Histopathology of the ICE syndrome.
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Biomedical subjects
Publications and source records attributed to C Kupfer.
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Public health ophthalmology, often referred to as preventive or community ophthalmology, focuses on three community based activities: preventive, curative, and promotive. The pioneering work of Professor Barrie Jones in this field is recognised. Examples of each activity are provided, with emphasis on the developing world.
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Neural crest cells give rise to all connective tissues anterior to the lens epithelium including corneal stromal cells, corneal and trabecular mesothelial cells, iris stromal cells, and iris melanophores. These cells constitute a continuous layer which extends from the corneal mesothelium to trabecular meshwork mesothelium and onto the anterior surface of the iris. Between the seventh and eighth month of gestation in man, the layer of cells loses its continuity. The present case suggests a defective terminal induction of a particular structural component of the angle derived from neural crest resulting in massive congenital adhesions in the anterior chamber angle. The persistence of a layer of abnormal cells lining the anterior chamber structures further reinforces the presence of defective mesothelial cells. Additional cases having such clinicopathologic correlation may provide further insight into this hypothesis.
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A previous study of 16 patients and 16 controls had indicated that the mean ocular rigidity value was significantly lower in Osteogenesis Imperfecta (OI) patients than in normal controls (p = 0.001). These preliminary results have been confirmed in the present study of 46 patients and 53 controls where the difference in mean ocular rigidity between the patient and control group was statistically significant at the p less than 0.0001 level. Of particular interest was the inverse correlation between blueness of sclera and ocular rigidity in patients with OI. The establishment of such a relationship depended upon the availability of a larger sample size than had previously been examined.
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Abnormalities in neural crest cell migration may have a role in the pathogenesis of the congenital glaucomas, with or without changes in the gonioscopic appearance of the anterior chamber angle. Neural crest cells also contribute to the bones of the face, dental papilla, cartilage, bone, and meninges; this may explain the association of craniofacial, dental, and upper spinal malformations with some of the congenital glaucomas.
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The group of congenital and developmental anomalies of the anterior chamber associated with increased intraocular pressure has been difficult to interpret from an embryologic perspective which would take into account the ocular defects as well as associated systemic abnormalities. A new hypothesis is presented which suggests that the ocular and systemic defects can be explained by abnormalities in migration or terminal induction of neural-crest cells. These cells represent the initial population which differentiates into the diversity of cell types that appear to be involved in both the ocular and systemic manifestation of this group of anterior chamber anomalies.