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Biomedical subjects

S M Stewart

Publications and source records attributed to S M Stewart.

At least 19 recordsLinked to original sources

Evolution of hereditary non-polyposis colorectal cancer.

The prevalence of colorectal adenoma in 23 affected patients with hereditary non-polyposis colorectal cancer (HNPCC) was compared with that in an age matched forensic autopsy population. The mean ages at diagnosis of colorectal cancer in men (13) and women (10) were 39 and 37 years respectively. In HNPCC patients who presented below the age of 50 years, at least one adenoma was found in 3 of 10 (30%) men and in 4 of 9 (44%) women. In the age matched autopsy specimens of large bowel, a solitary adenoma was found in 2/42 (5%) men (p = 0.015) and 1/21 (5%) women (p = 0.0075). Of the 11 adenomas from HNPCC patients, nine were greater than 1 cm and six showed a pure villous architecture. Contiguous adenocarcinoma was observed with six adenomas. Adenomas do not occur in large numbers in HNPCC, but develop at a young age, attain a large size, often show a villous configuration, and are more prone to malignant conversion than sporadic adenomas.

Adenocarcinoma

Cognitive patterns in school-age children with end-stage liver disease.

Although children with end-stage liver disease (ESLD) have been found to have cognitive delays, the relationship between patterns of cognitive function and diagnostic category, age of onset, duration and severity of disease has not been assessed before transplantation. Verbal and performance IQ (VIQ, PIQ) scores and scores on Bannatyne's cognitive factors for 43 children with ESLD were compared with those of 15 control children with cystic fibrosis (CF) and with existing normative data. Children with biliary atresia had deficits in PIQ, spatial and sequential scores. Children with alpha-1 antitrypsin deficiency did not differ significantly from CF controls but did show deficits compared with normative data. Children with onset of disease in the first year of life had deficits on all cognitive measures compared with both control groups. In contrast, children with later onset differed from the normative population only on VIQ and the acquired knowledge factor. In multiple regression analyses, duration of disease and indexes of liver dysfunction combine to predict cognitive scores. These preliminary findings suggest that children with early onset of liver disease are at high risk for cognitive impairment.

Biliary Atresia

Emotional adaptation in children after liver transplantation.

The emotional adaptation of 25 children who had undergone successful liver transplantation at least 1 year previously was evaluated and compared with that of a control group of 26 children with diabetes mellitus, matched for age, gender, and socioeconomic status, and with reported normative values. With the use of parent- and self-report measures, emotional adaptation was assessed in terms of behavioral adjustment (Child Behavior Checklist), depressive experiences (Children's Depression Inventory), anxiety (State-Trait Anxiety Inventory for Children), and self-concept (Piers-Harris Children's Self-concept Scale). Similar variables were also assessed by a projective personality measure (Rorschach). We found minimal differences in emotional adjustment between liver transplant and diabetic groups; on self-report measures, the adjustment of those with liver transplants was as good as, or better than, that of normative samples. However, on other tests, children with transplants differed from normative samples. On projective measures, liver transplant recipients had an increased number of depressive experiences, situationally related internal distress, and a greater tendency toward negative self-focus and evaluation. We conclude that in pediatric liver transplant recipients, behavioral and emotional adjustment is as good as in children with another chronic illness; in comparison with physically well children, the transplant recipients have subtle signs of emotional difficulties.

Adaptation, Psychological

Neuropsychological function in young children who have undergone liver transplantation.

Presented neuropsychological data from 20 patients between the ages of 4 and 9 years, who had undergone liver transplantation at least 12 months prior to study participation, and compared them to a control group of 20 children with cystic fibrosis. The liver transplant group showed deficits in VIQ, PIQ, visual-spatial and abstraction/reasoning skills, but not in alertness/concentration, motor, or sensory-perceptual functions. On motor and sensory-perceptual tests, no differences were found in direction of lateralization of deficits. Visual-spatial deficits found in this study sample are similar to those found in adults with end-stage liver disease and in a previous report of an overall older group of children following liver transplantation. However younger children have greater evidence of generalized impairment and VIQ deficits which have not been found in older groups. Findings are discussed in relation to possible etiology, location of brain damage, and clinical implications.

Brain Damage, Chronic

Neuropsychological outcome of pediatric liver transplantation.

Children with end-stage liver disease who undergo liver transplantation may have unrecognized neuropsychological and academic deficits, for which remediation programs may be available. Intellectual, academic, and neuropsychological measures of 28 pediatric patients who had received successful liver transplantation at least 1 year previously were compared with those of 18 patients with cystic fibrosis (to control for effects of growth retardation and chronic illness) matched for age, age at diagnosis, physical growth, and parents' socioeconomic status. Liver transplant patients had significantly lower scores on nonverbal intelligence tests (mean +/- SD for liver transplant vs cystic fibrosis patients: 89.1 +/- 19.1 vs 105.8 +/- 17.6), lower academic achievement, and lower zeta scores for age in the areas of learning and memory (-0.68 +/- 1.09 vs 0.19 +/- 1.24), abstraction and concept formation (-1.73 +/- 1.58 vs -0.79 +/- 1.37), visual-spatial function (-0.66 +/- 1.09 vs 0.10 +/- 0.69), and motor function (-0.13 +/- 0.85 vs 0.36 +/- 0.57). No differences were found on tests of verbal intelligence, or in alertness and concentration, perceptual-motor, and sensory-perceptual areas. Cyclosporine levels were found to correlate positively with motor speed (r = .41, P less than .05). Thorough psycho-educational and neuropsychological evaluations should be considered for pediatric patients who receive liver transplantation to allow these children to maximize their potential.

Adolescent

Identifying factors in post-operative successful adaptation to pediatric liver transplantation.

In order to identify variables contributing to successful adaptation to liver transplantation, 38 families, at least six months post-transplant, were divided according to transplant team members' rating into two groups: those with successful adaptation to transplant and those families at risk for difficulties. Families who had successful follow-up periods tended to be characterized by intact marriages, private insurance coverage, less subjective financial stress, and higher intellectual and developmental functioning in the child. Single-parent families who had no pre-evaluation involvement with liver transplant organizations and had lengthy post-operative hospital stays more frequently had complications post-transplant. Case studies are presented illustrating the application of this information.

Adaptation, Psychological

Mental and motor development, social competence, and growth one year after successful pediatric liver transplantation.

We measured intellectual and motor function, social competence, and growth in 29 children (mean age 4 years 7 months) before liver transplantation and 1 year later. We used either the Bayley Scales, the Stanford-Binet Intelligence Scale, and the Minnesota Child Development Inventory (MCDI), Motor Age Quotient, or the Wechsler Scales, depending on the age of the child at testing. Social function was measured with the MCDI or the Child Behavior Checklist. All anthropometric measures were expressed relative to normal values for age and sex. Patients whose intellectual and motor scores were less than 80 before transplantation gained an average of 8 points, but these changes were not statistically significant, nor were the changes on these measures for the group as a whole. The development of children with onset of liver disease in the first year of life was more likely to remain delayed after transplantation. Older subjects improved significantly in social competence (p less than 0.008). There were significant increments after transplantation in weight, head circumference, and arm anthropometrics (p less than 0.0001 to 0.04), but there was no change in linear growth rate. Increments in length correlated negatively with steroid dosage, and change in head circumference was associated with age at time of transplantation (p less than 0.005 to 0.10).

Adolescent

Dexamethasone therapy for bacterial meningitis. Results of two double-blind, placebo-controlled trials.

We enrolled 200 infants and older children with bacterial meningitis in two prospective double-blind, placebo-controlled trials to evaluate the efficacy of dexamethasone therapy in addition to either cefuroxime (Study 1) or ceftriaxone (Study 2). Altogether, 98 patients received placebo and 102 received dexamethasone (0.15 mg per kilogram of body weight every six hours for four days). At the beginning of therapy, the clinical and demographic characteristics of the patients in the treatment groups were comparable. The mean increase in the cerebrospinal fluid concentration of glucose and the decreases in lactate and protein levels after 24 hours of therapy were significantly greater in those who received dexamethasone than in those who received placebo (glucose, 2.0 vs. 0.4 mmol per liter [36.0 vs. 6.9 mg per deciliter], P less than 0.001; lactate, 4.0 vs. 2.1 mmol per liter [38.3 vs. 19.8 mg per deciliter], P less than 0.001; and protein, 0.64 vs. 0.25 g per liter [64.0 vs. 25.3 mg per deciliter], P less than 0.05). One patient in the placebo group in Study 1 died. As compared with those who received placebo, the patients who received dexamethasone became afebrile earlier (1.6 vs. 5.0 days; P less than 0.001) and were less likely to acquire moderate or more severe bilateral sensorineural hearing loss (15.5 vs. 3.3 percent; P less than 0.01). Twelve patients in the two placebo groups (14 percent) had severe or profound bilateral hearing loss requiring the use of a hearing aid, as compared with 1 (1 percent) in the two dexamethasone groups (P less than 0.001). We conclude that dexamethasone is beneficial in the treatment of infants and children with bacterial meningitis, particularly in preventing deafness.

Bacterial Infections

Mental development and growth in children with chronic liver disease of early and late onset.

Comparison was made of the mental function and physical growth of 21 children in whom liver disease occurred in the first year of life with 15 patients with late (17 months of age to 12 years of age) manifestation of liver disease. Ages (mean +/- SD) at testing for the two groups was 8 +/- 3 years for the early group and 11 +/- 5 years for the late group. Wechsler verbal, performance, and full-scale IQ scores were lower for the early group (range of mean scores: early, 85 to 86 v late, 96 to 103). Growth measures were significantly different in the two groups. Means +/- SD (percentage of standard) were: length for early group, 92 +/- 9; for late, 99 +/- 7; and head circumference for early, 98 +/- 4; for late, 101 +/- 2. The groups were similar in severity of liver disease and acute nutritional status, however. Patients with intellectual impairment had a longer duration of illness, poor nutritional status, and vitamin E deficiency; 82% of impaired patients were in the early group. The data suggest that liver disease during early life has pernicious effects on intellectual function and linear growth. Careful monitoring of nutritional status of children with early-onset liver disease and aggressive nutritional support beginning at the time of diagnosis may help reduce delays in growth and mental development.

Adolescent

Mental and motor development correlates in patients with end-stage biliary atresia awaiting liver transplantation.

We measured mental and motor development in 27 infants and 14 children with end-stage biliary atresia who were awaiting liver transplantation, and we measured disease-related variables (physical growth, liver function, and severity and duration of disease) that may relate to development. We then determined the relationship between development and the disease-related variables. Infants were assessed with the Bayley scales, and children were assessed with the Stanford-Binet scales of intelligence and the Minnesota Child Development Inventory. The mean +/- SD mental and motor scores for infants were 79.5 +/- 19.6 and 69.7 +/- 17.6, respectively. The mean +/- SD IQ and motor development scores for children were 76.1 +/- 16.6 and 56.9 +/- 18.1, respectively. For infants, mental and motor development were related significantly to height and weight (r values ranged from .42 to .72). Mental development in infants was also related significantly to serum vitamin E levels (P = .03). Multiple regression analyses combining growth and disease-related indices predicted 70% of the variance in mental and motor development in infants (P = .001). For children, development was related to measures of liver function (ie, to serum bilirubin, r = -.51, P = -.08, and to serum albumin, r = .54, P = .06) but not to growth. Careful nutritional support during infancy and aggressive management of liver disease prior to transplantation may be important in optimizing the development of children with biliary atresia who subsequently undergo liver transplantation.

Biliary Atresia