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Holoprosencephaly (Arhinencephaly) Summary

Description: 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 Show All >>

Networked: 312 relevant articles (2 outcomes, 8 trials/studies) for this Disease

Key Drugs and Agents for Holoprosencephaly

Efficacy Chart >>
Drugs and Important Biological Agents (IBA) related to treatments:
  1. Megaduodenum : 1 outcome 2 studies in 9 results : IBA
  2. Patau syndrome : 1 study in 30 results : IBA
  3. Cholesterol : 1 study in 11 results : IBA
  4. Single upper central incisor : 1 study in 11 results : IBA
  5. trans- 1,4- Bis(2- chlorobenzaminomethyl)cyclohexane Dihydrochloride : 1 study in 6 results : IBA
  6. cyclopamine : 1 study in 5 results : IBA
  7. Hereditary renal agenesis : 1 study in 3 results : IBA
  8. trisomy Chromosome 22 : 1 study in 2 results : IBA
  9. Ectrodactyly : 14 results : IBA
  10. Ethanol (Ethyl Alcohol) : 11 results : IBA
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Diseases Related to Holoprosencephaly

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

Efficacy Chart >>
  1. Tracheotomy : 1 outcome in 1 result
  2. Sutures (Suture) : 6 results
  3. Ventriculoperitoneal Shunt : 4 results
  4. Colonic Pouches (S Pouch) : 2 results
  5. Anesthesia : 2 results
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