Successful pregnancies in the presence of spinal muscular atrophy: two case reports

We report two cases of successful pregnancy in women with chronic, infantile onset, or type II spinal muscular atrophy, both of whom delivered healthy, unaffected babies. The patients required concurrent management by a physiatrist, pulmonologist, and perinatologist throughout the pregnancy. Complications included recurrent urinary tract infections, dyspnea and worsening of pulmonary function, wheelchair seating and positioning problems, and musculoskeletal and low back pain. These problems resolved postpartum. One woman had vaginal delivery, the other had caesarean section, both of which were well-tolerated. Because of severe musculoskeletal deformity, pelvic assessment is necessary to determine the mode of delivery. The uterus has normal contractility and effective labor patterns can be established. Spinal/epidural anesthesia may be contraindicated because of spine deformity. The pregnancies had no deleterious effect on the progression of the disease in our patients, both of whom reported a positive experience with great personal fulfillment.     

Cytoplasmic tail deletion of T cell receptor (TCR) beta-chain results in its surface expression as glycosylphosphatidylinositol-anchored polypeptide on mature T cells in the absence of TCR-alpha

Surface expression of the T cell antigen receptor (TCR) in mature T cells requires the association of a variable heterodimer (alpha.beta or gamma.delta) with six invariant CD3 polypeptides (gamma, delta, epsilon-epsilon, zeta-zeta, or zeta-eta). We described here that deletion of the cytoplasmic tail polypeptide sequence (Lys-Lys-Lys-Asn-Ser) of TCR beta-chain (beta CT) results in expression of the truncated beta-chain on the surface of a mature T cell hybridoma line, in the absence of TCR-alpha, as a glycophosphatidylinositol (GPI)-anchored monomeric polypeptide. The GPI-anchored TCR-beta CT is not associated with CD3-epsilon and is incapable of conventional signal transduction. Association with TCR-alpha prevents beta CT from GPI-linkage formation. The alpha beta CT heterodimer binds the CD3 polypeptides, and the resultant TCR alpha beta CT/CD3 complex is capable of signal transduction. Our data show that a signal sequence for GPI-linkage formation is present in TCR-beta, and this alternative membrane anchoring mechanism can be utilized by beta-chain polypeptide lacking the CT sequence. We conclude therefore that in the absence of TCR-alpha expression, the beta-chain CT sequence plays an essential function in hindering GPI-linkage formation, thereby preventing escape of incompletely assembled TCR beta-chain to the cell surface of mature T cells.     

A cdc2-like kinase associated with commitment to division in Paramecium tetraurelia

Cell division in higher eukaryotes is mainly controlled by p34cdc2 or related kinases and by other components of these kinase complexes. We present evidence that cdc2-like kinases also occur in Paramecium. Two polypeptides reacted with an antibody directed against the perfectly conserved PSTAIR region found in cdc2 kinases in other eukaryotes. Only the less abundant peptide bound to p13suc1 from Schizosaccharomyces pombe. Using centrifugal elutriation to select cells on the basis of size, we isolated highly synchronous Paramecium G1 cells. With this procedure, we demonstrated that the p13suc1-associated cdc2-like histone H1 kinase was activated before cell division at the point of commitment to division in Paramecium. Further, we show that Paramecium cdc2-like proteins occurred principally as monomers and that these monomers were active as histone H1 kinases in vitro.     

Automatic generation of IDEF0 models

This paper looks into a new area for knowledge-based system application, that of manufacturing modelling. Manual generation of IDEF0 models of manufacturing systems is time-consuming and inconsistent. However, the process can be automated to improve timeliness and consistency. In this paper, a knowledge-based manufacturing modelling system for the automatic generation of IDEF0 models is proposed. The system will not only greatly reduce the IDEF0 modelling time but will also eliminate the inconsistency problem of conventional IDEF0 modelling systems. The paper explains the knowledge-based approach and identifies the kinds of domain knowledge that are required for the construction of the knowledge-based manufacturing modelling system.     

Effects of pramipexole on contraversive rotation and functional motor impairments in 1-methyl-4-phenyl1,2,3, 6-tetrahydropyridine-induced chronic hemiparkinsonian monkeys

Rotational and functional motor behavioral changes were studied in five MPTP lesioned chronic hemiparkinsonian Macaca nemestrina monkeys after i.m. pramipexole, a predominant D2 subfamily agonist. Pramipexole induced contraversive rotations in a dose-dependent manner with an optimal dose of 56 microg/kg for approximately 2 to 4 hr after injection. Three different rating scales were used to determine drug-induced functional improvement. They included a monkey parkinsonism rating scale, volitional responses to fruit presentations, and number of hand movements that appeared volitional. A dose of 56 microg/kg of pramipexole produced functional improvements on hand disability, and on a parkinsonian rating scale for monkeys in a dose-dependent manner from 32 to 100 microg/kg. These doses produced an increase in significant hand movements in the affected (contralateral) as well as in the normal (ipsilateral) hand to the side of the brain lesion compared with 5% dextrose in water vehicle control. With a dose of 100 microg/kg, the therapeutic effects of pramipexole on hand movements were less than with 56 microg/kg, due to side effects such as scratching.     

A controlled evaluation of an elementary school primary prevention program for eating problems

Researchers have recently called for the development of primary prevention of eating disorders programs aimed at elementary school students. The present study reports on the development of a curriculum for fifth graders designed to encourage healthy eating, exercise, and body image while discouraging calorie-restrictive dieting, exercising for weight loss, and the development of body dissatisfaction. The program consisted of ten lessons taught by the classroom teachers. The influence of the curriculum on (1) knowledge about nutrition, body fat, and dieting; (2) attitudes about fat people and own body (body esteem); and (3) behaviors, including attempts at weight reduction, consumption of fruits and vegetables, and exercising, were evaluated in a pre-post controlled experimental design. There were 222 white public school children who participated in both the pre- and posttesting, 167 of whom were in the classrooms receiving the curriculum. Results indicated that knowledge was broadly improved by the curriculum. There were information improvements in terms of nutrition, effects of dieting, and causes of body fat. Attitudinal changes were less pronounced, although the curriculum did positively affect attitudes about fat people. Behavior, including eating patterns, exercise patterns, weight reduction attempts, and teasing of fat children, was not changed by participation in the curriculum.     

Salmonella excretion in adult cattle on the Maltese island of Gozo

A cross-sectional study was conducted, between June and November 1993, to determine the prevalence of Salmonella excretion in a representative sample (n = 300) of adult cattle, chosen by strict random sampling of the isolated bovine population in Gozo. Salmonellae were found in 41.3% of the cattle investigated, and seven animals yielded multiple Salmonella serovars. The total number of Salmonella isolates was 131. These included Salmonella Croft (55 isolates), S. Telaviv (29), S. Montevideo (17), S. Kpeme (7), S. Infantis (2) and S. Abadina (1). A seventh serovar (14 isolates) was found to have a novel antigenic structure: the name S. Gozo was proposed, and was accepted by the World Health Organisation Salmonella Reference Centre. All isolates were sensitive to four antibiotics, while 43% of the Salmonella strains were sensitive to nine antibiotics. No resistance was detected to cefuroxime, ciprofloxacin and gentamicin.     

Functional palatoraphy and modified genioplasty in obstructive sleep apnea

10 patients with obstructive sleep apnea syndrome (OSAS) have been treated with the new surgical procedure functional palatoraphy and modified genioplasty. 5 months after surgery 7 patients with an apnea hypopnea index under 10 were cured. Three therapy refractory patients were all overweight with a body mass index of more than 29 kg/m2. Excessively overweight patients should therefore not be operated. Following the selection criteria we introduced an effective new treatment method for OSAS.     

A resource-saving technology for manufacturing unfired articles for bottom casting of steel

Basic parameters of a resource-saving technology for manufacturing unfired articles for bottom steel casting that was developed under laboratory conditions are presented. Industrial tests of an experimental batch of unfired bottom tubes produced by the suggested technology have shown high performance properties for them and applicability of them for bottom casting of steel.Translated from Ogneupory i Tekhnicheskaya Keramika, No. 8, pp. 27–29, August, 1996.