Éditeur(s) : HAL CCSD Wiley
Résumé : International audience
Sickle cell anemia (SS) is characterized by a reduced cerebral microvascular oxygen saturation (cerebral TOI), which is not associated with hemoglobin concentration. Cerebral TOI has never been studied in sickle cell-hemoglobin C disease (SC). We focused on the relationships between hemorheological alterations and cerebral TOI in sickle cell patients with no cerebral vasculopathy and on the usefulness of TOI variability to assess the cerebral vasomotion activity. The blood rheological profile, the level of cerebral TOI (spatial resolved spectroscopy) and the cerebral TOI variability, which reflects vasomotion activity, were compared between 20 healthy subjects (AA), 21 SC patients, and 21 SS patients. Cerebral TOI exhibited the following order: AA > SC > SS. The low cerebral TOI in SS patients was related to red blood cell aggregation and deformability properties. The cerebral TOI variability of SS and SC patients was increased above healthy values and vasomotion activity was negatively associated with the reduced cerebral TOI in SS patients. We demonstrated that (1) blood rheology could be involved in the reduced cerebral TOI in SS patients but not in SC patients; (2) vasomotion activity is increased in SS and SC patients to compensate for the reduced cerebral TOI.
ISSN: 0361-8609

inserm-00838966
http://www.hal.inserm.fr/inserm-00838966
http://www.hal.inserm.fr/inserm-00838966/document
http://www.hal.inserm.fr/inserm-00838966/file/2013_Waltz_American_Journalsur Hematology.pdf
DOI : 10.1002/ajh.23318

Éditeur(s) : HAL CCSD Elsevier
Résumé : International audience
Infective endocarditis is defined by a focus of infection within the heart and is a feared disease across the field of cardiology. It is frequently acquired in the health care setting, and more than one-half of cases now occur in patients without known heart disease. Despite optimal care, mortality approaches 30% at 1 year. The challenges posed by infective endocarditis are significant. It is heterogeneous in etiology, clinical manifestations, and course. Staphylococcus aureus, which has become the predominant causative organism in the developed world, leads to an aggressive form of the disease, often in vulnerable or elderly patient populations. There is a lack of research infrastructure and funding, with few randomized controlled trials to guide practice. Longstanding controversies such as the timing of surgery or the role of antibiotic prophylaxis have not been resolved. The present article reviews the challenges posed by infective endocarditis and outlines current and future strategies to limit its impact. (C) 2017 by the American College of Cardiology Foundation.
ISSN: 0735-1097

hal-01489511
https://hal.archives-ouvertes.fr/hal-01489511
DOI : 10.1016/j.jacc.2016.10.066

Éditeur(s) : HAL CCSD Elsevier
Résumé : International audience
Vascular resistance and tissue perfusion may be both affected by impaired vascular function and increased blood viscosity. Little is known about the effects of vascular function on the occurrence of painful vaso-occlusive crises (VOC) in children with sickle cell anemia (SCA). The aim of the present study was to determine which side of the balance (blood viscosity or vascular function) is the most deleterious in SCA and increases the risk for frequent hospitalized VOC. Microvascular function, microcirculatory oxygenation and blood viscosity were determined in a group of 22 SCA children/adolescents at steady state and a group of 13 healthy children/adolescents. Univariate analyses demonstrated blunted microvascular reactivity during local thermal heating test and decreased microcirculatory oxygenation in SCA children compared to controls. Multivariate analysis revealed that increased blood viscosity and decreased microcirculatory oxygenation were independent risk factors of frequent VOC in SCA. In contrast, the level of microvascular dysfunction does not predict VOC rate. In conclusion, increased blood viscosity is usually well supported in healthy individuals where vascular function is not impaired. However, in the context of SCA, microvascular function is impaired and any increase of blood viscosity or decrease in microcirculatory oxygenation would increase the risks for frequent VOC.
ISSN: 1079-9796

hal-01668335
https://hal.univ-antilles.fr/hal-01668335
DOI : 10.1016/j.bcmd.2015.10.005

Éditeur(s) : HAL CCSD Masson
Résumé : International audience
In French Guiana, the age-standardized incidence rate of cervical cancer is four times higher than in France and the mortality rate 5.5 times higher. A survival study revealed that stage at diagnosis was the main factor influencing the prognosis, showing that early detection is crucial to increase cervical cancer survival. The present study aimed at evaluating the cervical cancer screening rate between 2006 and 2011 by age and for 3-years period in French Guiana. All pap smears realised in French Guiana were analysed in two laboratories allowing exhaustive review of screening data. The screening rate was estimated at about 54% from 2006 to 2011, with a statistical difference between coastal and rural area (56.3% versus 18.7%).Although the methodological difference did not allow comparisons with mainland France, these results could be used to evaluate the impact of organised cervical cancer screening by the French Guiana Association for Organized Screening of Cancers implemented in French Guiana since 2012.
ISSN: 0037-9085

hal-01260391
https://hal.archives-ouvertes.fr/hal-01260391
https://hal.archives-ouvertes.fr/hal-01260391/document
https://hal.archives-ouvertes.fr/hal-01260391/file/2015%2C%20Douine%20-%20Servical%20cancer.pdf

Éditeur(s) : HAL CCSD Elsevier
Résumé : International audience
A recent study suggested that adenosine signaling pathway could promote hemolysis in patients with sickle cell anemia (SCA). This signaling pathway involves several gene coding enzymes for which variants have been described. In this study, we analyzed the genotype-phenotype relationships between functional polymorphisms or polymorphisms associated with altered expression of adenosine pathway genes, namely adenosine deaminase (ada; rs73598374), adenosine A2b receptor (adora2b; rs7208480), adenylyl cyclase6 (adcy6; rs3730071, rs3730070, rs7300155), and hemolytic rate in SCA patients. One hundred and fifty SCA patients were genotyped for adcy6, ada, and adora2b variants as well as alpha-globin gene, a genetic factor known to modulate hemolytic rate. Hematological and biochemical data were obtained at steady-state. Lactate dehydrogenase, aspartate aminotransferase, reticulocytes and total bilirubin were used to calculate a hemolytic index. Genotype-phenotype relationships were investigated using parametric tests and multivariate analysis. SCA patients carrying at least one allele of adcy6 rs3730070-G exhibited lower hemolytic rate than non-carriers in univariate analysis (p=0.006). The presence of adcy6 rs3730070-G variant was associated with a decreased hemolytic rate in adjusted model for age and alpha-thalassemia (p=0.032). Our results support a protective effect of adcy6 rs3730070-G variant on hemolysis in SCA patients.
ISSN: 1079-9796

hal-01668332
https://hal.univ-antilles.fr/hal-01668332
DOI : 10.1016/j.bcmd.2016.02.006

Éditeur(s) : HAL CCSD Oxford University Press (OUP)
Résumé : International audience
Background: The objective of the study was to determine the incidence of transaminase elevation during dengue, and its predictive factors. Methods: In 2013, a longitudinal study was performed using data from all cases of dengue seen in Cayenne Hospital. Cox proportional modeling was used. Signs of major transaminase elevation were defined as an increase in aspartate amino transferase (AST) or alanine amino transferase (ALT) concentration over 10 times the normal value (10N). Results: There were 1574 patients and 13 249 person-days of follow-up. The incidence rate for signs of trans-aminase elevation (10N) was 0.55 per 100 person-days. Six patients had major transaminase elevation with AST.1000 units (0.43 per 1000 patient-days), and 73 patients (4.6%) developed transaminase elevation with AST .10N. The variables independently associated with major transaminase elevation were hyponatremia, low platelets, dehydration, hematocrit increase, food intolerance, positive nonstructural protein 1 (NS1), age over 15 years and the notion of paracetamol intake. Conclusions: Although very frequent, the incidence of major transaminase elevation was lower than reported elsewhere perhaps because of good access to care, or of the particular serotype causing this epidemic. The patients with transaminase elevation tended to be older, more severe and taking paracetamol.
ISSN: 0035-9203

inserm-01475594
http://www.hal.inserm.fr/inserm-01475594
http://www.hal.inserm.fr/inserm-01475594/document
http://www.hal.inserm.fr/inserm-01475594/file/2016%2C%20Djossou%20-%20Incidence%20and%20predictive%20factors%20of%20transaminase.pdf
DOI : 10.1093/trstmh/trv117

Éditeur(s) : HAL CCSD Wiley
Résumé : International audience
Oxidative stress and haemolysis-associated nitric oxide (NO) depletion plays a crucial role in the development of vasculopathy in sickle cell anaemia (SS). However it remains unknown whether oxidative stress and haemolysis levels influence vascular function in patients with sickle haemoglobin C disease (SC). Microvascular response to heat (using Laser Doppler flowmetry on finger), oxidative stress biomarkers, NO metabolites, endothelin-1 and haematological parameters were compared between patients with SS and SC. Vascular function, oxidative and nitrosative markers were also measured in healthy (AA) children. SS and SC had increased plasma advanced oxidation protein products (AOPP), malondialdehyde, plasma antioxidant activities and NO end products, compared to AA. SC had lower catalase activity compared to AA and SS. Haemolytic rate, glutathione peroxidase and nitrotyrosine concentrations were significantly increased in children with SS compared to SC and AA. SS and SC had impaired microvascular reactivity compared to AA. In SS, the plateau phase of the response to local thermal heating was negatively associated with nitrotyrosine and AOPP. No association between vascular function parameters and oxidative stress markers was observed in SC. Mild haemolysis in SC, compared to SS, may limit oxidative and nitrosative stress and could explain the better preserved microvascular function in this group.
ISSN: 0007-1048

hal-01668314
https://hal.univ-antilles.fr/hal-01668314
DOI : 10.1111/bjh.14693

Éditeur(s) : HAL CCSD Public Library of Science
Résumé : International audience
Sickle cell anemia (SCA) and hemoglobin SC (HbSC) disease are the two most common forms of sickle cell disease (SCD), a frequent hemoglobinopathy which exhibits a highly variable clinical course. Although high levels of microparticles (MPs) have been consistently reported in SCA and evidence of their harmful impact on the SCA complication occurrences have been provided, no data on MP pattern in HbSC patients has been reported so far. In this study, we determined and compared the MP patterns of 84 HbSC and 96 SCA children, all at steady-state, using flow cytometry. Most of circulating MPs were derived from platelets (PLTs) and red blood cells (RBCs) in the two SCD syndromes. Moreover, we showed that HbSC patients exhibited lower blood concentration of total MPs compared to SCA patients, resulting mainly from a decrease of MP levels originated from RBCs and to a lesser extent from PLTs. We did not detect any association between blood MP concentrations and the occurrence of painful vaso-occlusive crises, acute chest syndrome and pulmonary hyper-tension in both patient groups. We also demonstrated for the first time, that whatever the considered genotype, RBC-derived MPs exhibited higher externalized phosphatidylserine level and were larger than PLT-derived MPs.
ISSN: 1932-6203

hal-01668322
https://hal.univ-antilles.fr/hal-01668322
https://hal.univ-antilles.fr/hal-01668322/document
https://hal.univ-antilles.fr/hal-01668322/file/journal.pone.0177397.pdf
DOI : 10.1371/journal.pone.0177397

Éditeur(s) : HAL CCSD Elsevier
Résumé : International audience
CONTEXT: Chlordecone, an environmentally persistent organochlorine insecticide used intensively in banana culture in the French West Indies until 1993, has permanently polluted soils and contaminated foodstuffs. Consumption of contaminated food is the main source of exposure nowadays. We sought to identify main contributors to blood chlordecone concentration (BCC) and to validate an exposure indicator based on food intakes. MATERIAL AND METHODS: We used a food frequency questionnaire (FFQ) completed by a sample of 194 pregnant women to estimate their dietary exposure to chlordecone and compared it to blood levels. In a first approach, chlordecone daily intake was estimated as the product of daily eaten quantity of 214 foodstuffs, multiplied by their chlordecone content, and summed over all items. We then predicted individual blood chlordecone concentration with empirical weight regression models based on frequency of food consumption, and without contamination data. RESULTS: Among the 191 subjects who had BCC determination, 146 (76%) had detectable values and mean BCC was 0.86 ng/mL (range < LOD-13.2). Mean per capita dietary intake of chlordecone was estimated at 3.3 microg/day (range: 0.1-22.2). Blood chlordecone levels were significantly correlated with food exposure predicted from the empirical weight models (r=0.47, p<0.0001) and, to a lesser extent, with chlordecone intake estimated from food consumption and food contamination data (r=0.20, p=0.007). Main contributors to chlordecone exposure included seafood, root vegetables, and Cucurbitaceous. CONCLUSION: These results show that the Timoun FFQ provides valid estimates of chlordecone exposure. Estimates from empirical weight models correlated better with blood levels of chlordecone than did estimates from the dietary intake assessment.
ISSN: 0013-9351

inserm-00517109
http://www.hal.inserm.fr/inserm-00517109
http://www.hal.inserm.fr/inserm-00517109/document
http://www.hal.inserm.fr/inserm-00517109/file/Pesticide_exposureofpregnantwomeninGuadeloupe.pdf
DOI : 10.1016/j.envres.2009.10.015

Éditeur(s) : HAL CCSD Elsevier
Résumé : International audience
This study aimed to compare the relative survival of patients with invasive breast cancer between women from French Guiana (a French territory in South America) and metropolitan France. No study hadever compared survival of breast cancer on the basis of immigrant status in France. Our study underlined that access to care for migrants is challenging whichwgenerates health inequalities. Background The prognosis of breast cancer in French Guiana is worse than in France with 23 deaths per 100 incident cases against 17 per 100 in metropolitan France. This study aimed to compare relative survival of patients with invasive breast cancer (IBC) between women from French Guiana and metropolitan France and to determine risk factors influencing breast cancer survival in French Guiana.Materials and methods Data were collected from the Cancer registry of French Guiana. We comparedthe relative survival of women with IBC between French Guiana and metropolitan France. We used Cox's proportional hazard regression to evaluate the effect of prognostic factors on cancer-specific mortality in French Guiana. Results We included all 269 cases of IBC in women diagnosed in French Guiana between 2003 and 2009. The overall 5-year relative survival rate of patients with IBC was 79% in French Guiana and 86% in metropolitan France. The place of birth (foreign country versus French territory), the tumor stage at the time of diagnosis, the mode of diagnosis (symptoms versus screening), the presence of hormone receptors in the tumor and the histologic type were the variables associated with survival differences. None of the other study variables were significantly associated with prognosis. Conclusion Access to care for migrants is challenging,which leads to health inequalities. Early detection through prevention programs is crucial to increaseIBC survival notably for foreign-born patients.
ISSN: 1526-8209

hal-01288657
https://hal.archives-ouvertes.fr/hal-01288657
https://hal.archives-ouvertes.fr/hal-01288657/document
https://hal.archives-ouvertes.fr/hal-01288657/file/2016%2C%20Roue%20-%20Predictive%20factors%20of%20the%20survival%20of%20women%20with%20invasive%20breast%20cancer%20in%20French%20Guiana.pdf

Éditeur(s) : HAL CCSD Wiley
Résumé : International audience
ISSN: 0007-1048

hal-01679026
https://hal.univ-antilles.fr/hal-01679026
DOI : 10.1111/bjh.14693

Éditeur(s) : HAL CCSD
Résumé : National audience
Contexte initial de prise en charge, qualité de vie à 3 mois et survie des patients dialysés pour insuffisance rénale chronique terminale dans deux départements français d'Amérique
Premier congrès de la Société francophone de néphrologie dialyse et transplantation (SFNDT)
Strasbourg, France

inserm-01567195
http://www.hal.inserm.fr/inserm-01567195
http://www.hal.inserm.fr/inserm-01567195/document
http://www.hal.inserm.fr/inserm-01567195/file/2017%2C%20Deloumeaux%20-%20Insuffisance%20r%C3%A9nale%20chronique%20terminale.pdf
DOI : 10.1016/j.nephro.2016.07.340

Éditeur(s) : HAL CCSD Public Library of Science
Résumé : International audience
Leg ulcer is a disabling complication in patients with sickle cell anemia (SCA) but the exact pathophysiological mechanisms are unknown. The aim of this study was to identify the hematological and hemorheological alterations associated with recurrent leg ulcers. Sixty-two SCA patients who never experienced leg ulcers (ULC-) and 13 SCA patients with a positive history of recurrent leg ulcers (ULC+) - but with no leg ulcers at the time of the study - were recruited. All patients were in steady state condition. Blood was sampled to perform hematological, biochemical (hemolytic markers) and hemorheological analyses (blood viscosity, red blood cell deformability and aggregation properties). The hematocrit-to-viscosity ratio (HVR), which reflects the red blood cell oxygen transport efficiency, was calculated for each subject. Patients from the ULC+ group were older than patients from the ULC- group. Anemia (red blood cell count, hematocrit and hemoglobin levels) was more pronounced in the ULC+ group. Lactate dehydrogenase level was higher in the ULC+ group than in the ULC- group. Neither blood viscosity, nor RBC aggregation properties differed between the two groups. HVR was lower and RBC deformability tended to be reduced in the ULC+ group. Our study confirmed increased hemolytic rate and anemia in SCA patients with leg ulcers recurrence. Furthermore, our data suggest that although systemic blood viscosity is not a major factor involved in the pathophysiology of this complication, decreased red blood cell oxygen transport efficiency (i.e., low hematocrit/viscosity ratio) may play a role.
ISSN: 1932-6203

inserm-00922105
http://www.hal.inserm.fr/inserm-00922105
http://www.hal.inserm.fr/inserm-00922105/document
http://www.hal.inserm.fr/inserm-00922105/file/2013_Connes_-_Decreased_Hematocrit.pdf
DOI : 10.1371/journal.pone.0079680

Éditeur(s) : HAL CCSD
Résumé : National audience
La Guyane française, de par sa situation géographique et climatique équatoriale – entraînant uneimmense biodiversité – et le caractère multiculturel et multiethnique de sa population, est le siège d’unlarge panel de pathologies infectieuses et tropicales, dont certaines sont assez mal connues.La fièvre Q, la toxoplasmose, la cryptococcose ou l’infection à VIH sont communes aux pays tempérés,mais présentent ici de grandes spécificités, entraînant une prise en charge et un raisonnement médicalparfois différents. De nombreuses pathologies tropicales sont par ailleurs présentes sur un mode endémiqueet/ou épidémique : paludisme, leishmaniose, maladie de Chagas, histoplasmose, dengue, chikungunya, etc.Des personnes ayant séjourné en Guyane peuvent présenter des problèmes infectieux spécifiques deretour sur le continent européen, et ces pathologies doivent pouvoir être évoquées par l’infectiologue.
La Lettre de l'Infectiologue

inserm-01407150
http://www.hal.inserm.fr/inserm-01407150
http://www.hal.inserm.fr/inserm-01407150/document
http://www.hal.inserm.fr/inserm-01407150/file/2016%2C%20Epelboin%20-%20L%27infectiologie%20en%20Guyane%20synth%C3%A8se%20med%20trop.pdf

Éditeur(s) : HAL CCSD SAGE Publications
Résumé : International audience
: French Guiana is the French territory that is most affected by HIV. AIDS incidence is much higher than in mainland France and sex work seems to be an important driver of the epidemic. The objective of this study was to describe consistent condom use among female sex workers with their clients and their intimate partners and to identify determinants of non-use of condoms. An HIV/AIDS Knowledge, Attitudes, Behaviours and Practices survey was conducted in 2009-2010 among sex workers in French Guiana. A total of 477 sex workers were interviewed. Female sex workers were more likely to use condoms with their clients (97%) than with their intimate partners (45%). The factors associated with non-consistent condom use with the intimate partner were having had an abortion, feeling at risk for HIV, not evaluating one's own risk for HIV, living as a couple, being Dominican, not feeling comfortable asking intimate partners to use condoms. Although a high proportion of female sex workers declared using condoms with commercial partners, there is still room for improvement in the prevention of transmission with both commercial and intimate partners.
ISSN: 0956-4624

inserm-01065111
http://www.hal.inserm.fr/inserm-01065111
http://www.hal.inserm.fr/inserm-01065111/document
http://www.hal.inserm.fr/inserm-01065111/file/Table_III_IJSA.pdf
http://www.hal.inserm.fr/inserm-01065111/file/2014_Parriault_-_Predictive_factorssur unprotected_sex_for_female_sex_workers.pdf

Éditeur(s) : HAL CCSD Oxford University Press (OUP)
Résumé : International audience
Introduction: Most studies on nematode-malaria interactions were conducted outside of the Americas. The objective of the present study was thus to study the relation between malaria and nematodes in a cohort of children in an Amazonian village. Methods: Odds ratios for intestinal nematode infections as an explanatory variable to malaria resistant vs. malaria sensitive were computed. Results: Ascaris lumbricoides was significantly more frequent in the ‘resistant’ malaria group than in the ‘sensitive’ one. Conclusions: Despite its low statistical power, the present results find that Ascaris was associated with less malaria, as observed by a number of studies.
ISSN: 0142-6338

inserm-01410100
http://www.hal.inserm.fr/inserm-01410100
http://www.hal.inserm.fr/inserm-01410100/document
http://www.hal.inserm.fr/inserm-01410100/file/worms%20malaria%20camopi%20j%20trop%20pediatrics.pdf
http://www.hal.inserm.fr/inserm-01410100/file/GI%20nematodes%20tab%20aurelia%29.pdf

Éditeur(s) : HAL CCSD
Résumé : International audience
Aim The global increase in the rate of cesarean sections (CS) is currently an issue. We aimed to assess the rate of CS locally and to identify cases in which this procedure may have been avoidable. Methods In this prospective consecutive series, we analyzed the 478 CS carried out in our unit in 2009. We analyzed the characteristics of each case, and classified each as potentially avoidable or unavoidable. Results The total rate of CS was 24.0%, including 1.7% that was scored as potentially avoidable. Parity, gestational age at birth, birthweight, cases requiring cervical ripening, cases of labor induction, and CS during labor were all significantly higher or more frequent among potentially avoidable CS. Multivariate analysis indicated that the risk of potentially avoidable CS was positively associated with gestational age and tended to be negatively associated with parity. The main indications for potentially avoidable CS were cervical dystocia and abnormal fetal heart rate, and for unavoidable CS they were abnormal fetal heart rate and history of previous CS. Conclusion Labor, especially when induced, seems to be the key period for the prevention of ‘avoidable’ CS. This is particularly important given that potentially avoidable CS are more frequently associated with uncomplicated pregnancies than are unavoidable CS. A woman's first CS increases the likelihood of CS for subsequent deliveries, so the prevention of the first CS is a key aim for reducing the overall rate of CS
Journal of Obstetrics and Gynaecology Research

hal-01305484
https://hal-univ-rennes1.archives-ouvertes.fr/hal-01305484
PUBMED : 27094021
DOI : 10.1111/jog.13007

Éditeur(s) : HAL CCSD Taylor & Francis (Routledge)
Résumé : International audience
The Maroni basin, an isolated region delineating the border between Suriname and French Guiana has been affected by the human immunodeficiency virus (HIV) epidemic 10 years after coastal French Guiana. However, the rise in HIV prevalence was sharp, exceeding 1% within 10 years. The aim of the present study is to compare, using the first quantitative data from the general population in remote villages, the knowledge, attitudes, and behaviors regarding HIV between Maroon and Amerindian populations, the two most frequent populations living along the Maroni. Data were collected in 2012 using a structured questionnaire among a random sample of 896 individuals residing in the remote villages on the Maroni river. Proportions were compared between the Maroni and the coastal general population, and between Maroon and Amerindian populations. The present study shows significant differences between territories and between communities living on the Maroni river: the multiple sexual partnerships, more common among population living on the Maroni river, were more frequently reported in Maroons than in Amerindians. Condom use was more frequently reported among men on the Maroni river than on the coast, but these findings were reversed for women. Moreover, among people living on the Maroni river, condom use was more frequently reported among Maroons than among Amerindians. Regarding genital factors that may affect transmission, penile implant seemed to have no ethnic boundaries, steam baths seemed specific to Maroon women. The present results should help to improve community-based specific interventions.
ISSN: 0954-0121

hal-01260915
https://hal.archives-ouvertes.fr/hal-01260915
https://hal.archives-ouvertes.fr/hal-01260915/document
https://hal.archives-ouvertes.fr/hal-01260915/file/2015%2C%20Van%20Melle%20-%20Knowledge%2C%20attitudes%2C%20behaviors%2C%20and%20practices%20differences%20regarding%20HIV.pdf
DOI : 10.1080/09540121.2015.1032203

Éditeur(s) : HAL CCSD Ferrata Storti Foundation
Résumé : International audience
BACKGROUND: Little is known about the effects of blood rheology on the occurrence of acute chest syndrome and painful vaso-occlusive crises in children with sickle cell anemia and hemoglobin SC disease. DESIGN AND METHODS: To address this issue, steady-state hemorheological profiles (blood viscosity, red blood cell deformability, aggregation properties) and hematologic parameters were assessed in 44 children with sickle cell anemia and 49 children with hemoglobin SC disease (8-16 years old) followed since birth. Clinical charts were retrospectively reviewed to determine prior acute chest syndrome or vaso-occlusive episodes, and rates of these complications were calculated. RESULTS: Multivariate analysis revealed that: 1) a higher steady-state blood viscosity was associated with a higher rate of vaso-occlusive crises in children with sickle cell anemia, but not in children with hemoglobin SC disease; 2) a higher steady-state red blood cell disaggregation threshold was associated with previous history of acute chest syndrome in children with hemoglobin SC disease and boys with sickle cell anemia. CONCLUSIONS: Our results indicate for the first time that the red blood cell aggregation properties may play a role in the pathophysiology of acute chest syndrome in children with hemoglobin SC disease and boys with sickle cell anemia. In addition, whereas greater blood viscosity is associated with a higher rate of vaso-occlusive crises in children with sickle cell anemia, no association was found in children with hemoglobin SC disease, underscoring differences in the etiology of vaso-occlusive crises between sickle cell anemia and hemoglobin SC disease.
ISSN: 0390-6078

inserm-00849038
http://www.hal.inserm.fr/inserm-00849038
http://www.hal.inserm.fr/inserm-00849038/document
http://www.hal.inserm.fr/inserm-00849038/file/inserm-00849038.pdf
DOI : 10.3324/haematol.2012.066670

Éditeur(s) : HAL CCSD Elsevier Masson
Résumé : International audience
Background. An association with cancer is described in 17–32% of cases of dermatomyositis (DM) and in 5–16% of cases of anti-synthetase syndrome (ASS). The literature contains very few studies involving Afro-Caribbean patients with DM or ASS. The aim of our retrospective study was to determine the prevalence of cancer in a series of patients with DM or ASS at the University Hospital of Pointe-à-Pitre between 1st January 2000 and 31st December 2012. The secondary objective was to review the clinical and laboratory features as well as the course of DM/ASS in these patients.Patients and methods. The inclusion criteria were as follows: Afro-Caribbean origin; age >15 years; patient living in Guadeloupe; screening for malignancy.Results. Twenty-two patients were included (15 DM, 7 ASS). Only one case of cancer was diagnosed in the entire study population at a mean follow-up of 6±4 years (prevalence: 6.7%, CI95% [1.7–31.9]). Of the 15 patients presenting DM (sex ratio F/M: 4, mean age: 45±14 years), 6 (40%) had associated connective tissue disease.Conclusion. Our study suggests a weak association between DM and cancer in Afro-Caribbean patients. These results may be explained by the features of the disease seen in these patients (female gender, young age at onset, associated connective tissue disease) and the low prevalence in the Caribbean region of cancers typically associated with DM.
Introduction. Dix-sept à 32 % des cas de dermatomyosite (DM) et 5 à 16 % des cas de syndrome des anti-synthétases (SAS) sont associés à un cancer. Les séries de patients afro-caribéens atteints de DM ou de SAS sont exceptionnellement rapportées dans la littérature. Le but de notre étude rétrospective était d’estimer la prévalence des cancers dans une série de patients guadeloupéens atteints de DM ou de SAS. L’objectif secondaire était de préciser les caractéristiques cliniques, biologiques et évolutives de la maladie chez ces patients.Patients et méthode. Les critères d’inclusion étaient les suivants : patient afro-caribéen ; âge >15ans ; patient vivant en Guadeloupe ; réalisation d’un bilan d’extension à la recherche d’une néoplasie.Résultats. Vingt-deux patients ont été inclus (15 atteints de DM, 7 de SAS). Un seul cancer a été diagnostiqué, avec une durée moyenne de suivi de 6±4ans sur l’ensemble de la série (prévalence de 6,7 % ; IC 95 % [1,7 ; 31,9]). Parmi les 15 patients atteints de DM (sex-ratio H/F=1/4 ; âge moyen : 45±14ans), 6 (40 %) avaient une connectivite associée.Conclusion. Notre étude suggère une faible association entre DM et cancer chez les patients afro-caribéens. Ces résultats s’expliquent principalement par les caractéristiques de la maladie chez ces patients (sexe féminin, jeune âge, connectivite associée) et la faible prévalence, aux Antilles, des cancers classiquement associés aux DM.
ISSN: 0151-9638

inserm-01110999
http://www.hal.inserm.fr/inserm-01110999
http://www.hal.inserm.fr/inserm-01110999/document
http://www.hal.inserm.fr/inserm-01110999/file/2014%2C%20Tersiguel%20-%20Epid%C3%A9miologie%20de%20la%20dermatomyosite%20et%20du%20syndrome%20des%20anti-synth%C3%A9tases%20en%20population%20afro-carib%C3%A9enne.pdf
PUBMED : 25288059