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Holoprosencephaly (Arhinencephaly)

Anterior midline brain, cranial, and facial malformations resulting from the failure of the embryonic prosencephalon to undergo segmentation and cleavage. Alobar prosencephaly is the most severe form and features anophthalmia; cyclopia; severe MENTAL RETARDATION; CLEFT LIP; CLEFT PALATE; SEIZURES; and microcephaly. Semilobar holoprosencepaly is characterized by hypotelorism, microphthalmia, coloboma, nasal malformations, and variable degrees of mental retardation. Lobar holoprosencephaly is associated with mild (or absent) facial malformations and intellectual abilities that range from mild mental retardation to normal. Holoprosencephaly is associated with CHROMOSOME ABNORMALITIES.
Also Known As:
Arhinencephaly; Alobar Holoprosencephalies; Arhinencephalies; Holoprosencephalies; Holoprosencephalies, Alobar; Holoprosencephalies, Lobar; Holoprosencephalies, Semilobar; Holoprosencephaly, Alobar; Holoprosencephaly, Lobar; Holoprosencephaly, Semilobar; Lobar Holoprosencephalies; Semilobar Holoprosencephalies; Alobar Holoprosencephaly; Lobar Holoprosencephaly; Semilobar Holoprosencephaly
Networked: 312 relevant articles (2 outcomes, 8 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Umbilical Hernia (Omphalocele)
2. Paralysis (Palsy)
3. Aneuploidy (Aneuploid)
4. Epiglottitis
5. Apnea

Experts

1. Chen, Chih-Ping: 9 articles (09/2013 - 09/2003)
2. Muenke, Maximilian: 8 articles (10/2012 - 03/2003)
3. Wang, Wayseen: 7 articles (09/2013 - 09/2003)
4. Chern, Schu-Rern: 5 articles (09/2013 - 09/2003)
5. Ueta, Etsuko: 5 articles (09/2007 - 12/2002)
6. Naruse, Ichiro: 5 articles (09/2007 - 12/2002)
7. Nicolaides, K H: 4 articles (07/2010 - 12/2005)
8. Sulik, Kathleen K: 3 articles (01/2014 - 01/2010)
9. Roessler, Erich: 3 articles (01/2012 - 04/2009)
10. Richieri-Costa, Antonio: 3 articles (01/2012 - 12/2006)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Holoprosencephaly:
1. MegaduodenumIBA
2. Patau syndromeIBA
3. CholesterolIBA
4. Single upper central incisorIBA
5. trans- 1,4- Bis(2- chlorobenzaminomethyl)cyclohexane DihydrochlorideIBA
6. cyclopamineIBA
7. Hereditary renal agenesisIBA
8. trisomy Chromosome 22IBA
9. EctrodactylyIBA
10. Ethanol (Ethyl Alcohol)IBA

Therapies and Procedures

1. Tracheotomy
2. Sutures (Suture)
3. Ventriculoperitoneal Shunt
4. Colonic Pouches (S Pouch)
5. Anesthesia