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2018-03-22
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THE PHENOTYPIC SPECTRUM OF CONGENITAL ZIKA SYNDROME
Del Campo, Miguel et al. | Date Issued: 2017
Author
Del Campo, Miguel
Feitosa, Ian M. L.
Ribeiro, Erlane M.
Horovitz, Dafne D. G.
Pessoa, André L.S.
França, Giovanny V. A.
García-Alix, Alfredo
Doriqui, Maria J. R.
Wanderley, Hector Y. C.
Sanseverino, Maria V. T.
Neri, João I. C. F.
Pina-Neto, João M.
Santos, Emerson S.
Verçosa, Islane
Cernach, Mirlene C. S. P.
Medeiros, Paula F. V.
Kerbage, Saile C.
Silva, André A.
van der Linden, Vanessa
Martelli, Celina Maria Turchi
Cordeiro, Marli T.
Dhalia, Rafael
Vianna, Fernanda S. L.
Victora, Cesar G.
Cavalcanti, Denise P.
Schuler-Faccini, Lavinia
Affilliation
University of California San Diego. Department of Pediatrics. Division of Dysmorphology and Teratology. San Diego, California.
Universidade Federal de Rio Grande do Sul. Departamento de Genética. Porto Alegre, Rio Grande do Sul, Brazil / Universidade Federal de Pernambuco. Departamento de Medicina Clínica. Recife, Brazil.
Hospital Infantil Albert Sabin, Fortaleza, Ceara, Brazil.
Fundação Oswaldo Cruz. Instituto Nacional de Saúde da Mulher, da Criança e do Adolescente Fernandes Figueira. Rio de Janeiro, RJ, Brasil.
Hospital Infantil Albert Sabin, Fortaleza, Ceara, Brazil.
Ministry of Health. Secretariat of Health Surveillance. Brasilia, Brazil.
Universitat de Barcelona, Institut de Recerca Pediàtrica Sant Joan de Déu. Barcelona, Spain.
Hospital Infantil Dr. Juvêncio Mattos, São Luiz, Brazil.
Secretaria de Estado da Saúde do Espírito Santo, Vitória, Brazil.
SIAT-Brazilian Teratogen Information Service, Medical Genetics Service, Hospital de Clinicas de Porto Alegre, Porto Alegre, Rio Grande do Sul, Brazil.
Universidade Potiguar, Natal, Brazil.
Faculdade de Medicina de Ribeirao Preto, Departamento de Genetica, Universidade de Sao Paolo, Ribeirao Preto, Brazil.
Universidade Federal de Sergipe, Lagarto, Brazil.
Centro de Aperfeiçoamento Visual Ver a Esperança Renascer. Fortaleza, Brazil.
Universidade Federal de Sao Paulo. Departamento de Genetica Medica. Sao Paolo, Brazil.
Universidade Federal de Campina Grande, Campina Grande, Paraiba, Brazil.
Hospital Infantil Albert Sabin, Fortaleza, Ceara, Brazil.
Universidade Federal de Rio Grande do Sul. Departamento de Genética. Porto Alegre, Rio Grande do Sul, Brazil / Universidade Federal de Pernambuco. Departamento de Medicina Clínica. Recife, Brazil. / Brazilian Teratogen Information Service, Medical Genetics Service. Hospital de Clinicas de Porto Alegre, Porto Alegre, Rio Grande do Sul, Brazil / UNIVATES University, Porto Alegre, Rio Grande do Sul, Brazil.
Associação de Assistência à Criança Deficiente, Recife, Brazil.
Fundação Oswaldo Cruz. Centro de Pesquisas Aggeu Magalhães, Recife, Brazil.
Fundação Oswaldo Cruz. Centro de Pesquisas Aggeu Magalhães, Recife, Brazil.
Fundação Oswaldo Cruz. Centro de Pesquisas Aggeu Magalhães, Recife, Brazil.
Universidade Federal de Rio Grande do Sul. Departamento de Genética. Porto Alegre, Rio Grande do Sul, Brazil / Universidade Federal de Pernambuco. Departamento de Medicina Clínica. Recife, Brazil. / Brazilian Teratogen Information Service, Medical Genetics Service, Hospital de Clinicas de Porto Alegre, Porto Alegre, Rio Grande do Sul, Brazil.
Graduate Program in Epidemiology, Federal University of Pelotas, Pelotas, Rio Grande do Sul, Brazil.
Universidade de Campinas. Departamento de Genetica Medica. Campinas, Brazil.
Universidade Federal de Rio Grande do Sul. Departamento de Genética. Porto Alegre, Rio Grande do Sul, Brazil / Universidade Potiguar, Natal, Brazil.
Abstract
In October 2015, Zika virus (ZIKV) outbreak the Brazilian Ministry of Health (MoH). In response, the Brazilian Society of Medical Genetics established a task force (SBGM-ZETF) to study the phenotype of infants born with microcephaly due to ZIKV congenital infection and delineate the phenotypic spectrum of this newly recognized teratogen. This study was based on the clinical evaluation and neuroimaging of 83 infants born during the period from July, 2015 to March, 2016 and registered by the SBGM-ZETF. All 83 infants had significant findings on neuroimaging consistent with ZIKV congenital infection and 12 had confirmed ZIKV IgM in CSF. A recognizable phenotype of microcephaly, anomalies of the shape of skull and redundancy of the scalp consistent with the Fetal Brain Disruption Sequence (FBDS) was present in 70% of infants, but was most often subtle. In addition, features consistent with fetal immobility, ranging from dimples (30.1%), distal hand/finger contractures (20.5%), and feet malpositions (15.7%), to generalized arthrogryposis (9.6%), were present in these infants. Some cases had milder microcephaly or even a normal head circumference (HC), and other less distinctive findings. The detailed observation of the dysmorphic and neurologic features in these infants provides insight into the mechanisms and timings of the brain disruption and the sequence of developmental anomalies that may occur after prenatal infection by the ZIKV.
Keywords in Portuguese
Zika virus
Keywords
Congenital Zika sequence
Congenital Zika Sequence
Congenital Zika Ssyndrome
Disruption
Disruptive Sequence
Dysmorphology
Fetal Brain Disruption Sequence
Teratogen
Teratology
Zika Virus
 
DeCS
Zika virus
Publisher
Wiley
Citation
DEL CAMPO, Miguel. etal. The phenotypic spectrum of congenital Zika syndrome. American Journal of Medical Genetics Part A. v. 173, n. 4, p. 841-857, 2017
DOI
10.1002/ajmg.a.38170
ISSN
1552-4833