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Query: UMLS:C0086543 (cataract)
 29,165 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two brothers showed severe and persistent hyperchloraemic metabolic acidosis (capillary blood pH 7.07--7.15) due to a low renal bicarbonate threshold at 11 mmol/l. The maximal tubular capacity for bicarbonate reabsorption was reduced to about half the normal. A high dose of acetazolamide (25 mg/kg) lowered the tubular bicarbonate reabsorption substantially, indicating the presence of carbonic anhydrase. Both the glomerular filtration rate, the renal blood flow and the renal concentrating capacity were slightly reduced. The clinical characteristics were: growth retardation, mental retardation, nystagmus, corneal opacities, cataract, glaucoma and enamel defects of the permanent teeth. Serum thyroxine was pathological low without clinical signs of hypothyreosis. The erythrocytes showed an increased osmotic resistance. Autopsy of the younger brother, who died 4 1/2 years old, revealed thyroid and thymus weights of 25% of the normal. The kidney tubular cells were swollen with vacuoles. The glomeruli had a normal appearance.
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PMID:Congenital persistent proximal type renal tubular acidosis in two brothers. 4 68

Among 200 children who had received routine ophthalmic and orthoptic examinations as part of their evaluation by a multidisciplinary assessment team, it was found that there was a high incidence of ocular defects. These defects included refractive errors (98 children), squint (74 children), nystagmus (15 children), cataract, retinopathy and optic atrophy. This high incidence of ocular defects emphasized the need for the routine ophthalmic examination of developmentally handicapped children in order to detect and treat ocular conditions which might otherwise have gone undetected, and enabled the visual sense of these children to be evaluated in relationship to their general development.
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PMID:The ophthalmologist's role in multidisciplinary assessment of developmentally handicapped children. 13 Sep 99

The first one was a 13 months old lewish boy with typical features of the rhizometic type; clinicaly the patient demonstrated cataract, esotropia, nystagmus, malnutrition, micromelia, contracture, multiple stippled calcifications about the periphery of the cartilagenous structures of the vertebral bodies, sternum, wrists, knees, heels, ribs; and profound mental retardation, After cataract operation, the head of the optic nerve demonstrate to be narrower than usualy and white. Pathologic studies of the lens' fragments after surgery showed the cataract to be without any specific characters. The child now 2 yrs and 1/2 old is still alive but in poor condition. The second was a 16 months old Arabic boy without typical features but with a cataract and stippled calfications of a limited number of epiphysae. Pathologic studies of the lens after cataract surgery showed the cataracts to be without any specific characters, the optic nerves were normal. The child now 3 yrs old is normally developed and in good conditions. It is remarquable to note that in both cases there was an history of consanguinity (mother and father were first cousins).
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PMID:[Two cases of chondrodysplasia punctuate are reported]. 14 72

The principal occular complications of chromosomal aberrations are : strabismus, cataract, ptosis, nystagmus. Each of these can benefit from surgical treatment but one has to take into account the unfavorable prognosis due to mental deficiency.
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PMID:[Therapeutic possibilities in occular complications of chromosomal aberrations (author's tansl)]. 31 3

Sixty-eight soft cataracts were removed utilizing the O'Gawa double-bore aspirating-irrigating cannula. The postoperative visual acuity was found to be 20/100 or better in 83% and 20/40 or better in 59% of the 64 cases in which visual acuity was obtainable. In congenital cataracts, associated ocular defects such as strabismus or nystagmus reduced the visual prognosis. All cases of traumatic cataract had associated defects, usually a corneal laceration, however, visual improvement was noted in 81% of the cases. Significant postoperative corneal edema was not seen. Complications attributable to the operation included: rupture of the posterior capsule, insufficient removal of lens cortex, and pupullary block glaucoma. Discission of the posterior capsule was necessary in 26% of the eyes with an intact capsule. Retinal detachment followed the operation in three instances. The O'Gawa double-bore cannula is recommended for the removal of soft cataracts.
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PMID:Cataract aspiration with the O'Gawa double-bore cannula. 60 Apr 84

Of 76 members of a large pedigree with familial aniridia, 61% of the 38 affected patients had visual acuity of 6/9 (20/30) or better and only 5% had acuity of 6/60 (20/200) or worse. Cataracts were present in 18% of affected patients; glaucoma in 13%; and strabismus in 34%. Sixteen percent of affected patients had strabismic amblyopia. No patient had nystagmus or corneal pannus. The good visual acuity in this family, as compared to that in others, indicates that the absence of iris tissue alone cannot account for the severe visual problems usually associated with aniridia.
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PMID:Familial aniridia with preserved ocular function. 86 70

Eighteen patients who, years earlier, had undergone operations for congenital cataracts were discovered to have high intraocular pressures. Several had profound glaucomatous loss of vision. All had deep anterior chambers with flat iris planes and wide open angles. The uveal meshwork was unusually coarse and pigmented in some cases. Nystagmus, aftercataracts, and small pupils made visual field testing and observation of optic discs difficult. Most cases were bilateral, which suggested a hereditary origin; some had a family history of glaucoma. In none did we observe signs of congenital glaucoma or rubella syndrome. The incidence of this disease is not yet known, but it is common enough in our clinic to indicate lifetime surveillance for glaucoma in all patients who undergo congenital cataract surgery.
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PMID:Open-angle glaucoma following surgery for congenital cataracts. 92 76

It is hoped that the results of surgery for retinal detachment after surgery for congenital cataract, when discission and aspiration or phacoemulsification under the microscope are used, will approach the present reattachment rate that has been achieved with improved examination, instrumentation, and surgical technique in cases of senile retinal detachment. Further studies of the role of retinal pathology as lattice degeneration, vitreous pathology, and the place for prophylactic cryotherapy all must be reevaluated. In summary, the low incidence of retinal detachment following the extraction of congenital cataracts as seen in major retinal referral centers suggests that the risks of detachment are not as high as previously reported, that the cure rate approaches that of senile aphakic detachment; therefore early operative intervention to prevent amblyopia, nystagmus, and strabismus is indicated.
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PMID:Retinal detachment following congenital cataract surgery. 92 30

During the period 1970-73, 1,046 children under 20 years of age were registered with the Canadian National Institute for the Blind. The three most common registration diagnoses were Cataract (13%), Optic Atrophy (12%) and Nystagmus (10%); Retrolental Fibroplasia was responsible for a smaller proportion (6%). Twenty per cent of the registration diagnoses were non-specific and included "Nystagmus", "Site or Type not Established", "Affection of Visual Centre" and "Amblyopia". Without a specific diagnosis one could not decide whether the blindness was due to genetic or environmental causes. It is recommended that the C.N.I.B. establish a procedure by which children with a non-specific registration diagnosis can be referred for further investigation.
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PMID:Causes of blindness in children. 1046 cases registered with the Canadian National Institute for the Blind 1970-1973. 119 67

Microphthalmos is a developmental disorder of the eye consisting of a smaller than normal eye. This disorder can present as an isolated condition or associated with other systemic alterations. It is not uncommon for patients with microphthalmos to have congenital cataracts along with other ocular and systemic abnormalities. This paper reports the experience with 11 microphthalmic eyes of seven patients who had primary or secondary intraocular lens (IOL) implantation over a six-year period from 1985 to 1991. In all cases the IOL had a 13.5 mm or 14.0 mm overall diameter and a 6.0 mm or 6.5 mm optic. It was difficult to obtain documentation of objective visual improvement in many of these cases because of the associated nystagmus. However, all patients reported subjective improvements. These results suggest that with proper technique and lens selection microphthalmic patients should be considered for IOL implantation with relative safety and success.
J Cataract Refract Surg 1992 Sep
PMID:Intraocular lens implantation in microphthalmic patients. 833 40


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