Subtle neuropsychological deficits in children with sickle cell disease

Forty-three children and adolescents (8-16 years old) with sickle cell disease (SCD) and no overt neurologic dysfunction were evaluated with the Wechsler Intelligence Scale for Children-Revised (WISC-R), the Wide Range Achievement Test (WRAT), and an age-appropriate version of the Luria-Nebraska Neuropsychological Battery (LNNB). Thirty similar-aged siblings were controls. WISC-R performance and full-scale IQs were lower (p less than 0.05) for patients than sibling controls. No significant differences were seen on the WRAT. Patients with SCD missed more school days than controls (p less than 0.001) but did not differ from controls in academic performance or the proportion in special education. On the LNNB, scores for patients and controls 13 years and older did not differ. However, young patients had significantly elevated (abnormal) scores compared with controls on the following scales: expressive speech, writing, reading, arithmetic, memory, and pathognomonic. The possibility that subtle neuropsychological deficits in younger patients with SCD reflect either subclinical strokes or a predisposition for strokes merits further investigation in a large multi-institution study.     

Social competence and behavioral adjustment of children who are long-term survivors of cancer

Relatively little is known about the special needs of long-term survivors of childhood cancer or the factors that make this growing population vulnerable to chronic psychologic disorders. We therefore surveyed 183 children who had been treated for cancer at St Jude Children's Research Hospital and were free of the disease for greater than or equal to 2 years after completing therapy. Parental responses to the Child Behavior Checklist, a standardized inventory of social competence and behavioral problems, were analyzed in relation to demographic and medical variables, as well as the children's appearance and functional status. School-related problems and somatic complaints of undetermined origin were increased fourfold relative to age- and gender-adjusted rates for peer groups in the general population. The presence of functional but not cosmetic impairments increased the risk of academic and adjustment problems. An older age at evaluation, treatment with cranial irradiation, and residence in a single-parent household were also associated with an increased risk of psychologic problems. General pediatricians must eventually assume responsibility for the extended follow-up care of children who have survived a malignancy. Their awareness of the high-risk groups described in this report should facilitate more timely identification of problems and referral of the children to appropriate services.     

Differences in twins: the importance of birth order

Despite the clinical impression that firstborn twins do better than second-born twins, recent reports have shown no difference in perinatal mortality between them. In order to evaluate differences in twins, more sensitive means than perinatal deaths are necessary. This study examines differences between 80 firstborn and second-born twin pairs with respect to Apgar score, umbilical venous and arterial blood gas, and acid-base data. The umbilical venous and arterial blood PO2, PCO2, base deficit, pH, and lactic acid concentration were measured in paired samples and compared with the paired t test and chi 2 when applicable. Statistically significant differences favoring twin A, the firstborn, were found in 1-minute Apgar score, umbilical venous pH, PO2, and PCO2, and umbilical arterial PO2. The other factors in umbilical venous and arterial blood did not show statistically significant differences. When these parameters were examined with respect to route of delivery, monochorionic and dichorionic twins, interval between twins, and vertex twins only, with the possible effects of malpresentation eliminated, the results persistently favored the firstborn twin. Thus it is unequivocally demonstrated that there are substantial differences at birth favoring the first twin, despite similar perinatal mortality for both. The data suggest that the second-born twin has potentially greater susceptibility to hypoxia and trauma.     

Attitudes of Physicians Regarding Receiving and Storing Patients' Genetic Testing Results for Cancer Susceptibility

In order to determine interest in and support for a genetic counseling program for heritable cancers, a four-item questionnaire was sent to 700 physicians in San Diego County likely to encounter patients with significant family histories of cancer. Included in the questionnaire was an item requesting information about physician attitudes and practices regarding their record keeping for patient results of genetic testing for cancer susceptibility. Ninety-two questionnaires were returned for a response rate of 13%. The low response rate introduces caution when interpreting the results, particularly if the physicians most interested in the topic were the most likely to respond. In this light, of note was the marked variability found in the attitudes of respondents regarding where the results of patients' genetic testing results should be placed in relation to the medical record. Whereas one group of physicians would place the testing results into the medical record, just as they would any laboratory test result, other physicians do not even want written notice of the results in order to maintain patient confidentiality. Another group acknowledges the sensitivity of the information, but prefers to store genetic testing results separately, as they would results of HIV testing or history of psychiatric treatment. Genetic testing for cancer susceptibility is associated with patient concerns regarding confidentiality of testing results and fears of the consequences of release of this information to insurance companies. While the small and possibly biased sample must be considered when interpreting the results, the lack of consistency among physicians about where to store genetic testing results in terms of the patient medical record underscores the need for both a consensus statement and legal protection for both patient and physician. Variability in physician practices suggests that the process of obtaining informed consent for genetic testing should include a discussion with the patient about how the confidentiality of test results will be maintained.     

The psychological status of survivors of childhood/adolescent Hodgkin's disease

To assess psychosocial late effects of childhood/adolescent cancer, semi-structured interviews were conducted with 40 subjects who had achieved complete remission from Hodgkin's disease and completed therapy at least five years previously. Mean ages were 12.8 years at diagnosis and 24.7 years at interview. Side effects of treatment were most often mentioned as the "worst thing" about having had Hodgkin's disease. Although subjects had missed a mean of six months of school, and 40% had reported unpleasant school experiences, their educational levels exceeded those expected in sex-, age-, and state-matched populations. Almost all subjects said that they had benefited in some way from the experience of having cancer. In contrast to the female subjects, male subjects expressed little interest in having their reproductive status assessed. Current concerns included discrimination in employment or in obtaining life or health insurance.     

Pediatric clinicians' support for parents makes a difference: an outcome-based analysis of clinician-parent interaction

Pediatric clinicians frequently must offer support (eg, reassurance) to anxious, stressed parents. Supportive clinician behaviors were studied to determine their impact on parents. Forty initial health supervision visits to a pediatric clinic were videotaped through a one-way mirror. Mothers were interviewed immediately before and 1 week after the visits to ascertain changes in concerns, opinions of clinicians, perceptions of infants, and self-confidence. Mothers also completed a postvisit satisfaction questionnaire. Coders blinded to these outcomes identified and enumerated three supportive clinician behaviors: encouragement, reassurance, and empathy. Analyses compared visit outcomes according to high and low levels of maternal exposure to clinician support. Mothers exposed to high levels of encouragement had significant improvement in their opinions of clinicians and higher satisfaction (P = .02). Mothers exposed to high levels of empathy had higher satisfaction and greater reduction in concerns (P less than .05). No significant differences in outcome were found for exposure to reassurance. Differences in visit outcomes were not related to either maternal demographic factors or clinician type (pediatricians v pediatric nurse practitioners). These results suggest that pediatric clinicians' support for parents makes a difference. Additional outcome-based analyses are needed to identify the full range of effective pediatric communication.