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Biomedical subjects

S Stahl

Publications and source records attributed to S Stahl.

At least 19 recordsLinked to original sources
PubMed1995

Transfer of the human glucocerebrosidase gene into hematopoietic stem cells of nonablated recipients: successful engraftment and long-term expression of the transgene.

In trying to develop methods of gene therapy for Gaucher disease that will avoid the morbidity and mortality associated with bone marrow (BM) ablation, we transplanted BM stem cells transduced with a retroviral vector containing the human glucocerebrosidase cDNA into normal, nonablated, syngeneic mice. Donor BM from untreated male mice or treated with 5-fluorouracil (5-FU) was transduced ex vivo using a standard 4-day transduction protocol. Recipient female mice were injected one time only or once daily for 5 consecutive days or once a week for 5 consecutive weeks using 2 x 10(7) (untreated BM) or 2 x 10(6) (5-FU-treated BM) cells per injection. Initial transduction efficiency into colony-forming unit-spleen (CFU-S) was 80% to 100%. Recipient analysis was performed at least 6 months after the last transplantation. The best engraftment of donor stem cells, up to 5% by secondary CFU-S analysis, was obtained with multiple injections of transduced BM not previously treated with 5-FU. Polymerase chain reaction (PCR) amplification for both the transgene and the Y chromosome identified the progeny of transduced stem cells in various hematopoietic and non-hematopoietic organs. The copy number of the transgene in stem cells was 0.13 to 2.8. Transgene expression was shown by reverse transcriptase-PCR, in situ hybridization, and immunohistochemistry. No serious side effects of the procedure were noted. We conclude that multiple transplants of retrovirally transduced BM cells into nonablated recipients may be a safe and effective therapeutic modality for a number of genetic hematopoietic disorders.

Animals↗
PubMed1995

Retaining cocaine-abusing women in a therapeutic community: the effect of a child live-in program.

A clinical trial examined whether retention of cocaine-abusing women in a therapeutic community can be improved by permitting their children to live with them during treatment. Fifty-three women were randomly assigned to either the standard community condition (n = 22), in which children were placed with the best available caretaker, or the demonstration condition (n = 31), in which one or two of the children lived with their mother in the community. Survival analysis distributions indicated that demonstration women remained in treatment significantly longer than standard treatment women. (Mean length of stay was 300.4 days vs 101.9 days, respectively.)

Adult↗
PubMed1995

Transvaginal sonographic diagnosis of fetal finger abnormalities in early gestation.

The prenatal sonographic diagnosis of fetal finger abnormalities was recorded in a study population of 20,000 pregnant patients. All scans were performed with a vaginal transducer at 13 to 17 weeks' gestation. The following abnormalities were observed: six cases of polydactyly, three of syndactyly, nine cases of overlapping fingers, two cases of cleft hands, two cases of adactyly, one case of aphalangia, and one case of clasped thumb. In 15 of the 25 fetuses with finger abnormalities, we observed associated malformations or chromosomal disorders, or both. We therefore recommend that examination of the fingers and hands should become an integral part of the early ultrasonographic evaluation of the fetus.

Chromosome Aberrations↗
PubMed1995

Expression of hemidesmosomes and component proteins is lost by invasive breast cancer cells.

Hemidesmosomes are multiprotein structures that attach basal cells of stratified epithelia to basement membranes. Although normal human breast epithelia are not stratified, we observed expression of electron-dense hemidesmosomes and hemidesmosome protein components by breast epithelial and myoepithelial cells at the basal lamina in vivo. Primary cultured normal human breast epithelial cells also contained hemidesmosomes and component proteins, and could be used as a model for hemidesmosome assembly and regulation. In these cultured cells, hemidesmosome proteins were expressed and localized basally in an unvaried temporal pattern, and electron-dense hemidesmosomes were not seen until the final protein was localized to the cell base. In addition, rate of localization was influenced by confluence, doubling time, and extracellular matrix. Invasive breast cancer cells did not express hemidesmosomes or most of the component proteins in vivo. In carcinoma in situ, cells away from the basement membrane lacked hemidesmosomes and hemidesmosome proteins, and cells at the basement membrane exhibited abnormalities of hemidesmosome protein expression. Primary human malignant breast cells in culture exhibited a mix of hemidesmosome phenotypes. These data suggest that hemidesmosomes may be important subcellular structures in determining the cytoarchitecture of the breast epithelium. Further, their downregulation may influence cytoarchitecture remodeling closely linked with cell cycle, motility, and extracellular matrix interactions; and their loss in carcinoma may be associated with loss of normal cytoarchitecture.

Autoantigens↗
PubMed1992

Peripheral ischaemia and gangrene presenting at birth.

The aetiology of non-iatrogenic causes of peripheral ischaemia and gangrene presenting either at birth or within a few hours of delivery is unknown in the majority of 56 confirmed cases. In this review of 47 cases occurring since 1941 the aetiology was clear in only 6, four due to compression by the encircling umbilical cord. There was no clear association with gestational age, birth weight, maternal age or type of delivery. Seven were infants of poorly controlled diabetic mothers and these may constitute a subgroup due to altered haemostatic mechanisms. Pregnancy hypertension was an association in 7 cases, oligohydramnios in 6. There is only limited support for birth trauma, sepsis, and thrombo-emboli from the ductus arteriosus as causes. There is indirect evidence that thrombo-emboli can migrate from the placental bed to the fetus. In recent years death from this condition has been rare with surgical thrombectomy increasingly successful in late presenting cases. When gangrene is established at birth surgical amputation, autoamputation, or some loss of function is usual. Peripheral ischaemic insults presenting at birth may be part of a wider spectrum of disorders, both prenatal and perinatal, attributable to occlusive vascular disruption.

Extremities↗
PubMed1992

Comparison of two new immobilization collars.

STUDY OBJECTIVE: To evaluate the limitation of movement of four cervical collars, with emphasis on two new extrication collars. DESIGN: Ranges of motion permitted by four extrication collars, measured by two goniometric techniques, were compared. Times required to apply each collar were noted and compared. SETTING: In a laboratory setting, volunteers were asked to flex, extend, laterally bend, and rotate their necks, first without restriction and then with each of the collars applied. TYPE OF PARTICIPANTS: Participants were healthy volunteers who worked either in the Department of Physical Therapy or in the Emergency Department of Tulane Medical Center Hospital. INTERVENTIONS: The collars used were the Nec-Loc Extrication Collar, Philadelphia Collar, Philadelphia Red EM Collar with Immobilizer, and Vacuum Splint Cervical Collar. MEASUREMENTS: Measurements were performed first using the head goniometer and then the hand-held goniometer. Time required for application was measured in seconds. Statistical evaluation was performed using repeated measure analysis of variance and then Newman-Keuls multiple comparison procedure. MAIN RESULTS: The Vacuum Splint Cervical Collar restricted range of motion of the cervical spine most effectively. CONCLUSION: A cervical collar with design characteristics similar to the Vacuum Splint Cervical Collar (ie, a rigid collar that incorporates part of the thorax) will restrict movement of the neck more effectively than shorter, less rigid collars.

Adult↗
PubMed1990

Intramuscular haemangioma of the forearm.

Intramuscular haemangioma, though benign, may be a serious disorder, because its large size can cause functional impairment, pain and disfigurement, which may require surgical excision. These, as well as the baffling histopathological aspects, are exemplified by this report of an intramuscular haemangioma of the forearm in an adolescent girl. Clinical recognition of a cutaneous angioma overlying a deeply-situated soft tissue tumour may aid the diagnosis of an intramuscular haemangioma.

Adolescent↗
PubMed1990

[Dermatologic aspects of anorexia and bulimia nervosa].

Anorexia and bulimia nervosa are common in western civilized countries. They are frequently associated with disorders of the skin. Up to now, no dermatological studies of large numbers of patients with eating disorders have been published. We examined 145 patients with eating disorders (65 anorexia nervosa, 80 bulimia nervosa) regarding skin alterations. Simultaneously we controlled the moisture content and the superficial lipids of the skin. Several symptoms relevant to dermatology were found. In detail we report on: xerosis, hair changes, nail disorders, pathognomonic hand lesions, sialadenosis, lesions of the oral mucosa and the teeth, peripheral oedema, acrocyanosis, petechiae and artefacts.

Adult↗
PubMed1989

Genetic engineering applied to the development of vaccines.

The simplest application of the modern genetic manipulation methods to vaccine development is the expression in microbial cells of genes from pathogens that encode surface antigens capable of inducing neutralizing antibodies in the host of the pathogen involved. This procedure has been exploited successfully for development of a vaccine against hepatitis B virus (HBV) that is now widely used. Similar approaches have been directed towards formulations for immunization against several other animal and human diseases and some of these preparations are now presently in trials. Of no less importance is the impact of biotechnology in providing reagents for fundamental studies of topics such as the determination of virulence, antigenic variation, virus receptors and the immunological response to viral antigens. The core antigen of HBV is a good example of a product of genetic engineering that is a valuable diagnostic reagent, and that is finding important use in immunological studies of particular pertinence to vaccine development.

Amino Acid Sequence↗
PubMed1989

An alpha 2 adrenoceptor antagonist, Org 3770, enhances nocturnal melatonin secretion in man.

Nocturnal plasma melatonin concentration was significantly increased in ten normal volunteers following the administration of an alpha 2 adrenoceptor antagonist, Org 3770 (30 mg). This result supports the existence of an alpha 2 adrenergic influence on melatonin secretion in man and provides a possible clinical neuroendocrine marker of the action of an alpha 2 antagonist.

Adrenergic alpha-Antagonists↗
PubMed1988

Comparative immunogenicity of hepatitis B virus core and E antigens.

The nucleocapsid (hepatitis B core Ag (HBcAg] of the hepatitis B virus is a particulate Ag composed of a single polypeptide (p21). Although a non-particulate form of HBcAg designated hepatitis B e Ag (HBeAg) shares significant amino acid identity, the immune responses to these Ag appear to be regulated independently. This report describes the use of recombinant HBcAg and HBeAg to examine and compare murine T cell and B cell recognition of these related Ag. The HBcAg preparation was stable at pH 7.2 and 9.6 and expressed HBc antigenicity. However, the antigenicity of the HBeAg preparation was pH dependent. At pH 9.6 the HBeAg preparation was non-particulate and expressed HBe antigenicity exclusively; however, at pH 7.2 it was particulate and expressed both HBc and HBe antigenicities. Although this "hybrid" particle most likely does not exist naturally, it is a unique research reagent to investigate the interrelationship between HBcAg and HBeAg. HBcAg was significantly more immunogenic in terms of in vivo antibody production as compared to either the non-particulate or particulate forms of HBeAg. Nevertheless, in most murine strains HBcAg and HBeAg were equivalently immunogenic and crossreactive at the level of T cell activation. The disparity between anti-HBc and anti-HBe antibody production is best explained by the observation that HBcAg can function as a T cell-independent Ag whereas HBeAg is T cell dependent even when present within the same particulate structure as HBcAg. Furthermore, HBcAg was shown to function efficiently as an immunologic carrier moiety for the DNP hapten in athymic as well as euthymic mice in contrast to conventional carrier proteins. These results have implications relevant to the human immune responses to HBcAg and HBeAg during infection, and to vaccine development.

Animals↗
PubMed1988

Delayed rupture of the extensor pollicis longus tendon after nonunion of a fracture of the dorsal radial tubercle.

Several apparently conflicting mechanisms have been proposed to explain the seemingly spontaneous delayed rupture of the extensor pollicis longus tendon (EPL). The following case, the first of its kind of which we are aware, may help to clarify the relationships between these mechanisms. Traumatic hyperextension of a patient's wrist brought the styloid process of the third metacarpal into contact with Lister's tubercle, fracturing it. Chronic chafing of the EPL on the nonunited fracture's jagged surface seemed to cause its spontaneous rupture 18 months later. The long period between accident and rupture is evidence that rupture was not caused by crush injury. Because a fracture of Lister's tubercle will not normally be visible on radiographs, after accidents in which this may have occurred or when the EPL ruptures more than 3 months after injury, we recommend that special radiographs of Lister's tubercle be taken to determine if such a fracture exists.

Adult↗
PubMed1987

The design of a simple competitive ELISA using human proinsulin-alkaline phosphatase conjugates prepared by gene fusion.

The gene encoding human proinsulin has been fused in-frame with the E. coli alkaline phosphatase gene (pho A) (EC 3.1.3.1). Two constructions are described. One construction consists of the entire proinsulin gene fused to the 5'-terminal end of pho A. In the other construction a 42 base pair DNA fragment has been deleted from the 3'-terminal end of the proinsulin gene. The two purified fusion proteins are enzymatically active showing a specific activity of 10-15 U/mg and 18-25 U/mg, respectively. The first construction exhibited insulin antigenicity and was used to design a simple competitive ELISA for insulin. The lower detection limit was found to be at least 2.5 ng/ml. Both fusion proteins were also shown to have potential for use in a competitive ELISA for proinsulin.

Alkaline Phosphatase↗
PubMed1987

Antiparkinsonian activity of (+)-PHNO in the MPTP-treated common marmoset.

Administration of MPTP (1-4 mg/kg ip daily for 5-7 days) to common marmosets induced persistent parkinsonian motor deficits. The subcutaneous administration of (+)-PHNO [(+)-4-propyl-9-hydroxynaphthoxazine; 1-4 micrograms/kg] caused a dose-dependent reversal of the akinesia and incoordination of movement. Similarly, oral administration of (+)-PHNO (5-20 micrograms/kg) caused an equivalent reversal of the motor abnormalities. No dyskinetic phenomena were induced by (+)-PHNO on oral or subcutaneous administration. Oral or subcutaneous administration of (+)-PHNO to normal control marmosets also increased the usual repetoire of motor behaviour, but this was not as marked as in MPTP-treated animals. (+)-PHNO is a potent dopamine agonist drug of potential use in the treatment of Parkinson's disease.

1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine↗