Anesthetic considerations of an infant with Beckwith-Wiedemann syndrome

The case of a 3-day-old infant with Beckwith-Wiedemann syndrome who required anesthetic care during closure of an abdominal wall defect is presented. Beckwith-Wiedemann syndrome comprises a constellation of clinical features, including macroglossia, macrosomia, omphalocele, visceromegaly, mild microcephaly, facial nevus flammeus, horizontal earlobe creases, and renal medullary dysplasia. Due to the high rate of omphalocele in this syndrome, anesthetic care is frequently required during the neonatal period. Many of these infants (greater than 50%) are born prematurely. Therefore, their anesthetic care may be further complicated by associated diseases of prematurity, such as hyaline membrane disease. Additional anesthetic implications of this syndrome relate to the occurrence and management of hypoglycemia and polycythemia. Careful intraoperative management of glucose homeostasis is particularly important, since eventual neurologic outcome and intelligence will be normal provided prolonged neonatal hypoglycemia is avoided. Preoperative evaluation of the cardiac and genitourinary system, including echocardiography and renal ultrasound, are recommended because of the frequent occurrence of associated anomalies with omphalocele.     

The structure of wandering in dementia

‘Wandering’ is one of the most troublesome of behavioural problems in dementia. The term ‘wandering’ covers many different types of behaviour. We examined the hypothesis that the different types of wandering behaviour seen in dementia from a scale using data collected on 83 elderly subjects suffering from either Alzheimer's disease or multiinfarct dementia. We reject the scaling hypothesis. Our data suggest that there are three main categories of wandering behaviour, and that one of these categories is usefully divided into four subcategories.     

Loss of cables, a novel gene on chromosome 18q, in ovarian cancer.

Cables, a cyclin-dependent kinase (cdk) interacting protein, has recently been identified and mapped to human chromosome 18q11. Cables appears to be primarily involved in cell cycle regulation and cell proliferation. Overexpression of Cables in Hela and other cell lines inhibits cell proliferation and tumor formation. We hypothesize that loss of Cables expression is associated with ovarian cancer. To test our hypothesis, we examined Cables expression in the four most common subtypes of ovarian carcinomas: serous, endometrioid, mucinous, and clear cell. In addition, mucinous and serous borderline tumors were also included. Loss of Cables expression was observed at high frequency in ovarian serous (11 of 14 cases, 79%) and endometrioid (5 of 10 cases, 50%) carcinomas. In contrast, strong Cables staining was detected in all clear cell carcinomas (10 cases) and mucinous tumors (5 carcinomas and 5 borderline tumors). The majority of serous borderline tumors (11 of 14 cases, 79%) showed positive Cables staining, with the rest showing focal loss of Cables expression. Furthermore, RT-PCR revealed the lack of Cables mRNA in a human ovarian cancer xenograft. No correlation was noted between loss of Cables and histologic grade, tumor stage, and survival. In conclusion, our results indicate that loss of Cables is common in ovarian serous and endometrioid carcinomas and imply that Cables may be involved in the pathogenesis of these two types of ovarian carcinomas.     

Inhibition of in Vitro Generation of Cytotoxicity Against Tumor Cells by Monoclonal Antibody to Thy-1

The Thy-1 molecule on murine T lymphocytes has been suggested to play a role in cellular activation events leading to a variety of immunologic functions. We present evidence that this molecule may be involved in signals leading to the in vitro generation of cytotoxic T cells against several tumor cell lines used as stimulators in mixed tumor-lymphocyte culture. The presence of monoclonal antibody against a polymorphic determinant on the Thy-1 molecule markedly reduced the generation of cytotoxicity after three days of culture of murine splenocytes with stimulator tumor cells bearing low levels of Ia antigen. In contrast, no effect was seen when the stimulators were either allogeneic splenocytes, or a tumor cell line expressing large amounts of Ia. These results suggest that the Thy-1 molecule is critically involved in events leading to the generation of cytotoxic effectors under some, but not all conditions.     

Changes in cognitive and Language functioning of Preschool children with autism

Preschool children with autism and their normally developing peers were compared on the Stanford-Binet IV and Preschool Language Scale before and after 1 school year. Both measures showed that although the children with autism functioned at a lower level than their normally developing peers, the children with autism had narrowed this gap after treatment, making a nearly 19-point increase in IQ and an 8-point gain in language quotient. The IQ measure remained stable for the normally developing peers while their language showed a 7.73-point increase. The data support the notion that young children with autism can make very significant developmental gains.Special thanks to the parents and the children who participated in this study. Thanks also to Michael Alessandri, Jean Burton, David Celiberti, Nancy Gera-Moglia, Anne Nathan, and Lisa Kamean who assisted in the data collection process. We appreciate the cooperation of Alicia MacWright and Leisa Tomchek who taught two of the classes from which participants were drawn.     

Treatment of experimental osteomyelitis by surgical debridement and the implantation of bioerodable,polyanhydride-gentamicin beads

Osteomyelitis was induced in the radius in 77 rabbits and confirmed by histological examination and culture. At 4 weeks, the wounds were debrided and the animals were treated with (a) fatty acid dimersebacic acid beads (a bioerodable composite) impregnated with 20% or (b) 10% gentamicin sulfate, (c) placebo beads and intramuscular gentamicin sulfate. (d) placebo beads alone, or (e) debridement only. After 4 weeks, eradication of infection was determined by histological examination and culture. Osteomyelitis was eradicated in 93% of the animals treated with the beads and 20% gentamicin, in 67% of those treated with the beads arid 10% gentamicin, in 25% of those treated with placebo beads and intramuscular gentamicin, in 7% of those treated with placebo beads alone, and in 12.5% of those treated with debridement only (p values from <0.001 to 0.02). Fatty acid dimer-sehacie acid beads with gentamicin were then implanted in noninfected rabbits, and gentamicin sulfate concentrations in bone, serum, urine, and wound exudate were measured. Gentamicin sulfate was detectable in bone for as long as 8 weeks after implantation. Levels as high as 4,746 g/ml were present in the wound exudate for the first 7 days. Levels in the serum peaked at 1.03 μg/ml. Urine levels peaked at 135 μg/ml.     

Cognitive impairment after unilateral hemispheric injury of congenital or adult origin.

OBJECTIVE: The purpose of this study was to assess and compare cognitive functioning in adults with unilateral hemispheric injury due to either congenital damage or an ischemic event in young adulthood. METHOD: Adults with cerebral palsy resulting from left hemispheric brain damage were compared with adults who had a unilateral stroke in either the left or the right hemisphere. Our primary interest was to determine the impact on hemispheric dominance as revealed by dichotic listening, a task that assesses the bias for preferential listening and processing of sounds. Performance also was determined on a language-related task (word finding) and a spatial task (dot localization). RESULTS: Scores on the Quick Neurological Screening Test indicated that all participants demonstrated significant neuromotor deficits, whereas scores on the Barthel Index indicated that the participants were functional in basic activities of daily living. On cognitive assessments, healthy control participants demonstrated a pronounced left-hemisphere dominance and right-ear advantage; participants with injury to the left hemisphere showed a strong shift toward a right-hemisphere and left-ear dominance. In particular, injury of congenital origin appeared to foster this neural reorganization and localization of language-related functions into the healthy hemisphere. This shift was associated with a deterioration of performance on both the language and the spatial tasks. CONCLUSION: The importance of appreciating subtle deficits after unilateral injury is important in therapy. The dichotic listening test may provide a simple and useful means for evaluating persistent unilateral brain dysfunction in the clinical setting.     

An Improved Method of Removing Polyurethane Foam-Covered Gel Prostheses

One of the advantages of polyurethane foam-covered prostheses has been that in the first 5 to 10 years after their use, the amount of capsular contraction was found much less than when similar ``slick' prostheses were used. Another advance was their fixation to the surrounding tissue thus giving a more natural appearance and movement with the muscles when the arms were moved in any direction. The formation of a thick capsule also acted as a protection against gel granuloma due to rupture of the prosthesis and has been thought to be a factor in the lower capsule contraction rate. The greatest disadvantage has been that its removal was extremely difficult and this has continued up until the technique described in this paper has been introduced.