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Globally, autosomal recessive IFNAR1 deficiency is a rare inborn error of immunity underlying susceptibility to live attenuated vaccine and wild-type viruses. We report seven children from five unrelated kindreds of western Polynesian ancestry who suffered from severe viral diseases. All the patients are homozygous for the same nonsense IFNAR1 variant (p.Glu386*). This allele encodes a truncated protein that is absent from the cell surface and is loss-of-function. The fibroblasts of the patients do not respond to type I IFNs (IFN-α2, IFN-ω, or IFN-β). Remarkably, this IFNAR1 variant has a minor allele frequency >1% in Samoa and is also observed in the Cook, Society, Marquesas, and Austral islands, as well as Fiji, whereas it is extremely rare or absent in the other populations tested, including those of the Pacific region. Inherited IFNAR1 deficiency should be considered in individuals of Polynesian ancestry with severe viral illnesses., (© 2022 Bastard et al.)

Congenital Zika virus syndrome consists of a large spectrum of neurologic abnormalities seen in infants infected with Zika virus in utero. However, little is known about the effects of Zika virus intrauterine infection on the neurocognitive development of children born without birth defects. Using a case-control study design, we investigated the temporal association of a cluster of congenital defects with Zika virus infection. In a nested study, we also assessed the early childhood development of children recruited in the initial study as controls who were born without known birth defects,. We found evidence for an association of congenital defects with both maternal Zika virus seropositivity (time of infection unknown) and symptomatic Zika virus infection during pregnancy. Although the early childhood development assessment found no excess burden of developmental delay associated with maternal Zika virus infection, larger, longer-term studies are needed.

The Zika virus crisis exemplified the risk associated with emerging pathogens and was a reminder that preparedness for the worst-case scenario, although challenging, is needed. Herein, we review all data reported during the unexpected emergence of Zika virus in French Polynesia in late 2013. We focus on the new findings reported during this outbreak, especially the first description of severe neurological complications in adults and the retrospective description of CNS malformations in neonates, the isolation of Zika virus in semen, the potential for blood-transfusion transmission, mother-to-child transmission, and the development of new diagnostic assays. We describe the effect of this outbreak on health systems, the implementation of vector-borne control strategies, and the line of communication used to alert the international community of the new risk associated with Zika virus. This outbreak highlighted the need for careful monitoring of all unexpected events that occur during an emergence, to implement surveillance and research programmes in parallel to management of cases, and to be prepared to the worst-case scenario., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

Background: The emergence of Zika virus in the Americas has coincided with increased reports of babies born with microcephaly. On Feb 1, 2016, WHO declared the suspected link between Zika virus and microcephaly to be a Public Health Emergency of International Concern. This association, however, has not been precisely quantified., Methods: We retrospectively analysed data from a Zika virus outbreak in French Polynesia, which was the largest documented outbreak before that in the Americas. We used serological and surveillance data to estimate the probability of infection with Zika virus for each week of the epidemic and searched medical records to identify all cases of microcephaly from September, 2013, to July, 2015. Simple models were used to assess periods of risk in pregnancy when Zika virus might increase the risk of microcephaly and estimate the associated risk., Findings: The Zika virus outbreak began in October, 2013, and ended in April, 2014, and 66% (95% CI 62-70) of the general population were infected. Of the eight microcephaly cases identified during the 23-month study period, seven (88%) occurred in the 4-month period March 1 to July 10, 2014. The timing of these cases was best explained by a period of risk in the first trimester of pregnancy. In this model, the baseline prevalence of microcephaly was two cases (95% CI 0-8) per 10,000 neonates, and the risk of microcephaly associated with Zika virus infection was 95 cases (34-191) per 10,000 women infected in the first trimester. We could not rule out an increased risk of microcephaly from infection in other trimesters, but models that excluded the first trimester were not supported by the data., Interpretation: Our findings provide a quantitative estimate of the risk of microcephaly in fetuses and neonates whose mothers are infected with Zika virus., Funding: Labex-IBEID, NIH-MIDAS, AXA Research fund, EU-PREDEMICS., (Copyright © 2016 Elsevier Ltd. All rights reserved.)

Introduction: The care of premature infants in French Polynesia is complicated by this country's geographic isolation. We undertook an evaluation of the medical care of very premature infants (VPIs) to find local solutions to this problem., Objectives: The objectives were to determine the incidence, mortality, and the short- and long-term outcome of very preterm infants in French Polynesia., Methods: We retrospectively reviewed the medical charts of all infants born alive at<32 gestational age (GA) and>24 GA from January 2007 to December 2011. Perinatal characteristics and outcomes were examined by univariate and multivariate analysis., Results: In total, 204 VPIs were born during the 5-year study period, comprising 0.9% of all births. Infants less than 28 GA comprised 0.1% of all births. Sixty-two percent of mothers were of extreme age including 43% less than 25 years old. Prematurity was attributed to spontaneous preterm labor in 63% of cases and preeclampsia in 29%. Spontaneous multiple pregnancies comprised 15% of the cases. Alcohol and tobacco consumption were frequently noted (>8% and 26% mothers, respectively). Seventy-eight percent of VPIs had received prenatal steroids. Intrauterine growth was normal in 89%. Mortality occurred in 9.3% (19 patients). Mortality was higher with lower gestational age (P<0.05) and absence of prenatal steroids (P<0.05) in univariate and multivariate analysis. The primary cause of death was sepsis. Hyaline membrane disease occurred in 44% of patients, 80% of whom received surfactant therapy. In total, 16.2% newborns developed bronchodysplasia, 3.4% necrotizing enterocolitis, 3% cerebral hemorrhage, and 1.5% leukomalacia. Long-term outcome was marked by 52% of the patients lost to follow-up by 2 years of age, mostly because of geographic isolation. For the 72 patients followed-up, four developed asthma and three cerebral palsy; 70% were attending school by 3 years of age., Conclusions: The incidence, mortality, and morbidity of very preterm birth in French Polynesia are comparable to reports from metropolitan centers in France. Conversely, nearly one-half of the patients were lost to follow-up, precluding meaningful information on intellectual development and other outcomes. We recommend organizing a long-term follow-up network to detect cognitive sequelae and adapting such a system to the geographical residence of French Polynesian families., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

We report here the first isolation and genotyping of two human Toxoplasma gondii strains from French Polynesia. The parasites had new and atypical genotypes, and were responsible for asymptomatic congenital toxoplasmosis. Both genotypes were divergent from the common strains isolated in Europe, North America, South America, Africa and China., (Copyright © 2014 Elsevier B.V. All rights reserved.)