Clinical manifestations in semilobar holoprosencephaly

Holoprosencephaly (HPE) is a brain malformation caused by a primary defect in induction and patterning of the rostral neurotube (basal forebrain) during the first 4 weeks of embryogenesis. T his defect results in incomplete separation of the cerebral hemispheres. Based on the degree of hemispheric n...

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Main Authors: Elisa Elisa, A. H. Putranti, E. Mulyono
Format: Article
Language:English
Published: Indonesian Pediatric Society Publishing House 2011-06-01
Series:Paediatrica Indonesiana
Subjects:
Online Access:https://paediatricaindonesiana.org/index.php/paediatrica-indonesiana/article/view/722
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author Elisa Elisa
A. H. Putranti
E. Mulyono
author_facet Elisa Elisa
A. H. Putranti
E. Mulyono
author_sort Elisa Elisa
collection DOAJ
description Holoprosencephaly (HPE) is a brain malformation caused by a primary defect in induction and patterning of the rostral neurotube (basal forebrain) during the first 4 weeks of embryogenesis. T his defect results in incomplete separation of the cerebral hemispheres. Based on the degree of hemispheric nonseparation, HPE traditionally has been classified into three types: alobar, semilobar, and lobar.! In 1963, DeMyer et al. mentioned that defects in brain development may frequently coexist with abnormalities on the midfacial region. T he median facio-cerebral anomalies appear in various associated gradations and combinations. When combined in patterns, these facies always predict a severe, highly characteristic brain anomaly.2
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spelling doaj.art-50c184ab8d2341f498b9f91c71328f302022-12-21T20:29:10ZengIndonesian Pediatric Society Publishing HousePaediatrica Indonesiana0030-93112338-476X2011-06-01513182610.14238/pi51.3.2011.182-6589Clinical manifestations in semilobar holoprosencephalyElisa Elisa0A. H. Putranti1E. Mulyono2Pediatric Intensive Care Unit, ]ogja International Hospital, Yogyakarta, Central JavaDepartment of Child Health, Diponegoro University Medical School/Dr. Karyadi Hospital, Semarang, Central JavaDepartment of Radiology, Gadjah Mada University Medical School/Dr. Sardjito Hospital, Yogyakarta, Central JavaHoloprosencephaly (HPE) is a brain malformation caused by a primary defect in induction and patterning of the rostral neurotube (basal forebrain) during the first 4 weeks of embryogenesis. T his defect results in incomplete separation of the cerebral hemispheres. Based on the degree of hemispheric nonseparation, HPE traditionally has been classified into three types: alobar, semilobar, and lobar.! In 1963, DeMyer et al. mentioned that defects in brain development may frequently coexist with abnormalities on the midfacial region. T he median facio-cerebral anomalies appear in various associated gradations and combinations. When combined in patterns, these facies always predict a severe, highly characteristic brain anomaly.2https://paediatricaindonesiana.org/index.php/paediatrica-indonesiana/article/view/722HoloprosencephalyHPEbrain malformation
spellingShingle Elisa Elisa
A. H. Putranti
E. Mulyono
Clinical manifestations in semilobar holoprosencephaly
Paediatrica Indonesiana
Holoprosencephaly
HPE
brain malformation
title Clinical manifestations in semilobar holoprosencephaly
title_full Clinical manifestations in semilobar holoprosencephaly
title_fullStr Clinical manifestations in semilobar holoprosencephaly
title_full_unstemmed Clinical manifestations in semilobar holoprosencephaly
title_short Clinical manifestations in semilobar holoprosencephaly
title_sort clinical manifestations in semilobar holoprosencephaly
topic Holoprosencephaly
HPE
brain malformation
url https://paediatricaindonesiana.org/index.php/paediatrica-indonesiana/article/view/722
work_keys_str_mv AT elisaelisa clinicalmanifestationsinsemilobarholoprosencephaly
AT ahputranti clinicalmanifestationsinsemilobarholoprosencephaly
AT emulyono clinicalmanifestationsinsemilobarholoprosencephaly