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Updated: Jun 21, 2026
13:08
Measurement of Factor V Activity in Human Plasma Using a Microplate Coagulation Assay
Published on: September 9, 2012
Anterior segment dysgenesis in a child with factor VII deficiency
Francis Beby1, Sandrine Meunier, Pierre Cochat
1Department of Ophthalmology, Edouard Herriot Hospital, Rue du Professeur Florence, Lyon, France. beby.francis@neuf.fr
Clinical Ophthalmology (Auckland, N.Z.)
|August 12, 2009
Summary
This study details the first case of anterior segment dysgenesis linked to congenital factor VII deficiency (hypoproconvertinemia). Early coagulation screening is recommended for children with unexplained anterior segment dysgenesis.
Area of Science:
- Ophthalmology
- Hematology
Background:
- Congenital factor VII deficiency (hypoproconvertinemia) is a rare bleeding disorder.
- Anterior segment dysgenesis encompasses a spectrum of developmental abnormalities of the eye's anterior structures.
Observation:
- A 2-month-old infant with diagnosed severe factor VII deficiency presented with bilateral corneal opacities.
- The infant's parents were consanguineous, and the deficiency was noted early due to gastrointestinal bleeding.
Findings:
- Ocular examination revealed bilateral corneal opacities, iris strands adherent to the cornea, and peripheral anterior synechiae.
- Intraocular pressure, lens clarity, and optic nerves were normal, indicating no secondary glaucoma or cataracts.
Implications:
- This case highlights potential ocular manifestations of hypoproconvertinemia.
- Suggests routine coagulation screening for children with idiopathic anterior segment dysgenesis to detect underlying bleeding disorders.
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