Alport syndrome is a hereditary, progressive disease characterized by progressive nephritis, sensorineural deafness, and ocular abnormalities, including anterior lenticonus. The ultrastructure of the lens capsule abnormalities in Alport syndrome is reported. Four anterior lens capsules from 31-year-old patient and 26-year-old patient with lenticonus who were affected by the Alport syndrome were obtained at capsulectomy. And all four anterior lens capsules were examined by transmission electron microscopy. The histopathologic findings showed that the thickness of the anterior lens capsules was decreased (4~13 micrometer) and that there were many vascular dehiscences localized at the inner part of the lens capsule. There were large numbers of capsular dehiscences containing fibrillar materials and vacuoles. The anterior capsules were clearly fragile in this disease, forming the basis for the progressive lenticonus and anterior polar cataract.
Adult ; Epithelial Cells/ultrastructure ; Humans ; Lens Capsule, Crystalline/*ultrastructure ; Lens Diseases/genetics/*pathology ; Lens Implantation, Intraocular ; Male ; Nephritis, Hereditary/genetics/*pathology ; Phacoemulsification

Recently iridectomy using an argon or Nd-YAG laser to treat narrow angle glaucoma has become popular, and is now the procedure of choice over the standard surgical technique. However, the shock wave of the Nd-YAG laser causes hemorrhage in almost all cases and the high energy level of the Nd-YAG laser, which is required for iridectomy, causes injury to the lens and cornea. Furthermore, there is a tendency toward closure of the iridectomy site after argon laser application. We performed iridectomies by a combined application of argon and Nd-YAG lasers in pigmented rabbits to improve iris bleeding, iridectomy patency, and lens and corneal damage. The iridectomy patency and the lens and corneal damage were examined with a scanning electron microscope. The rabbits that underwent laser iridectomies with only the Nd-YAG laser were used as a control group. Based on the results, it can be concluded that laser iridectomy by a combined application of argon and Nd-YAG lasers results in a lower rate of bleeding, a higher rate of patency, and less damage to the lens and cornea as compared with iridectomy performed by Nd-YAG laser only.
Animals ; Cornea/ultrastructure ; Endothelium, Corneal/ultrastructure ; Eye Hemorrhage/etiology ; Iris/blood supply/*surgery/ultrastructure ; *Laser Therapy/adverse effects ; Lens, Crystalline/ultrastructure ; Microscopy, Electron, Scanning ; Rabbits ; Random Allocation

OBJECTIVETo observe the correlation of gammaD-crystallin P23T mutant with lens ultrastructure of the hereditary coralliform cataract.

METHODSComplete ophthalmologic examinations were performed before lens extraction and lens samples were studied by transmission and scanning electric microscope respectively. Protein molecular modeling was performed using SWISS-MODEL(version 2.0).

RESULTSProtein structure modeling demonstrated that the mutant caused a decrease in molecular final total energy and changes in the surface structure of gammaD-crystallin. Ultrastructure study revealed crystals deposited in lens, extensive granules dispersed in uncommon oval structure and the disorganization of lens epithelial cells.

CONCLUSIONIt is possible that the gammaD-crystallin P23T mutant is associated with abnormal crystals in lens and disorganization of lens epithelial cells.

Cataract ; congenital ; genetics ; pathology ; Female ; Humans ; Lens, Crystalline ; ultrastructure ; Male ; Pedigree ; Phenotype ; Point Mutation ; gamma-Crystallins ; genetics

A 45-year-old man with retinitis pigmentosa (RP), who had undergone uneventful extracapsular cataract extraction (ECCE) in his right eye eight years previously, and phacoemulsification in his left eye six years previously, had spontaneously dislocated intraocular lenses (IOL) within the capsular bag in both eyes one month apart. We removed the dislocated IOLs, and performed anterior vitrectomy and scleral fixation of the new IOLs. Mild contraction of the capsular bags and uneven distribution of the zonular remnants' clumps along the equator of the capsules were found by scanning electron microscopic (SEM) examination. In this study, we propose the correlation between RP and zonular weakness. To our knowledge, this is the first case report of bilateral spontaneous dislocation of IOLs within the capsular bag of an RP patient.
Adult ; Foreign-Body Migration/*etiology ; Humans ; Lens Capsule, Crystalline/*pathology ; Lens Implantation, Intraocular ; *Lenses, Intraocular ; Ligaments/ultrastructure ; Male ; Microscopy, Electron, Scanning ; Phacoemulsification ; Reoperation ; Retinitis Pigmentosa/*complications ; Vitrectomy

To investigate if the surface modification of intraocular lens (IOL) is efficient in the prevention of posterior capsular opacification (PCO), the acrylic surface of intraocular lens (Acrysof(R)) was polymerized with polyethylene glycol (PEG-IOL). The human lens epithelial cells (1x10(4) cells/mL) were inoculated on PEG grafted or unmodified acrylic lenses for the control. The adherent cells on each IOL surface were trypsinized and counted. The every PEG-IOL was implanted in 20 New Zealand rabbits after removal of crystalline lens. The formations of PCO were checked serially through retroilluminated digital photography, and the severity scores were calculated using POCOman(R). The cell adherence patterns on each IOL were examined by scanning electron microscopy. As a result, the mean number of adherent cells of PEG-IOL (3.2+/-1.1x10(3)) tended to be smaller than that of the acrylic controls (3.6+/-1.9x10(3)) without a statistical significance (p=0.73). However, the mean severity of PCO formation in PEG-IOL was significantly lower than that in the control during the third to sixth weeks after surgery. Scanning electron microscopy revealed that the more patch-like cells were found firmly attached to the IOL surface in control than in the PEG-IOL. Conclusively, PEG polymerization to the acrylic IOL would possibly lessen the formation of PCO after cataract removal.
Acrylic Resins/chemistry ; Biocompatible Materials ; Cataract/metabolism/*therapy ; Cell Adhesion ; Humans ; Lens Implantation, Intraocular/*methods ; Lens, Crystalline/cytology/ultrastructure ; Microscopy, Electron, Scanning ; Polyethylene Glycols/*chemistry/metabolism ; Time Factors

BACKGROUNDCongenital cataract is a sight-threatening disease that affects about 1 - 6 cases per 10000 live births and causes 10% - 30% of all blindness in children. About 25% of all cases are due to genetic defects. We identified autosomal dominant congenital coralliform cataracts-related genetic defect in a four-generation Chinese family.

METHODSComplete ophthalmological examinations were performed prior to lens extraction. Lens samples were then studied by electron microscopy. Genomic DNA from family members were examined using whole-genomic linkage analysis, with two-point logarithm of odds (LOD) scores calculated using the Linkage program package (version 5.1). Mutation analysis of candidate genes was performed by direct sequencing. Finally, a three-dimensional protein model was predicted using Swiss-Model (version 2.0).

RESULTSEleven of the 23 examined individuals had congenital cataracts. Ultrastructure studies revealed crystal deposits in the lens, and granules extensively dispersed in transformed lens fiber cells. The maximum two-point LOD score, 3.5 at theta = 0.1, was obtained for the marker D2S325. Mutation analysis of the gamma-crystallin (CRYG) gene cluster identified a mutation (P23T) in exon 2 of gammaD-crystallin (CRYGD). Protein structure modeling demonstrated that the P23T mutation caused a subtle change on the surface of the gammaD protein.

CONCLUSIONSThe results suggest that the coralliform cataract phenotype is due to a mutated CRYGD gene, and that this sequence change is identical to one reported by Santhiya to be related to another distinct clinical condition, lamellar cataract. This study provides evidence that this same genetic defect may be associated with a different phenotype. This is the first report identifying the genetic defect associated with an autosomal dominant congenital coralliform cataract.

Cataract ; genetics ; pathology ; Female ; Genes, Dominant ; Genetic Linkage ; Humans ; Lens, Crystalline ; ultrastructure ; Male ; Microscopy, Electron ; Mutation, Missense ; gamma-Crystallins ; chemistry ; genetics