Biomedical subjects
Publications and source records attributed to M G Pike.
Three children are described in whom herpes simplex encephalitis (HSE) followed a clearly biphasic course. The secondary deterioration may be due to a resurgence of the viral infection and calls into question the adequacy of current treatment regimens for HSE. Alternatively, a postinfectious neuroallergic process may be active in which case immunomodulatory treatment might be more appropriate than further antiviral treatment.
An infant girl had the clinical and immunologic findings of congenital rubella syndrome but also had arthrogryposis multiplex and calcific epiphyseal stippling. Spastic quadriparesis developed, and both physical and behavioral development were slow. Increased spasticity of the legs at 5 1/2 years was related not to progressive rubella encephalomyelopathy but to spinal cord compression by abnormal cartilaginous tissue. The presence of a peroxisomal disorder was demonstrated by a greatly increased level of phytanic acid and slightly increased levels of hexacosanoate in serum and by reduced activity of peroxisomal dihydroxyacetone phosphate acyltransferase and a slightly increased ratio of cytosolic to peroxisomal catalase activity in cultured fibroblasts. A reduction in the number and size of peroxisomes was demonstrated in cultured fibroblasts, and a needle biopsy specimen of the liver also showed the peroxisomes to have a smaller diameter than usual. We recommend that any child with epiphyseal stippling be assessed for peroxisomal disease and that the potential for spinal cord compression by dysplastic bone or cartilage be recognized. The association of peroxisomal dysfunction with congenital rubella has not been described previously. The interaction between rubella virus infection and peroxisomal function may need further investigation.
To determine the value of computed tomography and electrophysiologic studies in predicting neurologic outcome, we prospectively studied 41 children with acute bacterial meningitis, using clinical examination, computed tomography of the head, electroencephalography, brain-stem auditory evoked response, and visual evoked potential mapping during the acute illness. Two children died; 32 of the remaining 39 children were reviewed clinically, electrophysiologically, and with computed tomography between 5 and 38 months after the illness. The electrophysiologic data obtained during the illness were not found to alter the acute-stage management. Focal or generalized suppression, demonstrated on the electroencephalogram, was associated with a poor outcome. Cerebral infarction and edema, demonstrated by computed tomography of the head, were predictive of a poor outcome, but enlarged ventricular and subarachnoid spaces and increased subdural effusions were of no predictive value. Neither computed tomographic scans nor electrophysiologic data were better indicators of neurologic prognosis than the clinical examination.
Ninety-seven children who were born between 1954 and 1986 and presented to the Visually Impaired Program of British Columbia's Children's Hospital, Vancouver, Canada, with a primary ophthalmologic diagnosis of cataracts, were assessed neurologically, ophthalmologically, audiologically, and developmentally. Causal factors included prenatal infection (35 cases), hereditary cataracts (22 cases), various syndromes and metabolic disorders (9 cases), trauma (1 case), and unknown (30 cases). Ninety children were diagnosed to have congenital cataracts while 7 acquired them. Findings indicated that prenatal infection continues to be a cause of infantile cataracts, despite rubella immunization; that prematurity is not, as has been stated in the past, a cause of infantile cataract; and that careful neurological, audiological, and developmental examination is vital in the assessment of likely causes of this condition.
We describe the clinical, biochemical and ophthalmic findings in four closely related males with X-linked ichthyosis and hypogonadism. Recognition of this association is important for both paediatricians and paediatric dermatologists.
Severe allergic protein-losing enteropathy has been described in childhood atopic dermatitis (AD). Minor morphologic and functional abnormalities of the gastrointestinal mucosa, including altered permeability to oligosaccharides, are not uncommon in this condition, but the prevalence of occult enteric protein loss is unknown. We measured the random fecal alpha 1-antitrypsin (AAT) concentration, an indicator of enteric protein loss, in children with and without AD and found no significant difference between the groups. In half of the patients with AD, gastrointestinal permeability to oligosaccharides was also measured, and no relationship between this and fecal AAT was found.
We describe a child in whom severe atopic dermatitis (AD) and a neutrophil chemotactic defect were complicated by acute bacterial endocarditis following recurrent staphylococcal skin infections.
The parents of 128 children with atopic eczema and 117 healthy control children responded to a questionnaire that included measurement of their own and their children's heights at home after standardised instructions. When cases and controls were compared there was no significant difference in parental height but the children with eczema were significantly shorter than the healthy control group. This difference remained significant when children with associated asthma were excluded from the analysis.
Sixty six children with severe atopic eczema were treated with highly restricted ('few food') diets followed, if they improved, by serial reintroduction of excluded foods. Twenty four patients (36%) improved considerably during the few food phase of the diet. Fifteen of these (23% of the study group) maintained this improvement on dietary treatment, of whom three abandoned the diet after periods ranging from six to 10 months, despite continued benefit, because they found the dietary restrictions too arduous. Thus 12 out of 66 children (18%) with severe eczema experienced prolonged and useful benefit from this dietary manoeuvre. Double blind food challenges performed in 10 patients failed to establish that parental identification of provoking foods is reliable. A search for historical and in vitro predictors of diet responsiveness was unsuccessful in this series.
We have measured the urinary excretion of trimethylamine in two sisters with trimethylaminuria (the fish-odour syndrome). On a restricted diet the patients still excreted increased quantities of trimethylamine, and this did not alter following a fourteen-day course of lactulose. Dietary provocation produced a rise in urinary trimethylamine which was abolished by fourteen days' pretreatment with lactulose. The case histories illustrate the tendency for delay in reaching this diagnosis and the psychological damage caused by the condition.
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We have investigated gastrointestinal permeability in children with atopic eczema by measuring the relative urinary excretion rates of the inert di- and monosaccharides lactulose and rhamnose following their oral administration. The median lactulose/rhamnose ratio was greater in 26 children with atopic eczema than in a control group of 29 children which included both healthy individuals and others with various noneczematous dermatoses. This increased permeability may be a primary abnormality of the gut or may reflect intestinal mucosal damage caused by local hypersensitivity reactions to food antigens.
A case of intrathoracic, extramediastinal cystic hygroma presenting with pneumonia in a 6 year old boy is described.