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: | , , |
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| Format: | Book |
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Indonesian Pediatric Society Publishing House,
2011-06-01T00:00:00Z.
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| Summary: | 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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| Item Description: | 0030-9311 2338-476X 10.14238/pi51.3.2011.182-6 |