Angiostrongylus cantonensis Infection of Central Nervous System, Guiana Shield.

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.

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

What's childhood asthma in French Guiana? A cohort study based on children referred for allergology consultations at the Cayenne hospital center.

Background: Asthma is a multifactorial chronic disease, whose most frequent etiology is allergy, especially to Blomia tropicalis. In French Guiana, the childhood prevalence of Blomia T sensitization is unkwown. The aim of this study was to determine the proportion of sensitization to Blomia T and other mites in asthmatic children, and to describe the characteristics of childhood asthma in French Guiana. Methods: A retrospective cohort study focused on children from 0 to 18 years of age, followed for asthma at the Department of Pediatrics of the Cayenne Hospital Center in French Guiana. All asthmatic children followed by the same paediatric allergist were systematically skin-tested with Bt total extract, and Bt-specific IgE tests were additionally performed to confirm specific sensitization. All follow-up variables were collected from medical records. The outcome was sensitization to Blomia tropicalis and other allergens, and the explanatory variables were those of asthma follow-up. Patients were categorized into Blomia tropicalis sensitization yes/no. Logistic regression analysis was used to assess the relationship between follow-up variables and the outcome. Results: 302 patients were followed: 177 cases of allergic rhinitis, 135 allergic conjunctivitis, 105 atopic dermatitis, 153 food allergy, and 14 cases of drug allergy. Poly-allergy (respiratory, food, skin, and medicinal) was present in 239 children. There were 158 children followed for asthma, of whom 103 (65%) were sensitized to Blomia tropicalis. The median age of the asthmatic children sensitized to Blomia tropicalis was 7 years, and 3 years for those who were not sensitized (p < 0.001). Among the girls (n = 58), 67% were sensitized to Blomia; 97 (92%) asthmatic children co-sensitized to Blomia tropicalis, Dermatophagoides pteronyssinus, and Dermatophagoides farinae. Multivariate analysis showed that the childhood asthma in French Guiana is characterized by a median age of 7 years (p < 0.001), a high prevalence of Blomia tropicalis (p < 0.001), co-sensitization to other mites (p < 0.001), and a high prevalence of co-sensitization to cockroaches (p = 0.006). The area under the ROC curve was close to 0.9, confirming the quality of our model. Conclusion: In French Guiana, asthma is characterized by a high prevalence of Blomia tropicalis sensitization.

Developmental and epileptic encephalopathy related to a heterozygous variant of the RHOBTB2 gene: A case report from French Guiana.

Here we report a case of developmental and epileptic encephalopathy related to RHOBTB2 gene mutation in a ten-month old infant in French Guiana. Although the 28 previously reported cases had early-onset epilepsy and severe intellectual disability, here the reported individual presented with late postnatal onset of microcephaly and the absence of cortical atrophy on MRI. The publication of cases of such a rare form of developmental and epileptic encephalopathy will eventually allow us to better understand the mechanism by which RHOBTB2 misregulation could induce severe and atypical neurological disorders.

Low Stroke Risk in Children With Sickle Cell Disease in French Guiana: A Retrospective Cohort Study.

One in every 227 babies born in French Guiana has sickle cell disease, which represents the greatest incidence in France. This study aimed to determine the incidence of stroke in children with sickle cell disease and its associated risk factors. This retrospective cohort study included all children with sickle cell disease diagnosed in the neonatal period who were born in French Guiana between 01/01/1992 and 12/31/2002. Of a total of 218 records, 122 patients were included. There were 70 HbSS/Sß0 (58%), 40 HbSC (33%), and 11 Sß + thalassemia (9%). The number of emergency admissions was significantly different between genotypes, with a higher number in SS/Sß0 children (p = 0.004). There were significantly more acute chest syndromes (p = 0.006) and more elevated Lactate Dehydrogenase in SS/Sß0 patients (p = 0.003). Three of these patients had ischemic strokes at a mean age of 6.9 years, and one had a hemorrhagic stroke at the age of 9,2 years. The incidence rate of ischemic stroke for SS/Sß0 children was 3.1 (95% CI: 1.0-9.7) per 1,000 patient-years, and the clinically apparent stroke risk by the age of 15 years and 3 months was 6,4%. The incidence of hemorrhagic stroke was 1.1 (95% CI: 0.1-7.4) per 1,000 patients-years. No patient with SC or Sß + thalassemia genotypes experienced any stroke.

Hemiplegic Migraine Associated With PRRT2 Variations: A Clinical and Genetic Study.

BACKGROUND AND OBJECTIVE: PRRT2 variants have been reported in a few cases of patients with hemiplegic migraine. To clarify the role of PRRT2 in familial hemiplegic migraine, we studied this gene in a large cohort of affected probands. METHODS: PRRT2 was analyzed in 860 probands with hemiplegic migraine, and PRRT2 variations were identified in 30 probands. Genotyping of relatives identified a total of 49 persons with variations whose clinical manifestations were detailed. RESULTS: PRRT2 variations were found in 12 of 163 probands who previously tested negative for CACNA1A, ATP1A2, and SCN1A variations and in 18 of 697 consecutive probands screened simultaneously on the 4 genes. In this second group, pathogenic variants were found in 105 individuals, mostly in ATP1A2 (42%), followed by CACNA1A (26%), PRRT2 (17%), and SCN1A (15%). The PRRT2 variations included 7 distinct variants, 5 of which have already been described in persons with paroxysmal kinesigenic dyskinesia and 2 new variants. Eight probands had a deletion of the whole PRRT2 gene. Among the 49 patients with variations in PRRT2, 26 had pure hemiplegic migraine and 16 had hemiplegic migraine associated with another manifestation: epilepsy (8), learning disabilities (5), hypersomnia (4), or abnormal movement (3). Three patients had epilepsy without migraine: 2 had paroxysmal kinesigenic dyskinesia without migraine, and 1 was asymptomatic. DISCUSSION: PRRT2 should be regarded as the fourth autosomal dominant gene for hemiplegic migraine and screened in any affected patient, together with the 3 other main genes. Further studies are needed to understand how the same loss-of-function PRRT2 variations can lead to a wide range of neurologic phenotypes, including paroxysmal movement disorder, epilepsy, learning disabilities, sleep disorder, and hemiplegic migraine.

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

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

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.

Salmonella enterica serovar Panama meningitis in exclusive breastfeeding infants: Report of 4 cases, clinical features and therapeutic challenges.

RATIONALE: The pathway of Nontyphoid Salmonella meningitis, especially in exclusive breastfeeding infants, has not been well characterized. PATIENT CONCERNS: We analyzed data related to nontyphoid Salmonella meningitis in 4 infants. DIAGNOSES: No diarrhea was observed and the coproculture was negative for all patients. INTERVENTIONS: Early diagnosis and treatment with combination of third-generation cephalosporins plus quinolones for a minimum of 3 weeks is necessary to avoid severe sequelae and death. OUTCOMES: The first 3 patients had a good evolution, whereas the last patient had multiple brain abscesses and hydrocephalus requiring treatment with a ventriculoperitoneal shunt. LESSONS: The highlights of our study are that all infants were exclusively breastfed, no diarrhea observed and the negative coproculture for all the 4 patients, which is relatively rare for Salmonella infection.

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

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.

Chikungunya Infection in Hospitalized Febrile Infants Younger Than 3 Months of Age.

BACKGROUND: Fever in infants younger than 3 months is generally a cause for concern because of the risk for a serious bacterial infection. The aim of this study was to describe clinical and biologic features of Chikungunya infection in infants <3 months of age hospitalized in Cayenne Hospital during the 2014-2015 outbreak. METHODS: We performed a preliminary retrospective study followed by a prospective study from March 2014 to February 2015. All infants younger than 3 months presenting with fever and hospitalized in Cayenne Hospital were included. The main diagnostic criteria were fever and positive Chikungunya polymerase chain reaction. RESULTS: One hundred and twenty infants were hospitalized with fever. The mean age was 46 days (standard deviation ± 22 days). The mean hospitalization duration was 7.4 days (standard deviation ± 6.1 days). Chikungunya infection was diagnosed in 26 children. The most important clinical findings were high [80.8% (77.5-84)] and prolonged fever [76.9% (73.4-80.4)], irritability [96.2% (94.5-97.7)] and skin rash [69.2% (65.4-73)]. Half of the infants presented edema of the extremities (hands and feet principally). However, in 15% of infants, Chikungunya infection was associated with a serious bacterial infection. Infants who presented with irritability, high fever and elevated PCT were at high risk for Chikungunya: OR 39 (9.2-243; P < .001), with a specificity of 96.7% and a negative predictive value of 89.4%. The area of the receiver operating characteristic curve was 0.96. CONCLUSIONS: Our results confirm that Chikunguyna infection is a cause of high fever in infants younger than 3 months. Our data should be confirmed by larger studies.