Holoprosencefalia: Análise do seu espectro morfológico em doze casos de autópsia

Lúcia De Noronha; Ramon Coral Ghanem; Fabíola Medeiros; José Knopfholz; Tiago Augusto Magalhães; Gilberto Antunes Sampaio; Maria José Serapião; Luiz Fernando Bleggi Torres

doi:10.1590/s0004-282x2001000600014

Holoprosencefalia: Análise do seu espectro morfológico em doze casos de autópsia

Translated title of the contribution: Holoprosencephaly: Morphological aspects of twelve cases of autopsy

Lúcia De Noronha, Ramon Coral Ghanem, Fabíola Medeiros, José Knopfholz, Tiago Augusto Magalhães, Gilberto Antunes Sampaio, Maria José Serapião, Luiz Fernando Bleggi Torres

Research output: Contribution to journal › Article › peer-review

Abstract

The term holoprosencephaly (HPC) is used to indicate the group of hemispheric deformities caused by a failure in the development of the prosencephalic vesicle. The purpose of this study is to explain the morphologic specter of twelve cases of HPC, qualify them, and compare them to the literature. It was evaluated 5837 pediatrics necropsies, and there were 12 cases of HPC. Data like gender, age and the presence of another associated malformations were evaluated and compared to another malformations and to the total number of necropsies. The majority of the cases was male (66.66%), and stillborns (75%). The most frequently type of HPC found was the lobar type (58.3%), and the most frequently type of facial alteration was the ciclopy (25%). There is a statistic tendency to HPC affect the male sex, in comparison with other neurologic malformations. Besides, the stillborns are more frequently observed in HPC than in other neurologic malformations.

Translated title of the contribution	Holoprosencephaly: Morphological aspects of twelve cases of autopsy
Original language	Portuguese (Brazil)
Pages (from-to)	913-919
Number of pages	7
Journal	Arquivos de Neuro-Psiquiatria
Volume	59
Issue number	4
DOIs	https://doi.org/10.1590/s0004-282x2001000600014
State	Published - Dec 2001
Externally published	Yes

Keywords

Autopsy
Holoprosencephaly
Nervous system malformations

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10.1590/s0004-282x2001000600014

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@article{ea4d21e6720a47dfbc6a3f19beff065d,

title = "Holoprosencefalia: An{\'a}lise do seu espectro morfol{\'o}gico em doze casos de aut{\'o}psia",

abstract = "The term holoprosencephaly (HPC) is used to indicate the group of hemispheric deformities caused by a failure in the development of the prosencephalic vesicle. The purpose of this study is to explain the morphologic specter of twelve cases of HPC, qualify them, and compare them to the literature. It was evaluated 5837 pediatrics necropsies, and there were 12 cases of HPC. Data like gender, age and the presence of another associated malformations were evaluated and compared to another malformations and to the total number of necropsies. The majority of the cases was male (66.66%), and stillborns (75%). The most frequently type of HPC found was the lobar type (58.3%), and the most frequently type of facial alteration was the ciclopy (25%). There is a statistic tendency to HPC affect the male sex, in comparison with other neurologic malformations. Besides, the stillborns are more frequently observed in HPC than in other neurologic malformations.",

keywords = "Autopsy, Holoprosencephaly, Nervous system malformations",

author = "{De Noronha}, L{\'u}cia and Ghanem, {Ramon Coral} and Fab{\'i}ola Medeiros and Jos{\'e} Knopfholz and Magalh{\~a}es, {Tiago Augusto} and Sampaio, {Gilberto Antunes} and Serapi{\~a}o, {Maria Jos{\'e}} and Torres, {Luiz Fernando Bleggi}",

year = "2001",

month = dec,

doi = "10.1590/s0004-282x2001000600014",

language = "Portugu{\'e}s (Brasil)",

volume = "59",

pages = "913--919",

journal = "Arquivos de Neuro-Psiquiatria",

issn = "0004-282X",

number = "4",

}

TY - JOUR

T1 - Holoprosencefalia

T2 - Análise do seu espectro morfológico em doze casos de autópsia

AU - De Noronha, Lúcia

AU - Ghanem, Ramon Coral

AU - Medeiros, Fabíola

AU - Knopfholz, José

AU - Magalhães, Tiago Augusto

AU - Sampaio, Gilberto Antunes

AU - Serapião, Maria José

AU - Torres, Luiz Fernando Bleggi

PY - 2001/12

Y1 - 2001/12

N2 - The term holoprosencephaly (HPC) is used to indicate the group of hemispheric deformities caused by a failure in the development of the prosencephalic vesicle. The purpose of this study is to explain the morphologic specter of twelve cases of HPC, qualify them, and compare them to the literature. It was evaluated 5837 pediatrics necropsies, and there were 12 cases of HPC. Data like gender, age and the presence of another associated malformations were evaluated and compared to another malformations and to the total number of necropsies. The majority of the cases was male (66.66%), and stillborns (75%). The most frequently type of HPC found was the lobar type (58.3%), and the most frequently type of facial alteration was the ciclopy (25%). There is a statistic tendency to HPC affect the male sex, in comparison with other neurologic malformations. Besides, the stillborns are more frequently observed in HPC than in other neurologic malformations.

AB - The term holoprosencephaly (HPC) is used to indicate the group of hemispheric deformities caused by a failure in the development of the prosencephalic vesicle. The purpose of this study is to explain the morphologic specter of twelve cases of HPC, qualify them, and compare them to the literature. It was evaluated 5837 pediatrics necropsies, and there were 12 cases of HPC. Data like gender, age and the presence of another associated malformations were evaluated and compared to another malformations and to the total number of necropsies. The majority of the cases was male (66.66%), and stillborns (75%). The most frequently type of HPC found was the lobar type (58.3%), and the most frequently type of facial alteration was the ciclopy (25%). There is a statistic tendency to HPC affect the male sex, in comparison with other neurologic malformations. Besides, the stillborns are more frequently observed in HPC than in other neurologic malformations.

KW - Autopsy

KW - Holoprosencephaly

KW - Nervous system malformations

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U2 - 10.1590/s0004-282x2001000600014

DO - 10.1590/s0004-282x2001000600014

M3 - Artículo

SN - 0004-282X

VL - 59

SP - 913

EP - 919

JO - Arquivos de Neuro-Psiquiatria

JF - Arquivos de Neuro-Psiquiatria

IS - 4

ER -

Holoprosencefalia: Análise do seu espectro morfológico em doze casos de autópsia

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Keywords

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