Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0032463 (polycythemia vera)
 3,374 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

To determine whether decreased alkaline phosphatase activity in the granules from neutrophils of patients with chronic myelogenous leukemia (CML) was due to an absence of enzyme or the production of defective enzyme, we compared the immunologic properties of granule alkaline phosphatase derived from patients with CML with that of normal subjects and patients with polycythemia vera (PRV). Antisera prepared in rabbits against granule alkaline phosphatase purified from the neutrophils of a patient with PRV produced a single precipitin line of antigenic identity when reacted with extracts of normal, PRV, and CML neutrophil granules. A histochemical stain for alkaline phosphatase activity (alpha-naphthyl acid phosphate coupled with Fast Blue RR) specifically stained the precipitin line. A variety of quantitative precipitin techniques failed to produce satisfactory precipitation of alkaline phosphatase activity. Comparative analyses were therefore performed by affinity chromatography using goat antirabbit-gammaglobulin linked to Sepharose 4B to adsorb alkaline phosphatase complexed with rabbit gamma globulin. With this method, 100% of CML, normal, and PRV alkaline phosphatase could be adsorbed. Using limiting concentrations of antibody, a proportionally smaller fraction of enzyme activity was absorbed as the concentration of PRV alkaline phosphatase or normal alkaline phosphatase was increased. Extracts of CML granules containing comparable amounts of protein but 200-fold less alkaline phosphatase activity per milligram did not specifically reduce adsorption. Thus, in CML, we found no evidence that the granulocytes contained a large amount of antigenically normal but enzymatically defective alkaline phosphatase. Examination of electron micrographs revealed no significant differences in the number or distribution of granules in the granulocytes of normal subjects or patients with PRV or CML. This suggests that the low level of neutrophil alkaline phosphatase in CML granulocytes is the result of decreased enzyme content and not a consequence of synthesis of catalytically defective enzyme.
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PMID:Neutrophil alkaline phosphatase: comparison of enzymes from normal subjects and patients with polycythemia vera and chronic myelogenous leukemia. 4 59

The replication of PRV DNA occurs in two phases, early and late. During the early stages of infection newly synthesized DNA is associated with molecules sedimenting with an S-value up to two-fold greater than that of mature viral DNA. These molecules represent unit-size linear or circular molecules, as well as small concatemers in the process of replication. Initiation of replication occurs at a site situated 20 micron from one of the ends as well as at or near the end of the molecules. At later times, newly synthesized DNA is associated with large, "tangled" concatemers containing single-stranded segments of DNA. Our results indicate that at least some of the single-stranded DNA may be produced during the extraction procedure. Analysis of the large, "tangled" concatemers with restriction enzymes shows that they consist of linear arrays of viral DNA molecules.
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PMID:Characterization of the replicative structures of the DNA of a herpesvirus (pseudorabies). 22 63

The equation that we proposed in this JOURNAL (1977), relating the postrepair ratio between peak right ventricular pressure and peak left ventricular pressure (PRV/LV) to the intraoperatively measured internal diameter of the narrowest point in the pulmonary arterial outflow tract (formerly called pulmonary "anulus"), performed well when prospectively tested in 25 patients undergoing repair of the tetralogy of Fallot. The observed PRV/LV in all 21 patients in whom no transannular patch was used fell within the 70 percent confidence limits (1 standard deviation) of that predicted from the measured diameter. A trivial difference (-0.03 +/- 0.069 PRV/LV units, P = 0.08) was found between predicted PRV/LV and that observed after repair, with body surface area (BSA) used for normalization. When the four additional patients who had secondary transannular patching are included, or where the normalization was with weight, the differences were not significant (p greater than 0.2). A slightly revised equation has been devised using the combined data from our original group and the patients used for prospective testing. This equation predicts with reasonable accuracy (r = -0.69, p less than 0.0001) postrepair PRV/LV without transannular patching from the intraoperatively measured diameter of the pulmonary arterial outflow tract. We believe it is therefore helpful in the operating room in making the important decision for or against using transannular patching.
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PMID:Decision-making in repair of tetralogy of Fallot based on intraoperative measurements of pulmonary arterial outflow tract. 42 86

The influence of chemical sympathectomy with 6-hydroxydopamine (6-OHDA) on regional alveolar hypoxic vasconstriction and on global hypoxic pulmonary vasoconstriction was investigated. In eight dogs a double-lumened endotracheal tube allowed ventilation of one lung with nitrogen as an alveolar hypoxic challenge while ventilation of the other lung with 100% O2 maintained adequate systemic oxygenation. Distribution of perfusion to the two lungs was determined with 133Xe and external counters. Mean perfusion to the test lung was 50.9 +/- 4.9% of total lung perfusion on room air and decreased by 32.4% (P smaller than 0.01) during alveolar hypoxia. Following 6-OHDA the test lung continued to reduce perfusion during alveolar hypoxia by 27.3%. In five dogs global hypoxia induced a 106% increase in pulmonary vascular resistance (PVR) prior to 6-OHDA and a 90% increase in PVR after 6-OHDA. After 6-OHDA no rise in PRV or systemic blood pressure occurred in response to tyramine, confirming effective sympathectomy by the 6-OHDA. Thus, sympathectomy with 6-OHDA failed to substantially block regional alveolar hypoxic vasoconstriction or global hypoxic pulmonary vasconstriction.
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PMID:Pulmonary hypoxic vasoconstriction: not affected by chemical sympathectomy. 43 23

To aid preoperative decision-making, we have related the ratio of postrepair peak pressure in the right and left ventricles (PRV/LV) to preoperative cineangiographic measurements in a retrospective study of 135 patients undergoing complete repair of tetralogy of Fallot or tetralogy of Fallot with pulmonary atresia. Postrepair PRV/LV was related to the preoperative diameter of right (DRPA) and left (DLPA) pulmonary arteries normalized to the descending thoracic aorta (DescThAo) in patients undergoing repair with transannular patching or a valved external conduit by the dquation: PRV/LV = 0.4840/(DRPA/DescThAO + DLPA/DesThAo) + 0.2007. Stenosis of the right pulmonary artery orifice and pulmonary artery arborization abnormalities incrementally increased postrepair PRV/LV. When a transannular patch was not used in classical tetralogy of Fallot, an increment of postrepair PRV/LV usually resulted, depending upon the size of the "anulus" measured intraoperatively: Incremental PRV/LV = 0.09437 . exp(-0.6344 . Z) where Z is a normalized expression in circumference terms of the diameter of the pulmonary arterial outflow tract (DPAOT) measured intraoperatively after infundibular dissection and valvotomy. DPAOT is itself related to the cineangiographically measured pulmonary valve anulus diameter (DPVA): DPAOT = 3.357 . DPVA0.5789 . BSA0.1551. In toto, these relations allow postrepair PRV/LV without transannular patching to be estimated from preoperative cineangiographic measurements. This allows preoperative predictiom in classical tetralogy of Fallot of the need for transannular patching, and in infants this can determine the choice between primary one-stage repair and two-stage repair. Prediction of postrepair PRV/LV when transannular patching or an external conduit is planned allows identification of patients in whom right and left pulmonary arteries are too small for safe complete repair, and in them an initial palliative operation should be done to enlarge the arteries.
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PMID:Preoperative prediction from cineangiograms of postrepair right ventricular pressure in tetralogy of Fallot. 48 Sep 61

Thirteen (16 percent) of 80 patients with tetralogy of Fallot and pulmonary atresia undergoing corrective operations between Jan. 1, 1967, and Jan. 1, 1978, died in the hospital. The hospital mortality rate was 13 percent (10 deaths) among the 77 patients with confluent right and left pulmonary arteries. The risk of operation was not significantly affected by age at operation or by use of a valved external conduit versus a transannular outflow patch. It was affected (p = 0.008) by the ratio of peak right ventricular to left ventricular pressure (PRV/LV) immediately after repair. This (PRV/LV) was determined primarily by size of left and right pulmonary arteries. An equation was developed relating postrepair PRV/LV to diameter of right and left pulmonary artery (normalized by dividing by size of descending thoracic aorta), body surface area, and possible arborization abnormalities and stenoses of the right and left pulmonary arteries. Cardiac performance after repair was better in those in whom a transannular patch was used rather than a valved external conduit. Important pulmonary dysfunction postoperatively occurred more often in patients with large "bronchial" arteries than in those without them, but was less when these were not ligated. Four (8 percent) of 48 traced hospital survivors died late postoperatively. Reoperations late postoperatively were required only in patients receiving valved external conduits. Forty (91 percent) of 44 living traced patients are asymptomatic.
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PMID:Surgical treatment of tetralogy of Fallot with pulmonary atresia. 68 64

Fifty-five consecutive patients with the tetralogy of Fallot underwent intracardiac repair in a 12 month period. A standardized protocol for outflow tract enlargement was used. When the measured diameter of the pulmonary valve ring was at least the "minimum acceptable pulmonary valve ring diameter," primary patch-graft enlargement across the valve ring was not done (Group I, 34 patients); when it was less, primary patch-graft enlargement was done (Group II, 21 patients). The right ventricular-left ventricular systolic pressure ratio (PRV/LV) after repair without patch-graft enlargement across the ring was related to the size of the ring. Sixteen (94 percent) of 17 such patients with normal-sized or large pulmonary valve rings (according to the criteria of Rowlatt, Rimoldi, and Lev) had PRV/LV equal to or less than 0.65; four (80 percent) of five patients with smaller rings but within the 50 percent confidence limits had PRV/LV equal to or less than 0.65; five (56 percent) of nine with still smaller rings but within the 72.5 percent confidence limits had PRV/LV equal to or less than 0.65. In Group II, 15 patients had abnormally small rings, but after patch-graft enlargement the mean PRV/LV was 0.44 +/- 0.140. A surgical protocol based on these data has been developed.
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PMID:Surgical management of pulmonary stenosis in tetralogy of Fallot. 89 72

The peak systolic pressure ratio PRV/PLV of the right and left ventricle after correction of the outflow tract (OFT) in Tetralogy of Fallot (TF) yields reliable dates about the efficiency of the outflowtract correction and the probability of survival. In 110 patients (2 to 57 years) the ratio after correction PRV/PLV was measured and compared with different methods of reconstruction of the OFT. Infundibulectomy (54) pericard-patch insertion across the pulmonary valve ring (43) and a valve bearing Hancock-Conduit (13) were used. To calculate the statistical differences the U-test according to Wilcoxon, Whitney, Mann was applied on the 95% level. Moreover the pulmonary insufficiency (PI) was evaluated in 60 patients within 15 to 60 days, after correction with a videodensitometric method. There is no PI after use of a valve bearing Hancock-Conduit. In severe TF a valve bearing Hancock-Conduit is hemodynamically superior to a pericard-patchreconstruction of OFT to relief right ventricular hypertension, particularly if hypoplasia of pulmonary vessels and pulmonary vascular disease after shunt-operation is present.
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PMID:[Hemodynamics after reconstruction of the outflow tract in tetralogy of Fallot. Infundibulectomy, patchreconstruction or valve rearing conduit (author's transl)]. 92 67

Between 1960 and 1975, 1 078 operable mammary carcinomas were treated by caesium therapy with the objective of conserving the breast. The authors analyse the indications for and results of this treatment in the 403 cases which have had more than 5 years follow up (241 cases were irradiated as primary treatment, 162 after having a simple tumorectomy). For the whole group 92 mastectomies were ultimately carried out: in 30 of them no tumour was found in the operative specimen. The tumorectomy cases, restricted to stage 1 of the UICC classification (pre 1962) -- PRV 0 IGR yielded the best results: 86 per cent 5 yeat cures with 9 out of 10 women retaining both breasts. The primary irradiation cases, all types considered, gave results comparable with those for primary radical surgery: 58 per cent 5 year cures-but 2 out of 3 women retained both breasts. By itself caesium therapy is capable of eradicating tumour in breast and axilla in half of the cases. It really amounts to a "double chance strategy"--an attempt at cure if possible with avoidance of mastectomy, this being in reserve in the event of failure.
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PMID:[Curative radiotherapy with hope of saving the breast in operable breast cancers 403 cases with 5-year survival]. 99 May 16

In 38 children with typical tetralogy of Fallot recatheterization was performed 15 days to 410/12 years after correction. Their age ranged from 47/12 to 181/12 years and their weigh? 14;8 TO 54.4 KG. The biplane angiocardiograms were especially evaluated with respect to thcy to decrease from intraoperative to postoperative values. We did not find a parameter, determined during the operation, which can predict with certainty the operative result, found at the time of recatheterization. The peak pressure ration PRV/PLV seems to be most suitable but in an individual case one cannot rely on it. A diastolic murmur was heart postoperatively in 25 children, 9 of whom had a regurgitation index greater than 10%. In all 38 patients an infundibulectomy was performed, which in every case resulted in a disturbance of the contraction pattern (asynergy) of the right ventricular outflow tract. In the area of the ostium infundibuli small aneurysms were demonstrated in 50% of the cases. They were divided according to their angiographic appearance, the type of asynergy and their visibility on the two standard x-ray projections. In 11 children we observed incompletely resected muscle bundles of the infundibulum. Hemodynamically they were insignificant. Residual muscular infundibular stenoses, causing a pressure gradient, were observed in 8 cases. Aortal indentations of varying degree at the site of cannulation for the extracorporal circulation were present in 91%. In 13 patients an outflow tract reconstruction was necessary. In 8 children the patch was clearly identifiable as such. The necessity of recatheterization after correction of tetralogy of Fallot is discussed.
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PMID:[Videodensitometric, angiocardiographic and hemodynamic examinations in corrected tetralogy of fallot]. 114 72


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