shukoor65/lab-rag-model huggingface dataset:
Reflex anal dilatation: effect of parting the buttocks on anal function in normal subjects and patients with anorectal and spinal disease. Anal dilatation in response to gentle parting of the buttocks has been advocated as a sign of sexual abuse in children, but nothing is known of the physiology of this response or its existence in normal subjects, in patients with spinal disease, and in patients with a weak sphincter and whether it can be elicited after training. To answer these questions we investigated the effect of parting the buttocks on anal function. Combined anal manometry and electromyography was conducted in six normal subjects (five men, one woman, aged 19-53 years), in 18 patients with faecal incontinence (three men, 15 women, aged 30-80 years), and in seven paraplegic patients (six men, one woman, aged 25-36 years), in four of whom the posterior sacral roots had been cut. Parting the buttocks in normal subjects reduced the pressure in the anal canal from 102 (20) to 14 (3) cm H2O (mean (SEM), p less than 0.00001), but did not cause the anus to gape. This drop in pressure was associated with increased electrical activity in the external anal sphincter. Normal subjects could consciously relax the external anal sphincter and reduce the anal pressure but not so as to result in anal gaping during traction on the buttocks, even after anal dilatation. Stimulation of the anal lining by moving a probe in and out of the anal canal increased the activity of the external anal sphincter, raising anal pressures. Paraplegic patients who had lost conscious control of their external sphincters showed anal gaping when the buttocks were parted.
Maple syrup urine disease in Mennonites. Evidence that the Y393N mutation in E1 alpha impedes assembly of the E1 component of branched-chain alpha-keto acid dehydrogenase complex. Maple Syrup Urine Disease (MSUD) in Mennonites is associated with homozygosity for a T to A transversion in the E1 alpha gene of the branched-chain alpha-keto acid dehydrogenase complex. This causes a tyrosine to asparagine substitution at position 393 (Y393N). To assess the functional significance of this missense mutation, we have carried out transfection studies using E1 alpha-deficient MSUD lymphoblasts (Lo) as a host. The level of E1 beta subunit is also greatly reduced in Lo cells. Efficient episomal expression in lymphoblasts was achieved using the EBO vector. The inserts employed were chimeric bovine-human cDNAs which encode mitochondrial import competent E1 alpha subunit precursors. Transfection with normal E1 alpha cDNA into Lo cells restored decarboxylation activity of intact cells. Western blotting showed that both E1 alpha and E1 beta subunits were markedly increased. Introduction of Y393N mutant E1 alpha cDNA failed to produce any measurable decarboxylation activity. Mutant E1 alpha subunit was expressed at a normal level, however, the E1 beta subunit was undetectable. These results provide the first evidence that Y393N mutation is the cause of MSUD. Moreover, this mutation impedes the assembly of E1 alpha with E1 beta into a stable alpha 2 beta 2 structure, resulting in the degradation of the free E1 beta subunit.
In vivo regulation of virulence in Pseudomonas aeruginosa associated with genetic rearrangement. A chronic pulmonary infection model was used to induce conversion to the mucoid phenotype by Pseudomonas aeruginosa PAO. At 6 months after initial inoculation, organisms isolated from infected lungs demonstrated the mucoid phenotype. Significant decreases (P less than .01) were seen in the levels of exotoxin A, exoenzyme S, phospholipase C, and pyochelin produced by the mucoid P. aeruginosa PAO rat lung isolates that returned to parental levels after reversion to the nonmucoid phenotype. In addition, lipopolysaccharide of the mucoid PAO lung isolates failed to react with serotype B-specific antibody in contrast to the original PAO and the revertant PAO organisms. Digestion of chromosomal DNA and hybridization with P. aeruginosa virulence factor-specific probes demonstrated that conversion to the mucoid phenotype was associated with rearrangement of chromosomal DNA upstream of the exotoxin A gene. Analysis of DNA from revertant organisms revealed hybridization patterns identical to the original PAO organism.
Delay to invasive investigation and revascularisation for coronary heart disease in south west Thames region: a two tier system? OBJECTIVES--To assess the accessibility of invasive investigation and revascularisation procedures in the management of coronary heart disease in a defined population in the South West Thames region over 10 years, and to audit the performance of both the NHS and the private sector. DESIGN--Analysis of all patient referrals to the regional cardiothoracic centres for coronary heart disease during 1979-88. SETTING--North West Surrey District Health Authority, which had a mean catchment population of 205,000 during the study period. The health district is one of the 13 in the South West Thames region. PATIENTS--823 patients aged 34-80 years with suspected coronary heart disease, 204 of whom were referred for private investigation and 619 were referred within the NHS. The NHS referrals were mainly to St Thomas's Hospital during 1979-83 and to St George's Hospital during 1984-8. MAIN OUTCOME MEASURES--Difference in time to investigation and intervention between the NHS and the private patients. RESULTS--After some variation in earlier years the mean (SD, range) waiting times from referral to cardiac catheterisation and then revascularisation increased progressively in NHS patients, to 115.8 (126.5, 22-482) days and 305.9 (164.4, 22-620) days respectively in 1988. There was no significant change over the 10 years in waiting times within the private sector, with a mean of 17.2 (18.2, 1-62) days to angiography and 22.8 (14.5, 2-152) days to surgery. Fifteen people on the NHS waiting list died of probable cardiac causes. No people on the private waiting list died. CONCLUSIONS--The performance of the NHS system in South West Thames region in response to emergency referral is adequate. The waiting time to routine investigation and revascularisation is prolonged and seems to be worsening despite increased investigative and surgical activity by the regional centre. The delays may subject NHS patients to unnecessary risk, which is not shared by private patients.
Prevention of retrovirus infection after injury with contaminated instruments: an experimental study. Owing to similarities between human immunodeficiency virus and feline retroviruses, the feline model was chosen for the study to investigate the efficacy of timely topical treatment of accidental human immunodeficiency virus infection in the operating room. Cats were subcutaneously inoculated with either feline leukemia virus or feline immunodeficiency virus. An effort was made to neutralize the virus in loco either by infiltration of the inoculation site with povidone-iodine or with monoclonal antibodies, or by cauterization and excision. The animals were periodically monitored for feline leukemia virus antigens or for feline immunodeficiency virus antibodies. The results indicated that in the feline model, the development of generalized virus infection may be prevented by local measures if applied immediately.
Nuclear exercise testing and the management of coronary artery disease. Three hundred seventy-eight patients referred for nuclear exercise testing were classified using demographics and symptoms into low, intermediate, and high coronary disease likelihood categories. These likelihood groups constituted 15%, 41%, and 15% of referrals, respectively. Patients with prior infarction or disease at angiography (proven disease) made up the remaining 29% of patients. Only 2% of low likelihood patients had typical angina, but physicians diagnosed coronary disease in 64%, prescribed antianginal therapy in 50%, and were considering catheterization in 28% of these patients, all as frequently as for patients with intermediate or high likelihoods for disease. Patients with proven disease were treated differently in that 79% were receiving antianginal therapy and 56% were considered for catheterization (p less than 0.001). Nuclear exercise test results reduced the perceived need for catheterization in all groups, on average by 49%. Nuclear exercise tests are a standard by which patients are managed, sometimes substituting for the traditional role of the history in physician decision making.
Role of vasopressin in salt-induced hypertension in baroreceptor-denervated uninephrectomized rabbits. To elucidate the contributions of renal, humoral, and arterial baroreceptor reflex components to salt-induced hypertension, we administered 10% NaCl intravenously for 10 days to sinoaortic-denervated rabbits with unilateral nephrectomy (n = 7), sinoaortic-denervated rabbits with intact kidneys (n = 7), and sham-operated sinoaortic-denervated rabbits with unilateral nephrectomy (n = 7). Serial changes in mean arterial pressure (MAP), heart rate, and blood pressure variability were recorded. In sinoaortic-denervated rabbits with unilateral nephrectomy, MAP increased significantly from 109 +/- 2 to 124 +/- 3 mm Hg (day 4) and remained elevated for the rest of the experiment. This elevation of MAP was accompanied by a reduction in the standard deviation of MAP, with significant elevations in plasma vasopressin, norepinephrine, and atrial natriuretic peptide concentrations and in sodium retention. In the other groups, there were no significant changes in these vasoactive hormones. In the sham-operated sinoaortic-denervated rabbits with unilateral nephrectomy, sodium retention was similar to that of sinoaortic-denervated rabbits with unilateral nephrectomy. Continuous infusion (1 microgram/kg/hr) of a V1 antagonist prevented the elevation of blood pressure and plasma norepinephrine, the accumulation of sodium, and the reduction of blood pressure lability, whereas a bolus injection (10 micrograms/kg) on day 4 reduced blood pressure from 128 +/- 3 to 115 +/- 2 mm Hg (p less than 0.005). These results imply that vasopressin plays a crucial role in the expression of salt-induced hypertension in rabbits with compromised baroreceptor and renal function.
Giant adult malignant sacrococcygeal teratoma. Successful treatment by combined abdominosacral resection. This report describes the successful removal of the largest adult sacrococcygeal teratoma (18.75 kg) the authors could find on record. The patient was 58 years of age. The tumor had been present at birth and had been biopsied at the time of her cesarean section 34 years earlier without further treatment. Special planning was necessary for moving and positioning the patient for operation to prevent injury due to the size and weight of the tumor. A combined abdominosacral resection with preliminary ligation of the internal iliac arteries and a diversionary colostomy were performed without difficulty or undue blood loss. The defect was closed primarily and drained. The tumor proved to be malignant on pathologic examination.
Ultrasonic decalcification of the aortic annulus during aortic valve replacement. Aortic valve replacement for calcifica aortic stenosis requires meticulous debridement of the aortic annulus to effect optimal valve seating. Since 1987, we have used ultrasonic energy to debride the aortic annulus during aortic valve replacement in 56 patients. In our experience, ultrasonic debridement of the annulus is superior to traditional methods of debridement, affords improved seating of the valve, and may allow placement of a larger valve. Our follow-up ranges from 2 to 32 months (mean follow-up, 13 +/- 9 months) with 0% incidence of paravalvular leak or valve failure. We advocate the use of ultrasonic debridement as an adjunctive tool in aortic valve replacement.
Unilateral lumbar fusion. Unilateral fusion of the lumbar spine is rarely necessary or indicated. However, in patients with a "far-out syndrome" requiring decompression or in cases where unilateral posterior element resection is necessary for any reason, it may be both necessary and indicated. This unilateral destabilization effectively removes one leg of the tripod, rendering that intervertebral joint potentially unstable. The charts and radiographs of 13 patients (seven men, six women) with an average age of 60 years (range, 25 to 76) who underwent unilateral fusion were retrospectively reviewed. Follow-up time ranged from 12 1/2 to 2 years with a median follow-up of 8 years. Eleven of the fusions were at one level, and two were at two levels. Seven patients had a far-out syndrome secondary to degenerative scoliosis; four were secondary to spondylolisthesis. Two patients had an osteoid osteoma involving a pedicle. A paraspinal approach was used in the majority of patients. Autologous bone graft was used in all patients. Unilateral pedicle screw fixation was used in the last patient in the series. The fusion rate was 85% (11/13). Three patients were smokers, two of whom developed pseudarthrosis. Disc space height did not appear to affect fusion rate. There was no progression of slip noted in any of the patients. One complication was noted in this group: a moderate postoperative infection, which cleared spontaneously.
Double compartment hydrocephalus in an adult. A case of double-compartment hydrocephalus in an adult is presented. Although this entity is infrequently noted in children, it is diagnosed even more rarely in the adult population. A discussion of the spectrum of abnormal cerebrospinal fluid dynamics of the 4th ventricle and treatment options are presented.
Carpal tunnel syndrome: surgical treatment using the Paine retinaculatome. The operative technique for the relief of carpal tunnel syndrome has remained controversial. This report presents the results of 445 patients or 577 hands operated on using a transverse technique that varies little from that described by Paine and Polyzoidis. The patients were followed for 9 months to 3 years. In addition to the data received from follow-up visits, a questionnaire was sent to each patient for evaluation of their results. All surgery was performed under local anesthesia, sometimes with intravenous supplement. Of the 445 patients, 313 had unilateral operations and 132 had bilateral operations at one sitting. Postoperatively, normal hand function was achieved in 59.4% of patients in 1 week or less. Of the 577 hands operated on, 535 (92.7%) exhibited satisfactory results from surgery. No patient required postoperative physical therapy or splinting. There were no injuries to the median nerve or any of its branches.
Motor responses after transcranial electrical stimulation of cerebral hemispheres with a degenerated pyramidal tract. Motor responses were evoked in the thenar muscles by transcranial electrical cortex stimulation in 5 stroke patients with an isolated lacuna in the internal capsule, in whom wallerian degeneration of the pyramidal tract was demonstrated in vivo. Suprathreshold stimulation of the affected hemisphere elicited bilateral motor responses; whereas, stimulation at identical intensities of the undamaged hemisphere yielded strictly unilateral responses in the contralateral hand, like the responses of all normal control subjects. Focused magnetic brain stimulation was performed in 1 patient and gave identical results. Because muscular excitability to cortical stimulation is preserved in spite of pyramidal tract disruption, other pathways must bypass the lesion. Because of the bilaterality of responses, we suggest polysynaptic corticoreticulospinal connections.
Treatment of constitutional growth delay in prepubertal boys with a prolonged course of low dose oxandrolone. Forty six prepubertal boys who had constitutional growth delay were treated with oxandrolone. Mean age at the onset of treatment was 11.9 years (range 9.0-14.0) and bone age delay was 1.9 'years'. The dose of oxandrolone used was a mean of 0.05 mg/kg (range 0.03-0.18) for a mean of 0.9 years (range 0.2-3.6). Height velocity increased from a mean (SD) before treatment of 4.0 (1.0) to 7.5 (1.2) cm/year with oxandrolone. Growth rate was sustained at 7.6 (2.2) cm/year in the period after treatment. Those boys who attained a testicular volume of 4 ml or greater at the end of the treatment period had the most pronounced sustained growth acceleration. Height for bone age SD score did not alter significantly from a mean of -1.0 before treatment to -1.2 after treatment. Oxandrolone is a safe and effective treatment for prepubertal boys with constitutional growth delay.
Use of duplex scanning in the diagnosis of arteria profunda femoris stenosis. To evaluate the accuracy of duplex scanning in diagnosing arteria profunda femoris stenoses in patients with concomitant superficial femoral artery occlusions, 123 femoral artery bifurcations were examined in 103 patients. Peak systolic and time-averaged maximal flow velocity parameters were measured in the arteria profunda femoris and compared with independently performed angiography. For detecting stenoses greater than 30% diameter reduction (50% by area) of the arteria profunda femoris, duplex scanning had a sensitivity of 91% and 96%, a specificity of 85% and 98%, a positive predictive value of 86% and 98%, and a negative predictive value of 91% and 96%, for a peak systolic velocity of 180 cm/sec and more, and for a time-averaged maximal velocity of 50 cm/sec and more in the arteria profunda femoris, respectively. The day-to-day variability for peak systolic and time-averaged maximal velocity parameters was low with correlation coefficients between velocity measurements on both days of 0.96 and 0.98 (n = 20), respectively. In 10 patients with arteria profunda femoris stenoses and superficial femoral artery occlusions, undergoing percutaneous transluminal angioplasty of arteria profunda femoris stenosis, the duplex scan revealed a reduction in stenotic peak systolic velocity from 330 +/- 84 to 163 +/- 50 cm/sec and a decrease in stenotic time-averaged maximal velocity from 156 +/- 47 to 54 +/- 17 cm/sec after the interventional procedure. These results show that peak systolic and time-averaged maximal velocities are accurate parameters to detect significant arteria profunda femoris stenosis in patients with superficial femoral artery occlusions.
Southern blot analysis in a case of Richter's syndrome. Evidence for a postrearrangement heavy chain gene deletion associated with the altered phenotype. Richter's syndrome (RS) can be defined as the emergence of an aggressive lymphoma in patients suffering from chronic lymphocytic leukemia (CLL). The authors performed immunophenotypic and Southern blot analysis of the peripheral blood and tissue specimen of a patient with RS. Using immunoperoxidase and immunogold-silver staining techniques and a panel of monoclonal antibodies, the authors found that the large cells characteristic of RS showed an altered immunophenotype as compared with the CLL cells and did not express mu heavy chain. Southern blot analysis revealed identical kappa light chain rearrangements in both tumoral cell populations consistent with a common clonal origin. Using the JH probe and several restriction enzymes, the authors also found evidence for a postrearrangement deletion of the heavy chain mu gene. These findings suggest that in this case of RS, a deletion of the heavy chain mu gene resulted in loss of mu expression by the larger cells that were characteristic of RS and was associated with their altered phenotype.
Arterial insufficiency in hemodialysis access procedures: correction by "banding" technique. Arterial insufficiency distal to an antecubital access is fortunately an infrequent but disturbing and often debilitating complication resulting in suboptimal access. During an 8 1/2-year period, 335 vascular access procedures were performed, 108 of which were antecubital brachiocephalic Gore-Tex conduits. Of these Gore-Tex conduits, 1.8% (2/108) had developed symptoms of vascular insufficiency 2 to 3 1/2 months after such access surgery. As previously published, arterial steal after the creation of an AV fistula could be corrected by the placement of an interposition Gore-Tex loop that would cure the problem and save the access. We have presented a procedure to correct a steal syndrome once a Gore-Tex conduit is already in place. Proximal banding and distal ligation with division of the cephalic vein below an end-to-side Gore-Tex loop is a simple surgical solution. It is not only curative but the procedure also maintains the existing Gore-Tex graft as a sustained avenue for access.
Clinical significance of the V-shaped space in the subluxed shoulder of hemiplegics. Recently, it has been proposed that shoulder subluxation in hemiplegia is accompanied by 1) the appearance of a V-shaped articular configuration occurring between the humeral head and glenoid fossa and 2) the presence of chronic pain. The main purpose of this study was to investigate the validity of these statements. We evaluated 40 hemiplegic subjects over 3 months. Radiographs of the affected and nonaffected shoulders were taken at both a frontal plane (0 degree) and a 45 degree incidence. From these patients, subluxed (n = 19) and nonsubluxed (n = 21) groups were formed. Pain was evaluated using the Present Pain Intensity index of the McGill Pain Questionnaire. On these x-ray films, measurements were taken of the V-shaped space, abduction of the arm, and rotation of the scapula. The statistical analysis (analysis of variance for repeated measures) contrasted the results obtained from the nonaffected side with those from the affected side over the 3 months studied. At the 45 degree angle, which better exposes the articular configuration of the shoulder, the difference in the V angle between the affected and nonaffected shoulders was significant for the subluxed group (p less than 0.01), indicating that such a V-shaped space can be identified. The measures taken also indicate that a downward subluxation of the humeral head occurs relative to the scapula without any systematic abduction of the humerus or downward rotation of the scapula. None of the results obtained from the frontal plane x-ray films was significant. Finally, no significant relation was found between subluxation and shoulder pain.
High-dose medroxyprogesterone acetate versus estramustine in therapy-resistant prostatic cancer: a randomised study. A series of 105 patients with metastatic prostatic cancer, having progressed on first-line hormonal treatment, were randomised to high-dose medroxyprogesterone acetate (MPA) 1000 mg i.m. daily for 15 days, followed by 1000 mg weekly (53 patients), or to estramustine 280 mg per os twice daily (52 patients). The treatment was discontinued because of side effects in 3 of 51 evaluable MPA-treated patients and in 8 of 51 evaluable estramustine-treated patients. Progression-free survival was short in both groups and no statistically significant difference between them was observed. After 1 year, 70% of the patients had died and there was no statistically significant difference between the 2 treatments in the cumulative observed survival rates. According to modified SPCG criteria, remissions lasting from 12 to 56 weeks were noted in 13 MPA-treated patients and in 4 estramustine-treated patients. This difference was statistically significant. After cross-over, 6 of 33 patients in the MPA group had a remission compared with 1 of 24 in the estramustine group. It was concluded that the response rate, considering both subjective and objective response criteria, was better with MPA and the side effects were fewer.