Recommandations françaises de prise en charge de la drépanocytose de l'adulte : actualisation 2015

Habibi, Anoosha; Arlet, J; Stankovic, K.; Gellen-Dautremer, Justine; Ribeil, Jean‐Antoine; Bartolucci, Pablo; Lionnet, François

doi:10.1016/s0248-8663(15)60002-9

Cited by 111 publications

(88 citation statements)

References 26 publications

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“…La drépanocytose est une maladie génétique autosomique récessive caractérisée par la présence d'une hémoglobine anormale, qui favorise la falciformation irréversible des hématies en situation d'hypoxie prolongée et aboutissant à des manifestations vaso-occlusives [ 1 ]. On regroupe sous le terme « syndrome drépanocytaire majeur », la forme homozygote SS et les formes hétérozygotes composites SC et S-β + thalassémie ou S-β 0 thalassémie qui ont une expression clinique voisine [ 2 ].…”

Section: Introductionunclassified

“…De nombreuses complications aiguës et chroniques, causes de morbidité font partie intégrante de cette affection. Les complications aiguës sont principalement représentées par les crises vaso-occlusives, l'anémie hémolytique chronique avec des épisodes d'aggravation aiguë et la susceptibilité aux infections bactériennes à germes encapsulés en raison de l'asplénie fonctionnelle [ 1 - 6 ]. Si de nombreuses études ont fait l'inventaire des complications de la drépanocytose, peu de données récentes sont disponibles sur les urgences drépanocytaires dans notre contexte.…”

Section: Introductionunclassified

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Urgences drépanocytaires au Service des Maladies du Sang du Centre National Hospitalier Universitaire-Hubert Koutoukou Maga de Cotonou, Benin

Dodo¹,

Zohoun²,

Baglo³

et al. 2018

Pan Afr Med J

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IntroductionL'évolution de la drépanocytose est marquée par la survenue de complications aigues dont certaines constituent de véritables urgences pouvant mettre en jeu le pronostic vital ou fonctionnel. Notre travail avait pour objectif d'étudier la fréquence et l'évolution des urgences drépanocytaires dans le Service des Maladies du Sang (SMAS) du Centre National Hospitalier Universitaire-Hubert Koutoukou Maga (CNHU-HKM) de Cotonou.MéthodesNous avons mené une étude rétrospective et descriptive de janvier 2014 à décembre 2015 et portant sur l'ensemble des patients hospitalisés pour une urgence drépanocytaire. Nous avons exclu les patients hospitalisés pour des complications drépanocytaires chroniques.RésultatsDeux cent quatre (204) urgences drépanocytaires pour un nombre total de 813 hospitalisations ont été enregistrés (prévalence de 25%). L'âge moyen des patients était de 24,2 ans. La tranche d'âge la plus représentée était celle de 20-30 ans (45,6%). Le sexe masculin prédominait à 60,8%. Les élèves/étudiants étaient les plus représentés à 55,9%. La douleur aigue était le motif principal d'hospitalisation à 63,7%. L'homozygotie SS était majoritairement représentée à 72,1%. Les complications vaso-occlusives ostéo-articulaires prédominaient à 70,1%. Les complications infectieuses documentées étaient dominées par le paludisme (27,5%). La décompensation anémique était notée à 30,4%. Sur le plan thérapeutique, l'hydratation a été utilisée à 85,3%. La durée moyenne de séjour était de 5,4 jours. L'évolution a été favorable dans 96,5% des cas. La létalité était de 2,5%.ConclusionLes urgences drépanocytaires sont fréquentes. Elles nécessitent un diagnostic rapide et une prise en charge précoce et efficace. La formation continue du personnel médical à la prise en charge des urgences drépanocytaires s'avère nécessaire pour réduire leur mortalité.

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Section: Introductionunclassified

Urgences drépanocytaires au Service des Maladies du Sang du Centre National Hospitalier Universitaire-Hubert Koutoukou Maga de Cotonou, Benin

Dodo¹,

Zohoun²,

Baglo³

et al. 2018

Pan Afr Med J

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“…Several authors agree that medullary crowding by postnecrotic endosteal bone apposition makes medullary access difficult and increases the risk of miscarriage and intraoperative fractures, and SY [16] points out that these difficulties make it necessary to resort to the cautious bore under image intensification using rigid reamers of diameter 8 or 6 and to have a wide range of implants. difficulties in preparation of the acetabulum and femoral shaft [24,25].…”

Section: Discussionmentioning

confidence: 99%

Total Hip Arthroplasty in Patient with Sickled Cell Trait Ss: The Cases of 14 Patients

Cva¹

2017

JOBD

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Osteo-articular complications of sickle cell disease are multiple but mostly dominated by epiphyseal aseptic necrosis.Conservative at the beginning, the surgical treatment becomes radical in the last stage with the performance of a total hip arthroplasty (THA). We report the case of total hip arthroplastyin a patient with sickle cell traitSSat Ordre de Malte Hospital (CHOM) in Dakar, focusing on the peri-operative complications encountered in this particular case in order to prevent them or to treat them if necessary. This is a retrospective, mono-centric study including 15 total hip arthroplasty (THA) performed in 14 SS sickle cell patients over a 66-month period. The postero-lateral Moore mini open approach was used exclusively. The clinical evaluation was based on the Aubigné Postel-Merle (PMA) and Harris (HHS) scores and the radiographic score on the Ficat and Arlet classification. The corticodiaphyseal index as well as the Noble flare index channel made it possible to appreciate the medullary congestion and the shape of the femur.All together, THA in Sickle Cell Disease represented 7.57% of all hip prosthetic activity during the period. The average age of patients (10 women and 4 men) was 29.06 years (18-50). The overall functional result was good and very good in 77% of cases. We observed 4 femoral fissures, an early vesicular lithiasis infection with psoas syndrome and acetabular loosening in the same patient and one death at 5 days postoperatively.

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“…Analgesics are frequently prescribed, but steroidal antiinflammatory drugs are contraindicated [7]. French guidelines for the management of adult with SCD recommend an annual dental check-up [9]. A systematic review was performed to evaluate methods of treating dental complications in people with SCD; however, no randomized clinical trial or quasi-RCT was identified.…”

Section: Anemia and Oral Healthmentioning

confidence: 99%

“…Patients may neglect dental care due to concerns of bleeding, which can result in the need for invasive dental procedures associated with a higher risk of bleeding [8]. The need for regular examinations and adequate preventive dental care is therefore emphasized for these patients [9,10]. Clinical guidelines also recommend to plan and manage required dental procedures with the patient's hematologist [8].…”

Section: Introductionmentioning

confidence: 99%

Non-Malignant Blood Disorders and Their Impact on Oral Health: an Overview

Boström

Lira-Júnior

2019

Curr Oral Health Rep

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Purpose of Review This review aims to summarize the literature on inherited hemoglobin and coagulation disorders, focusing on sickle cell disease (SCD), thalassemia, hemophilia, and von Willebrand disease (vWD), and their impact on oral health. Clinical dental management of patients with these conditions is also reviewed. Recent Findings There is no evident association between caries and SCD. Despite limited available studies, there may be a possible association between caries and thalassemia, which could relate to salivary changes in immunoglobulin A and Streptococcus mutans reported in these patients. Controversial results on the caries experience of hemophilia and vWD patients are found in the literature, which are likely to reflect differences in the provided preventive dental care of these patients rather than a biological association. Current evidence does not support any association between periodontal disease and SCD. The literature on periodontal disease and thalassemia shows controversial results although some evidence suggests differences in the gingival inflammation of thalassemia patients with increased levels of inflammatory cytokines and matrix metalloproteinases, deserving further investigation. The available literature does not provide evidence of an increased risk for gingival bleeding or periodontal disease in patients with hemophilia or vWD. Dental management of these patients requires appropriate measures to prevent infection and excessive bleeding. The use of local hemostatic may often be sufficient; however, in severe bleeding disorders, systemic hemostatic or replacement therapy may be required, and procedures are recommended to be planned with the patient's hematologist. Summary Maintaining good oral health through preventive dentistry is important for patients with inherited hemoglobin and coagulation disorders as that may prevent the risk of infection and the need for invasive dental treatments that can result in bleeding complications. Keywords Hematologic diseases. Anemia. Blood coagulation disorders. Oral health. Periodontal diseases. Dental caries This article is part of the Topical Collection on Systemic Diseases * Elisabeth A.

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Recommandations françaises de prise en charge de la drépanocytose de l'adulte : actualisation 2015

Cited by 111 publications

References 26 publications

Urgences drépanocytaires au Service des Maladies du Sang du Centre National Hospitalier Universitaire-Hubert Koutoukou Maga de Cotonou, Benin

Urgences drépanocytaires au Service des Maladies du Sang du Centre National Hospitalier Universitaire-Hubert Koutoukou Maga de Cotonou, Benin

Total Hip Arthroplasty in Patient with Sickled Cell Trait Ss: The Cases of 14 Patients

Non-Malignant Blood Disorders and Their Impact on Oral Health: an Overview

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