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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 INTELLECTUAL DISABILITY; CLEFT LIP; CLEFT PALATE; SEIZURES; and microcephaly. Semilobar holoprosencepaly is characterized by hypotelorism, microphthalmia, coloboma, nasal malformations, and variable degrees of INTELLECTUAL DISABILITY. Lobar holoprosencephaly is associated with mild (or absent) facial malformations and intellectual abilities that range from mild INTELLECTUAL DISABILITY to normal. Holoprosencephaly is associated with CHROMOSOME ABNORMALITIES.
Also Known As:
Arhinencephaly; Holoprosencephaly, Familial Alobar; 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: 219 relevant articles (2 outcomes, 4 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Paralysis (Palsy)
2. Epiglottitis
3. Apnea
4. Hydrocephalus (Hydrocephaly)
5. Neural Tube Defects (Exencephaly)

Experts

1. Muenke, Maximilian: 12 articles (01/2021 - 03/2003)
2. Roessler, Erich: 6 articles (02/2012 - 04/2009)
3. Lipinski, Robert J: 5 articles (01/2021 - 02/2010)
4. Dubourg, Christèle: 5 articles (01/2020 - 10/2007)
5. Everson, Joshua L: 4 articles (01/2021 - 01/2014)
6. Odent, Sylvie: 4 articles (01/2020 - 10/2007)
7. Sulik, Kathleen K: 4 articles (11/2016 - 01/2010)
8. Flores-Sarnat, Laura: 3 articles (03/2021 - 07/2013)
9. Sarnat, Harvey B: 3 articles (03/2021 - 07/2013)
10. Kruszka, Paul: 3 articles (01/2021 - 01/2019)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Holoprosencephaly:
1. Vasopressins (Vasopressin)IBA
2. TolvaptanFDA Link
3. CholesterolIBA
4. trans- 1,4- Bis(2- chlorobenzaminomethyl)cyclohexane DihydrochlorideIBA
5. SterolsIBA
6. cyclopamineIBA
7. Proteins (Proteins, Gene)FDA Link
8. Transcription Factors (Transcription Factor)IBA
9. Ethanol (Ethyl Alcohol)IBA
10. Minocycline (Cyclops)FDA LinkGeneric

Therapies and Procedures

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