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Biomedical subjects

D Taylor

Publications and source records attributed to D Taylor.

At least 379 records · Page 21Linked to original sources

Follow-up and diagnostic reappraisal of 75 patients with Leber's congenital amaurosis.

We reexamined 75 children in whom Leber's congenital amaurosis had been previously diagnosed. On review, 30 of these patients had an ocular or systemic disorder other than Leber's congenital amaurosis. The most common of these revised diagnoses were congenital stationary night blindness, achromatopsia, infantile-onset retinitis pigmentosa, Joubert's syndrome, Zellweger syndrome, and infantile Refsum's disease. Of the 45 patients with Leber's congenital amaurosis, mental retardation occurred in six patients, and visual deterioration in six patients. Leber's congenital amaurosis should only be diagnosed if other known ocular and systemic disorders have been carefully excluded.

Blindness↗

Ocular manifestations of the congenital varicella syndrome.

Three children developed the congenital varicella syndrome following a maternal varicella infection during the second trimester of pregnancy. Systemic findings included a bulbar palsy, mild hemiparesis, cicatricial skin lesions, developmental delay, and learning difficulties. Ocular findings included chorioretinitis, atrophy and hypoplasia of the optic discs, a congenital cataract, and Horner's syndrome. Electroretinograms and pattern-reversal visual-evoked potentials recorded from the four eyes with chorioretinitis were attenuated and degraded. Two of the children had been misdiagnosed as having congenital toxoplasmosis. Chorioretinal scars probably occur more frequently with the congenital varicella syndrome than has been previously recognized and should be included in the differential diagnosis of congenital chorioretinal scars. Hypoplasia of the optic disc may develop after an intrauterine varicella infection during the 16th week of gestation. Children with the congenital varicella syndrome should be examined by an ophthalmologist to exclude ocular abnormalities. These abnormalities are often subtle and difficult to diagnose correctly.

Chickenpox↗

Joubert syndrome.

Joubert syndrome is an autosomal recessive condition in which there is a variable combination of central nervous system defects with a distinctive congenital retinal dystrophy, ocular motor abnormalities, and respiratory abnormalities in early infancy. The retinal dystrophy has been previously classified as a variant of Leber's congenital amaurosis. We report electrophysiologic and eye movement findings in a series of seven consecutive children with Joubert syndrome. Unlike patients with Leber's congenital amaurosis, all but one of these children had preserved flash and pattern-reversal visual evoked potentials. Six of the seven children had abnormalities of smooth pursuit, optokinetic nystagmus, and saccades. Six of the children had nystagmus: three had a pendular torsional nystagmus and three had a form of see-saw nystagmus. An alternating hyperdeviation was present in five of the patients, two of whom also had a tonic deviation of their eyes laterally. All seven patients had cerebellar vermis hypoplasia on a magnetic resonance imaging scan. Developmentally delayed children with an absent or highly attenuated electroretinogram should be investigated for Joubert syndrome.

Blindness↗

Delayed visual maturation. A longitudinal clinical and electrophysiological assessment.

Delayed visual maturation is an idiopathic condition characterized by visual inattention during infancy. The authors followed longitudinally nine children with an initial diagnosis of delayed visual maturation and compared their electroretinograms (ERGs) and visual evoked potentials with those of age-matched controls. Eight of the nine patients consistently had normal visual evoked potentials to flash and to pattern stimulation. All of the children had normal ERGs. Visually mediated behavior gradually developed in all of these children when they were between 3 and 8 months of age (mean, 5.5 months). Five patients also were delayed in other spheres of development. Visual evoked potentials are helpful in formulating a visual prognosis for children with delayed visual maturation.

Age Factors↗

Amino acid requirements for the growth and production of some exocellular products of Staphylococcus aureus.

A simple, defined synthetic medium has been developed for the growth of a toxic-shock-syndrome-associated Staphylococcus aureus strain (FRI 1187) in continuous culture, which gives a high growth yield and assayable amounts of acid phosphatase, haemolytic activity, hyaluronate lyase and toxic-shock-syndrome toxin-1. It consists of multiple ions, ammonium sulphate, glucose, cystine, aspartate, glutamate, arginine, glycine, proline, nicotinic acid and thiamine.

Amino Acids↗

Acceptance of the mental illness label by psychotic patients: effects on functioning.

Labeling theory predicts that psychotic patients who accept the label of mental illness will function less well than those who reject their diagnosis. Accepted psychotherapeutic theory suggests the reverse. Two predictive models were tested. Results supported the central hypothesis of the psychotherapeutic model but not that of the labeling model, although some elements of the latter model were affirmed. Besides acceptance of diagnosis, an internal locus of control appears important for good outcome in psychosis.

Adaptation, Psychological↗

Visual evoked potentials in dissociated vertical deviation: a reappraisal.

Pattern reversal and flash evoked potentials were recorded in 13 children with dissociated vertical deviation (DVD). No electrophysiological evidence was found to support the notion that patients with DVD have an anomalous (albinoid) projection of visual fibres originating from the temporal retina of each eye. However, DVD patients had significantly smaller monocular and binocular pattern evoked responses than age matched controls. Explanations are given for this finding and for the occipital VEP asymmetries reported by other workers.

Adolescent↗

Childhood leukaemia: relapse in the anterior segment of the eye.

A proved first relapse occurred in the anterior segment of eight children with acute leukaemia, two of whom had concurrent central nervous system or bone marrow relapse. A further child developed uveitis after remission was induced, but in this patient no causal relationship with leukaemia was established. Uveitis in children who have had acute leukaemia should be regarded as evidence of relapse, and anterior chamber aspiration and iris biopsy are essential procedures in their evaluation. The outlook for children with anterior segment relapse remains poor despite intensive local and systemic treatment.

Child, Preschool↗

Reinforcement of bone cement using metal meshes.

Following on from previous work reported in this journal, a practical system has been developed for in situ fibre reinforcement of bone cement in the hip joint prosthesis. A fibre preform is inserted during the operation, surrounding the metal stem of the prosthesis, forming a composite with the cement. Tests performed on full-scale models of the joint show improvements in fatigue life by more than an order of magnitude when a preform of metal fibres is used; this significantly delays the onset of cement cracking and stem loosening. Initial results reported previously suggest that other fibre materials such as carbon may give even greater improvements. Clinical trials have begun, using a form of the metal mesh.

Bone Cements↗

Combined epidural and general anesthesia versus general anesthesia in patients having colon and rectal anastomoses.

Three retrospective studies were conducted at St. Vincent's Hospital to compare the outcomes of colorectal anastomoses, with and without resections, with respect to anesthetic technique. Operations were performed upon patients anesthetized with either combined regional (epidural) and general anesthesia (CRAG) or general anesthesia alone (GA). Postoperative pain relief was achieved with either continuous epidural analgesia (CEA) in the CRAG group or with postoperative narcotics in the GA groups (GA/PN). In one group, a different regimen was introduced: combined epidural and general anesthesia with postoperative epidural morphine (CRAG/EDM). Overall, anastomotic leak rates and death rates were lower in the CRAG group, and the lowest incidence of anastomotic leak was reported in the patients receiving CEA. Thus the reduced leak rate was associated more with the postoperative analgesia regimen than with the anesthetic technique. An increased incidence of wound dehiscence occurred with postoperative epidural morphine analgesia.

Analgesia, Epidural↗