123rc/medical_text huggingface dataset:
Right-to-left shunting through a patent foramen ovale caused by pulmonary hypertension associated with rheumatoid arthritis and Sjogren's syndrome: a case report. This case report presents a fifty-four-year-old woman with right-to-left shunt in the atrium. It seemed clinically at first that the shunt was due to atrial septal defect. However, she also had pulmonary disease associated with rheumatoid arthritis and Sjogren's syndrome. At autopsy atrial septal defect was not evident, but a patent foramen ovale and pulmonary artery disease were observed. This case suggests that pulmonary hypertension secondary to rheumatoid arthritis and Sjogren's syndrome could lead to right-to-left shunting through a patent foramen ovale.
Defective dystrophin in Duchenne and Becker dystrophy myotubes in cell culture. We examined normal and dystrophic human myotubes in cell culture for expression of dystrophin, the protein product of the Duchenne muscular dystrophy locus. Dystrophin levels in developing myotubes detected by Western blotting increased after 24 hours and reached maximum levels after 10 days in fusion medium. We did not detect dystrophin in myotubes cultured from Duchenne myoblasts (7 cases). Myotubes from a Becker muscular dystrophy patient's biopsy produced a lower molecular weight (approximately 408 kd) dystrophin, which was the same size in a whole muscle preparation from the same biopsy. This 408-kd dystrophin was the expected size for this Becker patient whose DNA was deleted for exons 45-48 of the Duchenne gene. This cell culture system will allow a detailed analysis of the effects of potential pharmacologic agents on steady-state dystrophin levels.
The use of fibrin sealant to reduce blood loss during Cotrel-Dubousset instrumentation for idiopathic scoliosis. The increased complexity of Cotrel-Dubousset instrumentation has, as an unexpected side effect, a potential increase in blood loss. A prospective randomized study was undertaken to test the hypothesis that application of a fibrin sealant to exposed cancellous bone can significantly reduce blood loss during Cotrel-Dubousset instrumentation for idiopathic scoliosis. A significant difference was demonstrated in total body loss, loss per level fused, and loss per kilogram of body weight when comparing the sealant group with random controls. A significant difference was demonstrated in loss per level fused when comparing the sealant control with a historical control group. Thirty-three patients were randomly assigned to the fibrin sealant or nonsealant groups; another 10 patients operated on before planning the study were included as historical controls. The sealant was used to control bleeding at the bone graft donor site and in the spine after decortication. All patients underwent Cotrel-Dubousset instrumentation for idiopathic scoliosis. There were no significant differences between groups with respect to degree of curvature, number of levels fused, age or weight of patient, or operating time. Total blood loss in the sealant group averaged 672 ml compared with 894 ml in the sealant control group. No patient in the sealant group required homologous blood. Given the increasing awareness of the complications of blood transfusion, the authors conclude that fibrin sealant is a useful adjunct to spinal surgical technique.
Temporal arteritis presenting as a submandibular swelling. Temporal arteritis (giant cell arteritis) is a disease of protean manifestation. A case which presented as a submandibular swelling is reported.
Lack of bone accretion and amenorrhea: evidence for a relative osteopenia in weight-bearing bones. Bone mineral density (BMD) was studied in young exercising amenorrheic girls to determine if density was compromised and the change related to injury. Ninety-eight volunteers (professional ballet dancers and controls) were studied in a cross-sectional study. Dancers and controls were further subdivided into normally cycling and amenorrheic subjects. Amenorrhea significantly lowered bone density of the spine (P less than 0.0001), wrist (P less than 0.03), and metatarsal (P less than 0.01); effects on the wrist were eliminated by controlling for age while controlling for weight eliminated all effects of amenorrhea at three sites. BMD of the metatarsal, a weight-bearing bone, showed an interaction between amenorrhea and dancing (exercising) P less than 0.035); surprisingly, dancing was associated with a further lowering of bone density. This interaction was eliminated when controlling for age, but not when controlling for weight. With multiple comparisons of the groups, spine, wrist, and metatarsal bone density was significantly lower in amenorrheic dancers when compared to normal dancers (P less than 0.05), even when controlling for age and weight in the metatarsal (P less than 0.05), and age in the spine (P less than 0.05). Estradiol levels correlated with bone density of both the wrist and the spine (r = 0.25, r = 0.23, P less than 0.02). Metatarsal density correlated with estradiol levels only in the dancers (r = 0.34, P less than 0.02). The only variable found to correlate with the occurrence of stress fractures was age of menarche. This was also the only variable of 9 (BMD of the wrist, spine or foot, calories ingested and expended, amount of calcium ingested, involvement in high energy activity, age of menarche or presence of amenorrhea) to predict stress fractures. Thus, BMD is significantly affected by the presence of amenorrhea but the effects are generally weight dependent. The compensatory increase in bone density generally seen in stressed bones, such as the metatarsal in ballet dancers, is deficient in amenorrheic premenopausal women even when controlling for weight but this effect may be age and estrogen dependent. Bone mass may not accumulate in the same manner in adolescents as in the mature women, thus putting them at risk for injury.
Node-negative breast cancer: prognostic subgroups defined by tumor size and flow cytometry Adjuvant systemic therapy for women with node-negative breast cancer is most easily justified for those patients at highest risk of relapse. We have examined the impact of tumor size, histologic grade, estrogen receptor (ER) status, tumor ploidy, and S-phase fraction (SPF) on relapse-free survival (RFS) for 169 patients with node-negative breast cancer in order to identify groups of patients at high and low risk of relapse. Patients with small tumors (less than or equal to 1.0 cm) had a significantly better RFS than those with larger tumors (P = .005), with 96% remaining relapse-free at 5 years. Patients with tumors less than or equal to 1.0 cm were thus excluded from analysis when attempting to define a group with a poor prognosis. Within the group of patients with tumors greater than 1.0 cm, tumor ploidy (P = .63), ER status (P = .3), or progesterone receptor (PgR) status (P = .24) did not predict for RFS. Patients with grade 1 or 2 infiltrating ductal tumors had a significantly better prognosis than those with grade 3 tumors (P = .04). The prognostic factor that gave the widest separation between subgroups, however, was SPF. Patients whose tumors were greater than 1.0 cm with an SPF less than or equal to 10% had a 5-year RFS of 78% compared with a 5-year RFS of 52% for those with an SPF greater than 10% (P = .006). We have combined tumor size and SPF to identify three prognostic groups: (1) tumor less than or equal to 1.0 cm, 5-year RFS 96%; (2) tumor greater than 1.0 cm plus SPF less than or equal to 10%, 5-year RFS 78%; 3) tumor greater than 1.0 cm plus SPF greater than 10%, 5-year RFS 52%. These prognostic groupings may help identify patients most suitable for adjuvant therapy.
Primary bone tumours of the thoracic skeleton: an audit of the Leeds regional bone tumour registry. An audit of the Leeds regional bone tumour registry found that primary bone tumours of the thoracic skeleton constituted 90 of the 2004 cases (4.5%). Thirty seven per cent occurred in the ribs, 32% in the scapulae, 11% in the thoracic vertebrae, 11% in the sternum, and 9% in the clavicles. Malignant tumours were more common than benign (54 v 36) and occurred in an older population (mean ages 47 and 31 years). The scapula was the most common site for malignant lesions and the ribs the most common site for benign tumours. Chondrosarcoma was the commonest tumour in older patients, fibrous dysplasia and plasmacytoma in the middle age group, and eosinophilic granuloma in children. Presenting symptoms were a poor guide to whether the lesion was malignant or not. This and the small proportion of correct preoperative diagnoses indicate the need for early biopsy. Bone tumour registries provide a valuable source of cumulative information about uncommon tumours and facilitate accurate diagnosis, teaching, and research.
Macrophages stimulate bone resorption when they phagocytose particles. We investigated in vitro a mechanism by which particulate debris may induce bone resorption and cause implant loosening. We first studied two standard particles: latex, which is considered to be inert, and zymosan, which is inflammatory. Macrophages that phagocytosed either particle became activated, and stimulated 15 times as much bone resorption as did control macrophages. For activation to occur, 100 times more latex than zymosan had to be phagocytosed. We also found that bone cement and polyethylene particles activated macrophages in a similar manner, and that the necessary amounts of these were intermediate between those of latex and zymosan. None of the particles were toxic. It was concluded that implant loosening may result from bone resorption stimulated by mediators released by macrophages that have phagocytosed particles of bone cement or polyethylene.
Hepatic fibrin-ring granulomas in a patient with hepatitis A. Hepatic fibrin-ring granulomas were found in a 30-year-old patient with serologically confirmed hepatitis A. Other causes associated with the presence of fibrin-ring granulomas in the liver, such as Hodgkin's and non-Hodgkin's lymphoma, cytomegalovirus infection, visceral leishmaniasis, and consumption of allopurinol, were ruled out. It is suggested that hepatitis A must be included in the differential diagnosis of a patient with hepatic fibrin-ring granulomas.
Radiation-associated valvular disease. The prevalence of radiation-associated cardiac disease is increasing due to prolonged survival following mediastinal irradiation. Side effects of radiation include pericarditis, accelerated coronary artery disease, myocardial fibrosis and valvular injury. We evaluated the cases of three young patients with evidence of significant valvular disease following mediastinal irradiation. One patient underwent the first reported successful aortic and mitral valve replacement for radiation-associated valvular disease (RAVD) as well as concurrent coronary artery revascularization. A review of the literature revealed 35 reported cases of RAVD, with only one successful case of valve replacement that was limited to the aortic valve. Asymptomatic RAVD is diagnosed 11.5 years after mediastinal irradiation compared with 16.5 years for symptomatic patients, emphasizing that long-term follow-up is important for patients receiving mediastinal irradiation. This study defines a continuum of valvular disease following radiation that begins with mild asymptomatic valvular thickening and progresses to severe valvular fibrosis with hemodynamic compromise requiring surgical intervention.
Signs, complications, and platelet aggregation in familial exudative vitreoretinopathy. Between 1979 and 1989, I examined 106 patients (16 pedigrees) with signs of familial exudative vitreoretinopathy. Of these patients, 101 had familial exudative vitreoretinopathy, and five had a sporadic manifestation. The complications of familial exudative vitreoretinopathy, deformation of the posterior retina, vitreous hemorrhage, amblyopia, and retinal detachment, caused diminished visual acuity. Of 170 eyes, retinal neovascularization was observed in 18 eyes (11%), and retinal exudates were observed in 16 eyes (9%). Several forms of retinal detachment occurred in 37 of 180 eyes (21%), which often took an unfavorable course. A falciform retinal fold was observed in 14 eyes (8%). Retinal surgery was performed in 14 eyes; reattachment of the retina was successful in only seven eyes. Platelet aggregation studies disclosed no significant differences between seven patients with familial exudative vitreoretinopathy and ten control subjects. The pathogenesis of the disease is based on a premature arrest of the vascular development of the retina.
Endothelial-derived relaxing factor released by endothelial cells of human umbilical vessels and its impairment in pregnancy-induced hypertension. The principal objective of this study was to determine and compare the capability of human umbilical vessels of normal and pregnancy-induced hypertensive parturients to produce and to release the endothelial-derived relaxing factor. A bolus of bradykinin injected in the perfusion system of human umbilical vessels induces a release of a relaxant factor, detectable by bioassay, that is pharmacologically similar to the endothelial-derived relaxing factor. Human umbilical cords were collected from normal and pregnancy-induced hypertensive parturients. In the latter group the release of endothelial-derived relaxing factor is extremely reduced. In fact, in umbilical vessels collected from normal parturients, bradykinin at a dose of 20 pmol produces a release of endothelial-derived relaxing factor equivalent to a relaxation induced by 59.9 +/- 11.0 and 30.8 +/- 11.4 pmol of glyceryl trinitrate for the artery and vein, respectively. The same dose of bradykinin in umbilical vessels, collected from pregnancy-induced hypertensive parturients, produces a release equivalent to 6.6 +/- 2.2 and 5.7 +/- 3.5 pmol of glyceryl trinitrate equivalent for the artery and vein, respectively. Neither an increasing bolus of exogenous bradykinin or an infusion of superoxide dismutase or L-arginine was able to restore the production of endothelial-derived relaxing factor to normal levels. Our results indicate a probable alteration of endothelial cell numbers or an alteration of the enzymatic pathway, probably due to cytotoxic endogenous factors produced in pregnancy.
Left recurrent laryngeal nerve paralysis: an unusual presentation of histoplasmosis. A case of histoplasmosis seen as left recurrent laryngeal nerve paralysis in a 49-year-old man is described. The patient had roentgenographic findings of a solitary, noncalcified left upper lobe mass and mediastinal adenopathy. Tissue diagnosis of histoplasmosis was obtained using a thoracotomy, allowing institution of appropriate treatment.
Digitalis-like activity in human plasma: relation to blood pressure and sodium balance. PURPOSE: On the assumption that renal tubular cells are more important as the target cells for a natriuretic factor than blood cells, we used a well-characterized cultured renal tubular cell line, Madin-Darby canine kidney (MDCK), cells to monitor the circulating digitalis-like factor in human plasma and examine its role in the regulation of blood pressure and sodium balance. SUBJECTS AND METHODS: We investigated the effects of plasma on binding of radioactive ouabain to monolayered MDCK cells in order to determine the level of a circulating digitalis-like factor. First, we measured specific 3H-ouabain binding to MDCK cells in the presence of plasma from 71 outpatients (34 normotensive subjects and 37 hypertensive patients) after incubation for 4 hours. Second, we measured specific 3H-ouabain binding after incubation of cells with plasma from 16 hospitalized subjects (eight normotensive subjects and eight hypertensive patients) receiving low and high sodium diets. RESULTS: In Study 1, ouabain binding was lower by 30% with plasma from hypertensive patients than with plasma from normotensive subjects (p less than 0.01). There was a significant negative correlation between individual subject's systolic or mean blood pressure and ouabain binding (r = -0.34, p less than 0.01 or r = -0.29, p less than 0.01). In Study 2, ouabain binding was also significantly reduced by 25% in the presence of plasma from hypertensive subjects as compared with plasma from normotensive subjects irrespective of sodium intake (p less than 0.01). A significant negative correlation was also found for all subjects between either systolic, diastolic, or mean blood pressure and ouabain binding (r = -0.58, p less than 0.01, r = -0.51, p less than 0.01, or r = -0.55, p less than 0.01, respectively). With the changes from low to high sodium intake, there was a corresponding decrease in ouabain binding (p less than 0.01) and an increase in sodium excretion (p less than 0.01). A significant negative correlation was observed between these two parameters (r = -0.47, p less than 0.05). CONCLUSIONS: These findings suggest that a circulating digitalis-like factor, which may act on renal tubular cells as the ouabain-displacing compound, is increased in patients with essential hypertension and also demonstrate that plasma levels may be influenced by changes in dietary sodium intake.
Catheterization laboratory events and hospital outcome with direct angioplasty for acute myocardial infarction To assess the safety of direct infarct angioplasty without antecedent thrombolytic therapy, catheterization laboratory and hospital events were assessed in consecutively treated patients with infarctions involving the left anterior descending (n = 100 patients), right (n = 100), and circumflex (n = 50) coronary arteries. The groups of patients were similar for age (left anterior descending coronary artery, 59 years; right coronary artery, 58 years; circumflex coronary artery, 62 years), patients with multivessel disease (left anterior descending coronary artery, 55%; right coronary artery, 55%; circumflex coronary artery, 64%), and patients with initial grade 0/1 antegrade flow (left anterior descending coronary artery, 79%; right coronary artery, 84%; circumflex coronary artery, 90%). Cardiogenic shock was present in eight patients with infarction of the left anterior descending coronary artery, four with infarction of the right coronary artery, and four with infarction of the circumflex coronary artery. Major catheterization laboratory events (cardioversion, cardiopulmonary resuscitation, dopamine or intra-aortic balloon pump support for hypotension, and urgent surgery) occurred in 10 patients with infarction of the left anterior descending coronary artery, eight with infarction of the right coronary artery, and four with infarction of the circumflex coronary artery (16 of 16 shock and six of 234 nonshock patients, p less than 0.001). There was one in-laboratory death (shock patient with infarction of the left anterior descending coronary artery).
Neuroendocrine carcinoma of the ampulla of vater. A case of absence of somatostatin in a vasoactive intestinal polypeptide-, bombesin-, and cholecystokinin-producing tumor. A 31-year-old patient with a clinical picture of obstructive jaundice had surgical treatment, and a primary carcinoid of the ampulla of Vater (VA) was found. The tumor was studied with light microscopy, immunohistochemistry, and electron microscopy. The neoplasm had histopathologic and cytopathologic features similar to those encountered in typical neuroendocrine neoplasms. It is interesting that immunohistochemical techniques disclosed the presence of vasointestinal polypeptide, cholecystokinin, and bombesin; however, unlike most neuroendocrine neoplasms arising in VA, no somatostatin-immunoreactive cells were found.
US-guided fine-needle aspiration biopsy of gallbladder masses. Ultrasonically (US) guided fine-needle aspiration biopsy was performed in 88 patients who had gallbladder masses. All masses were less than 4.8 cm in diameter. A 22- or 23-gauge, Teflon-coated needle was placed into the mass with the transhepatic or transperitoneal route. By means of this technique, gallbladder malignancy was confirmed in 69 of the 78 cases of malignancy (88.5%). Ten of 10 benign lesions were properly categorized. One patient developed bile peritonitis following a single needle pass with the transperitoneal approach. Pain, a minor complication of the procedure, was noted in four cases. No instances of hemorrhage or vasovagal reactions occurred. From their experience in this large series, the authors conclude that US-guided fine-needle aspiration biopsy of gallbladder masses is a safe, reliable, and accurate technique for the diagnosis of malignancy.
Frequency of three Hex A mutant alleles among Jewish and non-Jewish carriers identified in a Tay-Sachs screening program. Mutations in the HEX A gene, encoding the alpha-subunit of beta-hexosaminidase A (Hex A), are the cause of Tay-Sachs disease as well as of juvenile, chronic, and adult GM2 gangliosidoses. We have examined the distribution of three mutations--a 4-nucleotide insertion in exon 11, a G----C transversion at a 5' splice site in intron 12, and a 269Gly----Ser amino acid substitution in exon 7--among individuals enzymatically diagnosed as carriers of Hex A deficiency. Mutation analysis included polymerase chain reaction (PCR) amplification of the relevant regions of genomic DNA, followed by allele-specific oligonucleotide hybridization; another test for heterozygosity of the exon 11 insertion was based on the formation of heteroduplex PCR fragments of low electrophoretic mobility. The percentage distribution of the exon 11, intron 12, exon 7, and unidentified mutant alleles was 73:15:4:8 among 156 Jewish carriers of Hex A deficiency and 16:0:3:81 among 51 non-Jewish carriers. Regardless of the mutation, the ancestral origin of the Jewish carriers was primarily eastern and (somewhat less often) central Europe, whereas for the non-Jewish carriers it was western Europe. Because a twelfth of the Jewish carriers and four-fifths of the non-Jewish carriers of Hex A deficiency had mutant alleles other than the three common ones tested, enzyme-based tests cannot be replaced by DNA-based tests at the present time. However, DNA-based tests for two-carrier couples could identify those at risk for the chronic/adult GM2 gangliosidoses rather than for infantile Tay-Sachs disease.
Flow cytometric analysis of terminal deoxynucleotidyl transferase. Terminal deoxynucleotidyl transferase (TdT) is a useful marker for lymphoid precursor cells. In this study, the authors used flow cytometry (FCM) to analyze TdT expression in human hematopoietic malignancies. Cells were fixed in 0.5% formaldehyde, briefly exposed to nonionic detergent, and subsequently labeled with mouse monoclonal anti-TdT antibodies followed by fluorescein-conjugated antimouse IgG and propidium iodide (PI), which was used for the simultaneous analysis of DNA content. Cells from 20 of 22 acute lymphoblastic leukemias (ALL), 4 of 7 mixed lineage leukemias, 2 of 21 acute myeloid and myelomonocytic leukemias, 1 of 2 chronic myeloid leukemias in blast crisis, 1 lymphoblastic lymphoma, and 1 thymoma were TdT positive. Cells from 13 nonlymphoblastic lymphomas, 3 myelodysplastic bone marrows, and peripheral blood mononuclear cells from 29 normal individuals were negative. An excellent correlation was seen between this assay, the conventional slide immunofluorescence technique, and an enzyme immunoassay method. The FCM assay detected as few as 2% blasts in mixing experiments of TdT-containing leukemic cells with normal peripheral lymphocytes. Furthermore, the combined analysis of TdT and DNA allowed the recognition of aneuploid TdT positive cells in four cases of ALL. The high sensitivity, the quantitative information obtained, and the capability of simultaneously analyzing DNA content make this method of TdT analysis more valuable than conventional techniques.