Biomedical subjects
Publications and source records attributed to H M Goodyear.
A female infant with failure to thrive, erythroderma and recurrent diarrhoea died at 10 months of age from a Haemophilus influenzae respiratory tract infection. Her clinical course was complicated by a persistent metabolic acidosis. Investigations revealed a low level of the fourth component of complement and reduced neutrophil mobility.
Developmental progress, hearing, and dysmorphic features were monitored prospectively in eight babies with Norrie's disease (an X linked form of congenital blindness believed to be associated with mental retardation, regression, sensorineural deafness, and dysmorphic features) and in six congenitally blind peers during their preschool years. No evidence of sensorineural deafness or dysmorphology was found in the group with Norrie's disease. No significant difference in the rate of developmental progress occurred between the two groups. All 14 children showed continuing developmental progress and in 10 this was at a normal or superior rate. Two cases and two controls showed slowing in their rate of progress; in both groups a suboptimal developmental climate had prevailed and may have been contributory. The emphasis on serious and progressive associated disabilities in past reports has led to considerable distress for families of children with this disease. Our study suggests that these anxieties may often be illfounded. Parental depression constrains development, particularly when a baby is blind. More optimistic counselling with developmental guidance is recommended for children who are not overtly retarded in infancy until the long term developmental perspective of this disease is further clarified.