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Biomedical subjects

A R Fielder

Publications and source records attributed to A R Fielder.

125 records · Page 7Linked to original sources
PubMed1985

Primary thalamic haemorrhage in the newborn: a new clinical entity.

4 healthy full-term babies presented between 11 and 14 days of age with neurological abnormalities. Eye signs in 3 suggested involvement of tracts closely related to the thalamus. Spontaneous intrathalamic haemorrhage was confirmed with computed tomography and real-time ultrasound scans. Ventricular dilatation occurred in 3 babies and 2 of these needed ventriculoperitoneal shunts. The short-term neurodevelopmental outcome was fairly good.

Cerebral Hemorrhage↗
PubMed1985

Direct and maternal aspects of the risk of cataract with partial disorders of galactose metabolism.

Partial deficiencies of the enzymes of galactose metabolism can be associated with cataract, both directly and through maternal effects during pregnancy on enzymatically normal children. However, the associations are modest, variable and not obviously expressing cause and effect. We have recorded ophthalmological and biochemical observations including oral galactose tolerance on families with established enzyme deficiencies and/or cataracts, including possible effects during pregnancy. With the partial disorders a simple relationship between the extent of biochemical abnormality and the risk of cataract is not apparent and the association may be substantially coincidental. Cataract is common, and the attractive possibility that expression is significantly due to heterozygous or lesser deficiency of the enzymes of galactose metabolism, amenable to early dietary control of children or mothers at risk, is on present evidence not well supported.

Adolescent↗
PubMed1985

Delayed visual maturation.

Fifty-three infants with delayed visual maturation (DVM) are presented. These have been classified according to their ocular and systemic features into three groups: DVM as an isolated anomaly, in association with mental retardation, and ocular abnormalities accompanied by DVM. The clinical features are discussed, particularly regarding the time and speed of visual improvement in the three groups. Infants with DVM who experienced difficulties in the perinatal period have an increased risk of developing permanent neurological sequelae.

Developmental Disabilities↗
PubMed1985

Microphthalmos in a family.

Details of 22 members from three generations of a family exhibiting systemic and ocular abnormalities are presented. The former include mild mental retardation and a high incidence of abortion or death in the neonatal period. Ocular features comprise: microphthalmos, strabismus, hypermetropia, reduced ocular axial length and abnormal peripapillary pigmentation. The microphthalmos was seen in three forms: bilateral, severe or mild and severe microphthalmos of one eye with the fellow eye mildly affected. No individual with any degree of microphthalmos had a normal sized fellow eye and no normal individual produced an affected child. The genetic implications are discussed. The possible aetiologies of the various features are discussed and that this condition is a neurocristopathy is also considered.

Genes, Dominant↗
PubMed1984

Severe ocular side effects of perhexilene maleate: case report.

We report a case of perhexilene maleate (PEXID) toxicity in which the presenting feature was loss of vision secondary to chronic papilloedema. Vortex keratopathy similar to that seen in amiodarone keratopathy was present, and corneal and conjunctival biopsy findings are presented. To our knowledge this is the first case report of a keratopathy occurring in perhexilene toxicity. After withdrawal of the drug the papilloedema and keratopathy subsided, but some visual deficit remains. The properties of perhexilene maleate and other amphiphilic drugs are described, and the possible aetiology of vortex keratopathy is discussed.

Conjunctiva↗
PubMed1982

Tapetoretinal degeneration in the cerebro-hepato-renal (Zellweger's) syndrome.

Electrophysiological and histopathological study of a baby suffering from Zellweger's syndrome and presenting progressive retinal dysfunction showed this to be related to degenerative changes in the photoreceptor cells and pigment epithelium and to defective myelination of the optic nerve. Disturbances of bile acid and lysine metabolism were also demonstrated, lending support to the concept that Zellweger's syndrome is attributable to a widespread inadequacy of intracellular oxidative function.

Brain Diseases↗
PubMed1981

Problems with corneal arcus.

Corneal arcus presents many puzzling features. The correlation between its incidence and serum lipid levels is poor and, using immunoelectrophoresis, we have only been able to identify low-density lipoprotein inconsistently in corneae containing this deposition. Infrared thermography has shown us that arcus commences in the warmest regions of the cornea. We have considered the possible relevance of our biochemical and thermographic findings to other problems with corneal arcus such as its irreversibility, anatomical distribution, and clear zone.

Aged↗
PubMed1980

Dissociated visual development: electrodiagnostic studies in infants who are 'slow to see'.

Four infants were studied who failed to show any visual interest or following responses in the first three months of life. Although no definite ocular abnormalities were found at the time of presentation, both the parents and their medical advisers initially had serious concerns about the infants' visual prognosis. Initial electroretinograms (ERG) were found to be unequivocally normal, but three of the infants showed absent or impaired cortical visual evoked responses (VER). The fourth infant had an initial VER which was immature. Subsequently, all the infants showed increasing visual responsiveness from three to four months of age and all now have visual behaviour and general development appropriate to their ages. The VERs were repeated after four months of age and all showed normal responses. It is concluded that poor or even absent VER responses in early infancy may not always indicate a poor prognosis for vision. The possible mechanisms of this visual maturational lag are discussed.

Attention↗
PubMed1980

Ophthalmic manifestations of primary oxalosis.

The clinical and pathological findings of a 6-month-old infant with primary oxalosis, who died in renal failure, are presented. The oxalate crystalline deposition in the retinal pigment epithelium corresponded to the flecked retinopathy observed ophthalmoscopically. The difficulties in establishing a precise biochemical diagnosis are discussed and the relevant ophthalmic literature is reviewed.

Calcium Oxalate↗
PubMed1979

Immunoglobulins of normal aqueous humour.

Immunoglobulin levels have been measured in aqueous humour and blood from 79 patients undergoing cataract extraction. A linear relationship between serum and aqueous IgG was found, but this was not so for IgA, and IgM was not detected in any of the aqueous samples. The morphological features of both the blood-ocular and the blood-brain-cerebrospinal fluid barriers, and the influences governing the passage of immunoglobulins across these barriers are considered. The similarity between two theories of protein penetration, diffusion through the iris root into the eye, and via the "functional leak" into the cerebrospinal fluid, are considered. We feel that the discrepancy between IgG and IgA penetrations into aqueous, despite their similar molecular weights, may be due in part to the larger hydrodynamic volume and consequently increased frictional ration of IgA, although the existence of selective IgG transfer has not been excluded.

Aqueous Humor↗
PubMedDate not supplied

Lipid-protein constituents of human corneal arcus.

Extracts of fresh senile human peripheral cornea with varying degrees of arcus were prepared by soaking minced tissue in buffered saline/EDTA. Apolipoprotein B was, at most, an occasional feature of these extracts; interactions involving glycosaminoglycans were not evident; and the lipid composition, particularly of the cholesterol ester fraction, was also not consistent with a recent origin from plasma components and particularly form low density lipoprotein. Assuming this origin, substantial secondary changes must follow insudation, involving protein loss and lipid reesterification, as is described for lipid deposits forming intracellularly at other sites. The manner of these changes in deposit forming extracellularly in the avascular peripheral cornea is not clear.

Apolipoproteins↗