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Article ; Online: [No title information]

Bartolucci, P

2023 Volume 44, Issue 4S1, Page(s) 4S7–4S11

Abstract: Sickle Cell Anemia is a disease with a strong vascular tropism. Beyond anemia, the pathophysiological mechanisms responsible for hemolysis, directly affect both acute and chronic vascular damages, thus resulting in a systemic disease. Understanding the ... ...

Title translation	Exploration de l’hémolyse associée à la drépanocytose et perspectives thérapeutiques spécifiques: Sickle cell disease hemolysis investigations and targeted therapeutics prospects.
Abstract	Sickle Cell Anemia is a disease with a strong vascular tropism. Beyond anemia, the pathophysiological mechanisms responsible for hemolysis, directly affect both acute and chronic vascular damages, thus resulting in a systemic disease. Understanding the different types of hemolysis underline the need for novel specific biomarkers. Targeted therapeutic approaches for these pathophysiological pathways are necessary to improve Sickle Cell patients' prognosis. Finally, given its complexity, Sickle Cell Disease is often used as a "proof of concept" for other pathologies. It seems likely that the rapidly evolving knowledge in this field will also benefit other diseases. © 2023 Société nationale française de médecine interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.
MeSH term(s)	Humans ; Hemolysis ; Anemia, Sickle Cell/complications ; Anemia, Sickle Cell/diagnosis ; Anemia, Sickle Cell/epidemiology ; Biomarkers
Chemical Substances	Biomarkers
Language	French
Publishing date	2023-11-21
Publishing country	France
Document type	English Abstract ; Journal Article
ZDB-ID	604679-4
ISSN	1768-3122 ; 0248-8663
ISSN (online)	1768-3122
ISSN	0248-8663
DOI	10.1016/S0248-8663(23)01303-6
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Article ; Online: Editorial.

Bartolucci, Pablo

Presse medicale (Paris, France : 1983)

2023 Volume 52, Issue 4, Page(s) 104208

Language	English
Publishing date	2023-11-14
Publishing country	France
Document type	Editorial
ZDB-ID	120943-7
ISSN	2213-0276 ; 0032-7867 ; 0755-4982 ; 0301-1518
ISSN (online)	2213-0276
ISSN	0032-7867 ; 0755-4982 ; 0301-1518
DOI	10.1016/j.lpm.2023.104208
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Article ; Online: Impact of hospitalized vaso-occlusive crises in the previous calendar year on mortality and complications in adults with sickle cell disease: a French population-based study.

Arlet, Jean-Benoît / Herquelot, Eléonore / Lamarsalle, Ludovic / Raguideau, Fanny / Bartolucci, Pablo

The Lancet regional health. Europe

2024 Volume 40, Page(s) 100901

Abstract: Background: Historically, sickle cell disease (SCD) patients experiencing frequent hospitalized vaso-occlusive crises (HVOC) have been associated with increased mortality, yet recent data reflecting the widespread use of hydroxyurea and advancements in ... ...

Abstract	Background: Historically, sickle cell disease (SCD) patients experiencing frequent hospitalized vaso-occlusive crises (HVOC) have been associated with increased mortality, yet recent data reflecting the widespread use of hydroxyurea and advancements in disease management remain limited. Our study aims to assess the association between HVOC and mortality or severe complications in patients with SCD in this new treatment landscape. Methods: This was a retrospective observational cohort study using the French national health data system. Between 01-01-2012 and 12-31-2018, all SCD patients ≥16 years old (ICD-10 codes D57.0-2) were included and followed until 12-31-2018. HVOC was defined as a hospitalization of ≥1 night with primary diagnosis of SCD with crisis, following an emergency room visit. The association between HVOC and severe complications was assessed with a Cox proportional hazards model. Findings: In total, 8018 patients (56.6% females; 4538/8018) were included. The 2018 SCD standardized one-year period prevalence was 17.9 cases/100,000 person-years [17.4; 18.3]. The mean rate was 0.84 (1.88) HVOC/person-year. In 2018, 70% (5323/7605), 22% (1671/7605), and 8% (611/7605) of patients experienced 0, 1-2, or 3+ HVOCs, respectively. The median survival time between HVOCs was 415 days [386; 439]. Overall, 312 patients died (3.9%) with a mean age of 49.8 (19.4). Compared to patients without HVOC, the hazard ratios of death in patients with 1-2 or 3+ HVOCs the year prior to death were 1.67 [1.21; 2.30] and 3.70 [2.30; 5.93], respectively. Incidence of acute chest syndrome, pulmonary embolism, osteonecrosis, and sepsis increased with the HVOCs category, but not stroke. In 2018, 29.5% (180/611) of patients with 3+ HVOCs did not take hydroxyurea. Interpretation: Patients must be closely monitored during their hospitalizations to intensify treatment and check treatment compliance. Innovative therapies are also required. Funding: The study was funded by Novartis.
Language	English
Publishing date	2024-04-06
Publishing country	England
Document type	Journal Article
ISSN	2666-7762
ISSN (online)	2666-7762
DOI	10.1016/j.lanepe.2024.100901
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Article: Hémoglobinopathies: un avantage contre le paludisme mais au risque de maladies sévères.

Bartolucci, Pablo

La Revue du praticien

2014 Volume 64, Issue 8, Page(s) 1110–1111

Title translation	Hemoglobin disorders: an advantage against malaria, but the risk of severe disease.
MeSH term(s)	Anemia, Sickle Cell/epidemiology ; Humans ; Malaria/epidemiology ; Risk Factors ; Severity of Illness Index ; Thalassemia/epidemiology
Language	French
Publishing date	2014-10
Publishing country	France
Document type	Journal Article
ZDB-ID	205365-2
ISSN	2101-017X ; 0035-2640
ISSN (online)	2101-017X
ISSN	0035-2640
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Article: Aspects épidémiologiques des maladies de I'hémoglobine.

Bartolucci, Pablo

La Revue du praticien

2014 Volume 64, Issue 8, Page(s) 1108–1109

Title translation	Epidemiology of hemoglobin disorders.
MeSH term(s)	Anemia, Sickle Cell/epidemiology ; Humans ; Thalassemia/epidemiology
Language	French
Publishing date	2014-10
Publishing country	France
Document type	Journal Article
ZDB-ID	205365-2
ISSN	2101-017X ; 0035-2640
ISSN (online)	2101-017X
ISSN	0035-2640
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Article ; Online: Tuberculosis in sickle cell disease patients.

Houist, A-L / Lafont, C / Gomart, C / Nebbad, B / Bartolucci, P / Vindrios, W / Melica, G / Habibi, A / Gallien, S

Infectious diseases now

2022 Volume 52, Issue 4, Page(s) 202–207

Abstract: Objective: Tuberculosis (TB) disease has rarely been reported in patients with sickle cell disease, but it is associated with an increased risk of bacterial infections. In France, sickle cell disease is frequent in populations with the highest ... ...

Abstract	Objective: Tuberculosis (TB) disease has rarely been reported in patients with sickle cell disease, but it is associated with an increased risk of bacterial infections. In France, sickle cell disease is frequent in populations with the highest prevalence of TB disease. We aimed to highlight clinical aspects of TB disease in patients with sickle cell disease. Patients and methods: Over a 10-year period, we retrospectively included all adults with sickle cell disease who had a positive culture for Mycobacterium tuberculosis managed in the adult sickle cell center of Henri-Mondor hospital. Sickle cell patients with TB disease were matched for comparison to adults without hemoglobinopathy and with documented TB disease in a 1:2 ratio. Logistic regression mixed models were performed. Results: Twelve patients with sickle cell disease and documented TB disease (median age: 29years; IQR [25-34]) were compared to 24 non-sickle cell patients (median age: 33years; IQR [27.5-38.5]). Baseline characteristics were similar between groups except for sickle cell disease. Ten of the 12 patients with sickle cell disease had pulmonary TB. TB disease characteristics were similar between sickle cell and non-sickle cell patients although sickle cell patients had fewer positive sputum smears for acid-fast bacilli (P=0.003) and fewer lung cavitations (P=0.03). Conclusions: TB disease in sickle cell patients was globally similar to non-sickle cell patients, even though less infectious. Regular follow-up in specialized centers might allow for earlier TB disease diagnosis in sickle cell patients.
MeSH term(s)	Adult ; Anemia, Sickle Cell/complications ; Anemia, Sickle Cell/epidemiology ; Humans ; Mycobacterium tuberculosis ; Retrospective Studies ; Tuberculosis/drug therapy ; Tuberculosis/epidemiology ; Tuberculosis, Pulmonary/drug therapy ; Tuberculosis, Pulmonary/epidemiology
Language	English
Publishing date	2022-03-05
Publishing country	France
Document type	Journal Article
ISSN	2666-9919
ISSN (online)	2666-9919
DOI	10.1016/j.idnow.2022.02.011
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Article ; Online: THromboprophylaxis In Sickle Cell Disease with central venous catheters (THIS): an internal pilot randomised controlled trial protocol.

Abdulrehman, Jameel / Forté, Stephanie / Tomlinson, George / Solh, Ziad / Bolster, Lauren / Sun, Haowei Linda / Bartolucci, Pablo / Kuo, Kevin H M

BMJ open

2024 Volume 14, Issue 1, Page(s) e079363

Abstract: Introduction: Individuals with sickle cell disease (SCD) and central venous catheters (CVC) are at high risk for venous thromboembolism (VTE). Minimal data exist regarding the use of anticoagulation as thromboprophylaxis of VTE in this demographic, and ... ...

Abstract	Introduction: Individuals with sickle cell disease (SCD) and central venous catheters (CVC) are at high risk for venous thromboembolism (VTE). Minimal data exist regarding the use of anticoagulation as thromboprophylaxis of VTE in this demographic, and as a result, clinical equipoise exists. Prophylactic dose rivaroxaban, a direct oral anticoagulant, is efficacious and safe as thromboprophylaxis in other demographics, and may be an optimal agent in SCD with CVC. Prior to conducting a full clinical trial to assess rivaroxaban as thromboprophylaxis in SCD with CVC, a pilot study is needed to gauge its feasibility. Methods and analysis: THromboprophylaxis In Sickle Cell Disease pilot trial is an investigator-initiated, multicentre, double-blinded, randomised controlled trial (RCT) assessing if it is feasible and safe to conduct an adequately powered RCT comparing rivaroxaban to matching placebo as thromboprophylaxis in those with SCD and CVC. Fifty adult patients with SCD and CVC will be randomised to receive either rivaroxaban 10 mg daily or matching placebo for the duration of the CVC in situ for up to 1 year. After randomisation, follow-up visits will occur every 3 months. The primary outcomes pertain to the feasibility of a full trial and include numbers of eligible and recruited participants. Exploratory outcomes include overall incidence of VTE and bleeding complications, as well as quality of life. If the full trial is feasible, blinding will be maintained and patients in the pilot study will be included in the full trial. Ethics and dissemination: The trial was initially approved by the University Health Network Research Ethics Board (REB) in Toronto, Canada. All sites will obtain approval from their respective REB prior to commencement of study activities. Study results will be disseminated through presentations at medical conferences and peer-reviewed publications. Trial registration number: NCT05033314.
MeSH term(s)	Adult ; Humans ; Pilot Projects ; Rivaroxaban/therapeutic use ; Central Venous Catheters/adverse effects ; Venous Thromboembolism/etiology ; Venous Thromboembolism/prevention & control ; Venous Thromboembolism/drug therapy ; Anemia, Sickle Cell/complications ; Anemia, Sickle Cell/drug therapy ; Anticoagulants/therapeutic use ; Randomized Controlled Trials as Topic
Chemical Substances	Rivaroxaban (9NDF7JZ4M3) ; Anticoagulants
Language	English
Publishing date	2024-01-03
Publishing country	England
Document type	Clinical Trial Protocol ; Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	2599832-8
ISSN	2044-6055 ; 2044-6055
ISSN (online)	2044-6055
ISSN	2044-6055
DOI	10.1136/bmjopen-2023-079363
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Article ; Online: Is chest X-ray still relevant for acute chest syndrome diagnosis?

Pelinski, Yanis / Guindo, Aldiouma / Kassasseya, Christian / Diallo, Dapa / Arlet, Jean-Benoît / Dessap, Armand Mekontso / Habibi, Anoosha / Bartolucci, Pablo

Health science reports

2024 Volume 7, Issue 5, Page(s) e2053

Language	English
Publishing date	2024-05-01
Publishing country	United States
Document type	Journal Article
ISSN	2398-8835
ISSN (online)	2398-8835
DOI	10.1002/hsr2.2053
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Article ; Online: Management of delayed hemolytic transfusion reaction in sickle cell disease: Prevention, diagnosis, treatment.

Pirenne, F / Bartolucci, P / Habibi, A

Transfusion clinique et biologique : journal de la Societe francaise de transfusion sanguine

2017 Volume 24, Issue 3, Page(s) 227–231

Abstract: Transfusion remains a key treatment of sickle cell disease complications. However, delayed hemolytic transfusion reaction, the most serious complication of transfusion, may be life-threatening if hyperhemolysis develops. This syndrome is generally ... ...

Abstract	Transfusion remains a key treatment of sickle cell disease complications. However, delayed hemolytic transfusion reaction, the most serious complication of transfusion, may be life-threatening if hyperhemolysis develops. This syndrome is generally underdiagnosed because its biological and clinical features resemble those of vaso-occlusive crisis, and red blood cell antibodies are frequently absent. Further transfusions may aggravate the symptoms, leading to severe multiple organ failure and death. It is therefore essential to prevent, diagnose and treat this syndrome efficiently. Prevention is based principally on the attenuation of allo-immunization through the provision of extended-matched RBCs or the use of rituximab. However, such treatment may be insufficient. Early diagnosis might make it possible to implement specific treatments in some cases, thereby avoiding the need for secondary transfusion. Diagnosis is dependent on the knowledge of the medical staff. Finally, many treatments, including steroids, immunoglobulins, erythropoietin and eculizumab, have been used to improve outcome. Improvements in our knowledge of the specific features of DHTR in SCD should facilitate management of this syndrome.
MeSH term(s)	Adrenal Cortex Hormones/therapeutic use ; Anemia, Sickle Cell/blood ; Anemia, Sickle Cell/immunology ; Anemia, Sickle Cell/therapy ; Antibodies, Monoclonal, Humanized/therapeutic use ; Blood Grouping and Crossmatching ; Humans ; Immunization ; Isoantibodies/blood ; Rituximab/therapeutic use ; Transfusion Reaction/diagnosis ; Transfusion Reaction/immunology ; Transfusion Reaction/prevention & control ; Transfusion Reaction/therapy
Chemical Substances	Adrenal Cortex Hormones ; Antibodies, Monoclonal, Humanized ; Isoantibodies ; Rituximab (4F4X42SYQ6) ; eculizumab (A3ULP0F556)
Language	English
Publishing date	2017-06-29
Publishing country	France
Document type	Journal Article
ZDB-ID	1204698-x
ISSN	1953-8022 ; 1246-7820
ISSN (online)	1953-8022
ISSN	1246-7820
DOI	10.1016/j.tracli.2017.05.016
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Article ; Online: Evaluation of a prophylactic transfusion program on obstetric outcomes in pregnant women with sickle cell disease: A single centre retrospective cohort study.

Sobczyk, O / Gottardi, E / Lefebvre, M / Canouï-Poitrine, F / Jebali, A / De Luna, G / Pirenne, F / Redel, D / Galacteros, F / Boutin, E / Bartolucci, P / Haddad, B / Habibi, A / Lecarpentier, E

European journal of obstetrics, gynecology, and reproductive biology

2023 Volume 290, Page(s) 103–108

Abstract: Objective: To evaluate the effects of a prophylactic transfusion program (TP) on obstetric and perinatal outcomes in pregnant women with sickle cell disease (SCD).: Methods: This retrospective cohort study included all singleton pregnancies among ... ...

Abstract	Objective: To evaluate the effects of a prophylactic transfusion program (TP) on obstetric and perinatal outcomes in pregnant women with sickle cell disease (SCD). Methods: This retrospective cohort study included all singleton pregnancies among women with SCD in a French university tertiary care center between 1 January 2004 and 31 December 2017. The TP group included patients selected according to the French guidelines who received regular red blood cell transfusions during pregnancy until delivery. The factors associated with TP indication [year of birth, SCD genotype, history of acute chest syndrome and delayed hemolysis transfusion reaction (DHTR) risk score] were taken into account in a propensity score. A composite obstetric adverse outcome was defined associating birth before 34 gestational weeks and/or pre-eclampsia and/or small for gestational age and/or abruption and/or stillbirth and/or maternal death and/or neonatal death. Results: In total, 246 pregnancies in 173 patients were analyzed. Twenty-two pregnancies with a history of DHTR were excluded. A higher frequency of TP was found before 2013 [119/148 (80.4%) vs 38/76 (50%); p < 0.001]. Rates of preterm birth before 34 gestational weeks (5.6% vs 19.7%; p = 0.001), vaso-occlusive crisis (36.5% vs. 61.8%; p < 0.001), and acute chest syndrome (6.1% vs. 14.5%; p = 0.04) during pregnancy were decreased significantly in the TP group. Among the groups with and without composite obstetric adverse outcomes, the frequency of TP was 52.6% and 74.7%, respectively [odds ratio (OR) 0.30, 95% confidence interval (CI) 0.09-1.02]. The multivariate analysis shows that the TP was associated with a significant reduction in the risk of composite obstetric adverse outcomes (OR 0.28, 95% CI 0.08-0.97; p = 0.04). Conclusion: A red blood cell TP may have an independent protective effect on maternal and perinatal adverse outcomes during pregnancy in women with SCD.
MeSH term(s)	Female ; Infant, Newborn ; Pregnancy ; Humans ; Pregnant Women ; Acute Chest Syndrome/complications ; Retrospective Studies ; Premature Birth ; Anemia, Sickle Cell/complications ; Anemia, Sickle Cell/therapy ; Stillbirth/epidemiology ; Pregnancy Outcome
Language	English
Publishing date	2023-09-09
Publishing country	Ireland
Document type	Journal Article
ZDB-ID	190605-7
ISSN	1872-7654 ; 0301-2115 ; 0028-2243
ISSN (online)	1872-7654
ISSN	0301-2115 ; 0028-2243
DOI	10.1016/j.ejogrb.2023.08.390
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