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Biomedical subjects

M O'Keefe

Publications and source records attributed to M O'Keefe.

117 records · Page 7Linked to original sources
PubMedDate not supplied

Ophthalmic findings in classical galactosemia--a screened population.

Classical galactosemia due to a deficiency of galactose-1-phosphate-uridyl transferase, is an autosomal recessive disorder of galactose metabolism with an incidence in Ireland of one in 30,000 births. It can result in cataract formation through the accumulation of galactitol within the lens. Seventeen children with transferase deficient galactosemia were studied. Early diagnosis followed by a galactose-free diet and tight biochemical control prevented cataract formation in 13 cases after a mean follow-up of 6.3 years. Cataracts did not regress in all patients commenced on diet by 6 weeks but early treatment prevented progression. The ophthalmologist may play an important role in the monitoring of patients with this disease as the recognition of new lens opacities by slit-lamp biomicroscopy may be the most sensitive initial index of inadequate biochemical control.

Adolescent↗
PubMedDate not supplied

Intellectual and educational attainment in albinism.

We studied the intellectual and educational ability of a group of 18 albino children as compared with that of a group of demographically matched controls. Intellectual ability was measured using Raven's Standard Progressive Matrices, and educational ability was measured by the reading, spelling, and arithmetic tests of the Wide Range Achievement Test. There was no statistically significant difference in intelligence of the two groups. However, there was a significant difference in reading, spelling, and arithmetic skills. No correlation was found between the clinical features of the albino children and their performance on the intelligence or educational tests.

Adolescent↗
PubMedDate not supplied

Uniocular childhood blindness: a prospective study.

PURPOSE: This prospective study examines uniocular blindness among children younger than 16 attending a large pediatric ophthalmology department. The aim was to identify the causes of uniocular blindness and determine how much is preventable. We defined blindness according to the World Health Organization definition of vision worse than or equal to 3/60. METHODS: All children who attended the department and were blind in one eye during the period of the study were included. A history was taken, visual acuity was assessed by an age-appropriate method, and an ocular examination was carried out. When necessary, the child also was seen by an orthoptist or pediatrician. RESULTS: A total of 71 patients were identified. At the time of diagnosis, the patients varied in age from a few weeks to 15 years. The causes were classified into one of the following diagnostic categories: chromosomal/genetic (8.5%), prenatal (47.9%), perinatal (7.0%), or childhood (36.6%). Many cases are prenatal in origin and are not preventable. Other causes that are difficult to prevent include retinoblastoma, toxocariasis, and trauma. One cause, amblyopia, may be preventable or treatable in most instances if detected early. CONCLUSIONS: Most cases of uniocular blindness are not preventable; however, protective glasses should be encouraged to prevent injury to the good eye.

Adolescent↗
PubMedDate not supplied

Randomized clinical trial of topical sodium hyaluronate after excimer laser photorefractive keratectomy.

BACKGROUND: Sodium hyaluronate (Healon) has been reported to promote corneal epithelial healing following severe eye burns in humans and rabbits. Endogenous hyaluronate has been shown to be significantly increased in the corneal tissue following extracapsular lens extraction, anterior segment trauma, and radial keratotomy. The authors report the use of topical sodium hyaluronate administration on the cornea of eyes which have been treated by photorefractive keratectomy for myopia. METHODS: Forty eyes of 40 patients undergoing excimer laser photorefractive keratectomy for myopia were studied to evaluate the effect of topical sodium hyaluronate versus placebo (Healon buffer) instillation on pain and corneal epithelial healing in an observer blind randomized clinical trial. RESULTS: There was no significant difference between patients whose eyes were treated with sodium hyaluronate and those treated with a placebo (Healon buffer), with regard to the amount of postoperative pain or the rapidity of corneal epithelial healing. CONCLUSIONS: Topical sodium hyaluronate administration showed no effect on pain or corneal epithelial healing following photorefractive keratectomy in this study.

Administration, Topical↗
PubMedDate not supplied

Ocular findings in glutaric aciduria type 1.

PURPOSE: To determine the nature and course of ocular abnormalities in glutaric aciduria (acidemia) type 1 (GA1). METHODS: Fifteen children with GA1 have been studied in the Republic of Ireland. A retrospective review of the records of the 6 children who died during their illness and prospective clinical examination of 9 survivors were performed. RESULTS: Seven of the 15 children had abnormal eye findings. Ocular complications included intraretinal hemorrhages, cataract, gaze palsy, strabismus, ametropia, and pigmentary retinopathy. CONCLUSION: Ocular involvement is common in glutaric aciduria. Complete ophthalmologic evaluation is recommended in all patients suspected to have this rare disease. Intraretinal hemorrhages due to GA1 could be misinterpreted as resulting from child abuse, and it is important to include this disorder with the differential diagnosis of child abuse.

Amino Acid Metabolism, Inborn Errors↗
PubMedDate not supplied

Two cases of hereditary optic atrophy associated with an enzymatic defect of the respiratory chain.

Hereditary optic atrophy is a significant cause of optic atrophy in children. We report two male siblings who suffered profound irreversible loss of vision over a 2-month period. Neither patient had any prior ophthalmic problems. Both siblings had a metabolic disorder characterized by transient episodes of ataxia and paresis, whereas in one brother a slight increase of lactate in blood and cerebrospinal fluid was detected during an acute episode. Respiratory chain defect in complex I was confirmed in one patient.

Adolescent↗
PubMedDate not supplied

Astigmatism following photorefractive keratectomy for myopia.

BACKGROUND: Astigmatism following photorefractive keratectomy for myopia has been reported as stable as early as 2 to 3 months. The authors report 36 out of 60 consecutive eyes with variations in the cylindrical component of their refraction at 6 months after laser treatment. METHOD: A standard photorefractive keratectomy was carried out on 60 consecutive eyes in 52 patients over a 7-month period. The manifest refraction of these eyes was followed for 6 months. RESULTS: Thirty-six eyes demonstrated a change in the cylindrical element of their refraction manifested as a change in cylinder power or axis, or both. The mean pretreatment cylinder power in the group that underwent a change in the cylindrical element was significantly higher than the mean of the group where this did not take place. The mean cylinder power change was 0.75 diopters (D) and in 9 eyes this change was 1.00 D or more. The corrected and uncorrected postoperative visual acuities were the same in the two groups. CONCLUSIONS: This observation implies meridional variability in the healing process of the anterior cornea following photorefractive keratectomy.

Adult↗
PubMedDate not supplied

Childhood penetrating eye injuries.

We reviewed 41 children with penetrating eye injuries from birth to 15 years of age between August 1982 to May 1990. Male to female ratio was 3.56:1. Thirteen (33%) cases resulted in monocular blindness. The distribution of penetrating injuries was based on the site of perforation: corneal, corneoscleral and scleral groups. The commonest cause of injury was child related from a thrown object (42%) and hazardous toys (21%).

Adolescent↗