetal loss are more frequent in SCD pregnancies. Therefore, there is a consensus on the need for a strict and multidisciplinary follow-up within specialized structures. Transfusion support remains the mainstay of treatment of SCD pregnancies, whereas more targeted modalities are still controversial: the benefit of prophylactic management, either by simple transfusions or by automated red blood cell exchange (aRBCX), is not unanimously recognized. We illustrate the cases of three SCD pregnant patients who underwent aRBCX procedures at our institution in different clinical scenarios. Moreover, we carried out a careful literature revision to investigate the management of pregnancy in SCD, with a particular focus on the viability of aRBCX. Our experience and the current literature support the use of aRBCX in pregnancy as a feasible and safe procedure, provided that specialized equipment and an experienced apheresis team is available. However, further research in this high-risk population, with appropriately powered prospective trials, is desirable to refine the indications and timing of aRBCX and to confirm the advantages of this approach on other transfusion modalities. Full article
(This article belongs to the Special Issue Acute and Chronic Sickle Cell Disease: Causes, Consequences, and Treatment: Volume II)
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