shukoor65/lab-rag-model huggingface dataset:
Expression of embryonic globins by erythroid cells in juvenile chronic myelocytic leukemia. Juvenile chronic myelocytic leukemia (JCML) is a rare hematopoietic neoplasia of early childhood with distinct hematologic and biochemical features. We studied the biologic properties and the globin synthetic profiles of JCML erythroid cells both in vivo and in vitro from a total of 24 patients. In these cases we observed the exuberant colony-forming unit-macrophage (CFU-M) colony growth, as reported previously. Furthermore, in contrast to previous reports, we found significant erythroid colony growth in most of our cases (average: 1,182 burst-forming unit-erythroid [BFUe] per 10(5) plated cells, range: 40 to 6,927). This growth was by and large erythropoietin-dependent and was not greatly influenced by other added cytokines. By several criteria all erythroid colony growth detected in vitro was derived from JCML progenitors. The globin synthetic profile of JCML erythroid cells showed high levels of fetal hemoglobin both in vivo and in vitro (gamma/gamma + beta: 53% to 94% in reticulocytes, 62% to 98% in BFUe-derived cells). In addition (in seven cases studied) we detected embryonic globins (epsilon and zeta) at the protein and messenger RNA level, a novel finding for primary leukemic cells. We speculate that the transformed erythroid cells in JCML harbor a trans environment supporting expression of developmentally earlier genes (fetal, embryonic). However, in contrast to other acute or subacute leukemias, JCML erythroid cells also have the ability to reach full maturation to the red cell level, thus allowing detection of this primitive program in vivo.
Prognostic factors in the conservative treatment of ureteric stones. A prospective study was made of 125 ureteric stones in order to detect factors which would predict the successful outcome of conservative treatment. The factors studied were the duration of pain prior to presentation, pyuria, haematuria, surface regularity of the stone and the degree of obstruction as seen radiologically. In stones less than or equal to 10 mm in size, conservative treatment was successful when the duration of pain was less than 30 days (39/45), when there was no significant pyuria (53/82), when the stones had an irregular surface (44/65) and when obstruction was only partial (55/88). Since most of these factors were interactive, further statistical analysis showed that duration of pain was the only significant factor in predicting the outcome of conservative treatment. In stones greater than 10 mm in size these factors had no predictive value and only 2/23 were passed spontaneously on conservative treatment.
Shoulder subluxation in hemiplegia: effects of three different supports. Shoulder subluxation in hemiplegia is a difficult problem to manage and it may be associated with pain and other complications. Measurements taken from x-rays have been used to obtain objective measures of shoulder subluxation, but have not been used to compare the effects of different shoulder supports. This study used x-ray measurements to evaluate different shoulder supports for subluxation in hemiplegia and to see if there was a significant difference between the Harris hemisling and the Bobath sling. The Harris hemisling gave good vertical correction of subluxation and compared closely to the uninvolved shoulder. The Bobath sling did not correct the subluxation as well, and the mean difference between the two slings was significant. The arm trough or lap board was less effective and tended to overcorrect. The Harris hemisling and arm trough or lap board had horizontal measurements similar to the uninvolved shoulders. The Bobath sling, however, distracted the glenohumeral joint horizontally and was more variable. The mean horizontal difference between the Harris hemisling and the Bobath sling was significant. These results support the effectiveness and specificity of shoulder support to decrease subluxation after hemiplegia.
Spinal muscular atrophy is not the result of mutations at the beta-hexosaminidase or GM2-activator locus. The disease locus for the clinically heterogeneous childhood spinal muscular atrophies (SMA) maps to the chromosome 5 subregion, 5q11.2-13.3. The beta-subunit of beta-D-N-acetylhexosaminidase (hexosaminidase) (EC 3.2.1.52) (Hex B) maps to the same region, and the protein required for substrate recognition by this enzyme, GM2-activator protein, likewise maps to chromosome 5. We have investigated the possibility of allelic variation among some forms of SMA and hexosaminidase deficiency. Recombination between the Hex B and SMA loci eliminates this enzyme as a candidate site for defects causing the illness. Furthermore, we show that, despite previous evidence to the contrary, the GM2-activator locus does not map to chromosome 5, thereby eliminating it as a candidate gene for SMA.
Preoperative hemostatic activity and excessive bleeding after cardiopulmonary bypass. The rationale for predicting the risk of excessive postoperative bleeding by assessing the hemostatic status of a patient before cardiopulmonary bypass was investigated. A novel, rapid, overall test (hemostatometry) consisting of a physiologically relevant test of platelet function (shear-induced hemostasis) and coagulation was performed using nonanticoagulated blood and compared with the routine coagulation screen. Two hundred five patients undergoing elective coronary revascularization were studied 3 to 4 days before operation. Forty-nine bled excessively for nonsurgical reasons; none were predicted by the routine coagulation tests. Using a stepwise discriminant analysis, hemostatometry correctly predicted 31 of 49 (63%). Thirty of 156 predicted as bleeders by hemostatometry did not bleed. Thus, preoperative hemostatometry predicted 77% of the true outcome. The false predictions suggest, however, that certain bleeding abnormalities probably acquired during cardiopulmonary bypass cannot be predicted. These findings do not justify the routine use of preoperative tests in assessing the bleeding risk in patients undergoing cardiopulmonary bypass.
Use of hydroxyurea in chronic myeloid leukemia during pregnancy: a case report. The concomitant occurrence of pregnancy and chronic myeloid leukemia is uncommon. The use of hydroxyurea in chronic myeloid leukemia during pregnancy is unknown. We report on a patient with chronic myeloid leukemia in whom hydroxyurea was used during pregnancy with a successful outcome for both mother and fetus.
Evidence for the segregation of a major gene in human susceptibility/resistance to infection by Schistosoma mansoni. Severe clinical disease caused by the major human parasite Schistosoma mansoni is the consequence of high and prolonged infections. Epidemiological studies indicate that, for individuals having frequent contacts with cercaria-infested waters, both infection intensities and reinfection after treatment depend, in large part, on their intrinsic susceptibility/resistance to infection, suggesting the role of genetic factors in human resistance to S. mansoni. To investigate whether a major gene controls human susceptibility/resistance to infection by S. mansoni, segregation analysis of infection intensities, adjusted for the factors relevant in schistosomiasis (water contact, age, sex), was performed on 20 Brazilian pedigrees (269 individuals), using both the unified mixed model and the regressive model of analysis. The results are consistent with the hypothesis that there is a codominant major gene controlling human susceptibility/resistance to infection by S. mansoni. Parameter estimates indicate a frequency of .20-.25 for the deleterious allele; thus, about 5% of the population is predisposed to high infections, 60% is resistant, and 35% has an intermediate, although fairly good, level of resistance. These findings provide a genetic basis for earlier observations on the lower resistance and the predisposition to reinfection of certain individuals. In addition to the detection of a major gene effect, the data suggest that immunity to S. mansoni develops progressively during childhood to reach a maximum around the age of puberty. The implications of these results for the strategy to be used in endemic areas to reduce morbidity and to control parasite transmission are discussed.
Effect of NG-monomethyl L-arginine on endothelium-dependent relaxation in arterioles of one-kidney, one clip hypertensive rats. Dose-response curves to topically applied acetylcholine, bradykinin, and nitroprusside were obtained by intravital microscopy in arcading arterioles of the spinotrapezius muscle of control (n = 9) and one-kidney, one clip hypertensive (1K1C) rats (n = 11) of 4 weeks' duration before and during superfusion with the specific inhibitor of nitric oxide formation NG-monomethyl L-arginine (LNMMA) (10(-4) M) and both LNMMA (10(-4) M) and indomethacin (2.8 x 10(-5) M). Resting arteriolar tone was higher in 1K1C rats than in controls, and vasodilation to acetylcholine and bradykinin, but not to nitroprusside, was reduced (p less than 0.05) in 1K1C rats compared with controls. LNMMA increased arteriolar tone (p less than 0.05) and inhibited the vasodilator responses to acetylcholine and bradykinin (p less than 0.05) in controls but not in 1K1C rats. LNMMA did not alter the response to nitroprusside in either group. Addition of indomethacin to LNMMA increased arteriolar tone and markedly reduced the response to bradykinin, but not to acetylcholine or nitroprusside, in both groups. These findings suggest that resting arteriolar tone is increased in 1K1C rats partially because of the decreased basal release or synthesis of nitric oxide. Responses to the endothelium-dependent vasodilators acetylcholine and bradykinin were attenuated in 1K1C rats, possibly because of changes in synthesis or release of nitric oxide for acetylcholine and of prostacyclin for bradykinin, because the response to the endothelium-independent vasodilator nitroprusside did not differ between the groups.
Mastoid surgery at the Red Cross War Memorial Children's Hospital 1986-1988. Eighty-three children between ages 0-12 years had mastoid surgery in the three year period 1986-88. The indications were acute mastoiditis (30 children), uncontrolled chronic suppurative otitis media (CSOM) (24 children), cholesteatoma (22 children) and a miscellaneous group (7 children). Forty-seven per cent of those with acute mastoiditis were under one year but the 27 per cent with cholesteatoma as the precipitating cause were 4-12 years. Twenty-three per cent had significant complications. Typmano-mastoid surgery was employed to resolve uncontrolled CSOM. Children were between 2-12 years and 62 per cent were below the 25th centile for weight. Seventy-nine per cent of the tympanic membrane grafts were successful. The children with cholesteatoma were between 3-12 years and 76 per cent were below the 25th centile for weight. Eight of them only presented after the onset of acute mastoiditis. One child had a definite congenital cholesteatoma. Only one had a pars flaccida origin of the cholesteatoma sac and only eight an origin from the postero-superior pars tensa. All had open cavity surgery. When the cavity was lined by temporalis fascia, healing was improved markedly.
Adrenal pheochromocytoma presenting with persistent abdominal and flank pain. Pheochromocytoma is classically associated with paroxysms of headache and hypertension. Its presentation, however, may be more varied and subtle. We present a case of an adolescent female who complained of right upper quadrant and flank pain. A right adrenal pheochromocytoma was diagnosed and subsequently removed. The pertinent literature is discussed.
Significance of anti-phospholipid antibodies in patients with lupus nephritis. Anti-phospholipid antibodies (APA) as markers or mediators of thrombosis in lupus could be of pathogenetic significance in nephritis, since glomerular capillary thrombi are an indicator of subsequent renal dysfunction. Isotype specific APA antibodies were measured, using cardiolipin as the antigen, in 76 patients with lupus nephritis. Twenty-nine percent of the patients had elevated IgG APA. Overall, 43% of patients showed raised levels of at least one isotype. In general, APA had specificity for anionic phospholipids. In vitro lupus anticoagulant activity was associated with all three isotypes of APA, but only the IgM isotype correlated with the biological false positive test for syphilis. APA were not associated with thromboses or neurological involvement, and only the IgA isotype correlated with thrombocytopenia. We confirmed an association between the presence of intraglomerular thrombi and serum IgG APA. However, we found no association between APA and renal histological pattern, or long-term renal function. Our data, therefore, do not support a major pathogenetic role for APA in the nephritis of lupus in treated patients.
Submaximal exercise thallium-201 SPECT for assessment of interventional therapy in patients with acute myocardial infarction. Submaximal thallium-201 stress testing has been shown to provide important diagnostic and prognostic information in patients with acute myocardial infarction. The purpose of this investigation was to evaluate the diagnostic value of early submaximal stress testing and thallium-201 single photon emission computed tomography (SPECT) after interventional therapy. Scintigraphic results from 56 patients with infarctions, who underwent acute thrombolytic therapy, angioplasty, or both, were compared with late (6 weeks) functional outcome as assessed by radionuclide ventriculography and with results of discharge coronary angiography. A linear correlation was found between the extent of thallium-201 SPECT perfusion defect and late ventricular function (r = 0.74, p less than 0.01). Forty-two percent of patients with large SPECT perfusion defects had normal left ventricular ejection fractions, suggesting an overestimation of infarct size by early imaging. Sensitivity and specificity of thallium-201 SPECT for detection of coronary artery stenosis in noninfarct territories was 57% and 46%, respectively, indicating limited diagnostic definition of extent of underlying coronary artery disease. Results of follow-up coronary angiography showed a significant relationship between the size of the initial perfusion defect and early restenosis or reocclusion of the infarct artery. Thus the extent of early thallium-201 perfusion defects correlates with late functional outcome but appears to overestimate the degree of injury. Submaximal thallium-201 stress testing allows only limited characterization of underlying coronary artery disease. Early assessment of infarct size may identify a patient population at high risk for reocclusion of the infarct artery.
Inverted colonic diverticulum: air contrast barium enema findings in six cases. A polypoid elevation of the colonic wall was identified on air contrast barium enema in six patients. The abnormality in each case was found to be an inverted colonic diverticulum. Inverted diverticula appeared as broad-based, smooth, sessile polyps measuring 1.5-2.0 cm. In five of the six patients a characteristic central umbilication and/or evidence of barium within the polyp could be identified. The diagnosis was confirmed in three patients by demonstrating an everted diverticulum replacing the suspected lesion. In the remaining three patients, two of whom had normal findings on colonoscopy, the diagnosis was strongly suggested by the presence of barium extending into the substance of the mass. Additional radiologic maneuvers or a second study may permit accurate diagnosis if the possibility of inverted diverticulum is considered. However, even when the diverticulum cannot be demonstrated, a central umbilication or barium within a smooth intraluminal projection should suggest inverted diverticulum and prevent unnecessary endoscopy or inadvertent diverticulectomy.
Persistent polyclonal B lymphocytosis with Epstein-Barr virus antibodies and subsequent malignant pulmonary blastoma. Persistent polyclonal B lymphocytosis (PPBL), a rare benign lymphoproliferative disorder, occurred in a 25 year old woman. The lymphocytes showed the phenotype cIgM+, FMC7+, CD19+, CD20+, which was similar to that seen in Waldenstrom's macroglobinaemia, but the proliferation was repeatedly shown to be polyclonal both immunologically and by immunoglobulin gene rearrangement studies. Eleven years after presentation the patient developed a malignant pulmonary blastoma, a rare pulmonary tumour of combined epithelial and mesenchymal origin. The failure to develop a lymphoid malignancy over 11 years together with immunological and genetic evidence of polyclonality confirms that PPBL is a benign lymphoproliferative disorder. The subsequent occurrence of a nonhaemopoietic malignancy suggests that benign lymphoid proliferations, like their malignant counterparts, may predispose to the development of neoplasia. A role for Epstein-Barr virus in the pathogenesis of one or both of these rare conditions is suggested by the finding of raised Epstein-Barr viral capsid antigen titres.
Hypertension, cardiac disease, and compliance in minority patients. In minorities, as in the general population, hypertension is taken seriously because it is a risk factor for cardiovascular disease. Until recently, our understanding of the role that hypertension plays in the heart disease seen in minorities has been limited by a paucity of prospective data regarding the prevalence, natural history, and pathophysiology of the disease process in minority populations. In the last few years large-scale epidemiologic studies and well-controlled clinical studies alike have confirmed usually high rates of hypertension-related morbidity and mortality in minorities, particularly blacks and Hispanics. The severity of end-organ damage, both cardiac and renal, that is seen in these patients--especially when coupled with the severe cerebrovascular damage that is also more common in black and Hispanic hypertensives--mandate that more effective public health measures be taken to reduce the incidence of hypertension in these patient populations. Because hypertension is usually without significant clinical symptoms, noncompliance with drug therapy and high dropout rates are common in all patient populations. They are strikingly higher in inner-city populations, however, where illiteracy, poverty, homelessness, and high rates of chemical dependency combine to exacerbate an already serious problem in treating hypertensive patients. Inner-city patients are, increasingly, black and Hispanic patients, and these patients are more likely to be underinsured or uninsured, to be functionally illiterate in English, to be disinclined to seek health care, and to be less capable of following a prescribed regimen than the populace as a whole. The nature of the therapeutic regimen itself is probably the most important determinant of compliance, and compliance with drug therapy will be improved if the clinic chooses a simplified drug regimen and avoids drugs that produce intolerable side effects. Once-a-day--or, with transdermal clonidine, one-a-week--single-drug therapy may not be possible in all patients, but multiple drug therapy and multiple daily dosing schedules should be avoided wherever possible.
Fetal death associated with measles virus infection of the placenta. A case of fetal death at 25 weeks' gestation, which was associated with maternal measles infection, is described. Immunohistochemical study revealed measles virus antigen in the syncytial trophoblastic cells and decidua but not in the fetus. Results suggest that fetal death was caused by measles virus infection in the placenta. As far as we know, this is the first report in which a measles virus antigen was detected in the placenta by the immunohistochemical method.
Effect of mitral regurgitation on pulmonary venous velocities derived from transesophageal echocardiography color-guided pulsed Doppler imaging. The effect of mitral regurgitation on pulmonary venous flow velocity was studied in 66 patients undergoing transesophageal echocardiography. Nine patients were studied intraoperatively before and after surgery, so that 75 pulmonary venous flow tracings were analyzed. Fifty-four patients had no significant (0 to 1+) mitral regurgitation and 21 had significant (2 to 3+) mitral regurgitation. Comparison of both groups revealed significant differences in the pulmonary venous flow pattern. In patients with no significant mitral regurgitation, the peak systolic velocity was higher (55 +/- 16 vs. -4 +/- 16 cm/s; p less than 0.0001) and the peak diastolic velocity was lower (43 +/- 13 vs. 59 +/- 17 cm/s; p less than 0.01) when compared with values in patients with significant mitral regurgitation. Consequently, the peak systolic/diastolic velocity ratio was significantly higher in the patients without significant mitral regurgitation (1.4 +/- 0.5 vs. 0.4 +/- 1.3; p less than 0.0001). The same trend was noted with respect to the systolic and diastolic velocity integrals. As the degree of mitral regurgitation increased, the peak diastolic velocity and diastolic velocity integral increased, whereas the peak systolic velocity and systolic velocity integral decreased. In patients with severe mitral regurgitation, the systolic flow became reversed (retrograde). The sensitivity of reversed systolic flow for severe mitral regurgitation was 90% (9 of 10), the specificity was 100% (65 of 65), the positive predictive value was 100% (9 of 9), the negative predictive value was 98% (65 of 66) and the predictive accuracy was 99% (74 of 75).
Relation of cellular drug resistance to long-term clinical outcome in childhood acute lymphoblastic leukaemia. The clinical relevance of cellular drug resistance in children with acute lymphoblastic leukaemia (ALL) is unknown. The relation between in-vitro sensitivity to chemotherapeutic drugs at initial diagnosis and long-term clinical outcome was investigated in 44 children with ALL. The short-term MTT assay was used to assess sensitivity to prednisolone, vincristine, colaspase (asparaginase), daunorubicin, and thioguanine (instead of mercaptopurine which is unstable in vitro). For vincristine and colaspase there was no difference in outcome (probability of continuous complete remission) between sensitive and resistant patients. However, the probability of continuous complete remission was significantly lower in patients with resistant cells than in those with sensitive cells for thioguanine (p less than 0.01), daunorubicin (p less than 0.02), and prednisolone (p less than 0.05). For prednisolone there was a significant worsening of the prognosis (p less than 0.05) from the extremely sensitive patients through an intermediate group to the most resistant group. The prognostic significance of cellular drug resistance was independent of white-blood-cell count, age, sex, and hepatosplenomegaly. Leukaemic cells from boys were more resistant to thioguanine than those from girls. Thus, the short-term highly efficient MTT assay can help to predict long-term response to chemotherapy in childhood ALL.
Rationale for a primary prevention study using low-dose aspirin to prevent coronary and cerebrovascular disease in the elderly. The benefits of prophylactic aspirin therapy to prevent cardiovascular and cerebrovascular disease in asymptomatic individuals remains unclear. The rationale for developing a multicentered, double-blind, placebo-controlled clinical trial to determine whether low-dose aspirin (100 mg daily) prevents cardiovascular and cerebrovascular morbidity and mortality in persons aged 70 years and over with no evidence of pre-existing cardiovascular or cerebrovascular disease is described. Sample size calculations have indicated that 15,000 subjects would be required over a 4-year follow-up period in order to demonstrate a 20% reduction in overall cardiovascular mortality at the 0.01 level with a power of 0.8. Such a large-scale community-based clinical trial has never been conducted in Australia in this age group. Therefore the PACE (prevention by low-dose aspirin of cardiovascular disease in the elderly) pilot study has been developed to test recruitment strategies and methods and ascertaining disease end-points.