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Holoprosencephaly

Background

Holoprosencephaly: HPE

Holoprosencephaly is a series of brain malformations in which there is incomplete development of the cerebrum. Its effects on the child range from severe and lethal to mild and almost undetectable.

In the most severe form, alobar HPE, the cerebrum is a single U-shaped mass rather than being divided into right and left hemispheres. Death most often occurs before, during or soon after birth. Among liveborn children with alobar HPE, half will have died by four months of age, but survival for several years has been noted in a number of children. 

What are the symptoms? View What are the symptoms?

Medical text written May 1998 by Dr M Barr Jr, University of Michigan, USA. Last reviewed March 2006 by Dr A Habel, Consultant Paediatrician, Great Ormond Street Hospital, London, UK and Dr M Lees, Consultant Geneticist, Institute of Child Health, London, UK.

 

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