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91.
Thrombosis in inflammatory bowel disease: clinical setting, procoagulant profile and factor V Leiden 总被引:7,自引:0,他引:7
Jackson LM; O'Gorman PJ; O'Connell J; Cronin CC; Cotter KP; Shanahan F 《QJM : monthly journal of the Association of Physicians》1997,90(3):183-188
Patients with inflammatory bowel disease have an increased frequency of
thromboembolism, and microvascular thrombosis has been proposed as a
contributory pathogenic factor. The mechanism of enhanced procoagulant
activity is not understood. We examined the clinical setting of
thromboembolic events in 52 patients with Crohn's disease or ulcerative
colitis, and assessed the procoagulant laboratory profile, including Factor
V Leiden, in a subset of 20 patients to identify procoagulant risk factors.
Patients who developed thrombosis tended to be young; 60% of thrombotic
events occurred in patients under 50 years. Multiple thromboembolic
episodes occurred in 13% and unusual sites of thrombosis (e.g.
intracardiac, cerebral, inominate veins) in 11%. No risk factor was
identifiable in 52% of cases and two-thirds of thromboses occurred in an
out-patient setting. The mortality rate was 8%. Evidence for inflammatory
disease activity was found in only 45% of patients with ulcerative colitis
at the time of the thromboembolic event, in contrast to 89% of those with
Crohn's disease. Assays for specific coagulation defects were negative in
all cases tested (protein S, C were normal in 17/17; anti-thrombin III,
anti-phospholipid antibodies and activated protein C resistance were
negative in 20/20, and only 1/20 patients was found to be heterozygous for
Factor V leiden. Thrombosis in inflammatory bowel disease is important
because it occurs in a young population, often in unusual sites, and has a
high mortality. The development of thrombosis is related to active
inflammatory disease in most patients with Crohn's disease but apparently
not in those with ulcerative colitis. Since approximately half of the
patients had no other identifiable risk factor, there remains a substantial
group of patients with IBD who develop thrombosis for unknown reasons.
相似文献
92.
93.
Genetic factors affecting the consistency and magnitude of changes in plasma cholesterol in response to dietary challenge 总被引:3,自引:0,他引:3
Humphries SE; Talmud PJ; Cox C; Sutherland W; Mann J 《QJM : monthly journal of the Association of Physicians》1996,89(9):671-680
We examined the role of common genetic variation in determining the
consistency and magnitude of change in plasma total cholesterol (TC) levels
in response to two separate changes from a high-saturated (SFA) to a
low-saturated/high-polyunsaturated-fat (PUFA) diet, in a group of
free-living healthy men and women. Consistent responders were defined as
those whose mean difference in the change in TC was within one SD of the
mean for all participants, and the remainder were defined as variable
responders. DNA was obtained from 55 individuals and genotype determined at
the apolipoprotein (apo) B locus (signal peptide, SP), apoCIII (C1100-T)
and lipoprotein lipase (LPL) gene loci (HindIII). In the 38 consistent
responders, the apoBSP24 allele was significantly more common than in the
17 individuals with a variable response (0.29 vs. 0.12; p < 0.05). No
other polymorphism showed a significant frequency difference between
groups. In the group as a whole, the correlation between the change in TC
level in response to the first and second dietary change was 0.28 (p =
0.05), but those with one or more apoB SP24 alleles and those with the
apoCIII genotype CC had a significantly higher correlation than those with
other genotypes (0.46 (p = 0.05) vs. 0.12 (NS) and 0.31 (p = 0.05) vs. 0.02
(NS), respectively). In the group as a whole, mean response left TC 10%
higher on the SFA than on the PUFA diet, and neither apoB nor apoCIII
genotypes affected the magnitude of this response. However, individuals
with the LPL HindIII genotype H+ H+ had a significantly smaller change in
mean TC in response to diet than those with one or more H- allele (9.3% vs.
14.4%; p = 0.03). Thus variation at the apoB and apoCIII loci affects the
consistency of response to change in dietary fat content, while variation
at the LPL gene locus affects magnitude of response.
相似文献
94.
Wierzbicki AS; Lumb PJ; Semra YK; Crook MA 《QJM : monthly journal of the Association of Physicians》1998,91(4):291-294
Lipid targets can be difficult to attain in familial hypercholesterolaemia.
To compare atorvastatin with simvastatin- fenofibrate and
simvastatin-cholestyramine therapy, we studied 54 patients with familial
hypercholesterolaemia over periods of 2-6 months on each therapeutic
regimen. The atorvastatin regimen reduced total cholesterol by 41.2 +/-
11.2%, LDL by 45.6 +/- 15.5%, triglycerides by 33.8 +/- 24.8%, and
increased HDL by 2.3 +/- 37.0%. Simvastatin- fenofibrate therapy achieved
reductions of 33.9 +/- 8.5% in cholesterol, 42.0 +/- 12.2% in LDL, 34.7 +/-
38.3% for triglycerides, and a 25.4 +/- 55.1% increase in HDL.
Simvastatin-cholestyramine gave a reduction of 31.3 +/- 11.8% in
cholesterol, 36.0 +/- 14.4% in LDL, 13.7 +/- 36.3% in triglycerides, and a
1.1 +/- 30.3% rise in HDL. The atorvastatin regimen was marginally but not
significantly better than simvastatin-fenofibrate in improving the LDL:HDL
ratio, LDL:apoB and and apolipoprotein B:A1 ratios. Eleven patients (20.4%)
had side- effects: two discontinued atorvastatin due to side-effects; two
patients had rashes; six had myalgia and two had diarrhoea.
Gastrointestinal side-effects were described in 16 (30.1%) patients on
simvastatin-cholestyramine therapy and four cases of myalgia (11.2%) were
seen with simvastatin-fenofibrate. In nine patients on atorvastatin (20.4%)
a 30% or greater fall in HDL was observed, compared to five patients with
resin therapy (9.2%) and two with fibrate therapy (5.5%). There were no
significant differences in liver or muscle biochemistry between the
regimens, but atorvastatin did raise transaminase and creatine kinase
concentrations significantly compared to pre-treatment values (p = 0.001).
Atorvastatin significantly improves the lipid profile in most patients
compared with other regimens. It has a comparable incidence of side-effects
to combination therapy regimens.
相似文献
95.
H-2 effects on cell-cell interactions in the response to single non-H-2 alloantigens. II. H-2 D region control of H-7.1-specific stimulator function in mixed lymphocyte culture and susceptibility to lysis by H-7.1- specific cytotoxic cells
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The relative immunogenicity of the H-7.1 alloantigen has been shown in a previous communication to be regulated by a gene in the D region of the mouse major histocompatibility (H-2) complex. The level of relative immunogenicity was inferred from survival times of H-7.1-incompatible skin grafts donated by donors with different H-2 haplotype origins of H-2D region genes. In this communication we report the results of an extension of these previous investigations into the possible role of H-2D region genes in controlling the capacity of H-7.1-incompatible lymphocytes to stimulate H-7.1-speciflc mixed lymphocyte culture proliferation and generation of cytotoxic effector cells. The results reported herein demonstrate that the H-2D genotype of H-7.1-incompatible stimulator cells determines the relative H-7.1-specific capacity of those lymphocytes to stimulate H-7.1-specific proliferation of in vivo primed responder T cells in secondary mixed lymphocyte culture. H-2D(b)-bearing, H-7.l-incompatible stimulators were significantly more effective in stimulating H-7.1-specific proliferation than H-2D(d)-bearing stimulators. As expected, H-2D(b), H-7.1-in-compatible stimulators were also more effective than H-2D(d) a stimulators in generating H-7.1- specific cytotoxic effector cells. Further, the susceptibility of (51)Cr- labeled, H-7.1-incompatible lymphoblast targets to H-7.1-specific lysis was similarly regulated by an H-2D gene. Reciprocal H-2 restriction (F(1) cells are capable of killing only the cells bearing the immunizing cell parental H-2 haplotype) observed by other investigators for cytolysis of non-H-2-incompatible targets was not observed. H-2D a-bearing, H-7.1- incompatible stimulators stimulated generation of cytotoxic effectors capable of detectably lysing H-2D(b) but not H-2D(a)-bearing, H-7.1- incompatible targets. The impact of these observations on the proposed models for H-2 restriction of non-H-2 histocompatibility antigen-specific cytolysis is discussed. 相似文献
96.
OSTLERE L; WARNER T; MEUNIER PJ; HULME P; HESP R; WATTS RWE; REEVE J 《QJM : monthly journal of the Association of Physicians》1991,79(3):503-515
Two patients with Type 1 (adult) Gaucher's disease and majorskeletal involvement with multiple fractures have been treatedwith the second generation bisphosphonate pamidronate for extensiveperiods. There was evidence of an immediate reduction in boneresorption, with increased calcium absorption (delayed in Patient1), improved calcium balance and maintained or improved bonedensity indices in the axial and peripheral skeleton. Therewas no evidence of immediate relapse on treatment cessation.No toxic effects of pamidronate treatment were identified andsubjective skeletal pain diminished in both patients. Histomorphometryof transiliac bone biopsies obtained before the start of treatment,after double in vivo tetracycline labelling, represents oneof the earliest reports of the quantitative findings in iliacbone invaded by Gaucher cells. 相似文献
97.
FcR gamma-chain is essential for both surface expression and function of human Fc gamma RI (CD64) in vivo 总被引:5,自引:0,他引:5
van Vugt MJ; Heijnen AF; Capel PJ; Park SY; Ra C; Saito T; Verbeek JS; van de Winkel JG 《Blood》1996,87(9):3593-3599
Most Ig receptors exist as hetero-oligomeric complexes with separate ligand binding (alpha) and signal transducing (beta, gamma, or zeta) subunits. For Fc gamma RIIIa and Fc epsilon RI, association with the FcR gamma-chain is essential for surface expression. However, the human high affinity IgG receptor, hFc gamma RI, was found to be surface- expressed by itself in transient transfection models. We have now analyzed the integrity of hFc gamma RI expression in more detail in stable transfectants. In vitro we noted that, in the absence of FcR gamma-chain, surface expression of hFc gamma RI rapidly declined to background levels, in both IIA1.6 B cells and NIH3T3 fibroblasts. The effect of FcR gamma-chain on hFc gamma RI surface expression in vivo was evaluated by using two newly generated transgenic mouse lines, selectively expressing hFc gamma RI on myeloid cells. These transgenic mice were crossed with FcR gamma-chain-deficient mice. Analysis of blood monocytes and peritoneal macrophages showed that surface expression of hFc gamma RI was reduced by approximately 80%. The remaining approximately 20% of receptors were still capable of binding IgG-opsonized RBC, suggesting FcR gamma-chain not to be critical for hFc gamma RI ligand-binding capacity. Importantly, however, hFc gamma RI signaling capacity was lost in FcR gamma-chain-deficient cells. No phagocytosis could be observed using either ligand sensitized (EA- IgG2a) or CD64-targeted erythrocytes (using a bispecific antibody) in both hFc gamma RI transgenic lines. This documents the FcR gamma-chain to be indispensable for both surface membrane expression and function of human Fc gamma RI in vivo. 相似文献
98.
Mycobacterial Infections After Renal Transplantation 总被引:5,自引:0,他引:5
HIGGINS RM; CAHN AP; PORTER D; RICHARDSON AJ; MITCHELL RG; HOPKIN JM; MORRIS PJ 《QJM : monthly journal of the Association of Physicians》1991,78(2):145-153
Mycobacterial infections occurred in 11 of 633 (1.7 per cent)recipients of successful renal transplants. There were no casesof tuberculosis in patients receiving chemoprophylaxis, butamongst those who did not receive prophylaxis disease occurredin six of the 27 (22 per cent) high-risk patients. The majorcause of morbidity during treatment was renal allograft rejection,largely due to reduction in immunosuppressive drug therapy. 相似文献
99.
WY Khoder AJ Becker B Schlenker S Tritschler PJ Bastian CG Stief 《European journal of medical research》2009,14(7):320-322
Introduction
Rectal polypectomy causes thinning (or even perforation) of the rectal wall in addition to thermic injury at the polypectomy site.Case report
We present a rare case of spontaneous rectal perforation after uncomplicated nerve sparing endoscopic extraperitoneal radical prostatectomy in a patient with a previous history of rectal polypectomy at the perforation site. The patient could be treated conservatively. There was complete healing of the fistula without any effect on functional results. This Conservative therapy for such rectal perforations is indicated if the patient''s general condition remains stable without any signs of infection.Conclusions
Polypectomy is an important risk factor for rectal perforation during nsEERPE. Adequate time interval should be given to allow healing and avoid adding further thermal wall damage which may obscure healing leading to complications like fistula. Conservative therapy for small missed rectal perforations constitutes an attractive, feasible and non invasive treatment entity. Following this principle we have not faced this complication in following similar cases. 相似文献100.
Background: Van der Woude syndrome (VWS) is the most common clefting syndrome in humans. It is characterized by the association of congenital lower lip fistulae with cleft lip and/or cleft palate. VWS individuals have a high prevalence of hypodontia. Although caused by a single gene mutation, VWS has variable phenotypic expression. This study aimed to describe the range of clinical presentations in 22 individuals with VWS to facilitate its diagnosis.
Methods: A retrospective study of 22 patients with a diagnosis of VWS was undertaken at the Australian Craniofacial Unit (ACFU) in Adelaide. Three extended families with affected members were included in the study cohort.
Results: The overall prevalence of lip pits in this study cohort was 86%. Cleft phenotypes included bilateral cleft lip and palate (32%); unilateral cleft lip and palate (32%); submucous cleft palate (23%); and isolated cleft hard and soft palate (9%). Missing permanent teeth were reported in 86% of affected individuals.
Conclusions: Submucous cleft palate in VWS may go undiagnosed if the lower lip pits are not detected. Associated hypodontia and resultant malocclusions will also require management by a dental team. 相似文献
Methods: A retrospective study of 22 patients with a diagnosis of VWS was undertaken at the Australian Craniofacial Unit (ACFU) in Adelaide. Three extended families with affected members were included in the study cohort.
Results: The overall prevalence of lip pits in this study cohort was 86%. Cleft phenotypes included bilateral cleft lip and palate (32%); unilateral cleft lip and palate (32%); submucous cleft palate (23%); and isolated cleft hard and soft palate (9%). Missing permanent teeth were reported in 86% of affected individuals.
Conclusions: Submucous cleft palate in VWS may go undiagnosed if the lower lip pits are not detected. Associated hypodontia and resultant malocclusions will also require management by a dental team. 相似文献