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991.
992.
DDAVP enhances platelet adherence and platelet aggregate growth on human artery subendothelium 总被引:5,自引:0,他引:5
Sakariassen KS; Cattaneo M; v.d. Berg A; Ruggeri ZM; Mannucci PM; Sixma JJ 《Blood》1984,64(1):229-236
The effect of intravenous 1-deamino (8-D-arginine)vasopressin (DDAVP) administration on platelet interaction with human artery subendothelium was investigated with flowing blood from five normal individuals and 12 patients with von Willebrand's disease (vWD). Three of the patients were diagnosed as vWD subtype I, four as subtype IIa, and five as subtype IIb. DDAVP administration to normals enhanced platelet adherence, in parallel with increasing plasma levels of factor VIII- related antigen ( FVIIIR :Ag) and ristocetin cofactor activity ( FVIIIR :RCF). Platelet aggregate formation was transiently increased within 90 minutes. Platelet adherence in patient blood before DDAVP infusion was subnormal. In patients with subtype I, administration of DDAVP normalized the bleeding time, enhanced the platelet adherence, and transiently improved the platelet aggregate formation. The platelet adherence was more corrected than would have been expected on the basis of the FVIIIR :Ag and FVIIIR :RCF levels. In patients with subtype IIa, infusion of DDAVP increased the FVIIIR :Ag levels approximately threefold, without affecting the FVIIIR :RCF levels, and in only two of four patients was a transiently enhanced platelet adherence with a corresponding shortening of the bleeding time observed. In patients with subtype IIb, administration of DDAVP increased the FVIIIR :Ag levels about threefold and the FVIIIR :RCF levels five to tenfold, but decreased the platelet adherence significantly. The bleeding time values were not normalized. A close association between the bleeding time values and corresponding platelet adherence values before and after DDAVP infusion was observed. Normalization of the bleeding time was paralleled with normalization of platelet adherence. We conclude that DDAVP improves the primary hemostasis by causing enhanced FVIII- vWF-mediated platelet adherence. DDAVP has little or no effect on the bleeding time in patients with subtype IIa and subtype IIb, because the platelet adherence is not normalized. 相似文献
993.
Acne-associated spondylarthropathy: radiographic features 总被引:3,自引:0,他引:3
Experience with six patients with severe acne and associated axial and peripheral arthritis is described. Four of the patients had a dermatologic triad of severe acne, hidradenitis suppurativa, and dissecting cellulitis of the scalp, the so-called follicular occlusion triad. All were black men with episodic peripheral arthropathy and low back pain. One had inflammatory bowel disease. Rheumatoid factor and HLA-B27 were absent in five patients who had these determinations. An erosive and proliferative arthritis of the axial and appendicular skeleton is described. The radiographic findings were indistinguishable from those of the seronegative spondylarthropathies. We found no previous reports in the radiologic literature describing this articulocutaneous entity. 相似文献
994.
995.
996.
997.
López Sánchez E Francés Muñoz E Mondéjar García JJ España Gregori E Menezo JL 《Archivos de la Sociedad Espa?ola de Oftalmología》2000,75(6):421-424
PURPOSE/METHODS: We present the case of a 69 year-old male patient who developed an anterior pole ischemia syndrome in his right eye. He did not have any personal history of diabetes or previous ocular pathology. Indirect ophthalmoscopy did not show any pathological finding. RESULTS/CONCLUSIONS: Carotid Duplex scanning and Nuclear Magnetic Resonance detected a severe carotid stenosis on the same side, which explained the case. We reviewed different forms of presentation of venous stasis syndrome in carotid stenosis and present this uncommon case of anterior segment ischemia syndrome without posterior segment findings. 相似文献
998.
999.
Schollen E; Pardon E; Heykants L; Renard J; Doggett NA; Callen DF; Cassiman JJ; Matthijs G 《Human molecular genetics》1998,7(2):157-164
The search for the carbohydrate-deficient glycoprotein syndrome type I
(CDG1) gene has revealed the existence of a family of phosphomannomutase
(PMM) genes in humans. Two expressed PMM genes, PMM1 and PMM2 , are located
on chromosome bands 22q13 and 16p13, respectively, and a processed
pseudogene PMM2 psi is located on chromosome 18p. Mutations in PMM2 are the
cause of CDG type IA whereas no disorder has been associated with defects
in PMM1 as yet. Here, we describe the genomic organization of these
paralogous genes. There is a 65% identity of the coding sequence, and all
intron/exon boundaries have been conserved. The processed pseudogene is
more closely related to PMM2 . Remarkably, several base substitutions in
PMM2 that are associated with disease are also present at the corresponding
positions in the pseudogene. Thus, mutations that occur at a slow rate in
the active gene in the population have also accumulated in the pseudogene.
相似文献
1000.
Prognostic factors for an unsatisfactory primary methotrexate treatment of cervical pregnancy: a quantitative review 总被引:17,自引:0,他引:17
Hung TH; Shau WY; Hsieh TT; Hsu JJ; Soong YK; Jeng CJ 《Human reproduction (Oxford, England)》1998,13(9):2636-2642
To determine the risks when the primary methotrexate (MTX) treatment of
cervical pregnancy has an unsatisfactory outcome, we conducted a Medline
search on relevant literature published from January 1983 to June 1997. The
search yielded 28 publications of 48 cases of cervical pregnancy. These and
four new cases from our institutions were used in our study. A cervical
pregnancy that presented with a serum beta-human chorionic gonadotrophin
concentration of > or = 10,000 mIU/ml [odds ratio (OR) 10.82, 95%
confidence interval (CI) 2.59, 45.14], gestational age at > or = 9 weeks
(OR 6.44, 95% CI 1.46, 28.52), embryonic cardiac activity (OR 14.29, 95% CI
2.95, 76.92), and crown- rump length of >10 mm (OR 13.33, 95% CI 1.46,
120.48) was considered to be associated with a higher unsatisfactory rate
of primary MTX treatment. A concomitant feticide was found to enhance the
therapeutic effect of MTX treatment if embryonic cardiac activity was
evident (OR 0.13, 95% CI 0.02, 0.68). Administration of a high dose of MTX
did not seem to be more effective than a lower one. Our findings supported
some previous observations and, more importantly, provided useful clinical
information in selecting appropriate candidates for MTX treatment in cases
of cervical pregnancy.
相似文献