Publications by authors named "Narcisse Elenga"

57 Publications

A call to start hydroxyurea by 6 months of age and before the advent of sickle cell disease complications.

Pediatr Blood Cancer 2021 Nov 24:e29423. Epub 2021 Nov 24.

Pediatric Hematology and Oncology, Boston Children's Hospital, Boston, Massachusetts, USA.

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http://dx.doi.org/10.1002/pbc.29423DOI Listing
November 2021

Red blood cell exchange in children with sickle cell disease.

Int J Hematol 2021 Sep 22. Epub 2021 Sep 22.

Centre Hospitalier de Cayenne, Rue des Flamboyants, BP 6006, 97306, Cayenne Cedex, French Guiana.

The aim of our study was to assess the efficacy of red blood cell exchange (RBCx) using a Spectra Optia® automated apheresis system in children with sickle cell disease (SCD). We used automated RBCx to treat acute and chronic complications in 75 children with SCD who had a median age of 10 years [7-13]. We analyzed 649 RBCx sessions. Peripheral venous access was limited in a number of the children, and thus a femoral double-lumen central venous catheter was required. We recommend heparin locking with 500 units in each lumen of the catheter. To prevent complications, we ensured that all patients had achieved a post-RCE HbS level of < 30%. For chronic transfusion, with a post-RCE Hb level of approximately 10-11 g/dL, a blood exchange volume of ≥ 32 mL/kg, and an interval between each RBCx procedure of ≤ 30 days, the residual HbS level was maintained below 30%. For acute transfusion, a post-exchange Hb level ≥ 10 g/dL (p < 0.001) and a total exchange volume ≥ 35 mL/kg (p = 0.001) were the best way to reduce HbS to < 30%. AUC was 0.84. Our results show that erythrocytapheresis was useful and safe for children with SCD.
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http://dx.doi.org/10.1007/s12185-021-03221-8DOI Listing
September 2021

Unilateral diplopia and ptosis in a child with COVID-19 revealing third cranial nerve palsy.

J Infect Public Health 2021 Sep 10;14(9):1198-1200. Epub 2021 Aug 10.

Department of Radiology, Andrée Rosemon Regional Hospital, Rue des Flamboyants, BP 6006, 97306 Cayenne Cedex, French Guiana.

We report the case of a 10-year-old boy with acute-onset diplopia and ptosis in the right eye. CR was positive for SARS-CoV-2. The patient was managed successfully with corticosteroids. We highlight the need for heightened suspicion of occult COVID-19 infection among children presenting with unusual III nerve palsy.
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http://dx.doi.org/10.1016/j.jiph.2021.08.007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8352849PMC
September 2021

Increased Prevalence of Alloimmunization in Sickle Cell Disease? Should We Restore Blood Donation in French Guiana?

Front Med (Lausanne) 2021 11;8:681549. Epub 2021 Jun 11.

Sickle Cell Disease Center, Andrée Rosemon Regional Hospital, Cayenne, French Guiana.

Patients with sickle cell disease often undergo frequent blood transfusions. This increases their exposure to red blood cell alloantigens of donor units, thus making it more likely that they produce alloantibodies. This cross-sectional study aimed to describe the prevalence of allo-immunization in patients with sickle cell disease who were monitored at Cayenne Hospital in 2016. Of the 451 patients recruited during the study period, 238 (52.8%) were female. There were 262 (58.1%) homozygous sickle cell and 151 (33.5%) compound heterozygous sickle cell patients. The median age of the participants was 23.09 years (range, 0.5-68). We noted different red blood cell extended phenotypes: -in the Duffy system, the Fy- Fy-profile was found in 299 patients (66%);-for the Kidd system, the most represented profile was Jka+ Jkb-, with 213 patients (47%). The Jka antigen was present in 355 patients;-in the MNS system, the S-s+ profile was found in 297 patients (66%);-the Lea antigen of the Lewis system was absent in 319 patients. The most frequent Rh phenotype in our patients was D+ C- E- c+ e+ K-, representing 51% of the patients. A total of 6,834 transfused packed red blood cell units were recorded. Sixty-eight patients (23%; 95% confidence interval, 20-25%) had detectable RBC alloantibodies. In multivariate logistic regression, only the mean number of single transfusions was statistically higher for the alloimmunized patients ( < 0.04). Thirteen (19%) of the patients with alloimmunization developed a delayed hemolytic transfusion reaction, thus representing 4.4% of the total number of transfused patients. Whether differences between donors from France vs. recipients from French Guiana could explain this high prevalence of alloimmunization to be examined. In conclusion, careful transfusion strategies for patients with RBC alloantibodies should allow further reduction of the rate of alloimmunization.
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http://dx.doi.org/10.3389/fmed.2021.681549DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8226117PMC
June 2021

Contribution of Research in the West Indies and Northeast Amazonia to Knowledge of the 2014-2015 Chikungunya Epidemic in the Americas.

Curr Trop Med Rep 2021 Jun 19:1-9. Epub 2021 Jun 19.

Service des Maladies Infectieuses et Tropicales, Centre Hospitalier de Cayenne, Cayenne, French Guiana.

Purpose Of Review: Although the chikungunya virus was discovered more than 60 years ago, it has only really been studied since the outbreak in La Reunion in 2005-2006. Ten years later, between 2014 and 2015, the chikungunya virus spread throughout the Americas, affecting millions of people. The objective of this review is to describe the contributions of research on chikungunya virus infection gained from epidemic in the West Indies and the Guiana Shield.

Recent Findings: Prevalence data were similar to those found in the Indian Ocean or Asia during epidemics. Clinically, there is now a better understanding of the typical, atypical, and severe forms. Several studies have insisted on the presence of neurological forms of chikungunya infection, such as encephalitis or Guillain-Barré syndrome. Cases of septic shock due to chikungunya virus as well as thrombotic thrombocytopenic purpura were described for the first time. Given the magnitude of the epidemic and the large number of people affected, this has led to a better description and new classifications of chikungunya virus infections in specific populations such as pregnant women, the elderly, and children. Several studies also described the behavior of populations faced with an emerging disease.

Summary: Current epidemiological data from tropical regions highlights the risk of spreading emerging diseases at higher latitudes, especially concerning arboviruses, since the vector is already established in many parts of northern countries. A better understanding of the disease and its epidemic dynamics will foster better management, the crucial importance of which was demonstrated during the COVID-19 epidemic.
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http://dx.doi.org/10.1007/s40475-021-00242-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8214063PMC
June 2021

Case Report: Acute Kidney Failure due to Massive Envenomation of a Two-Year-Old Child Caused by Killer Bee Stings.

Am J Trop Med Hyg 2021 May 10. Epub 2021 May 10.

1Service de Médecine et Chirurgie Pédiatrique, Cayenne Hospital, Cayenne, French Guiana.

A hybrid species of Brazilian bee has proliferated on the South American continent since 1956. We describe a "killer bee" swarm attack on a 2-year-old girl in French Guiana. The patient weighed 10 kg, and approximately hundreds of bees' stingers were removed, that is, 10 stings/kg. Our patient survived without long-term sequelae. The management of her condition required admission into intensive care for renal failure due to acute tubular necrosis and severe rhabdomyolysis. We emphasize the importance of early medical intervention, clinical surveillance, and biological monitoring at the hospital to prevent a toxic chain reaction that could prove fatal within 72 hours.
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http://dx.doi.org/10.4269/ajtmh.20-1276DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8274777PMC
May 2021

Association between maternal human immunodeficiency virus infection and preterm birth: A matched case-control study from a pregnancy outcome registry.

Medicine (Baltimore) 2021 Jan;100(4):e22670

Centre d'Investigation Clinique Antilles-Guyane, Inserm 1424, Andrée Rosemon Regional Hospital, Rue des flamboyants, Cayenne Cedex, French Guiana.

Abstract: This study aimed to evaluate the relationships between different types of antiretroviral therapy (ART) and preterm birth.Preterm birth was studied among all singleton pregnancies and compared between human immunodeficiency virus (HIV)-infected and uninfected women.We performed a matched case-control study from the pregnancy outcome registry of Cayenne Hospital. HIV-infected and uninfected women who delivered in the maternity ward of Cayenne Hospital from January 1, 2013 to December 31, 2015 were studied. We conducted an initial analysis to determine the risk factors for preterm birth among HIV-infected pregnant women. We also evaluated associations between exposure to antiretroviral therapy (ART) and preterm birth.There were 8682 deliveries; of these, 117 involved HIV-infected women, representing a prevalence of 1.34%. There were 470 controls. The sociodemographic characteristics were comparable. HIV-infected women were more likely to experience preterm birth (adjusted odds ratio [AOR] = 3.9, 95% confidence interval [CI] 1.5-9.9). Overall, 95.73% of the women received antiretroviral therapy before becoming pregnant, and they were in good clinical condition. The median CD4 count at the beginning of pregnancy was 500 cells/mm3 (357-722). Additionally, 53% of HIV-infected women had an undetectable viral load count (<20 copies/mL). Their median haemoglobin level was 120 g/L (100-120). There were 2 human immunodeficiency virus-infected babies. A higher rate of preterm birth was associated with protease inhibitor-based ART than a reverse transcriptase inhibitor-based ART regimen. The sample size being small this result would be considered with caution.The preterm birth rate among HIV-infected pregnant women was twice that of the general population; this trend was not explained by sociodemographic characteristics. Preterm birth was independently associated with combination ART, especially with ritonavir-boosted protease inhibitor therapy during pregnancy.
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http://dx.doi.org/10.1097/MD.0000000000022670DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7850744PMC
January 2021

In utero Zika virus exposure and neurodevelopment at 24 months in toddlers normocephalic at birth: a cohort study.

BMC Med 2021 01 21;19(1):12. Epub 2021 Jan 21.

Emerging Diseases Epidemiology Unit, Institut Pasteur, Paris, France.

Background: In utero exposure to Zika virus (ZIKV) is known to be associated with birth defects. The impact of in utero ZIKV exposure on neurodevelopmental outcomes in early childhood remains unclear. The objective of this study was to determine the impact of in utero ZIKV exposure on neurodevelopment at 24 months of age among toddlers who were born normocephalic to women who were pregnant during the 2016 ZIKV outbreak in French territories in the Americas.

Methods: We conducted a population-based mother-child cohort study of women whose pregnancies overlapped with the 2016 ZIKV epidemic in Guadeloupe, Martinique, and French Guiana. Infants were included in this analysis if maternal ZIKV infection during pregnancy could be determined, the newborn had a gestational age ≥ 35 weeks, there were no abnormal transfontanelle cerebral ultrasound findings after delivery or no abnormal ultrasound findings on the last ultrasound performed during the third trimester of the mother's pregnancy, there was an absence of microcephaly at birth, and the parent completed the 24-month neurodevelopment assessment of the infant at 24 months (± 1 month) of age. ZIKV exposure of the toddler was determined by evidence of maternal ZIKV infection during pregnancy. Neurodevelopment assessments included the Ages and Stages Questionnaire (ASQ) for five dimensions of general development-communication, gross motor, fine motor, problem solving, and personal-social skills; the Modified Checklist for Autism on Toddlers (M-CHAT) for behavior; and the French MacArthur Inventory Scales (IFDC) for French language acquisition.

Results: Between June 2018 and August 2019, 156 toddlers with and 79 toddlers without in utero ZIKV exposure completed neurodevelopment assessments. Twenty-four (15.4%) ZIKV-exposed toddlers and 20 (25.3%) ZIKV-unexposed toddlers had an ASQ result below the reference - 2SD cut-off (P = 0.10) for at least one of the five ASQ dimensions.

Conclusion: In one of the largest population-based cohorts of in utero ZIKV-exposed, normocephalic newborns to date, there were minimal differences apparent in neurodevelopment outcomes at 24 months of age compared to ZIKV-unexposed toddlers at 24 months of age.

Trial Registration: ClinicalTrials.gov, NCT02810210 . Registered 20 June 2016.
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http://dx.doi.org/10.1186/s12916-020-01888-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7819189PMC
January 2021

Pediatric scorpionism in northern Amazonia: a 16-year study on epidemiological, environmental and clinical aspects.

J Venom Anim Toxins Incl Trop Dis 2020 Sep 11;26:e202000038. Epub 2020 Sep 11.

Intensive Care Unit, Centre Hospitalier de Cayenne, Cayenne 97300, French Guiana, France.

Background: The Amazon basin is one of the seven major geographical areas where scorpionism is recorded. In French Guiana, 90 stings per 100,000 inhabitants are registered per year. As the severity of cases is higher in children, descriptive studies are needed to have a better understanding of this pathology. The aim of the present study is to describe pediatric scorpionism in French Guiana.

Methods: We conducted a monocentric descriptive retrospective study on scorpion stings in all pediatric patients admitted to Cayenne General Hospital from January 1, 2002 to December 31, 2018.

Results: In this survey, 132 patients were included. Of them, 63% were male. Patients with general signs of envenomation were younger and lighter (p = 0.04). The picture was "one sting" (95.3%) by a "big" (47.6%), "black" (60%) and "small pincer" (58%) scorpion on the extremity of the body (84%). Stings occurred mainly during the day, while patients changed clothes. There was no envenomation during night. The monthly evaluation highlights that the number of stings and percentage of general signs of envenomation were closely connected to a composite variable including the variation of the level of rivers (p = 0.005). Cardiac symptoms were recorded in 82% of cases with general signs of envenomation. The presence of pulmonary; ear, nose, and throat (ENT); or gastrointestinal symptoms are related to major envenomation (p = 0.001, p = 0.01, and p = 0.02 respectively). Leukocytosis and glycemia increased according to the envenomation grade whereas serum potassium and alkaline reserve decreased. Forty-six patients needed hospitalization and seven of them required intensive care. No patient died nor presented sequelae at discharge from the hospital.

Conclusion: Pediatric scorpionism in French Guiana is closely associated with child activities and climatic conditions. Severe envenomation presented most of the time with cardiac, pulmonary, and gastrointestinal symptoms.
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http://dx.doi.org/10.1590/1678-9199-JVATITD-2020-0038DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7485965PMC
September 2020

Fatal viral infections in hemoglobin sickle cell C patients.

Pediatr Blood Cancer 2021 02 8;68(2):e28668. Epub 2020 Sep 8.

Department of Immunology, Academic Hospital Paramaribo, Paramaribo, Suriname.

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http://dx.doi.org/10.1002/pbc.28668DOI Listing
February 2021

How I Treat Acute Chest Syndrome in Asthmatic Children with Sickle Cell Disease. A Practical Review.

Hemoglobin 2020 Sep 6;44(5):307-310. Epub 2020 Sep 6.

Service de Médecine et Chirurgie Pédiatrique, Centre Hospitalier de Cayenne, Cayenne Cedex, Guyanne Française.

Asthma is associated with increased rate of acute chest syndrome (ACS), pain episodes and premature death. Differentiating between an acute asthma exacerbation and ACS is a challenge clinically as they can present with similar symptoms. Clinicians should be aware of symptoms of asthma or broncho spasm in any children with sickle cell disease, as adequate treatments are required. In this mini-review, we selected 16 clinical studies, published in English between 2004 and 2016, and reviewed all of the abstracts and references of the selected articles. We subsequently selected articles that were focused on asthma in children with sickle cell disease. Given the pathophysiological mechanisms of ACS and the association between asthma and sickle cell disease, the management approach of asthmatic children should be clarified. Bronchodilators should be used if there are clinical features suggestive of a history of asthma or evidence of acute broncho spasm. The indication for cortisone should be reassessed. This literature review failed to conclude on therapeutic modalities of ACS in asthmatic children with sickle cell disease. Only a well designed, multicenter adequately-powered randomized controlled study of each of them will allow assessing their real benefits and risks.
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http://dx.doi.org/10.1080/03630269.2020.1814321DOI Listing
September 2020

Influence of beta-cluster haplotypes, alpha-gene status and UGTA1 polymorphism on clinical and hematological data in sickle-cell disease children from French Guiana.

PLoS One 2020 3;15(9):e0238691. Epub 2020 Sep 3.

Pediatric Unit, Cayenne General Hospital, Cayenne, French Guiana.

Objectives: This cross-sectional study aimed to investigate the influence of haplotypes, alpha-gene status and UGTA1 polymorphism on the severity of sickle cell disease in children.

Methods: This cross-sectional study was conducted between 2012 and 2014 at the Cayenne Hospital, in French Guiana. Acute clinical complications were grouped into (i) severe SCD defined by the presence of stroke and/or abnormal-transcranial Doppler (TCD), (ii) moderate SCD defined by the presence of at least three annual events requiring hospitalization and/or at least one acute chest syndrome, (iii) no severe SCD (in the absence of the precited events).

Results: Among the 86 patients, 33.7% were female with a median age of 10 years (range: 6-12 years). The vast majority of patients had SCA (HbSS) phenotype (74.4%; n = 64). The severe haplotype was found in 40% of patients. 30% were BEN/BEN. Analysis of α-globin gene deletions revealed that 32 patients (37.2%) were heterozygous (loss of 2 genes in 2 cases and loss of 1 gene in 30 cases) for α-thalassemia (3.7 kb deletion). Homozygous (TA) n TA7/7 was found in 24 (28%). In the multivariate analysis, the factors associated with the severity of sickle cell disease were the first vaso-occlusive crisis before one year of age (OR 25, [95% CI = 6.0-107.0], p<0.001) and a baseline MCV >80 fL (OR 0.20 [95% CI = 0.04-0.96], p = 0.04). The area of the ROC curve was 0.90.

Conclusion: Prospective studies with greater statistical power would provide more knowledge on the relationship between UGT1A1 mutations and the clinical and hematological manifestations of SCA.
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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0238691PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7470392PMC
October 2020

High fetal hemoglobin level is associated with increased risk of cerebral vasculopathy in children with sickle cell disease in Mayotte.

BMC Pediatr 2020 06 20;20(1):302. Epub 2020 Jun 20.

Pediatric Medicine and Surgery, Cayenne General Hospital, Cayenne, French Guiana, France.

Background: Understanding the genetics underlying the heritable subphenotypes of sickle cell anemia, specific to each population, would be prognostically useful and could inform personalized therapeutics.The objective of this study was to describe the genetic modulators of sickle cell disease in a cohort of pediatric patients followed up in Mayotte.

Methods: This retrospective cohort study analyzed clinical and biological data, collected between January12007 and December 312017, in children younger than 18 years.

Results: We included 185 children with 72% SS, 16% Sβ0-thalassemia and 12% Sβ + thalassemia. The average age was 9.5 years; 10% of patients were lost to follow up. The Bantu haplotype was associated with an increase in hospitalizations and transfusions. The alpha-thalassemic mutation was associated with a decrease of hemolysis biological parameters (anemia, reticulocytes), and a decrease of cerebral vasculopathy. The Single Nucleotide Polymorphisms BCL11A rs4671393, BCL11A rs11886868, BCL11A rs1427407 and HMIP rs9399137 were associated with the group of children with HbF > 10%. Patients with HbF > 10% presented a significant risk of early onset of cerebral vasculopathy.

Conclusions: The most remarkable result of our study was the association of SNPs with clinically relevant phenotypic groups. BCL11A rs4671393, BCL11A rs11886868, BCL11A rs1427407 and HMIP rs9399137 were correlated with HbF > 10%, a group that has a higher risk of cerebral vasculopathy and should be oriented towards the hemolytic sub-phenotype.
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http://dx.doi.org/10.1186/s12887-020-02187-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7305627PMC
June 2020

The Imperative of Early Treatment for Children With COVID-19 Infection.

Authors:
Narcisse Elenga

Indian Pediatr 2020 06 30;57(6):587-588. Epub 2020 Apr 30.

Pediatric Medicine and Surgery, Centre Hospitalier Andrée Rosemon, Rue des flamboyants, BP 6006, 97306, Cayenne Cedex, French Guiana.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7340737PMC
June 2020

The Imperative of Dengue Vaccination for Children with Sickle Cell Disease.

J Trop Pediatr 2020 06;66(3):245-247

Departments of Pediatrics and Global Health, University of Washington, Seattle, WA, USA.

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http://dx.doi.org/10.1093/tropej/fmaa021DOI Listing
June 2020

Etiology and prognosis of encephalitis in French Guianese children: a retrospective record-based study.

J Infect Public Health 2020 Jul 10;13(7):1051-1053. Epub 2020 Apr 10.

Pediatric Unit Cayenne General Hospital, Cayenne, French Guiana.

Acute encephalitis is an important cause of mortality and morbidity in children. We retrospectively identified children (≤15 years of age) admitted with suspected encephalitis at the Intensive Care Unit of the Pediatric Department of Cayenne Hospital between January 2007 and December 2018. A total of 30 children with acute encephalitis were identified. The incidence rate varied from 0 to 10.40 cases/100000 children under 15 years. Proven encephalitis was diagnosed in 73% of patients. Nine cases of acute disseminated encephalomyelitis were diagnosed. The causes of infection (44%) were Haemophilus influenzae, followed by Cryptococcus spp and Varicella Zoster Virus. Four children (13%) died: one case of Streptococcus pneumoniae, one of Haemophilus influenzae, one of Mycobacterium tuberculosis and one with no identified cause. Seventeen percent of children had moderate to severe neurological sequelae. The only factor associated with poor outcome was young age at the time of hospitalization (p = 0.03). Conclusion: This study highlights both vaccine-preventable pathogens and acute disseminated encephalomyelitis as the leading causes of childhood encephalitis in French Guiana.
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http://dx.doi.org/10.1016/j.jiph.2020.01.315DOI Listing
July 2020

Dengue in hospitalized children with sickle cell disease: A retrospective cohort study in the French departments of America.

J Infect Public Health 2020 Feb 20;13(2):186-192. Epub 2019 Sep 20.

Centre de référence de la drépanocytose aux Antilles-Guyane, French Guiana; Unité Transversale de la Drépanocytose, pôle Parents-Enfants, CHU de Pointe-à-Pitre/Abymes, Centre de référence de la drépanocytose aux Antilles-Guyane, French Guiana; UMR Inserm 1134/Université des Antilles-Guyane, French Guiana.

Background: To describe the characteristics of dengue in sickle cell children and try to identify risk factors of severity.

Methods: In this retrospective study, we describe the evolution according to genotype (SS or SC and controls) and severity.

Results And Conclusions: From 2005 to 2013, 106 hospitalizations for dengue fever were recorded, 35 SS genotype, 35 SC and 36 without SCD or any other chronic disease. The clinical evolution was quite different. During hospitalization, SC patients were more likely to develop multiorgan failure (31.4% versus 25.7% for SS, and 0% for controls, p=0.001), or acute pulmonary complications than patients without SC sickle cell disease (14.3% versus 8.6% for SS, and 0% for controls, p=0.03). Level 3 analgesic treatment was more frequent in SC patients (22.9% versus 3% for SS, and 0% for controls, p<0.001). Patients with SC sickle cell disease had a higher proportion of severe forms of dengue (57.1% versus 37.1% for SS, and 0% for controls, p<0.001) than patients without SC sickle cell disease. Transfer in intensive care unit was required for most SC patients (22.9% versus 3% for SS, and 0% for controls, p=0.005).Fatal episodes were more frequent in SC patients than in patients without SC sickle cell disease (5 deaths versus 1 for SS and 0 for controls, p=0.02). Thirty-three patients (47.1%) were diagnosed as having severe dengue (13 SS and 20 SC). On univariate analysis, age >10 years, acute pulmonary complications, multiorgan failure, severe anemia requiring transfusion, use of antibiotic treatment, need for treatment with morphine, and longer hospital stay were statistically more frequent in severe dengue-associated cases. Multiple logistic regression analysis showed that HbSC genotype and acute pulmonary complications, were significantly associated with severe dengue. In the multivariate model, the area of the ROC curve was 0.831. Children with SC genotype, typically thought to have less severe disease, actually had a higher rate of severe dengue and death than those with SS genotype.
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http://dx.doi.org/10.1016/j.jiph.2019.07.015DOI Listing
February 2020

Air Drep-A Retrospective Study Evaluating the Influence of Weather Conditions and Viral Epidemics on Vaso-Occlusive Crises in Patients with Sickle Cell Disease Living in French Guiana.

Int J Environ Res Public Health 2019 07 31;16(15). Epub 2019 Jul 31.

Sickle Cell Disease Center, Andrée Rosemon General Hospital, Cayenne 97300, French Guiana.

(1) Objectives: French Guiana is the French territory most affected by sickle cell disease (SCD). This study investigates the associations between different environmental factors relative to climate, infectious outbreaks, and emergency visits or weekly hospital admissions for vaso-occlusive crisis (VOC). The identification of risk factors would lead to better patient care and patient management, and more targeted prevention and therapeutic education for patients with SCD in French Guiana. (2) Methods: This study was performed using data collected from the medicalized information system and emergency medical records of Cayenne General Hospital, between 1 January 2010 and 31 December 2016. ARIMA models were used to investigate the potential impact of weather conditions and flu epidemics on VOC occurrence. (3) Results: During the study period, 1739 emergency visits were recorded among 384 patients, of which 856 (49.2%) resulted in hospitalization, 811 (46.6%) resulted in hospital discharge, and 72 (4.2%) in another orientation. Decreased temperature and decreased humidity were both independent factors associated with an increase of VOC cases ( = 0.0128 and = 0.0004, respectively). When studying severe VOC (leading to hospitalization, with or without prior emergency visit), 2104 hospital admissions were recorded for 326 patients. The only factor associated with severe VOC, in the multivariate analysis, was flu epidemics ( = 0.0148). (4) Conclusions: This study shows a link between climate, flu epidemics, and VOC in French Guiana. Patient's awareness of risks related to climate and flu epidemics should be encouraged, as home prevention measures can help avoid painful crises. Moreover, physicians should encourage patients to get immunized for influenza every year.
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http://dx.doi.org/10.3390/ijerph16152724DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6695746PMC
July 2019

Bone and joint infections with Staphylococcus aureus strains producing Panton-Valentine Leukocidin in French Guiana.

Medicine (Baltimore) 2019 Jul;98(27):e16015

Department of Pediatric Medicine and Surgery, Cayenne Hospital, Cayenne Cedex, French Guiana.

The aim of this study was to describe the clinical features of bone and joint infections (BJI) due to Panton-Valentine Leukocidin producing (PVL+) Staphylococcus aureus (SA) in French Guiana.A multicenter study that consists of a retrospective charts review of children admitted for PVL+ S. aureus BJI between January 2010 and December 2015.Six patients with SA-PVL BJI were identified during the study period: 2 osteomyelitis, 1 septic arthritis, and 3 disseminated BJI. The median age was 11 years old (4-14 years), and fever lasted for 3.2 days (2-5 days) before diagnosis. An open skin wound preceded the BJI in 5/6 patients. One patient presented with a septic thrombophlebitis of the femoral-popliteal vein on admission. Methicillin-susceptible Staphylococcus aureus (MSSA) were identified for all patients. Three patients had complications: 2 cases of necrotizing pneumonia and 2 pericarditis, with 1 death caused by cardiac tamponade.SA-PVL BJI was not frequent. Strains were susceptible to methicillin, but responsible of severe BJI. Early diagnosis and a multidisciplinary management of these infections are essential to prevent further complications.
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http://dx.doi.org/10.1097/MD.0000000000016015DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6635172PMC
July 2019

Newborn Screening for Sickle Cell Disease in the Caribbean: An Update of the Present Situation and of the Disease Prevalence.

Int J Neonatal Screen 2019 Mar 8;5(1). Epub 2019 Jan 8.

UMR Inserm 1134 Biologie Intégrée du Globule Rouge, Inserm/Université Paris Diderot-Université Sorbonne Paris Cité/INTS/Université des Antilles, Hôpital Ricou, Academic Hospital of Guadeloupe, 97159 Pointe-à-Pitre, Guadeloupe.

The region surrounding the Caribbean Sea is predominantly composed of island nations for its Eastern part and the American continental coast on its Western part. A large proportion of the population, particularly in the Caribbean islands, traces its ancestry to Africa as a consequence of the Atlantic slave trade during the XVI-XVIII centuries. As a result, sickle cell disease has been largely introduced in the region. Some Caribbean countries and/or territories, such as Jamaica and the French territories, initiated newborn screening (NBS) programs for sickle cell disease more than 20 years ago. They have demonstrated the major beneficial impact on mortality and morbidity resulting from early childhood care. However, similar programs have not been implemented in much of the region. This paper presents an update of the existing NBS programs and the prevalence of sickle cell disease in the Caribbean. It demonstrates the impact of the Caribbean Network of Researchers on Sickle Cell Disease and Thalassemia (CAREST) on the extension of these programs. The presented data illustrate the importance of advocacy in convincing policy makers of the feasibility and benefit of NBS for sickle cell disease when coupled to early care.
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http://dx.doi.org/10.3390/ijns5010005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7510201PMC
March 2019

High mortality due to congenital malformations in children aged < 1 year in French Guiana.

BMC Pediatr 2018 12 22;18(1):393. Epub 2018 Dec 22.

Service de pédiatrie, Centre Hospitalier Andrée Rosemon, Cayenne, French Guiana.

Background: In French Guiana, pregnant women may be exposed to infectious, environmental, and social risks leading to congenital malformation. The objective of the study was to study mortality rates from congenital malformations among infants < 1 year and to compare them with those in mainland France.

Methods: We used the CEPI DC (INSERM) database, which compiles annual data from death certificates in all French territories using the International Classification of Diseases. Annual deaths for French Guiana and mainland France between 2005 and 2015 were compiled. The age category studied was children less than 1 year and deaths from congenital malformations, deformations and chromosomal abnormalities were compiled. Crude risk ratios and 95% confidence intervals were calculated to quantify the excess risk of disease in French Guiana.

Results: In French Guiana between 2005 and 2015 there were 666 deaths of children aged < 1 year, among which, 132 (19.8%) were due to congenital malformations and chromosomal anomalies. Overall the risk ratio of death from congenital malformations and chromosomal anomalies between French Guiana and mainland France was 2.7 (1.5-4.7), P < 0.001 for neurological congenital malformations it was 4.8 (1.2-19.7), P = 0.01 and for congenital malformations of the circulatory system it was 3.3 (1.5-6.9), P = 0.001.

Conclusions: The incidence of death from congenital malformations or chromosomal anomalies in French Guiana was significantly higher than in mainland France. Explanations for this may be infections, genetic causes, nutritional causes, and toxic causes that are prevalent. There is a need to identify factors that predispose children born in French Guiana to having a higher risk of congenital malformations and chromosomal anomalies.
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http://dx.doi.org/10.1186/s12887-018-1372-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6303914PMC
December 2018

Clinical Features, Diagnosis, and Outcome of Encephalitis in French Guiana.

Am J Trop Med Hyg 2019 02;100(2):452-459

Intensive Care Unit, Cayenne General Hospital, Cayenne, French Guiana.

The aim of our study was to describe the clinical features, the etiologies, and the factors associated with poor outcome of encephalitis in French Guiana. Our study was retrospective, including all cases of encephalitis hospitalized in the Cayenne General Hospital, from January 2007 to July 2017. Patients were included through the 2013 encephalitis consortium criteria and the outcome was evaluated using the Glasgow outcome scale at 3 months from the diagnosis of encephalitis. We included 108 patients, giving an approximate incidence rate of four cases/100,000 inhabitants/year. The origin of the encephalitis was diagnosed in 81 cases (75%), and 72 of them (66.7%) were from an infectious origin. The most common infectious causes were sp. (18.5%) independently of the immune status, (13.9%), and (5.5%). In the follow-up, 48 patients (46.6%) had poor outcome. Independent risk factors associated with poor outcome at 3 months were "coming from inside area of the region" ( = 0.036, odds ratio [OR] = 4.19; CI 95% = 1.09-16.06), need for mechanical ventilation ( = 0.002, OR = 5.92; CI 95% = 1.95-17.95), and age ≥ 65 years ( = 0.049, OR = 3.99; CI 95% = 1.01-15.89). The most identified cause of encephalitis in French Guiana was . The shape of the local epidemiology highlights the original infectious situation with some local specific pathogens.
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http://dx.doi.org/10.4269/ajtmh.18-0308DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6367637PMC
February 2019

Pediatric Amazonian Toxoplasmosis Caused by Atypical Strains in French Guiana, 2002-2017.

Pediatr Infect Dis J 2019 03;38(3):e39-e42

From the Parasitology-Mycology, EA 3593, Faculty of Medicine, French West Indies and French Guiana University, French Guiana.

Background: Amazonian toxoplasmosis is a recently described form of Toxoplasma gondii infection, characterized by severe clinical and biological features and involvement of atypical genetic strains circulating through a forest-based cycle. Though mostly reported in French Guiana since 1998, this disease is probably under-diagnosed in other areas of South America. Few data are available on its specific features in children.

Methods: We retrospectively included all children seen in Cayenne between 2002 and 2017, diagnosed with acute toxoplasmosis due to an atypical strain. Clinical and biological features and risk factors of Amazonian toxoplasmosis were recorded.

Results: Eleven children met the inclusion criteria. The main clinical features were fever, lymphadenopathy, hepatomegaly and splenomegaly, as reported in adults. Less expected signs such as rhinorrhea, pharyngitis and maculopapular rash were also frequent. Cutaneous signs were associated with the involvement of a genetically atypical strain. Respiratory involvement was rare but associated with respiratory distress. Outcome was always favorable. Treatment by pyrimethamine/sulfadiazine or trimethoprim/sulfamethoxazole was always justified, efficient and well tolerated. Genetic analysis suggested the involvement of wild mammals in disease transmission, and strain-dependent virulence as one of the prognostic risk factors.

Conclusions: As in adults, pediatric Amazonian toxoplasmosis requires a systematic treatment. Symptoms such as skin rash, rhinorrhea and pharyngitis should not be mistaken for viral infections, when associated with lymphadenopathy or fever. Pediatricians working in South America should be familiar with these clinical presentations and with the different risk factors of Amazonian toxoplasmosis.
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http://dx.doi.org/10.1097/INF.0000000000002130DOI Listing
March 2019

Does Procalcitonin Predict Bacterial Infection in Febrile Children with Sickle Cell Disease?

Indian J Pediatr 2019 01 23;86(1):95-96. Epub 2018 Jun 23.

Centre d'Investigation Clinique Antilles-Guyane, Cayenne Hospital, Rue des Flamboyants, BP 6006, 97306, Cayenne Cedex, French Guiana.

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http://dx.doi.org/10.1007/s12098-018-2717-xDOI Listing
January 2019

Mother's obesity and high child's waist circumference are predictive factors of severe child's obesity: an observational study in French Guiana.

BMC Pediatr 2018 06 9;18(1):188. Epub 2018 Jun 9.

Department of Pediatric Medicine and Surgery, Cayenne Hospital, Rue des flamboyants, BP 6006, 97306, Cayenne Cedex, French Guiana.

Background: This study aims to describe the predictive factors of severe obesity in children followed in French Guiana.

Methods: In this observational study, the patients from the French Guianese Childhood Obesity Group database were prospectively included, after giving a statement of patient's non opposition.

Results: Our group classifications revealed that 36 of 150 (24%) participants were classified as being metabolically abnormal obesity" (MAO), while 114 of 150 (76%) were categorized as metabolically normal obesity" (MNO). MAO-patients were older. Their mothers had more severe obesity. We also observed that their systolic blood pressure was higher. The median Z-score BMI of children with MAO was 4, 9 [4, 05-5, 38], which shows a more obese condition than the MNO group. The median waist-to-height ratio (WTHR) of our study population was high, either 0.63 [0.54-0.59]. No significant differences in the term of pregnancy, father's obesity, gender, birth weight, feeding, diastolic blood pressure and WTHR were found between the two groups. The predictors of MAO status, after adjusting for age and sex, were mother's obesity and high child's waist circumference. Among the comorbidity, there were two Down syndrome, one Cornelia de Lange syndrome, one Nephrotic Syndrome and one Epilepsy. The leptin hormone and insulin levels were higher in MAO than in MNO, while 25-OH D-vitamin was higher in MNO.

Conclusion: This study indicates the need to incorporate waist circumference into routine clinical practice, in addition to traditional measures of weight, height, body mass index and waist-to-height ratio.
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http://dx.doi.org/10.1186/s12887-018-1158-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5994247PMC
June 2018

Modeling of the HIV epidemic and continuum of care in French Guiana.

PLoS One 2018 24;13(5):e0197990. Epub 2018 May 24.

Department of Dermatology, Centre Hospitalier de Cayenne, Cayenne, French Guiana.

Background: In order to compute the continuum of care for French Guiana, it is necessary to estimate the total number of persons living with HIV. The main objective was to determine how many persons were infected with HIV and how many were unaware of it.

Methods: We used 2 different models to calculate the total number of persons infected with HIV: Spectrum's AIM module using CSAVR to compute incidence from case registration and vital statistics; and the ECDC model from the French Guiana HIV cohort data.

Result: The present results show that both models led to similar results regarding the incident number of cases (i.e. for 2016 174 versus 161) and the total HIV population (in 2016 3206 versus 3539) respectively. The ECDC modeling tool showed that the proportion of undiagnosed HIV infections declined from 50% in 1990 to 15% in 2015. This amounted to a stable or slightly increasing total number of undiagnosed patients of 520.

Conclusions: The estimations of the total HIV population by both models show that the HIV population is still growing. The incidence rate declined in 2000 and the decline of the number of newly acquired HIV infections, after a decline after 2003 is offset by population growth. The proportion of undiagnosed infections has declined to 15% but the number of undiagnosed infections remains stable. The HIV cascade shows that despite good results for treatment in care, reaching the 90*90*90 UNAIDS target may be difficult because a significant proportion of patients are lost to follow-up.
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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0197990PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5967714PMC
December 2018

Angiostrongylus cantonensis Infection of Central Nervous System, Guiana Shield.

Emerg Infect Dis 2018 06;24(6):1153-1155

We report a case of eosinophilic meningitis complicated by transverse myelitis caused by Angiostrongylus cantonensis in a 10-year-old boy from Brazil who had traveled to Suriname. We confirmed diagnosis by serology and real-time PCR in the cerebrospinal fluid. The medical community should be aware of angiostrongyliasis in the Guiana Shield.
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http://dx.doi.org/10.3201/eid2406.180168DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6004866PMC
June 2018

Clinical features and prognosis of paraquat poisoning in French Guiana: A review of 62 cases.

Medicine (Baltimore) 2018 Apr;97(15):e9621

Service de Médecine et Chirurgie Pédiatrique, Centre Hospitalier de Cayenne, Rue des flamboyants, Cayenne Cedex, Guyane Française Université de Lille, UFR Médecine CHU Lille, Infection Control EA 7366, Pseudomonas aeruginosa Host-Pathogen Translational Research Group, Lille Département des centres délocalisés de prévention et de soins, Rue des flamboyants, Cayenne Cedex, Guyane Française Service de maladies infctieueses et du voyageur, Centre Hospitalier de Tourcoing-Hôpital Gustave Dron - 135, Tourcoing Département de l'information médicale, Centre Hospitalier de l'Ouest Guyane "Franck Joly"16 avenue du Général de Gaulle, Saint-Laurent-du-Maroni Département de l'information médicale, Centre Médico-chirurgical de Kourou, Kourou Département de l'information médicale, Centre Hospitalier de Cayenne, Rue des flamboyants, Cayenne Cedex, Guyane Française Service de Réanimation Médicale et Médecine Hyperbare, Hôpital Albert Calmette, Lille Cedex Service de néphrologie Service de urgences -SAMU Service de réanimation polyvalente, Centre Hospitalier de Cayenne, Rue des flamboyants, Cayenne Cedex, Guyane Française Centre anti pison, Centre Hospitalier Régional Universitaire de Lille, 2, Lille Cedex, France.

Paraquat is a nonselective contact herbicide of great toxicological importance, being associated with high mortality rates. Because of its high toxicity, the European Union withdrew it from its market in 2007. The aim of this study is to analyze all cases of paraquat poisoning hospitalized in French Guiana in order to assess their incidence and main characteristics.Medical records of all paraquat intoxicated patients hospitalized from 2008 until 2015 were reviewed in this retrospective study.Demographics, clinical presentation, and laboratory data were evaluated.A total of 62 cases were reviewed. The incidence of paraquat poisoning was 3.8/100,000 inhabitants/year. There were 44 adults and 18 children younger than 16 years of age. The median ages were 31 years [18.08-75.25] in adults and 13.4 years [0.75-15.08] in children, respectively. The median duration of hospitalization was longer in children [15.5 days (1-24)] than in adults [2 days (1-30)], P < .01. The majority of cases was due to self-poisoning (84%).Children had ingested a lower quantity of paraquat [48.8 mg/kg (10-571.1)] than adults [595.8 mg/kg (6-3636.4), P = .03]. There were more deaths among adults (65%) than in children (22%), P = .004. The severity and outcome was determined primarily by the amount of paraquat ingested.In conclusion, French Guiana has the largest cohort of paraquat poisonings in the European Union. The major factor affecting the prognosis of patients was the ingested amount of paraquat. The administration of activated charcoal or Pemba, in situ, within the first hour after ingestion of paraquat is essential.
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http://dx.doi.org/10.1097/MD.0000000000009621DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5908553PMC
April 2018

Incidence of infantile Pompe disease in the Maroon population of French Guiana.

BMJ Paediatr Open 2018 9;2(1):e000182. Epub 2018 Jan 9.

Department of Biochemistry, APHP-Robert Debré University Hospital, Paris, France.

Objectives: The aim of this study was to describe the epidemiology of infantile Pompe disease (IPD) in French Guiana, a French overseas territory, by combining a retrospective case records study and a prospective anonymous genotyping in a sample of mothers followed in the two major maternity units of French Guiana.

Methods: We identified 19 newborns with IPD born within a 13-year-period in French Guiana, corresponding to 1/4528 births. All children were born within the African-American Maroon (Bushinengue) community originating from slaves who settled along the Maroni river in the 19th century. We also performed an anonymised screening for all women in postpartum, in the two main maternity units of French Guiana.

Results: Genetic investigations revealed that all patients with IPD were homozygotes or compound heterozygotes for two known pathogenic variations: c.2560C>T p.(Arg854*) that has already been reported in African-Americans and c.1942G>A p.(Gly648Ser), a rare previously considered to be variant. We identified no heterozygotes among 453 mothers of various ethnicities in Cayenne, but 15 heterozygotes among 425 mothers (1/27) in Saint-Laurent-du-Maroni (95% CI 1/45 to 1/17), all from the Maroon community, which corresponds to an expected IPD incidence in Maroons of 1/1727 (95% CI 1/1156 to 1/8100).

Conclusion: The incidence of IPD in the Maroon community is roughly 50 times higher than elsewhere in the world. The presence of only two different variants in all affected patients is compatible with a double founder effect in a relatively small population that has seldom mixed with other regional populations in the past and therefore has a reduced pool of genotypes.
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http://dx.doi.org/10.1136/bmjpo-2017-000182DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5842995PMC
January 2018

Cerebral injuries associated with Zika virus in utero exposure in children without birth defects in French Guiana: Case report.

Medicine (Baltimore) 2017 Dec;96(51):e9178

Department of Pediatrics, Andrée Rosemon Hospital Centre d'Investigation Clinique Antilles Guyane CIC INSERM 1424 Department of Neonatal Pediatrics and Intensive Care, Andrée Rosemon Hospital, Cayenne, French Guiana Pediatric and Neonatal Intensive Care Unit, MFME Hospital, Fort de France, Martinique Neonatal Intensive Care, Pointe à Pitre Hospital, Pointe à Pitre, Guadeloupe, France.

Rationale: A major epidemic of Zika virus (ZIKV) infection occurred in French Guiana and West Indies. French national epidemiological surveillance estimated that 1650 pregnant women contracted the ZIKV during epidemic period from January 2016 to October 2016 in French Guiana.

Patient Concerns: ZIKV infection during pregnancy is a cause of microcephaly and birth defects.

Diagnoses: In this report, we describe 2 children with proven in utero ZIKV exposure. Their mothers were both symptomatic and ZIKV infection occurred early in pregnancy. Ultrasonography monitoring in utero did not show any abnormality for both patient. They were born at full-term, healthy, without any birth defects and no sign of congenital ZIKV infection.

Interventions: ZIKV was neither found on placenta fragments nor children blood and urine at birth. Their neurodevelopment outcomes in early-life fitted the expectations. As recommended in national guidelines, we performed cerebral MRIs at 2 months old, showing severe brain abnormalities, especially of white matter areas. After a large screening, we did not find any differential diagnosis for their brain lesions.

Outcomes: We concluded it was due to their in utero ZIKV exposure.

Lessons: In this report, pathogenicity of ZIKV may involve mother's immunological response or metabolic disorder during the infection.
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http://dx.doi.org/10.1097/MD.0000000000009178DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5758157PMC
December 2017
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