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Biomedical subjects

A Q McCormick

Publications and source records attributed to A Q McCormick.

At least 19 recordsLinked to original sources

Visual prognosis in children with chronic anterior uveitis and arthritis.

OBJECTIVE: To determine the visual and ocular prognosis for children with uveitis and chronic arthritis and in patients with uveitis with juvenile rheumatoid arthritis (JRA) or juvenile psoriatic arthritis (JPsA) and to evaluate risk factors associated with ocular complications. METHODS: We studied 49 children with chronic arthritis having greater than 2 years ophthalmological followup from onset of uveitis. Visual acuity and ocular complications (band keratopathy, synechiae, cataracts, glaucoma, or phthisis bulbi) were documented. For the 45 patients with JRA/JPsA, the antinuclear antibody and HLA status, time and mode of onset, and the course of uveitis, were evaluated as risk factors for developing complications. RESULTS: Mean followup was 9.4 years from diagnosis of uveitis (82 affected eyes). Ocular complications developed in 27 eyes (33%). Visual impairment (corrected acuity 20/50 or worse), occurring only in the presence of complicated uveitis, was present in 12 eyes (15%). Of 45 patients with JRA/JPsA, over 95% developed uveitis within 5 years of onset of arthritis. Those with complicated uveitis (n = 13, mean followup 8.6 years) and uncomplicated uveitis (n = 32, mean followup 10 years) were compared: factors significantly associated with complicated uveitis were (1) a chronic course of uveitis (2) JPsA (3) diagnosis of uveitis prior to, or at the time of arthritis onset (4) symptomatic onset. CONCLUSION: The risk of developing uveitis 5 years after the onset of JRA/JPsA is small. Although ocular complications were common (33%) among patients with uveitis, normal vision was maintained or correctable for over half of them. Those with uveitis and risk factors for developing ocular complications may need close ophthalmological scrutiny.

Adolescent↗

Experimental arthritis and uveitis in rats associated with Mycobacterium butyricum.

OBJECTIVE: To determine if the anterior uveitis associated with adjuvant arthritis (AA) in the rat can be passively transferred with arthritis to syngeneic recipients using spleen cells or T cell lines prepared from animals given complete Freund's adjuvant (CFA) and Mycobacterium butyricum (M. butyricum) in incomplete Freund's adjuvant (IFA). METHODS: Spleen cells from Lewis or Lewis SsN rats given IFA, CFA, type I collagen in IFA (CI-IFA), or type II collagen in IFA (CII-IFA) were administered to naive rats or rats treated with pertussis toxin or bacterial endotoxin. Three CD4+ T cell lines, propagated from CFA injected rats and maintained in vitro with M. butyricum (M-1), bovine proteoglycan (PR-1) or an extract of M. butyricum (MBE-1) were administered to naive or immunosuppressed rats. The arthritogenic and uveitogenic properties of these cell preparations and intradermal MBE-IFA, CII-IFA and intraperitoneal (ip) M. butyricum without adjuvant were evaluated. RESULTS: Uveitis was observed in 15/69 (22%) arthritic rats given CFA. Spleen cells prepared from CFA injected rats caused arthritis in 55 (82%) and uveitis in 2 (3%) of 67 cell recipients. Uveitis occurred in 2/6 cell recipients pretreated with bacterial endotoxin. Neither uveitis nor arthritis was observed in rats given IFA (0/6) or spleen cells prepared from rats given IFA (0/27), CI-IFA (0/6), or CII-IFA (0/28). CII-IFA produced polyarthritis in 5/6 rats, but no uveitis. CII-IFA induced arthritis associated uveitis in 1/15 animals receiving spleen cells from rats given CII-IFA, but not those given CI-IFA (0/3) or IFA (0/13). Uveitis was observed in one recipient of the M-1 T cell line and in 2 recipients of the PR-1 T cell line. Immunization with 400 micrograms of MBE-IFA induced uveitis but not arthritis in 3/11 animals. The MBE specific T cell line was neither arthritogenic nor uveitogenic. A high frequency (5/6) of uveitis accompanied arthritis in male Lewis rats given ip M. butyricum. Arthritis occurred in 4/10 female Lewis rats given ip M. butyricum and 2 arthritic animals also developed uveitis. CONCLUSION: Uveitis occurs infrequently in arthritic rats given spleen cells from CFA injected animals. The ip administration of M. butyricum constitutes a novel disease model in which the immunopathological relationships between arthritis and uveitis may be more reliably studied.

Animals↗

Nonpigmented congenital iris stromal cyst.

A nonpigmented iris stromal cyst was observed in the right eye of a 6-week-old girl. There was no past history of trauma or use of topical miotics, and the remainder of the ocular examination was normal. The pathophysiology and management of this rare developmental anomaly are controversial.

Cysts↗

Retinopathy of prematurity-induced blindness: birth weight-specific survival and the new epidemic.

A recent population-based study in the Canadian province of British Columbia showed that, since the mid-1960s, there has been a significant increase in the incidence of retinopathy of prematurity-induced blindness in infants weighing 750 to 999 g at birth. To determine the impact of changing birth weight-specific survival on this new epidemic, all infants born in the province in the period 1952 through 1986 and known to the British Columbia Health Surveillance Registry as having retinopathy of prematurity-induced blindness were identified. In addition, the birth registration records for the 1,299 740 infants born in British Columbia in the same period and the death records of the 22,940 British Columbia-born infants who died in the province before the end of their first year of life were linked using a combination of probabilistic and manual record linkage techniques. These linked records and the records from the Health Surveillance Registry were used to calculate birth weight-specific incidence rates of retinopathy of prematurity-induced blindness in liveborn infants and first-year-of-life survivors. The rates, in 5-year intervals, showed that, in both liveborn infants and first-year survivors, the highest birth weight-specific rates occurred during the first epidemic of retinopathy of prematurity, which ended in British Columbia in 1954. Since the mid-to late-1960s, the incidence of retinopathy of prematurity-induced blindness in liveborn infants weighing less than 1000 g increased steadily whereas in infants weighting 1000 to 1499 g, incidence decreased slightly since the original epidemic ended. However, the experience of first-year-of-life survivors is substantially different.(ABSTRACT TRUNCATED AT 250 WORDS)

Birth Weight↗

Uveitis and arthritis induced by adjuvant: clinical, immunologic and histologic characteristics.

The intradermal administration of complete Freund's adjuvant (CFA) containing Mycobacterium butyricum to Sprague-Dawley (SD) and Lewis strain rats results in polyarthritis and uveitis. Over 90% of the eyes examined from the SD rats given CFA had histologic evidence of anterior uveitis, clinically evident in only 20 to 28%. Many rats developed arthritis without clinical uveitis, but uveitis was rare in the absence of arthritis. Histologically, the ocular inflammation was characterized by a polymorphonuclear, and later, a lymphocytic infiltration of the iris and ciliary body with cells and fibrinous exudate in the anterior chamber and cells in the vitreous. Antibodies and cellular immunity to ocular (S antigen, alpha crystallin), articular (type II collagen, proteoglycan) and bacterial components (MDP), were demonstrated in some rats, but positive tests did not correlate with either articular or ocular disease. Ten percent of rats given type II collagen in incomplete Freund's adjuvant developed uveitis. Thus, the pathogenesis of the arthritis and uveitis in the adjuvant model may be mediated by lymphocytes which exhibit crossreactivity with antigens in these structures, although the specificity of such antigens has not been identified in our studies.

Animals↗

Retinopathy of prematurity: a new epidemic?

This study provides the first empiric evidence for the existence of a new epidemic of retinopathy of prematurity-induced blindness. Data from a population-based register of handicapping conditions in the Canadian province of British Columbia, and a birth weight-specific census of live-born infants in British Columbia, were used to determine annual, population-level incidences of retinopathy of prematurity-induced blindness during 1952 to 1983. Changes in incidence since the end of the original epidemic (1954) were determined by subdividing the 29-year period (1955 to 1983) into two intervals (1955 to 1964 and 1965 to 1983). Standardized incidence ratio analyses revealed a marginally significant increase in the overall incidence of retinopathy of prematurity-induced blindness in the later as compared with the earlier period. Infants weighing 750 to 999 g at birth had a significantly increased standardized incidence ratio of 3.07 (95% confidence interval 1.26, 11.06). No increases in risk were observed in heavier or lighter weight infants. Because ascertainment and diagnostic changes do not explain the weight-specific increases in incidence, these results provide the first population-level evidence for a new epidemic.

Blindness↗

Juvenile galactosialidosis in a white male: a new variant.

We describe a 19-year-old white male with juvenile galactosialidosis. He presented with hip arthralgia and was found to have facial "coarseness," corneal clouding, mitral and aortic insufficiency, and hepatosplenomegaly. Ultrastructural studies of skin biopsy and peripheral blood lymphocytes showed membrane-bound inclusions containing sparse fibrillogranular material. Biochemical analysis showed elevated urinary sialyloligosaccharides and no free sialic acid. Fibroblast enzyme analysis showed low activities of both alpha-neuraminidase and beta-galactosidase. To date, most patients with juvenile galactosialidosis have been Japanese. However, unlike those patients, our patient did not have macular cherry-red spots, neurologic abnormalities, or mental retardation. We speculate that this young man represents a new subtype of juvenile galactosialidosis with a potentially different molecular defect from that of the Japanese variant.

Adult↗

Visual loss following traumatic asphyxia in children.

Two young children suffered loss of vision in separate incidents following traumatic asphyxia. Visual loss secondary to trauma can occur from a variety of causes. This is apparently the first description of traumatic asphyxia causing visual loss in children. In children, visual impairment is easily missed unless the degree of handicap is severe. All children should have their fundi and visual acuity assessed before discharge following significant trauma, including all episodes of traumatic asphyxia.

Asphyxia↗

The unequal nystagmus test.

In children who exhibit nystagmus secondary to visual impairment, the better eye determines the quality of the nystagmus. 40 children with different visual acuities and 10 with the same vision in each eye were studied. By inspecting the behaviour of the nystagmus in each eye, while covering the other, it was possible to identify which eye had better foveal vision. This so-called 'unequal nystagmus test' is very simple to do and it offers valuable information.

Adolescent↗

Changing pattern of retinopathy of prematurity: a 37-year clinic experience.

A retrospective analysis was done of multi-disciplinary neurodevelopmental assessments in 70 children who were legally blind because of cicatricial retinopathy of prematurity. The subjects lived in British Columbia and were born during a 37-year period between 1951 and 1987. The purpose of the study was to investigate changes in the perinatal characteristics and to evaluate the associated handicaps. All patients were assessed at least once in the Visually Impaired Program, British Columbia Children's Hospital. In the majority, the visual loss was profound. Since 1951, blinding retinopathy of prematurity has become a disease of progressively smaller and less mature infants. Since 1981, it has been almost entirely confined to infants of birth weight less than 1,000 g in British Columbia. The diagnosis of mild spastic diplegia was made more commonly in patients born after 1980 but, despite the progressive reduction in birth weight and gestational age during the study period, the number of patients without other associated handicaps remained constant (approximately 30%) during each successive decade.

Birth Weight↗

Proptosis and diplopia following traumatic asphyxia.

We describe a previously unreported complication of traumatic asphyxia. An 11-year-old boy developed proptosis and diplopia following a crush injury in an automatic garage door. Computed tomography scan confirmed displacement of the eye. There was no retrobulbar hemorrhage or skull fracture, and the proptosis appeared secondary to traumatic displacement of orbital fat. Proptosis and diplopia resolved completely over six weeks. Visual disorders may be overlooked as a complication of trauma.

Accidents, Home↗

Eye and head movements of visually impaired children.

Fixation, nystagmus, pursuit, rapid voluntary, other types of eye movements and associated head movements of visually impaired children were studied. It was concluded that their eye and head movements reflect the severity and onset of their visual loss.

Child↗

Permanent cortical visual impairment in children.

Fifty patients with permanent cortical visual impairment were evaluated. They had a characteristic behaviour profile, usually with residual sight but poor visual attention. 30 of the 50 also had damage to the anterior visual pathway. Visual evoked potential mapping was shown to have a clear advantage over visual evoked responses, and using that in conjunction with CT and clinical data enabled several subgroups of cortical visual impairment to be identified. The diagnosis probably is more common than previously recognised, and should be suspected when there is greater delay in visual development in other areas and the degree of visual loss is unexplained by ocular findings. Using traditional criteria for cortical blindness may mean that many children are not diagnosed, which has serious implications for their rehabilitation.

Adolescent↗

Permanent visual loss after shunt malfunction.

Fourteen (1.8%) of more than 800 children evaluated in a Visually Impaired Program over 10 years became permanently blind during an episode of raised intracranial pressure secondary to shunt malfunction. Visual symptoms and papilledema were recognized in only three children at the onset of blindness. Clinical and radiologic findings suggested that the loss of sight was caused by a lesion in the pregeniculate pathway in nine patients, probably due to ischemia; five children in the postgeniculate group sustained infarcts of the occipital lobes. These cases highlight the importance of parent education and prompt treatment of raised intracranial pressure due to shunt malfunction.

Blindness↗

Clinical spectrum of congenital optic nerve hypoplasia: review of 51 patients.

Fifty-one patients with congenital optic nerve hypoplasia (CONH) were reviewed. It was found that the risk of having an affected child is higher in an adolescent mother, and that maternal alcohol or drug abuse may be important factors. Frequently the disorder is associated with other neuropsychiatric handicaps, and with neuro-endocrine abnormalities. The findings suggest that CONH probably is not a homogeneous group of disorders; some may be caused by primary failure of differentiation of the retinal ganglion cells, while others may be the product of an acquired transsynaptic degeneration of optic-nerve fibres.

Abnormalities, Multiple↗

Whiplash shaking syndrome: retinal hemorrhages and computerized axial tomography of the brain.

Three cases of whiplash shaking injury of infants are presented. All children presented with seizures and had minimal signs of external injury. Examination of the retina revealed extensive retinal hemorrhages. These were missed on initial examination and were only discovered after pupillary dilatation. The presence of these palecentered retinal hemorrhages suggested the diagnosis of child abuse and skeletal surveys and thorough social histories confirmed the diagnosis. Despite extensive retinal hemorrhages, computerized axial tomography (C.T.) scan showed minimal inter-hemispheric bleeding. In contrast to the "Battered Child Syndrome," all the findings in whiplash shaking syndrome of infants are subtle and demand an awareness, an index of suspicion and a thorough examination which may include extensive retinal examination following dilatation of the pupils. This latter examination is frequently not performed by family physicians and residents so that the syndrome may be missed. A fourth case is also discussed where shaking is admitted on initial presentation but said to be done for resuscitation. This poses an immense diagnostic dilemma to the pediatrician since in this case the child presented later with all the signs of physical abuse.

Brain↗