Obituary. V. Everett Kinsey, Ph.D. 1909-1978.
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Biomedical subjects
Publications and source records attributed to F W Newell.
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A unique form of dichromatic color vision is described in a family with incomplete achromatopsia. In 1966, incomplete achromatopsia was diagnosed in 4 of 14 children of a consanguineous marriage. The 4 affected had best visual acuities of 6/60 or 6/180, pendular nystagmus, and aversion to bright lights. The ERG showed minimal photopic responses. No abnormality of rod function was present. There was a severe color vision defect. In 1976, one of the patients returned for further color testing. Color tests included measurement of the luminous efficiency function using heterochromatic flicker photometry and colorimetric evaluation. The luminous efficiency function resembled that of the protanope. From the colorimetric measurements, we conclude that the patient has a unique form of dichromatic color vision mediated by two visual photopigments: the normal MWS cone photopigment and a photopigment with the spectral characteristics of rhodopsin.
We studied the electroretinogram in normal patients, patients with opacities of the anterior segment, and patients with vitreous hemorrage, using a high-intensity light stimulus delivered either through the sclera or through the pupil. We found the response obtained with transscleral stimulation a consistent indicator of gross retinal function. Since the stimulus entered the eye through the sclera, it provided a method for studying the retinal responses independent of opaque or translucent media.
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A 9-year-old boy, the product of a consanguineous marriage, had visual acuity of 6/60, pendular nystagmus, and a bull's-eye type of macular atrophy. A sensorineural deafness was present. The photopic electroretinogram was extinguished; the electro-oculogram was normal. There was associated mental retardation and failure of inhibition of the pituitary gland by high levels of circulating thyroid hormone. Two older siblings although not examined, had similar endocrine abnormalities. Non-involvement of three half-siblings suggested autosomal recessive inheritance.
To record the electro-oculogram in infants without using general anesthesia and passive globe rotation, we used the static vestibular reflex in which compensatory eye movements occur in response to angular rotation of the semicircular canal. To produce these eye movements, we placed the infant in a supine position in his mother's arms in a rocking chair and, while he nursed from a bottle, directed his attention to a distracting stimulus. We recorded the electro-oculogram during rocking under conditions of light and dark adaptation. We used the electro-oculograms to calculate the conventional light-to-dark amplitude ratio.
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A 23-year-old man had slow psychomotor development at 6 months of age and developed intermittent corneal clouding at about 18 months. He developed a truncal ataxia and hypotonia of the limbs combined with spasticity and active deep reflexes that did not progress. His skeleton and facies were normal. Between 1 and 13 years of age, he developed severe optic atrophy, absence of retinal blood vessels, and an extinguished electroretinogram. Biochemical analysis of cultured fibroblasts indicated no lysosomal hydrolase deficiency; cellular metachromasia was absent and there was no mucopolysaccharidoses. Ultrastructural studies indicated single-membrane-limited vacuoles containing lamellated membranes and a polymorphous substance in tissue-cultured cells and conjunctiva.
General anesthesia permits surgery involving large operative fields without limit to time. It is essential in children and uncooperative adults and provides an immobile, quiet patient who does not recall the operative procedure. Local and regional anesthesia re indicated when the surgical field is limited in size and extent. Neuroleptanesthesia in ophthalmology usually involves the administration of droperidol followed by fentanyl followed in intubation, sometimes using a muscle relaxant, and maintenance of anesthesia with nitrous oxide. The terrifying dreams produced by ketamine may be minimized by preoperative administration of diazepam and a quiet recovery period. Attempts to eliminate the oculocardiac reflex are unpredictable and unreliable and, with careful monitoring of anesthesia, elimination does not seem essential. Malignant hyperthermia is an autosomal dominant disorder occurring mainly with the administration of halogenated fluoroethane and depolarizing muscle relaxants. It is often signaled by sudden and prolonged muscle spasm following the injection of the muscle relaxant. Treatment must be directed toward hyperventilation using oxygen, correction of the metabolic respiratory acidosis, and reduction of fever.