Embryogenesis of triple neural tube defects: sonic hedgehog—a key?

Abstract Neural tube closure is a complex spatio-temporal process. Multiple neural tube defects (NTDs) in a single patient are extremely rare. Only a few cases of multiple NTDs have been reported in the world literature, including less than 20 cases of double NTDs and 3 cases of triple NTDs. We repo...

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Bibliographic Details
Published in:Journal of pediatric surgery 2011-02, Vol.46 (2), p.e5-e8
Main Authors: Mahalik, Santosh Kumar, Vaze, Dhananjay, Lyngdoh, Toijam Soni, Tewari, Manoj Kumar, Narasimhan, Kannan Laksmi
Format: Article
Language:English
Subjects:
Cell Polarity - genetics
Embryonic Development - genetics
Hedgehog Proteins - genetics
Hedgehog Proteins - physiology
Humans
Infant, Newborn
Magnetic Resonance Imaging
Male
Multiple neural tube defects
Neural Tube - embryology
Neural tube closure
Neural Tube Defects - diagnosis
Neural Tube Defects - embryology
Neural Tube Defects - genetics
Neurulation
Neurulation - genetics
Pediatrics
Rare Diseases
Signal Transduction - genetics
Signal Transduction - immunology
Sonic hedgehog
Surgery
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Summary:Abstract Neural tube closure is a complex spatio-temporal process. Multiple neural tube defects (NTDs) in a single patient are extremely rare. Only a few cases of multiple NTDs have been reported in the world literature, including less than 20 cases of double NTDs and 3 cases of triple NTDs. We report a fourth case of triple NTDs and review the literature regarding the embryogenesis. An effort is made to understand the theories of neural tube closure in light of recent advances in molecular biology and identification of the signaling pathways involved.
ISSN:0022-3468
1531-5037
DOI:10.1016/j.jpedsurg.2010.09.103