Plain radiographic and MR imaging appearances of familial dermal cylindroma.
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Adenoma↗
Biomedical subjects
J Kramer
Publications and source records attributed to J Kramer.
Changing behavior. Practical lessons from the diabetes control and complications trial.
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Attitude to Health↗
[Molecular genetic markers of Alzheimer dementia].
Alzheimer's dementia (AD) is a progressive neurodegenerative disease which is one of the most common forms of dementia in the above 65 years old age group. Diagnosis is based upon evaluation of cognitive skills, elimination of other dementia associated diseases and on cranial CT examination. However, definitive diagnosis is proved only by neurohistological findings. The etiology of the disease is unknown and effective treatment is not available. Intensive research into the pathomechanism of the disease revealed a connection between increased frequency of apolipoprotein E4 allele and later onset of AD. The aim of the author's research was to characterize apolipoprotein E polymorphisms in AD patients and to examine immunological factors which could play an important role in the development of the disease such as the polymorphism of complement and lipoprotein(a) [Lp(a)] alleles previously had not been investigated. 35 AD patients (26 female, 9 male) were examined (mean age 66.4 years +/- 8.7 SD). 18 of these patients showed late onset of the disease. The E4 allele was significantly more frequent in both early and late onset groups compared to controls (0.29 vs. 0.14 and 0.33 vs 0.09). The high frequency of apo E4 found in the early onset group in this study differs from other international data. Serum lipid, lipoproteins, Lp(a) concentrations and Lp(a) phenotype distribution showed no significant difference compared to controls. Furthermore, it was found that the frequency of complement C4AQ0 (null) and Bf F alleles was higher in the late onset AD group compared to controls (0.35 vs 0.139 and 0.305 vs. 0.225) suggesting an active involvement of the complement system in the pathogenesis of this disease.
Age Factors↗
Vegetable oil as an MR contrast agent for rectal applications.
The purpose of this study was to evaluate the feasibility of pure vegetable oil as an MR contrast agent for rectal applications. The hypothesis was that vegetable oil highlights the lumen of the rectum after rectal application as a positive contrast medium and offers additional contrast qualities using fat suppression techniques. Eleven MRI examinations were performed on 11 subjects (five healthy volunteers, all males, mean age 35 yr; and six patients, three males, three females, mean age 49 yr). Peanut oil, 200 ml, was applied rectally. In addition, 0.1 mmol/kg GD-DTPA was administered intravenously to the six patients only. Conventional T1-weighted SE sequences and T1-weighted SE images with fat suppression were obtained. Criteria for image evaluation were: overall image quality; uniformity of contrast distribution; chemical shift artifact; and delineation of the rectal wall. Side effects were assessed. There were no complaints reported by the 11 subjects. The image quality was sufficient in all studies. In all five of the volunteers and five of the six patients, the distribution of oil was uniform. Chemical shift artifacts did not deteriorate image quality. After rectal application of vegetable oil, the delineation of the rectal wall was sufficient with and without fat suppression techniques. Vegetable oil highlights the lumen of the rectum in MRI studies and offers additional contrast qualities with fat suppression techniques, acting as a positive as well as a negative contrast agent, depending on the chosen sequence.
Administration, Rectal↗
Ethnic minorities, health care systems, and behavior.
This article presents an overview of research on health care use and provider behavior, on doctor-patient relationships, adherence to medical regimens, self-care, practices and avoidance health care behaviors, and attitudes of 4 ethnoracial groups: African Americans, American Indians, Asian Americans, and Latinos. Although issues within the groups varied, common themes between the groups emerged. It became apparent, after discussion, that whatever the issues and health problems, these can be resolved most effectively when addressed within the social contexts of each ethnoracial group.
Black or African American↗
Relationship between the reactivity to hepatitis B virus vaccination and the frequency of MHC class I, II and III alleles in haemodialysis patients.
To study the immunoreactivity genes in a heterogeneous human population needs a large number of individuals. Associations between HLA antigens and immunoresponse to viral or bacterial antigens have been studied with controversial results. As a homogeneous population, the MHC class I, II and III allele distribution was studied in 153 end-stage renal disease patients (ESRD, average duration of renal replacement: 8.2 + 5.1 years) immunized with a recombinant hepatitis B vaccine in accordance to the standard vaccination schedule. Thirty-four patients with an antibody titre of less than 10 U/l following the last booster injection were considered as non-responders while 119 patients with antibody titre equal to or more than 10 U/l were considered as responders. The responder group was divided into two subgroups: low responders (antibody titre: < or = 1000 U/l) and high responders (antibody titre: > 1000 U/1). Marked differences were observed between responders and non-responders in the occurrence of carriers of different MHC class I, II and III alleles. Homozygotes for HLA-A1, HLA-B8, HLA-DR3 and HLA-DQ2 were found almost exclusively in the non-responder group and significantly more heterozygotes for these alleles were found in the non-responder group compared to the responders. Similar albeit less marked differences were found in the frequency of some MHC class III alleles (C4A*6, C4A*QO, Bf*F, Bf*S0.7). Within the responder group, carriers of HLA-A2, HLA-B7 and HLA-DR4 were found to be clustered in the low responder sub-group whereas carriers of HLA-A1, HLA-B27, HLA-Cw2, C4A*6 and Bf*F were observed more frequently in the group of high responders. Similar differences were found with extended haplotypes as well. For example, the extended haplotypes HLA-A1, B8, BfS, C4AQO, C4B1, DR3, DQ2 and HLA-A1, B8, BfF, C4A6, C4B2, DR3, DQ2 were present in nine of 34 cases of non-responders but only in one of 119 case of responders (P < 0.000001). These observations indicate that the presence or absence of certain MHC alleles even in heterozygous form determine the responsiveness to hepatitis B vaccination in end-stage renal disease patients, and among responders, the intensity of antibody response is also markedly influence by immunogenetic factors.
Adult↗
Preoperative assessment of the carotid bifurcation. Can magnetic resonance angiography and duplex ultrasonography replace contrast arteriography?
BACKGROUND AND PURPOSE: Noninvasive studies are used with increasing frequency to assess the carotid bifurcation before endarterectomy. Therefore, assessment of their diagnostic accuracies is essential for appropriate patient management. We prospectively evaluate two noninvasive tests, magnetic resonance angiography (MRA) and duplex ultrasonography (DU), as potential replacements for contrast arteriography (CA). METHODS: A blinded comparison of three-dimensional time-of-flight (TOF) MRA, two-dimensional TOF MRA, and DU in 176 arteries was performed. CA was used as the standard of comparison. RESULTS: Three-dimensional TOF MRA had a sensitivity of 94%, a specificity of 85%, and an accuracy of 88% for the identification of 70% to 99% stenosis; two-dimensional TOF MRA had a sensitivity and specificity that were approximately 10% lower than those of three-dimensional TOF MRA. DU resulted in a sensitivity of 94%, a specificity of 83%, and an accuracy of 86%. Combining data from three-dimensional TOF MRA and DU, allowing for CA only for disparate results, yielded a sensitivity of 100%, a specificity of 91%, and an accuracy of 94% among concordant noninvasive tests, with CA required in 16% of arteries. MRA accurately differentiated 17 carotid occlusions from 16 high-grade (90% to 99%) stenoses, whereas with DU two patent arteries were identified as occluded and one occluded artery was identified as patent. CONCLUSIONS: Three-dimensional TOF MRA is the most accurate noninvasive test. Combined use of MRA and DU results in a marked increase in accuracy to a level that obviates the need for CA in a majority of patients.
Aged↗
Follow-up of spongiosa plugs with contrast enhanced magnetic resonance imaging.
The aim of this study was to evaluate the extent and duration of the revascularization process in spongiosa plugs when a fibrin sealant was used. 20 patients with tumour-like lesions, benign tumours and tumours with a potential for malignant transformation were studied. After intralesional tumour removal, the defect was filled with homologous spongiosa either combined with or without a fibrin sealant, according to a prospective randomization. Magnetic resonance imaging (MRI) follow-up examinations were performed within 1 week, and 1.5, 3, 4.5, 6, 8, 12, 24 and 36 months after surgery. Those patients without a fibrin sealant showed an increased revascularization zone up to the sixth week. Patients treated with fibrin, however, showed increased revascularization up to 3 months. In the 25% percentile the extent as well as the rate of revascularization is higher in those with a fibrin sealant. It is of clinical relevance that no revascularization should be expected at 3 months after surgery, which is easily demonstrated by MR follow-up.
Adolescent↗
Apolipoprotein E and complement C3 polymorphism and their role in the response to gemfibrozil and low fat low cholesterol therapy.
Three different allelic variants of apolipoprotein E determine, in concert with other gene products, the levels of plasma lipoproteins. Recently, cleavage products of the complement C3 molecule have also been implicated in determining plasma triacylglycerol concentrations. This study presents data of an ongoing study to dissect the role of the apolipoprotein E gene locus in the response to low fat/low cholesterol diet combined with gemfibrozil treatment. In addition, for the first time, the significance of C3 allelic variants to such hypolipidaemic therapy response was analysed. To this end data from 81 obese hyperlipoproteinaemic patients (Fredrickson type II/A and B and type IV and V) confirmed the usefulness of the combined gemfibrozil/diet treatment and unveiled apolipoprotein E allele group specific therapy responses. The mean changes of lipid properties due to combined treatment was 15% for total cholesterol, 48% for triacylglycerols and 28% for atherogenic index. Division into hyperlipidaemia types according to Fredrickson and subgrouping into E2, E3 and E4 groups (apolipoprotein E2/2 and 2/3, apolipoprotein E3/3 and apolipoprotein E4/2 and 4/3 phenotype groups respectively) exposed pronounced differences from these mean changes, suggesting substantial influence of apolipoprotein E variants on this therapy. We observed triacylglycerol reductions of from 17% in type IIA-apolipoprotein E3 group patients up to 78% in the type IV and V-apolipoprotein E2 group. Thus it might be concluded the apolipoprotein E genotyping aides therapy success prediction. Although, low sample number in some subgroups obscures significance in this pilot study, significant therapy success emerges for the E3 and E4 group in type IV and V hyperlipidaemia and type IIB-apolipoprotein E3 homozygous patients can be predicted to respond better than apolipoprotein E2 carriers. Finally, we present evidence that positive changes of lipid properties are also determined by the "fast" complement C3 allel (C3-F). Patients with complement factor C3-FS pattern respond better to treatment than patients with C3-SS configuration. In summary these data endorse the genotyping of apolipoprotein E alleles to predict maximal success of "fibrate" treatment. In addition they argue strongly for further assessment of the involvement of complement C3 allelic variations in lipid homeostasis.
Adult↗
Resurfacing versus not resurfacing the patella during total knee replacement.
One hundred patients with osteoarthritic knees were randomized either to have their patella resurfaced or not resurfaced using the same total knee replacement. These patients were assessed preoperatively and a minimum 2 years postoperatively using disease-specific (Knee Society Clinical Rating System) and functional capacity (30 second stair climbing and knee flexor and extensor torques) outcome measures. Two patients in the not resurfaced group required reoperation because of anterior knee pain. At 2 years' followup, the not resurfaced group had significantly less pain and better knee flexor torques than did the resurfaced group, whereas the results of the Knee Society Function Scores, 30 second stair climbing, and knee extensor torques were similar. These results suggest that longer-term followup is required, but that one should keep an open mind regarding patellar resurfacing during total knee replacement.
Activities of Daily Living↗
Clinical significance of longitudinal complement measurements in recipients of bone marrow transplant.
Overall activity of classical complement pathway, C3 and C4 levels, level of circulating immune complexes and concentration of serum immunoglobulins were measured in 38 patients transplanted with HLA-identical bone marrow before and after transplantation for at least 4 years. Changes of complement parameters and their association with acute and chronic GVHD and with infections were analysed. A strong association was found between the development of chronic GVHD and hypercomplementaemia measured in 16 long-term survivors. Low pre-transplantation C4 activity was found to predict the development of severe acute GVHD. These findings indicate that longitudinal complement measurements may have clinical value in BMT patients.
Acute Disease↗
[Osteochondral lesions].
MRI has demonstrated high diagnostic value in the assessment of musculoskeletal disease. Pathological joint changes, especially in the osteochondral region, are an important orthopedic problem because the signs and symptoms are vague and unspecific. Therefore, in early disease their extent and severity are difficult to ascertain clinically. Early diagnosis and exact staging are recommended for sufficient therapeutic management. MR imaging has proved to be the method of choice in the evaluation of osteochondral lesions and helps to prevent late-stage disease and joint damage.
Cartilage, Articular↗
Complement C4, factor B and C3 polymorphism in north India.
Allotype frequencies of complement C4, factor B and C3 proteins were determined in 192 healthy unrelated individuals from North India. The gene frequencies for the most common factor B and C3 alleles were close to the data already published. As expected, significant differences were observed in this first study of C4 allotype frequencies among the North Indian population in comparison to European Caucasians. According to historians, Hungarian Gypsies migrated from India. In this study we demonstrate that our data for some complement allele frequencies are in accordance with the opinion of historians.
Adult↗
Immunological and genetic studies on hereditary angioedema.
Ca. eighty patients with hereditary angioedema due to C1-inhibitor deficiency were diagnosed and treated in Poland during last the 15 yrs, mainly in centers from Warsaw, Cracow and Bydgoszcz. The detailed description concerns 17 patients, 14 females (82%) and 3 males (18%). Important diagnostic points, response to treatment, and critical serum values of C1-inhibitor were evaluated. In the experimental part, we have established fibroblasts cell lines from 5 patients with type I HAE, with characteristic profound deficits of C1-INH (antigenic and functional) and C4. Fibroblasts from all the patients synthesized only ca. 20% of the normal values of C1-INH. We have confirmed the data from other authors that C1-INH synthesis can be substantially increased (11 to 20-fold) by IFN-gamma at a pretranslational level.
Adult↗
[Reactivity to hepatitis B vaccination (HBV) in patients with uremia: association with defined HLA antigen configuration and complement protein allotypes C4A, C4B and B factor (Bf)].
Genes, which are responsible for the effectivity of immune response are located on the chromosome 6 similarly as the genes of the class I and II of the major histocompatibility complex (MHC) together with the genes of some complement proteins (C4A, C4B, B factor/Bf/). The study investigates the interdependence between HLA configuration as well as complement protein allotypes (C4A, C4B, Bf) with the reactivity to HBV vaccination of ESRD [end stage renal disease]-patients. 153 ESRD-patients (68 females, 85 males; mean duration of hemodialysis therapy 8.2 +/- 5.1 years) were studied. Subjects were vaccinated (Gen-H-B-Vax-D; Merck, Sharp and Dohme, 40 micrograms/dosis i.m.) in the schedule at 0, 1, 2, and 6 month. Non-responders (NR) were defined when the anti-HBs antibody titer was below 10 U/liter. HLA typing was performed by means of microlymphocytotoxicity assay, whereas complement allotypes were estimated by using high voltage gel electrophoresis with subsequent specific immunofixation with antibodies against C4A, C4B and Bf. In the non-responders group [NR = 34] a) the significantly higher occurrence of single HLA-A1 and/or HLA-B8 and/or Cw4 or DR3 or DR7 or DQ2 have been found. In about 40% of NR HLA-A1, -B8, -DR3, or -DQ2 antigens occurred as homozygotes.
Adult↗
SWAP pre-mRNA splicing regulators are a novel, ancient protein family sharing a highly conserved sequence motif with the prp21 family of constitutive splicing proteins.
Regulators responsible for the pervasive, nonsex-specific alternative pre-mRNA splicing characteristic of metazoans are almost entirely unknown or uncertain. We describe here a novel family of splicing regulators present throughout metazoans. Specifically, we analyze two nematode (Caenorhabditis elegans) genes. One, CeSWAP, is a cognate of the suppressor-of-white-apricot (DmSWAP) splicing regulator from the arthropod Drosophila. Our results define the ancient, conserved SWAP protein family whose members share a colinearly arrayed series of novel sequence motifs. Further, we describe evidence that the CeSWAP protein autoregulates its levels by feedback control of splicing of its own pre-mRNA analogously to the DmSWAP protein and as expected of a splicing regulator. The second nematode gene, Ceprp21, encodes an abundant nuclear cognate of the constitutive yeast splicing protein, prp21, on the basis of several lines of evidence. Our analysis defines prp21 as a second novel, ancient protein family. One of the motifs conserved in prp21 proteins--designated surp--is shared with SWAP proteins. Several lines of evidence indicate that both new families of surp-containing proteins act at the same (or very similar) step in early prespliceosome assembly. We discuss implications of our results for regulated metazoan pre-mRNA splicing.
Animals↗
C4B*Q0 allotype as risk factor for myocardial infarction.
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Adult↗
Method for the quantitative assessment of contrast agent uptake in dynamic contrast-enhanced MRI.
In previous papers relative signal intensity increase was used as a quantitative assessment parameter for contrast uptake in contrast-enhanced MRI. However, relative signal intensity increase does not only reflect contrast uptake but depends also on tissue parameters (native T1 relaxation time) and sequence parameters (repetition time and flip angle); thus, the contrast uptake cannot be assessed accurately using relative signal intensity increase. Based on an analysis of the contrast behavior of spoiled gradient echo sequences, a method is described in this paper that overcomes the limitations of relative signal intensity increase measurement. A parameter, called "enhancement factor" (EF) is introduced that approximates differential T1 relaxation rate. The enhancement factor scales linearly with contrast uptake and is independent of tissue and sequence parameters. The additional measurement time involved in determining the enhancement factor is less than 1 min and computation is straightforward. The practicality of the new method was confirmed by phantom measurements using T1-weighted and proton density-weighted spoiled gradient echo sequences (FLASH-2D). Enhancing tissues were simulated by water phantoms doped with increasing concentrations of Gd-DTPA.
Algorithms↗