Showing papers by "Hospital for Sick Children published in 1994"

Stability and Transmission of Attachment across Three Generations

Abstract: Stability of adult attachment and transmission of attachment across 3 generations were examined in a longitudinal study of 96 infants, mothers, and maternal grandmothers. The Adult Attachment Interview (AAI) was used to assess attachment in mothers (during pregnancy and when infants were 11 months old) and grandmothers (any time during the study). The Strange Situation (SS) was used to assess attachment in infants at 12 months. Both the 3- and 4-category classification systems of the AAI and SS were used. Mothers' AAI classifications were stable over 12 months in 90% (3-category) and 77% (4-category) of mothers. Mothers' AAI classifications during pregnancy predicted infants' SS classifications in 81% (3-category) and 68% (4-category) of cases, and grandmothers' AAI classifications in 75% (3-category) and 49% (4-category) of cases. Using log-linear analysis, we show that a simple parent-to-child model accounts for transmission of attachment.

An SH3 binding region in the epithelial Na+ channel (alpha rENaC) mediates its localization at the apical membrane.

Abstract: The amiloride-sensitive Na+ channel constitutes the rate-limiting step for Na+ transport in epithelia. Immunolocalization and electrophysiological studies have demonstrated that this channel is localized at the apical membrane of polarized epithelial cells. This localization is essential for proper channel function in Na+ transporting epithelia. In addition, the channel has been shown to associate with the cytoskeletal proteins ankyrin and alpha-spectrin in renal epithelia. However, the molecular mechanisms underlying the cytoskeletal interactions and apical membrane localization of this channel are largely unknown. In this study we show that the putative pore forming subunit of the rat epithelial (amiloride-sensitive) Na+ channel (alpha ENaC) binds to alpha-spectrin in vivo, as determined by co-immunoprecipitation. This binding is mediated by the SH3 domain of alpha-spectrin which binds to a unique proline-rich sequence within the C-terminal region of alpha rENaC. Accordingly, the C-terminal region is sufficient to mediate binding to intact alpha-spectrin from alveolar epithelial cell lysate. When microinjected into the cytoplasm of polarized primary rat alveolar epithelial cells, a recombinant fusion protein containing the C-terminal proline-rich region of alpha rENaC localized exclusively to the apical area of the plasma membrane, as determined by confocal microscopy. This localization paralleled that of alpha-spectrin. In contrast, microinjected fusion protein containing the N-terminal (control) protein of alpha rENaC remained diffuse within the cytoplasm. These results suggest that an SH3 binding region in alpha rENaC mediates the apical localization of the Na+ channel. Thus, cytoskeletal interactions via SH3 domains may provide a novel mechanism for retaining proteins in specific membranes of polarized epithelial cells.

Specific arithmetic calculation deficits in children with Turner syndrome

Abstract: Study 1 compared arithmetic processing skills on the WRAT-R in 45 girls with Turner syndrome (TS) and 92 age-matched female controls. Results revealed significant underachievement by subjects with TS, which reflected their poorer performance on problems requiring the retrieval of addition and multiplication facts and procedural knowledge for addition and division operations. TS subjects did not differ qualitatively from controls in type of procedural error committed. Study 2, which compared the performance of 10 subjects with TS and 31 controls on the Keymath Diagnostic Arithmetic Test, showed that the TS group had less adequate knowledge of arithmetic, subtraction, and multiplication procedures but did not differ from controls on Fact items. Error analyses revealed that TS subjects were more likely to confuse component steps or fail to separate intermediate steps or to complete problems. TS subjects relied to a greater degree on verbal than visual-spatial abilities in arithmetic processing while their visual-spatial abilities were associated with retrieval of simple multidigit addition facts and knowledge of subtraction, multiplication, and division procedures. Differences between the TS and control groups increased with age for Keymath, but not WRAT-R, procedures. Discrepant findings are related to the different task constraints (timed vs. untimed, single vs. alternate versions, size of item pool) and the use of different strategies (counting vs. fact retrieval). It is concluded that arithmetic difficulties in females with TS are due to less adequate procedural skills, combined with poorer fact retrieval in timed testing situations, rather than to inadequate visual-spatial abilities.

Improving Joint Engagement in Parent-Child Interaction: An Intervention Study.

Abstract: The current study tested the effects of parent training on children's joint engagement in play interactions with their mothers Seven preschool children with developmental delays and their mothers participated in a 12-week intervention program They were matched for developmental and communication status to seven children in a delayed-treatment control group Mothers were trained to encourage child participation in social interaction by being highly responsive and child-centered Following intervention, mother-child dyads experienced significantly more episodes of joint engagement than control dyads Moreover, the total duration of joint engagement and the mean length of joint engagement episodes also increased significantly No treatment-induced changes were detected on a series of semi-structured tasks designed to measure joint attention, suggesting that dyadic changes in joint engagement did not generalize to unfamiliar contexts The results of this pilot study indicate that joint engagement is amenabl

Primary prevention of child sexual abuse: A critical review. Part II

Abstract: This paper (Part II) and the previous paper (Part I) provide an overview of the primary prevention of child maltreatment. Part II focuses on the effectiveness of interventions aimed at the primary prevention of child sexual abuse. It includes prospective controlled trials published between January 1979 and May 1993. These studies were systematically identified and the quality of each trial was determined using criteria which assessed methodological rigor. Interventions aimed at the prevention of sexual abuse were classified into eight main categories based on the method of intervention. All programs had education as the primary focus. There is evidence that educational programs can improve safety skills and knowledge of children about sexual abuse but no study has produced data that education actually reduces the occurrence of sexual abuse.

Behavioral Phenotype in Children with Turner Syndrome

Abstract: Compared Child Behavior Checklist results in 103 girls with Turner syndrome (TS) ages 7 to 13 years and 52 age-matched normal girls. Subjects with TS were part of a national study in Canada to evaluate growth hormone therapy in TS. Present data were obtained at baseline. Girls with TS scored below the comparison group in Total Social Competence (p < .0001), and above it in Total Behavior Problems (p < .05), Social (p < .0001), and Attention Problems (p < .001) scales. Item analysis revealed that subjects with TS were more immature, hyperactive, and nervous, and had poorer peer relations, greater difficulty at schooling, and more problems concentrating. In children with TS, height was correlated with social competence while karyotypes involving structural abnormalities of the X chromosome were associated with more behavior problems than a missing X or mosaicism.

Brief report: combined liver and heart transplantation for end-stage iron-induced organ failure in an adult with homozygous beta-thalassemia

Abstract: Most patients with homozygous β-thalassemia require red-cell transfusions to survive beyond the first decade of life. Although this intervention clearly prolongs survival,1 it also results in the accumulation of iron in tissue, which is itself fatal without iron-chelating therapy2. Before the introduction of deferoxamine, iron-induced cardiac dysfunction was a predictable outcome in thalassemia,3 and it is still the leading cause of death, followed in incidence by hepatic disease1. Despite the successes with deferoxamine,4–7 many patients still have serious complications, because of either advanced age at the start of therapy or erratic compliance8–14. To date, the . . .

Serum levels of soluble interleukin-2 receptor. A marker of disease activity in localized scleroderma.

Abstract: Objective To determine whether circulating serum levels of soluble interleukin-2 receptor (sIL-2R) are elevated in patients with localized scleroderma, and if levels of sIL-2R can differentiate between active and inactive disease. Methods Seventeen patients with localized scleroderma were categorized by overall physician assessment into active, inactive, and indeterminate groups, according to disease activity. Serum sIL-2R levels were analyzed and correlated with disease activity. Results The mean sIL-2R level was significantly higher (P = 0.005) in those with active disease (1,675 +/- 823 units/ml) than in those with inactive disease (722 +/- 218 units/ml). Conclusion Serum sIL-2R levels are elevated in patients with localized scleroderma. When present, elevated sIL-2R levels appear to be able to differentiate active from inactive disease. This fact also suggests cell-mediated immune activation in this condition. Further serial studies are required to assess the value and sensitivity of sIL-2R levels in measuring changes in disease activity.

Swallowing/ventilation interactions during oral swallow in normal children and children with cerebral palsy.

Abstract: Many children with cerebral palsy (CP) suffer from feeding disorders. Twenty children with spastic CP and 20 neurologically normal children (age range 6.2–12.9 years) were monitored with ultrasound imaging of the oral cavity synchronized with surface electromyographic (EMG) recordings of masseter and infrahyoid museles and respiratory inductance plethysmograph (RIP) recordings during feeding tasks. A lip-cup contact detector signaled contact of the drinking cup on the lip during liquid tasks. Children with CP required more time than normals for collection and organization of 5 ml and 75 ml liquid boluses for swallowing. The ventilatory preparation phase, recovery to baseline resting ventilatory pattern after swallowing, and total time for task completion were longer in children with CP for 5-ml and 75-ml tasks. The interval from lip-cup contact until alteration of ventilation from baseline resting ventilatory pattern was longer for children with CP during 75-ml tasks but not for 5-ml tasks. The interval from completion of the task-related cookie swallow until initiation of the next swallow was longer in children with CP than in normal children. These data provide evidence than children with CP manage solid boluses more easily than liquid boluses and small liquid boluses more easily than large liquid boluses. This investigation statistically confirms empirically based recommendations that children with CP be allowed more time to complete feeding tasks and consume small volume drinks rather than large volume drinks.

A clinical follow-up of British patients with FG syndrome.

Abstract: The FG syndrome is an X-linked recessive mental retardation syndrome. Ten patients are reviewed with special emphasis on the natural history of the intellectual development, constipation, and the prognosis for growth and behaviour. Six out of 10 patients are still macrocephalic, and there is no evidence for a specific growth pattern with respect to height. The degree of mental retardation is is usually severe. The behaviour is characteristically friendly, sociable and over-talkative, with periodic aggression. Six patients have seizures. A characteristic progression seems to occur from congenital hypotonia with joint hyperlaxity at birth, to joint contractures with apparent spasticity and unsteady gait later in life. The constipation was a temporary problem in five cases. The cowlick and the fetal pads persist and are important, but not specific, for the diagnosis.

Report of the second international workshop on human chromosome 14 mapping 1994.

Abstract: The first International Workshop on Human Chromosome 14 mapping was held at Novotel in Toronto, Canada on June 9-12, 1993. There were 23 participants from nine countries. The goals of the workshop were to compile physical maps and a consensus linkage map, to consolidate available data on disease loci, to catalogue and facilitate distribution of resources and to encourage new collaborations and data sharing.

Characteristics of court-involved men and non-court-involved men who abuse their wives

Abstract: The characteristics of court involved (CI) (n=86) and non-court-involved (NCI) men (n=42) who abused their wives and attended a treatment program were investigated. The groups experienced similar childhoods concerning family violence. The NCI men had more years of education, were more likely to be employed full-time, and tended to earn more money than the CI men. The NCI men also had more social support and scored higher on interpersonal problems than the CI men. The CI men were more likely to be separated, more reported drinking during their most recent assaultive occasion. Compared to the norms on the Basic Personality Inventory, both groups scored significantly higher on depression, anxiety, feelings of alienation, hypochondriasis, and impulse expression. CI men had higher denial and social introversion scores and NCI men had higher interpersonal problem scores than the norm. These findings have some implications for the planning of treatment programs.

Refining the genetic map for the region flanking the X-linked hypophosphataemic rickets locus (Xp22.1-22.2).

Abstract: We have screened fourteen kindreds with X-linked hypophosphataemic rickets with four microsatellite markers, viz. AFM163yh2, DXS999 (AFM234yf12), DXS443 and DXS365, in order to refine the genetic map flanking the gene, and to define a close flanking interval for the construction of a yeast artificial chromosome (YAC) and cosmid contig. The genetic data were enhanced after the isolation of a large 1.2-megabase YAC derived from AFM163yh2, in which marker DXS274 was present but not DXS365 or DXS443. Against HYP, DXS365, AFM163yh2 and DXS443 showed no recombinants (Z max = 18.1, Z max = 9.9, and Z max = 16.0 respectively). DXS999 gave Z max = 9.6 at 4% recombination and lies distal to HYP but proximal to DXS197 and DXS43. The disease gene and markers AFM163yh2 and DXS365 are flanked by DXS443 and DXS274. Combining the genetic and physical data, we are able to propose the following gene marker order: Xptel-DXS43-DXS197-DXS999-DXS443-[(DXS365-AFM163yh2), HYP]-DXS274-DXS41-Xcen.

Functional differences in the promoters of the interferon-inducible (2'-5')A oligoadenylate synthetase and 6-16 genes in interferon-resistant Daudi cells.

Abstract: A clone of interferon-α-resistant (IFNr) Daudi cells retained much greater transcriptional inducibility of the (2′–5′) oligoadenylate synthetase than the 6–16 gene despite the fact that the response of both genes is mediated by highly similar interferon-stimulable DNA response elements (ISRE). The primary IFN-α activatable transcription factor E (ISGF3) and the additional IFN-α-inducible ISRE-binding complex M were greatly reduced in the IFNr cells. The defect in E was in the Eα subunit. In electrophoretic mobility-shift assays the 6–16 and (2′–5′) oligoadenylate synthetase ISRE competed approximately equivalently for E and M. Moreover although active in wild-type cells the (2′–5′) oligoadenylate synthetase ISRE was no more capable of conferring inducibility on a reporter gene in the IFNr cells than was the 6–16 ISRE. The contrasting response of the endogenous (2′–5′) oligoadenylate synthetase and 6–16 genes in the IFNr cells is, therefore, unlikely simply to reflect the slight difference in the sequence of their ISRE. Consistent with this, in addition to the ISRE, sequence 5′ to the ISRE in the (2′–5′) oligoadenylate synthetase promoter appeared necessary for good induction by IFNα in the IFNr cells. Subtle quantitative changes in the phenotype of the IFNr cells have, however, precluded a more precise definition of the DNA element(s) involved.

Cell immobilization by adsorption to glass fibre mats

Abstract: The immobilization of biocatalysts (e.g. cells, organelles and enzymes) onto, or within a support has become a powerful and well-accepted technology with biotechnological applications in biomedical, and industrial research and development. For example, various biosensors using immobilized enzymes are important diagnostic tools in medicine, and are useful probes in research and industry (Mosbach, 1988). Likewise, immobilized cells have broad applications in the production of useful biochemicals such as ethanol, amino acids, organic acids, antibiotics, enzymes and hormones, used by the commodity chemical, food and pharmaceutical industries (Mosbach, 1976, 1987a,b). The reasons for the utility of immobilized biosystems are that high biocatalyst densities with high catalytic activity are possible, within the bioreactor. The biocatalyst can be reused allowing for continuous or semi-continuous operation in which the system is maintained in the most active phase for extended periods, and immobilized systems allow for improved downstream processing. Immobilized biocatalysts include single enzymes or enzyme-systems comprising two or more enzymes coupled for sequential catalysis, and cells of plant, animal, fungal or bacterial origin that are anchored in some fashion to a support-matrix.

A Study on Children’s Condition Thalassemia Using Neutron Activation Analysis and Other Techniques

Abstract: In this study, 50 thalassemia patients were tested using dual-energy X-ray absorptiometry (DEXA) and in vivo neutron activation analysis (IVNAA) to determine their bone mineral status. Both techniques were suitable for this purpose. Lower age was found to correspond to lower liver iron content and higher bone mineral content in the normal range. Patients undergoing treatment with transfusion had higher bone mineral content. Osteopenic patients had higher hepatic iron content than those with normal bone status. In the case of DEXA, bone mineral content (BMC) divided by height cubed was found to be a better indicator of bone mineral status than the BMD usually given. Liver density as determined by DEXA correlates well with hepatic iron