Velo-cardio-facial syndrome and DiGeorge sequence with meningomyelocele and deletions of the 22q11 region.

Approximately 5% of children with neural tube defects (NTDs) have a congenital heart defect and/or cleft lip and palate. The cause of isolated meningomyelocele, congenital heart defects, or cleft lip and palate has been largely thought to be multifactorial. However, chromosomal, teratogenic, and single gene causes of combinations of NTDs with congenital heart defects and/or cleft lip and palate have been reported. We report on 3 patients with meningomyelocele, congenital heart defects, and 22q11 deletions. Two of the children had the clinical diagnosis of velo-cardio-facial syndrome (VCFS); both also have bifid uvula. The third child had DiGeorge sequence (DGS). The association of NTDs with 22q11 deletions has not been reported previously. An accurate diagnosis of the 22q11 deletion is critical as this micro-deletion and its associated clinical problems is transmitted as an autosomal dominant trait due to the inheritance of the deletion-bearing chromosome. We recommend that all children with NTDs and congenital heart defects, with or without cleft palate, have cytogenetic and molecular studies performed to detect 22q11 deletions.

Cover and efficacy of predator-based repellents for Townsend's voleMicrotus townsendii.

Predator-based repellents have been used experimentally to control wildlife damage in both agriculture and forestry, but they have not always been effective. We examined the relative importance of cover and predator odors in forage patch selection by Townsend's vole,Microtus townsendii, and its behavior related to cover and predator cues. Experiments were conducted in which forage patch and area choices were related to available habitat alternatives. Outdoor enclosures were divided into halves: one side was treated and the other used as a control. Treatments consisting of "cover," "repellent," and "cover plus repellent" were compared to controls (no cover, no repellent). In the absence of cover, voles preferred to feed on the side without repellents. When cover was present, voles preferred to feed on the side with cover, regardless of whether or not repellents were present. Voles visited more feeding stations on the side without cover when repellents and cover were present than they visited during cover-only treatments. These additional feeding stations, visited outside of cover, were used only lightly as food sources. The amounts of oats eaten by voles decreased with increasing distance from cover. This inverse relationship had a steeper slope in coveronly treatments compared to cover plus repellent treatments. A selection model based on forage patch selection and a habitat preference hierarchy is proposed. We conclude that predator odors are effective as repellents, but their efficacy depends on habitat conditions. Managers intending to use predator-based repellents must ensure that alternative sites available to pests are better quality habitat than in areas to be protected.

A pediatric chronic illness transition unit.

A four-bed Chronic Illness Transition Unit (CITU) for the care and developmental support of children who require long-term hospitalization is described. The goal is to discharge patients to the home or the best alternative environment as expeditiously as possible. The layout, staffing, and equipment; the importance of regional coordination; and components that increase viability of such a program and unit are discussed. The costs of construction and the projected costs of service are reviewed. It is hoped that others can use this experience to establish pediatric transition units, thereby improving care, developmental stimulation, family support, and placement of children with severe chronic illnesses.

The awareness of primary physicians of the psychosocial adjustment of children with a chronic illness.

This study compared primary care physicians' ratings of psychosocial adjustment of their chronically ill pediatric patients with parents' and children's reports. A series of measures of psychosocial adjustment were administered to 63 children with either a seizure disorder or a visible orthopedic condition, and their parents. Each child's physician was asked to rate the presence and severity of psychological adjustment problems in the child, as well as to rate six specific psychosocial domains. Forty-one physicians responded, and relationships between their ratings and child and parent psychosocial measures were determined using Pearson product-moment correlation coefficients. Ratings by physicians correlated with children's reports and parents' judgments about the children, but not with mothers' self-concept or the impact that the illness had on the family. However, physicians' ratings were interdependent. When congruence of physician ratings with child or parent reports was examined for each disease group, we found significant results for the seizure disorder group; however, congruence among ratings for the orthopedically handicapped group was poor. Student's t-tests revealed no statistically significant differences between the two disease groups in any of the demographic variables of the children or physicians, nor in their scores on the criterion measures. Hierarchical multiple regression analysis identified factors that contributed to physicians' significantly different congruence with parents regarding the adjustment of children in the two subsamples. The findings of this study suggested that primary care physicians were moderately aware of the presence and severity of psychosocial adjustment problems in some categories of their pediatric patients with chronic illness.(ABSTRACT TRUNCATED AT 250 WORDS)