Lateral transmission of natural scrapie to scrapie-free New Zealand sheep placed in an endemically infected UK flock.
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Animals↗
Biomedical subjects
D Drummond
Publications and source records attributed to D Drummond.
Derivation of a scrapie-free sheep flock from the progeny of a flock affected by scrapie.
The Cheviot flock at the Institute for Animal Health's Neuropathogenesis Unit (npu) has endemic scrapie, which affects primarily vrq/vrq sheep and at high frequency. A new flock with a full range of PrP genotypes, including the highly susceptible vrq/vrq, has been produced on a separate site, from animals in the npu breeding flock, and it remains scrapie-free after eight years. In contrast, in a parallel flock at the npu farm, scrapie has reappeared after five years, although the animals were kept in separate accommodation from the scrapie-affected sheep. During this time the npu breeding flock continued to have scrapie cases. Although it is known that highly susceptible sheep can remain free of infection in a clean environment, this is the first report of the infection being removed successfully from the bloodlines of scrapie-affected sheep. The results confirm that scrapie is not a genetic disease dependent only on the PrP gene sequence, but requires both genetic susceptibility and an infectious agent.
Animal Husbandry↗
Transcatheter closure of surgical shunts in patients with congenital heart disease.
The aim of this study was to review contemporary techniques, devices, and results of transcatheter occlusion of surgical shunts in 2 pediatric cardiac programs. Closure of superfluous surgical shunts may reduce cardiac work and risk of endocarditis. Previous studies have shown that transcatheter closure of shunts is feasible, but have not demonstrated acceptable efficacy or safety. In addition, the performance of new techniques and devices has not been reviewed. Between 1993 and 1998, 18 patients with congenital heart disease underwent transcatheter closure of 19 Blalock-Taussig shunts. Detachable and standard Gianturco coils and Gianturco-Grifka vascular occlusion devices were employed. All 19 shunts had complete closure. Eight shunts had initial placement of detachable coils. Five shunts had stents placed that bridged the pulmonary end of the shunts. These 5 and 4 additional shunts had closure by standard coils. Two shunts were closed with Gianturco-Grifka devices. There were no complications, no embolizations, and no requirement for surgery precipitated by the procedures. This review of contemporary techniques, devices, and results suggests that transcatheter occlusion of surgical shunts is effective and safe.
Blood Vessel Prosthesis Implantation↗
Transmissions to mice indicate that 'new variant' CJD is caused by the BSE agent.
There are many strains of the agents that cause transmissible spongiform encephalopathies (TSEs) or 'prion' diseases. These strains are distinguishable by their disease characteristics in experimentally infected animals, in particular the incubation periods and neuropathology they produce in panels of inbred mouse strains. We have shown that the strain of agent from cattle affected by bovine spongiform encephalopathy (BSE) produces a characteristic pattern of disease in mice that is retained after experimental passage through a variety of intermediate species. This BSE 'signature' has also been identified in transmissions to mice of TSEs of domestic cats and two exotic species of ruminant, providing the first direct evidence for the accidental spread of a TSE between species. Twenty cases of a clinically and pathologically atypical form of Creutzfeldt-Jakob disease (CJD), referred to as 'new variant' CJD (vCJD), have been recognized in unusually young people in the United Kingdom, and a further case has been reported in France. This has raised serious concerns that BSE may have spread to humans, putatively by dietary exposure. Here we report the interim results of transmissions of sporadic CJD and vCJD to mice. Our data provide strong evidence that the same agent strain is involved in both BSE and vCJD.
Animals↗
Progressive congenital kyphosis: report of five cases and review of the literature.
For over 60 years congenital kyphotic deformities of the spine have been categorized into two distinct groups, depending on the developmental defect. Those arising from a failure of formation of the vertebral bodies were classified as type 1, while those arising from a failure of segmentation were referred to as type 2. Recognition of the progressive and unstable nature of the type 1 defects alerted physicians to the need for early operative stabilization through decompression and stabilization through instrumentation. As the embryogenesis of the spinal column was further investigated, and as diagnostic imaging methods of the spine improved, unstable congenital kyphoses were further subdivided. Progressive congenital kyphotic deformities now may accompany a host of vertebral column developmental defects as well as genetically mediated mesenchymal tissue defect syndromes. This paper presents 5 patients from The Children's Hospital of Philadelphia with progressive and symptomatic congenital kyphotic deformities of the spine. Two of these lesions resulted from defects of formation of the vertebral bodies, while one resulted from segmental spinal dysgenesis, maldevelopment of both the anterior and posterior vertebral elements. One patient's kyphotic deformity was a result of caudal regression syndrome, and the final case presented experienced a high thoracic kyphosis from a syndrome associated diffuse midline mesenchymal tissue abnormalities known as cerebrocostomandibular syndrome. All patients showed evidence of progressive cord compression and required neural element decompression, fusion, and instrumentation. The cases are discussed individually, and the developmental and clinical aspects of each are explored.
Bone Transplantation↗
Spinal deformity in patients who have fibrodysplasia ossificans progressiva.
We reviewed roentgenograms and clinical records in order to characterize the spinal deformity in forty patients who had an established diagnosis of fibrodysplasia ossificans progressiva. Twenty-six (65 per cent) of the patients had scoliosis, which, according to the clinical records and the recollection of the patients, had been present during childhood. Twenty-three (88 per cent) of the twenty-six curves were unbalanced c-shaped curves, while the remaining three (12 per cent) were balanced s-shaped curves. Twenty-one (91 per cent) of the twenty-three c-shaped curves involved the thoraco-lumbar or lumbar spine. The c-shaped curves ranged in magnitude from 15 to more than 80 degrees. Curves became rigid by early adulthood and many resulted in severe pelvic obliquity with impaired sitting or standing balance. An osseous bridge developed between the posterolateral aspect of the iliac crest and the posterolateral aspect of the rib cage in twenty-two (55 per cent) of the forty patients. Nineteen (86 per cent) of these twenty-two patients had scoliosis; there was a significant association between the development of scoliosis and the presence of the osseous bridge (p < 0.005). Ossification of the paravertebral muscles and fascia during the first decade of life limited the development of a normal thoracic kyphosis in ten (42 per cent) of twenty-four patients for whom lateral roentgenograms of the spine were available. A spinal orthosis was used to treat the scoliosis in two patients, but this method resulted in breakdown of the skin and failed to halt progression of the curve.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
Design and evaluation of a posterior laminar clamp spinal fixation system.
A new spinal instrumentation system for posterior applications was designed with the intent of minimizing intrusion of hardware into the neural canal during and postinsertion. Basic biomechanical measurements of posterior pullout strength led to the establishment of a clamp as an effective mechanism for gaining purchase on the vertebral lamina. These experiments defined the appropriate instrumentation size, as well as establishing that there was no loss of strength with the spinous process removed and the posterior laminar cortex compromised. There was no statistically significant difference between the maximum pullout strength achievable, using a band around the lamina as a control, and that produced by the implant system, in the thoracic spine. Comparative testing of a prototype system in flexion, extension, lateral bending, and torsion showed that the system, in general, had stiffness greater than segmental wiring and less than or equal to Cotrel-Dubousset fixation.
Biomechanical Phenomena↗
Protein engineering and the study of muscle contraction in Drosophila flight muscles.
We describe an experimental approach to the use of genetics to study muscle contraction in Drosophila melanogaster. Mutations induced by in vitro mutagenesis are inserted into the genome of flies using P-element mediated transformation, permitting the effects of the mutant genes to be studied in vivo in the indirect flight muscles (IFMs). Details of how mechanical experiments can be performed on skinned IFMs, despite their small size, are provided. The effects of two in vitro actin mutations, G368E and E316K, are described. The problems of performing biochemical and biophysical experiments on the IFMs and their myofibrillar proteins are described, together with indications as to how these may be overcome.
Actins↗
Major injury as a unique opportunity to initiate treatment in the alcoholic.
A prospective study was performed on the use of a standard outpatient intervention technique to induce inpatient alcoholic trauma patients into accepting alcoholism treatment. Interventions were performed on 17 trauma patients. All patients who underwent intervention accepted treatment and were immediately transferred to a 28-day inpatient treatment facility. Alcoholic trauma patients are highly susceptible to intervention for their disease. We found that intervention performed upon discharge from the trauma service successfully initiates alcoholism treatment.
Alcoholism↗
The stability and movement of mRNA in Xenopus oocytes and embryos.
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Animals↗
Interspinous process segmental spinal instrumentation.
A method for interspinous segmental spinal instrumentation (ISSI) is described and the laboratory testing and early clinical results are reported. The method utilizes a button-wire implant that is passed through the thickest and strongest part of the base of the spinous process. Tension tests show the stress-relieving qualities of the implant improve pullout strength 47% over simple wire fixation of the spinous process. Tests on the scoliosis simulator show that the interspinous instrumented spine resists high compressive loads to failure, comparing favorably with other systems tested. Early clinical experience with the implant for a wide variety of uses has been encouraging. The authors use ISSI as their procedure of choice in idiopathic and congenital scoliosis.
Adolescent↗
Improved postoperative course following modified segmental instrumentation and posterior spinal fusion for idiopathic scoliosis.
Postoperative course following posterior spinal fusion was reviewed and compared for 31 patients who underwent Harrington distraction instrumentation and 30 patients who underwent modified segmental spinal instrumentation (SSI). The SSI patients, on average, received oral feedings 1 day earlier (p less than 0.046), walked 5 days earlier (p less than 0.001), and were discharged 5 days sooner (p less than 0.001) than patients undergoing Harrington distraction. Despite this more active postoperative course, the SSI patients did not require more analgesics or narcotics. When the hospital costs were compared, there was a savings of $2,825 for the average patient undergoing SSI.
Adolescent↗
Radiation hazards in scoliosis management.
Safe radiography in scoliosis management is based on a sound knowledge of 1) the radiographic imaging process, 2) the degree of risk to the patient from radiation exposure, and 3) the radiographic requirements to both evaluate and follow patients with spine deformity. This paper is a current review of the subject and work done at the authors' centers. It includes recommendations for reducing the radiation risk while maintaining necessary diagnostic information.
Child↗
Methylenetetrahydrofolate dehydrogenase - methenyltetrahydrofolate cyclohydrolase - formyltetrahydrofolate synthetase from porcine liver: evidence to support a common dehydrogenase-cyclohydrolase site.
The cyclohydrolase activity of the trifunctional enzyme methylenetetrahydrofolate dehydrogenase - methenyltetrahydrofolate cyclohydrolase - formyltetrahydrofolate synthetase is inhibited by NADP+, a substrate of the dehydogenase. This uncompetitive inhibition, shown also by 3-aminopyridine adenine dinucleotide phosphate (AADP), indicates formation of dead-end complexes consisting of enzyme-nucleotide-methenyltetrahydrofolate. Chemical modification with diethylpyrocarbonate inactivates the dehydrogenase and cyclohydrolase but not the synthetase. Folate, but neither NADP+ nor AADP, protects both activities against modification. However, NADP+ potentiates the protection by folate by decreasing the apparent Kd for that ligand approximately sixfold. Chemical modification with phenylglyoxal also inactivates both the dehydrogenase and cyclohydrolase activities. Neither activity was protected by NADP+ or folate alone; however, the combination of NADP+ and folate protected both activities. These results are consistent with a model in which the dehydrogenase and cyclohydrolase activities share a common folate binding site.
Aminohydrolases↗
Melorheostosis in children. Clinical features and natural history.
Experience in the management of fourteen children with melorheostosis has been reviewed. The principal and presenting clinical features were unilateral soft-tissue contractures associated with inequality of limb length. In contrast to the disease in adults, pain occurred infrequently and was never intense. The average interval between the discovery of the clinical features and the correct diagnosis was six years. The distinctive radiographic feature in the child was an endosteal pattern of hyperostosis marked by streakiness of the long bones and spotting of the small. This differs from the usual subperiosteal or extracortical pattern of hyperostosis seen in adults. The surgical treatment of the contractures proved difficult and recurrence of the deformity was the rule. Distal ischaemia occurred when the chronically contracted and flexed joint was rapidly extended.
Adolescent↗
Relationship of spine deformity and pelvic obliquity on sitting pressure distributions and decubitus ulceration.
The distribution of pressure points in 16 patients with paraplegia, nine with ulcers, and six who were ulcer free were compared with the distribution in 15 normal individuals using an instrument capable of simultaneously measuring multiple pressure points under the buttocks and thighs. The nine patients with ischial and sacral decubiti showed redistribution of their sitting pressures posteriorly, asymmetrical loading of the ischiae, and higher than normal pressures under the sacrococcygeum. These abnormal pressures were associated with unbalanced scoliosis, pelvic obliquity, and the loss of physiological lordosis following a spinal fusion. We defined four criteria of risk for decubitus ulceration.
Buttocks↗