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PubMed2006

Effects of social stories on prosocial behavior of preschool children with autism spectrum disorders.

Social Stories are a popular intervention for preschool children with autism spectrum disorders (ASD), but little research on Social Stories has been conducted with this population. This study investigated the effects of Social Stories on prosocial behavior of three preschool children with ASD in an inclusive setting. An ABAB design was used for two participants, while an ABACBC was used for the third. Social Stories increased appropriate behavior and decreased inappropriate behavior for two participants. The addition of verbal prompts (condition C) was necessary to increase appropriate behavior for the third participant. Maintenance probes were conducted to assess whether stories became imbedded in classroom routines. Results are discussed in relation to applications, study limitations, and areas for future research.

Autistic Disorder↗
PubMed2006

Social skills development in children with autism spectrum disorders: a review of the intervention research.

Social reciprocity deficits are a core feature of the autism spectrum disorders (ASD). This review summarizes the state of research in group-based social skills training programs for school-age children and adolescents with ASD. All published studies of group social skills interventions between 1985 and 2006 were reviewed, as well as dissertations examining group-based social skills intervention programs. To assess the state of the science, a template developed by an NIMH work group was applied to 14 identified studies. Based on this review, the empirical support for this approach is incomplete, but promising intervention strategies were identified. Recommendations for the design of future treatment trials to guide clinical practice are offered.

Adolescent↗
PubMed2006

Parental recognition of developmental problems in toddlers with autism spectrum disorders.

Symptoms of Autism Spectrum Disorders (ASD) begin to manifest during the first 2 years; there is limited evidence regarding type and timing of symptom onset. We examined factors related to parental age of recognition (AOR) of early abnormalities and the association between AOR and diagnosis and levels of functioning at 2 and 4 years in 75 toddlers with ASD. Results suggest significant differences between autism and PDD-NOS in the AOR and type of first concerns. Early social and motor delays as well as maternal age was associated with AOR. Later AOR was associated with poorer social-communicative and nonverbal cognitive functioning at 2 and 4. The findings are discussed in a context of identifying distinct developmental trajectories within the autism spectrum.

Age Factors↗
PubMed2007

Sex differences in toddlers with autism spectrum disorders.

Although autism spectrum disorders (ASD) prevalence is higher in males than females, few studies address sex differences in developmental functioning or clinical manifestations. Participants in this study of sex differences in developmental profiles and clinical symptoms were 22 girls and 68 boys with ASD (mean age = 28 months). All children achieved strongest performance in visual reception and fine motor followed by gross motor and language functioning. Sex differences emerged in developmental profiles. Controlling for language, girls achieved higher visual reception scores than boys; boys attained higher language and motor scores and higher social-competence ratings than girls, particularly when controlling for visual reception. Longitudinal, representative studies are needed to elucidate the developmental and etiological significance of the observed sex differences.

Affect↗
PubMed2007

The first year inventory: retrospective parent responses to a questionnaire designed to identify one-year-olds at risk for autism.

The First Year Inventory (FYI) is a parent questionnaire designed to assess behaviors in 12-month-olds that suggest risk for an eventual diagnosis of autism. We examined the construct validity of the FYI by comparing retrospective responses of parents of preschool children with autism spectrum disorders (ASD; n = 38), other developmental disabilities (DD; n = 15), and typical development (TD; n = 40). Children with ASD were rated at significantly higher risk on the FYI than children with DD or TD. The DD group was at intermediate risk, also significantly higher than the TD group. These retrospective data strengthen the validity of the FYI and have implications for refining the FYI to improve its utility for prospective screening of 12-month-olds.

Autistic Disorder↗
PubMed2004

Atrial septal defect with borderline pulmonary vascular disease: surgery and long-term oral prostacyclin therapy for recalcitrant pulmonary hypertension.

The hemodynamic determination of operability in atrial septal defect (ASD) with severe pulmonary hypertension is problematic. Therefore, we perform an open lung biopsy prior to the corrective surgery in cases with pulmonary vascular resistance greater than 8 units x m2 and/or pulmonary arterial peak pressure greater than 70 mmHg. We present 4 cases showing occlusion of more than 70% of the small pulmonary arteries and arterioles by musculoelastosis, thromboembolism and mixed-type (musculoelastosis and plexogenic arteriopathy) which was considered borderline in terms of operability. After complete closure of the ASD and postoperative long-term oral prostacyclin (PGI2) therapy, pulmonary artery peak pressure decreased from 110-72 (mean 84) to 105-45 (mean 74) mmHg immediately after operation and 65-40 (mean 57) mmHg after PGI2 therapy. The New York Heart Association functional status of the patients improved from class II-III to class I with oral PGI2 only. Our cases demonstrate that despite more than 70% occlusion of the small pulmonary arteries and arterioles, surgery and long-term PGI2 therapy can reduce pulmonary artery pressure and improve the quality of life.

Adolescent↗
PubMed2004

Minimal access surgery for the repair of simple congenital heart defects: factors affecting hospital stay after surgery.

OBJECTIVE: We reviewed our experience of minimal access surgery to elucidate the efficacy and safety of this approach and determine the factors affecting hospital stay. METHODS: Seventy-seven patients (age, 11.8 +/- 11.0 years), with body weight of more than 10 kg, were operated using various forms of minimal access approach for repair of simple congenital heart defects [atrial septal defect (ASD) in 40, ventricular septal defect in 37]. These included lower partial sternotomy (n = 68) and mini-thoracotomy (n = 9, ASD only) with limited skin incision of 4-11 cm. The anesthetic protocol was modified to wean all patients from ventilator soon after operation. The protocol of discharge from hospital (critical pass) was 14 days in the early period (n = 30) and 10 days in the late period (n = 47). RESULTS: There were no hospital or late death, and no hospital re-admission. None of patients required blood transfusion. The endotracheal tube was extubated in the operating room in 48 cases (62%). Twenty-four patients (31%) failed to fulfill conditions of the critical pass. Univariate analysis of factors affecting unfavorably the critical pass demonstrated that the median approach, retention of pericardial effusion and social reasons were statistically significant, while an opened pleura and aortic cross-clamp time were marginally significant. Multivariate analysis indicated that the retention of pericardial effusion was the only significant factor that failed critical pass [p = 0.007, odds ratio (OR) 5.7, 95% confidence interval (CI) 1.61 -19.8]. In addition, a pericardio-pleural fenestration was the only significant factor that affected favorably the pericardial effusion (p = 0.035, OR 0.2, 95% CI 0.47-0.89) by multivariate analysis. CONCLUSIONS: Our experience demonstrated that minimal access surgery of the simple congenital heart defects provided excellent cosmetic results. Retention of pericardial effusion, possibly due to pericarditis, was a major risk factor of the prolonged hospital stay. The pericardio-pleural fenestration could reduce the risk of retention of effusion.

Adolescent↗
PubMed2004

Cosmetic approach for correction of simple congenital heart defects in female patients.

OBJECTIVE: Much more concern is recently paid to the cosmetics when simple congenital heart defect is repaired. Here we present the reversed U-shaped skin incision with lower partial sternotomy for juvenile or adult female patients. METHODS: Four patients with atrial septal defect (ASD) and one patient with ventricular septal defect underwent the operation with this method. Patients' ages ranged from 5 to 46 years old. RESULTS: Postoperative recovery in all cases was uneventful. Only the middle portion of the operative scar was visible when the breast was well developed. The scar was completely covered by the brassiere. A numbness of the upper medial portion of the left breast was recognized in one initial patient, who underwent ASD closure and concomitant right-side Maze procedure, necessitating a wider dissection than that used for other patients. CONCLUSION: A reversed U-shaped skin incision with a lower sternotomy could provide relatively comfortable operative fields for cardiac surgeons, and be cosmetically appealing to female patients who require a repair of simple congenital heart defect.

Adolescent↗
PubMed2004

Transcatheter Closure of Intracardiac Shunts.

Intracardiac shunts, such as atrial septal defects (ASDs), patent foramen ovales (PFOs), and ventricular septal defects (VSDs), are common forms of congenital or acquired heart disease. Traditional treatment has consisted of surgical closure. Transcatheter closure using implantable devices is now an alternative approach to the treatment of these lesions. Transcatheter closure offers advantages over surgical closure: 1) it is less invasive, resulting in shorter recovery times, less hospital time, and no scarring; 2) it avoids the deleterious neurocognitive effects of cardiopulmonary bypass; 3) it avoids the proarrhythmic effects of atrial or ventricular incisions; and 4) it is potentially less costly. Device closure of secundum ASDs is the procedure of choice. Device closure of PFOs can be performed under humanitarian device exemption (HDE) guidelines, although the indications for this procedure and its effectiveness compared with medical management remain controversial. Ongoing studies are addressing these issues. Transcatheter closure of muscular VSDs is an alternative to surgical treatment, especially for apical defects or those anterior to the moderator band. The CardioSEAL device (NMT Medical, Boston, MA) has HDE status for this purpose and the Amplatzer muscular VSD device (AGA Medical Corp., Golden Valley, MN) is being reviewed by the US Food and Drug Administration for market approval. Phase 1 investigations are underway for device closure of perimembranous VSDs using the Amplatzer perimembranous VSD device (AGA Medical Corp.). Until more data are available, surgical closure of perimembranous VSDs is still the procedure of choice. Closure of postinfarct VSDs can also be accomplished using intracardiac devices; however, this remains a high-risk procedure. Early experience compares favorably with surgical closure. Transcatheter device closure of intracardiac defects is a relatively new procedure. Long-term data for these procedures are currently lacking and, therefore, recommendations regarding the use of these devices will continue to evolve with time.

Journal Article↗
PubMed1982

Effect of cardiac surgery on ventricular septal motion: assessment by intraoperative echocardiography and cross-sectional two-dimensional echocardiography.

Echocardiographic evidence of paradoxical septal motion frequently occurs after cardiac surgery. To assess possible etiologic factors 17 patients were studied preoperatively, intraoperatively, and 7 days after surgery. Preoperative septal motion was normal in 14 and paradoxical in three (two with previous cardiac surgery, one with atrial septal defect [ASD]). Intraoperative septal motion prior to surgical procedure was normal in 16 and paradoxical in one (ASD). Septal motion (excursion and thickening fraction) was normal in all patients prior to chest closure. Echocardiograms of adequate quality were obtained at 7 days post surgery in 15 patients; septal motion was paradoxical in nine (group A) and normal in six (group B). No significant differences were seen between the two groups in ischemic time or in the preoperative to postoperative change in left ventricular (LV) and right ventricular diastolic dimension, shortening fraction, or septal and posterior wall thickening fraction. A significant postoperative decrease in septal excursion was seen in group A but not in group B; significant postoperative increases in posterior wall excursion were seen in both groups. Cross-sectional two-dimensional echocardiograms performed in 20 patients (8 normal, 12 postoperative paradoxical septal motion) were analyzed. In normal controls no significant change was detected in the LV centroid position during systole. In contrast, the 12 postoperative patients showed significant anterior displacement of the LV centroid and right septum during systole. Thus, paradoxical septal motion after cardiac surgery appears to relate to excessive anterior cardiac mobility due to pericardiotomy rather than to myocardial ischemia resulting from cardiopulmonary bypass.

Adult↗
PubMed1983

Radionuclide analysis of right and left ventricular response to exercise in patients with atrial and ventricular septal defects.

In patients with ventricular or atrial septal defect, the ventricle which is chronically volume overloaded might not appropriately respond to increased demand for an augmentation in output and thereby might limit total cardiac function. In this study we simultaneously measured right and left ventricular response to exercise in 10 normal individuals, 10 patients with ventricular septal defect (VSD), and 10 patients with atrial septal defect (ASD). The normal subjects increased both right and left ventricular ejection fraction, end-diastolic volume, and stroke volume to achieve a higher cardiac output during exercise. Patients with VSD failed to increase right ventricular ejection fraction, but increased right ventricular end-diastolic volume and stroke volume. Left ventricular end-diastolic volume did not increase in these patients but ejection fraction, stroke volume, and forward left ventricular output achieved during exercise were comparable to the response observed in healthy subjects. In the patients with ASD, no rest-to-exercise change occurred in either right ventricular ejection fraction, end-diastolic volume, or stroke volume. In addition, left ventricular end-diastolic volume failed to increase, and despite an increase in ejection fraction, left ventricular stroke volume remained unchanged from rest to exercise. Therefore, cardiac output was augmented only by the heart rate increase in these patients. Right ventricular function appeared to be the major determinant of total cardiac output during exercise in patients with cardiac septal defects and left-to-right shunt.

Adolescent↗
PubMed1991

Cardiorespiratory exercise capacity after surgical closure of atrial septal defect is influenced by the age at surgery.

To study the influence of age at the time of the operation on long-term functional performance in children undergoing surgery for atrial septal defect (ASD) of the secundum type, exercise tolerance was assessed in 24 patients and values were compared with those of normal subjects. Patients were divided into two groups: 11 patients had surgery before the age of 5 years (group 1) and 13 patients had surgery at a later age (group 2). There were no significant differences between groups 1 and 2 with regard to the pulmonary-to-systemic flow ratio, pulmonary artery pressure, and the interval between surgery and exercise testing. Performance capacity was assessed by determination of the ventilatory threshold during submaximal exercise. The mean value for the ventilatory threshold in group 1 was normal (99.4 +/- 15.1% of the age-predicted normal value). In the children who were more than 5 years of age at the time of the operation, the ventilatory threshold was below normal (84.5 +/- 10.9% of the age-predicted normal value). Furthermore, in group 2 more patients (77%) had values that were below normal (below the 95% confidence limit of the age-predicted normal value) compared with group 1 (27%). It is concluded that functional performance capacity is better when surgical closure of ASD is performed in early childhood and before the age of 5 years rather than at a later age.

Adolescent↗
PubMed1986

A case of single umbilical artery with various fetal anomalies: correlations between prenatal ultrasonographic diagnosis and autopsy.

We report a case of a single umbilical artery with various fetal anomalies, and a comparative study between prenatal ultrasonographic diagnosis and autopsy was done. The prenatal ultrasonographic diagnosis included, atrial septal defect (ASD), ventricular septal defect (VSD), mitral atresia or stenosis and a single great vessel. At autopsy, ASD, VSD, mitral atresia, overriding aorta and pulmonary atresia were evident. In case of other anomalies, the findings in the prenatal diagnosis and at autopsy were encephalocele, adrenal hypoplasia and a single umbilical artery. The ultrasonic detection of these anomalies is discussed and the limitations of this diagnostic method are given attention.

Abnormalities, Multiple↗
PubMed1989

The influence of adaptive seating devices on vocalization.

This study examined the relationship between receipt of adaptive seating devices (ASD) and sophistication of early vocalizations/verbalizations. Eight nonverbal children with cerebral palsy were studied prior to and following receipt of an ASD. Total speech tokens increased for 7 of the 8 subjects and diversity of speech sounds increased for 6 subjects. A concomitant improvement in feeding independence was observed for those subjects who demonstrated the greatest increase in speech sound diversity.

Cerebral Palsy↗
PubMed1993

Electrophysiological abnormalities before and after surgery for atrial septal defect.

The authors evaluated the electrophysiological parameters, atrial-His interval, His-ventricular interval, Wenckebach cycle length, corrected sinus node recovery time (CSNRT), and sinoatrial conduction time (SACT) before and after operation in 28 children with atrial septal defect (ASD). Before operation, electrophysiological abnormalities were detected in 20 (79%) of the 28 patients. Preoperative comparison of the mean value in each parameter between the two age groups showed higher values in all parameters in the older group. In 17 patients who underwent operation, atrial-His interval, Wenckebach cycle length, CSNRT, and atrial effective refractory period (AERP) significantly decreased after operation; preoperatively abnormal Wenckebach cycle length, CSNRT, and AERP values were normalized in many of them. Thus, electrophysiological abnormalities were already present before operation in the ASD children and were severer in the older children, but were improved to some degree following operation.

Adolescent↗
PubMed1996

A continuous epitope from transmissible gastroenteritis virus S protein fused to E. coli heat-labile toxin B subunit expressed by attenuated Salmonella induces serum and secretory immunity.

Antigenic site D from the spike protein of transmissible gastroenteritis virus (TGEV), which is a continuous epitope critical in neutralization, has been expressed as a fusion protein with E. coli heat-labile toxin B subunit (LT-B) in attenuated S. typhimurium. Synthetic peptides containing the sequence of site D induced TGEV neutralizing antibodies when inoculated subcutaneously in both rabbits and swine. A synthetic oligonucleotide encoding residues 373-398 of TGEV S protein, including antigenic site D, was cloned in frame with the 3' end of LT-B gene, into a plasmid used to transform S. typhimurium delta asd chi 3730. A collection of 6 recombinant plasmids designated pYALTB-D I-VI encoding LTB-site D fusions with a variable number of site D sequences were selected. Four of the 6 LTB-site D fusion products expressed in S. typhimurium chi 3730 formed oligomers (pentamers) that dissociated at > 70 degrees. S. typhimurium chi 3730 (pYALTB-D) V and VI expressed the oligomer forming products with higher antigenicity. Partially purified LTB-site D fusion product expressed from S. typhimurium chi 3730 (pYALTB-D) V induced anti-TGEV neutralizing antibodies in rabbits. Recombinant vaccine strain S. typhimurium delta cya delta crp delta asd chi 3987 transformed with plasmid pYALTB-D V expressed constitutively products that formed oligomers presumably containing 20 copies of site D, and showed a high stability in vitro. This recombinant strain was orally inoculated in rabbits and induced TGEV specific antibodies in both serum and intestinal secretion.

Amino Acid Sequence↗
PubMed1993

Expression of a recombinant Entamoeba histolytica antigen in a Salmonella typhimurium vaccine strain.

The expression of a major surface antigen of the intestinal protozoal parasite Entamoeba histolytica in an attenuated Salmonella typhimurium vaccine strain is described. A polymerase chain reaction fragment derived from cDNA encoding the serine-rich Entamoeba histolytica protein, SREHP, was introduced into S. typhimurium chi 3987 (delta cya delta crp delta asd) using a plasmid expression vector (pYA292) containing the aspartate semialdehyde (asd) gene. S. typhimurium expressing recombinant SREHP as a SREHP/maltose binding protein fusion protein was administered orally to mice and gerbils (an important animal model for E. histolytica infection) and was recovered from splenic tissue in both species. Our study demonstrates the feasibility of expressing recombinant amoebic proteins in attenuated S. typhimurium strains, and shows that vaccine strains of S. typhimurium can successfully infect the gerbil, a widely used model for amoebic liver abscess and intestinal amoebiasis.

Animals↗
PubMed1995

Specialized ribosomes: highly specific translation in vivo of a single targetted mRNA species.

In previous studies [Hui and de Boer, Proc. Natl. Acad. Sci. USA 84 (1987) (1987) 4762-4766; Hui et al., Methods Enzymol. 153 (1987) 432-452], it was shown that efficient translation of the human growth hormone mRNA (hGH) species having an altered Shine-Dalgarno (SD) sequence, 5'-GUGUG-3', depends on the presence of specialized (spc) ribosomes containing the modified anti-SD (ASD) sequence, 5'-CACAC-3', near the 3' end of their 16S rRNA. In spite of the altered ASD sequence, spc ribosomes were not found to be committed exclusively to the translation of the hGH mRNA; no more than 30% of the total amount of protein synthesized by such ribosomes was hGH. Once we replace the coding sequence of the hGH mRNA with that of chloramphenicol acetyltransferase (CAT), the specificity of spc ribosomes for translation of a single targetted mRNA, relative to the endogenous mRNAs, is greatly enhanced; an estimated 80% of the total amount of protein synthesized by spc ribosomes is CAT. Using the inducible spc ribosome system containing the cat gene, we show that, upon induction, spc ribosomes accumulate in large excess over the number needed for optimal translation of the targetted cat mRNA. Despite the excess, only few spc ribosomes initiate translation on a limited number of endogenous mRNAs. The excessive accumulation of spc ribosomes, which are predominantly present as free 30S subunits, is neither deleterious to the cells, nor does it lead to a feedback inhibition of the synthesis of wild-type ribosomes.

Chloramphenicol O-Acetyltransferase↗