Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0423320 (iridodonesis)
24 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The inheritance of congenital miosis in 2 pedigrees is described. The inheritance was autosomal dominant in one and autosomal recessive in the other. The pupils were 0.5-2.5 mm diameter, reacted normally to light and accommodation but dilated poorly with mydriatics. In both pedigrees, the affected members had enlarged corneas. The five members in the autosomal dominant family were myopic and had translucent peripheral irides. Iridodonesis was presented in both members of the autosomal recessive pedigree. The embryology and innervation of the iris muscles together with the ocular abnormalities and syndromes associated with congenital miosis are reviewed.
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PMID:Congenital miosis. 42 Nov 61

A family with Marfan's syndrome is reported showing as outstanding features, demonstration of the hereditary character of the entity through the genealogical tree. The classical clinical characteristics of the disease are: long face, arachnodactyly, decreased subcutaneous tissue, muscular hypoplasia, somatometric values above normal percentiles for Mexicans, weight and perimeters of arm and leg showing low mass and height, upper and lower segments above percentil 50 and ocular disorders like bilateral ectopia lentis, spherical and small lens, iridodonesis and myopia.
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PMID:[Marfan's syndrome]. 75 94

Eighteen patients who presented with severe aortic regurgitation and dilatation of the ascending aorta were found to be formes frustes of Marfan's syndrome and formed the basis for this clinicogenetic study. All had aortic valve replacement and reconstruction of part of the ascending aorta. The diagnosis was confirmed by histological examination of the aortic tissue. There were 126 first degree relatives; 85 were living and 67 (78-8%) of these were examined. Limited information was available about 32 of the 41 relatives who had died. No relative had the classical clinical features of Marfan's syndrome but stigmata of the disease were found in 25 (37-3%) of the 67 first degree relatives examined. In 21, the abnormality was confined to the cardiovascular system, the skeleton, or the eye, but in 4, abnormalities involved 2 systems. Cardiovascular abnormalities affecting the aortic valve or aortic wall were present in 6 (9-0%) of the 67 first degree relatives examined. One or more of the skeletal indices measured (height-span difference, metacarpal index, phalangeal index) was abnormal in 18 (26-9%) and ocular abnormalities were found in 5 of 51 (9-8%) examined. There were no relatives with dislocation of the lens or iridodonesis. Using strict diagnostic criteria, a minimum of 37-3 per cent of the first degree relatives examined were affected; this involved 12 of the 18 families studied. There was nothing in our data to suggest that the formes frustes of the disease had a different mode of inheritance from the classical syndrome.
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PMID:Formes frustes of Marfan's syndrome presenting with severe aortic regurgitation. Clinicogenetic study of 18 families. 83 34

An infant girl with arachnodactyly, spontaneously resolving contractures, dolichostenomelia, iridodonesis, and mitral and tricuspid incompetence died in cardiac failure. We confirm that congenital contractural arachnodactyly may exhibit serious cardiovascular and ophthalmic complications like Marfan's syndrome. The presence of iridodonesis further obscures the differentiation between classical Marfan's syndrome and congenital contractural arachnodactyly.
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PMID:Contractural arachnodactyly with mitral regurgitation and iridodonesis. 227 Oct 65

Iridodonesis and pseudophakodonesis were examined in detail with a high-speed camera capable of picture rates up to 500/sec. Tilting, or "wobble," of iris-supported intraocular lenses was extreme, with a tilt of nearly 24 degrees with respect to the visual axis of the globe being documented, even with a moderate speed of globe rotation. Oscillations of the lens continued for several cycles after the globe stopped its rotation. Such lenses can, in some instances, tilt sufficiently to contact the endothelial surface of the cornea. In the case of some anterior chamber intraocular lenses, the iridodonesis is sufficient to cause constant rubbing of the iris on the posterior surface of the lens and its haptics , suggesting that vault, finish, haptic configuration, and placement of the lens may be critical factors in anterior chamber intraocular lens design.
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PMID:High-speed photographic evaluation of intraocular lens movements. 673 39

Studies of high-speed motion pictures of the eyes of patients after extracapsular (EC) and intracapsular (IC) cataract extraction show that pseudophacodonesis and iridodonesis are the result of oscillations of the fluids in the anterior segment of the eye. These oscillations, initiated by movement of the eye, result in shearing forces on the corneal endothelium which may result in damage. Similar motion of the vitreous causes shearing forces which may damage the retina.
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PMID:Physical forces involved in pseudophacodonesis and iridodonesis. 690 24

A pedigree analysis of a family of 15 related Chinese Shar Peis was conducted. This pedigree analysis, including affected and nonaffected dams, sires and offspring, was compiled to document and characterize the occurrence, common clinical signs, and age of onset of primary lens luxation while suggesting a possible mode of inheritance in this breed. Of the five offspring from the mating of an affected dam to two unrelated affected males, 100% of offspring were affected with bilateral primary lens luxations. Of the four viable offspring from the mating of the same affected dam to an unrelated, unaffected male, two dogs (50%) were affected. The average age of onset of affected animals (seven) in this first generation was 4.9 years (range 3-6 years). The six dogs in the second generation of the same pedigree line were 2-years-old at examination with none of these animals affected at the time of this study. The most common presenting complaints were a unilateral change in ocular appearance (5 of 7 dogs) and subjective vision impairment (4 of 7 dogs). The most common clinical sign upon ophthalmic examination was iridodonesis (unilateral 4 of 7 dogs; bilateral 3 of 7 dogs) and the presence of an aphakic crescent (3 of 7 dogs). Gonioscopy and tonometry of severely affected eyes revealed a narrow or closed iridocorneal angle and ocular hypertension. This study suggests that primary lens luxation does occur in the Chinese Shar Pei, resembling the clinical condition (age of onset, clinical signs) previously described in the terrier breeds, the Border Collie, and the Tibetan Terrier. Application of the phenotypic findings in this study to a Mendelian genetic model of inheritance suggests that primary lens luxation in the Chinese Shar Pei is inherited as a simple autosomal recessive trait.
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PMID:Primary lens luxation in the Chinese Shar Pei: clinical and hereditary characteristics. 1139 17

The Brown-McLean syndrome is defined by corneal edema that involves the peripheral 2.0 to 3.0 mm of cornea, sparing the central cornea. This syndrome is usually associated with previous cataract surgery but has been reported rarely in patients with other predisposing factors such as angle-closure glaucoma or spontaneous lens absorption with iridodonesis. We describe the clinical course of a 50-year-old man with myotonic dystrophy who had Brown-McLean syndrome with no identifiable predisposition for peripheral corneal edema. Although this syndrome appears to be the result of peripheral endothelial dysfunction, this patient was able to tolerate cataract extraction without developing central corneal edema.
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PMID:Cataract surgery in a patient with Brown-McLean syndrome. 1152 11

We present a case of traumatic lens subluxation that was evident only when the patient was in the supine position. This case illustrates that a crystalline lens with traumatic zonular rupture can change its position depending on the posture of the patient. If such a lens is suspended by the remaining zonules at the superior quadrant while the patient is erect, phacodonesis may be present while the lens remains in its normal position. However, when the patient is in the supine position, the lens drops back into the vitreous cavity. Examination in erect and supine positions is important in the preoperative assessment of patients with traumatic phacodonesis or iridodonesis.
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PMID:Traumatic subluxation causing variable position of the crystalline lens. 1203 60

We describe a 36-year-old female who suffered from presenile cataract (nuclear sclerosis and posterior subcapsular opacity, with more-severe disease in the right eye than in the left) and report the use of some ophthalmologic examinations to evaluate the anatomic structures and visual functions of both eyes. Slit-lamp biomicroscopy showed increasing horizontal and vertical corneal diameters (14.0/13.5 mm) and iridodonesis. The pupils could not be fully dilated, and the lenses exhibited cataractous changes. The postlimbal depths were 1.772 mm (OD) and 1.690 mm (OS) (normal value, < 0.20 mm). The results of gonioscopy, specular microscopy, Goldmann visual field, and intraocular pressure evaluation were all within normal limits. Because the patient had bilateral megalocornea in the absence of glaucoma, a very deep anterior chamber depth, secondary effects of iridodonesis, and changes to cataractous lenses, anterior megalophthalmos was diagnosed. This disease is rare. Secondary effects such as iridodonesis, miosis, atrophy of the iris stroma lens subluxation, and occurrence of cataractous lens should be kept in mind.
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PMID:Anterior megalophthalmos. 1594 27


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