Marital stability and congenital heart disease.

The incidence of divorce or legal separation was studied in 438 families of children born with heart disease who entered the New England Regional Infant Cardiac Program between 1968 and 1973. The parents were interviewed when the children were 5 1/2 years old. The rate of divorce in 438 families of children with critical congenital heart disease was not significantly different from the rate in two comparison groups: (1) 25 families of children whose cardiac defect was spontaneously cured, and (2) 26 families of children catheterized in infancy for suspected cardiac defect but who were found to be free of heart disease. Rates of divorce or legal separation for the three groups were: critical congenital heart disease, 12.1%, spontaneously cured, 4.2% free of heart disease, 11.5% these rates were not significantly different. The average national divorce rate was 20.3% for the same period.

Oral and written language abilities of XXY boys: implications for anticipatory guidance.

Previous studies of XXY boys suggest that they are at risk for certain communication disorders involving oral and written language. In this study, the language, reading, and spelling skills of a group of 14 XXY boys identified during neonatal cytogenetic screening were compared with those of a group of 15 control children. The two groups were matched for age, grade, race, parity, birth weight, parental age and education, and socioeconomic status. The mean age of the XXY group was 9 years 7 months and that of the control group was 9 years 3 months. The mean academic grade level for both groups was at the transition between third and fourth grade. There was selective reduction in verbal IQ scores for the XXY group and no significant differences apparent between groups for performance IQ scores. The decrease in verbal IQ was associated with a reduced full-scale IQ and also with significant problems in expressive language, auditory processing abilities, and auditory memory. Word-finding difficulties and problems in the production of syntax were major components of the specific expressive language deficit. Except for difficulties in the understanding of complex sentence structures, the receptive language skills of XXY boys did not differ significantly from those of the control group. These reductions in speech and language abilities correlated with decreased achievement by the XXY group on a variety of reading and spelling tasks. These results suggest that difficulty learning how to read and spell may be due to a preexistent language disability. Early attention to such expressive language problems may be essential to ameliorate secondary maladaptive behaviors due to chronic language-related learning disabilities.

Origin of the retrocorneal membrane in the rabbit.

Electron microscopic studies have offered presumptive evidence that the origin of both cellular and extracellular components of the retrocorneal membrane (RCM) derive from corneal endothelial cells. In an attempt to demonstrate conclusively the origin of the cell in the RCM, we performed 8-mm exchange, penetrating keratoplasties between male and female rabbits. After allowing for complete healing and using only clear corneas, we produced central RCM by freezing the central cornea after inducing intraocular inflammation. Care was taken to ensure that the membrane was surrounded by normal donor endothelium and Descemet's membrane was intact. Sex chromatin counts of the cells in the RCM of a female host with a male graft showed an average of 2% Barr bodies. Cells in the membrane of a male host with a female graft showed an average of 40% Barr bodies. The experimental and control sex chromatin counts were almost identical. This study provides conclusive evidence that central retrocorneal, fibroblast-like cells and membranes in rabbits are derived from corneal endothelial cells.

Psychological functioning in children with cyanotic heart defects.

The relationship between congenital heart disease (CHD) and psychological functioning was studied in 63 children with transposition of the great arteries (TGA) and 77 children with tetralogy of Fallot (TF). These youngsters were compared with a group of 36 children originally diagnosed with CHD, but who spontaneously recovered (SR) without medical intervention. All children were under one year of age when diagnosed and were given psychological testing between 5.5 and 6.3 years. Children with TGA and TF had poorer overall psychological functioning and significantly greater central nervous system (CNS) impairment than the SR children. However when the effects of IQ and CNS impairment were controlled, there were no differences between groups. The diagnosis of a severe cyanotic heart defect does not appear to make a child more likely to have emotional disorder in the absence of other factors.

Aspects of corneal wound healing in health and disease.

The paper reviews current aspects of repair processes of the epithelial, stromal, and endothelial layers for both the normal and diseased cornea.

Maintenance of mydriasis with topical indomethacin during and after penetrating keratoplasty in the rabbit.

The use of indomethacin eyedrops, 0.5%, in combination with topical atropine and phenylephrine, prior to and immediately following an 8-mm penetrating keratoplasty in rabbits maintains maximal pupillary dilation. With this technique, we achieved clear grafts without synechiae in virtually every instance.

Spatial and temporal processing in patients with Turner's syndrome.

Thirteen females with Turner's syndrome were examined for spatial abilitles and serial processing, and their performance was compared with that of normal females matched by age, intellignece, and socioeconomic class. Patients with Turners's syndrome performed significantly poorer on tests of spatial ability than controls, but only on spatial tests requiring the integration of isolated elements as synthetic wholes or the remembering of spatial configurations which could not be verbally mediated. Patients also performed less well than controls on tasks of serial processing when the tasks could not be mediated verbally. 2t was concluded that patients with Turner's syndrome may have a selective deficit in cortical functions that are lateralized to the right cerebral hemisphere.

Cognitive function and age at repair of transposition of the great arteries in children.

To assess the effect of the duration of chronic hypoxemia on cognitive function, we studied 38 children with d-transposition of the great arteries and an intact ventricular septum who underwent corrective surgery at six months to six years of age (median, 1.6 years). Tests included the Wechsler Preschool and Primary Scale of Intelligence (WPPSI) in 33 children and the visual-association and auditory-association subtests of the Illinois Test of Psycholinguistic Abilities in 38 and 37 children, respectively. When controlled for social index, age at repair was inversely associated with the WPPSI intelligence-quotient score (P less than 0.01), the visual-association subtest score (P less than 0.01), and the auditory-association subtest score (P less than 0.1). In contrast, age at repair correlated poorly with cognitive function in children with ventricular septal defect, an acyanotic congenital heart defect. These data suggest that postponing repair of a cyanotic congenital heart disease, such as transposition of the great arteries, is associated with progressive impairment of cognitive function.

Preliminary observations on language and learning in XXY boys.

Difficulties with processing, storing and retrieving, and producing linguistic information were particularly evident among the XXY boys. These decrements in abilities were associated with decreased achievement in a variety of reading and spelling tasks in this study as well as in that of Graham et al [4]. The data suggest that a continuum of language disability is present throughout the early life of these children and is associated with the XXY anomaly. Furthermore, when considered in relation to cross-sectional observations on older Turner syndrome patients, these data suggest the possibility that pathologic variations in sex chromosome complements may differentially affect brain development and subsequent functioning of the children.

Hodgkin's lymphoma presenting as a protein-losing enteropathy: a case report.

A 10-year-old boy presented with oedema. Panhypoproteinaemia due to excessive enteric protein loss was demonstrated, and further investigation revealed the cause to be lymphocyte-depleted Hodgkin's lymphoma. Appropriate chemotherapy resulted in a dramatic improvement in the serum protein level and resolution of the proptosis and lymphadenopathy.