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At least 307 records · Page 17Linked to original sources
PubMed2003

Abnormalities in the cerebral arterial system in osteogenesis imperfecta.

A 9-year-old girl with osteogenesis imperfecta (OI) type 4 was admitted to the hospital because of choreatic movement. Cerebral angiography demonstrated bilateral symmetrical stenosis of the proximal cavernous segment of internal carotid artery, prethrombotic occlusion of the M1 segment of left middle cerebral artery, and moyamoya-like collateral vascular structure arising from the right lenticulostriate arteries. After a literature review, these findings were interpreted as vasculopathic changes secondary to vascular fragility caused by the collagen abnormality in OI.

Capillary Fragility↗
PubMed1991

Studies in red blood cell preservation: 4. Plasma vesicle hemoglobin exceeds free hemoglobin.

Studies were designed to find out how much of the plasma hemoglobin ( Hb) in whole blood was in microvesicles and how much was free Hb after 21 days of storage in citrate-phosphate-dextrose anticoagulant and to determine the effect of the plasticizer, di-(2-ethylhexyl)phthalate (DEHP). The total plasma Hb in polyolefin (PO) containers without DEHP was much higher than in polyvinyl chloride (PVC) with the plasticizer (p = 0.004). Less than 30% of the Hb was in free solution in either type of container. The addition of 300 micrograms/ml of DEHP to the plasma in the PO containers resulted in marked reduction in the microvesiculation (p less than 0.01) but did not affect the level of free Hb. RBC hypotonic fragility and morphology scores were significantly improved. It is concluded that microvesiculation contributes more to plasma Hb concentration than free Hb during storage. Some hemolysis of red blood cells (RBC) is expected during blood bank storage. It has been shown that part of the hemoglobin (Hb) in the suspending medium is free and part is encapsulated in microvescicles shed by the RBC [1]. The amount of hemolysis and microvesiculation that occurs has been noted to be less when blood is stored in polyvinyl chloride (PVC) containers in which di-(2-ethylhexyl)phthalate (DEHP) is used as the plasticizer [1]. The DEHP that leaches into the plasma has been shown to decrease hemolysis, microvesiculation, and the increase in osmotic fragility which RBC undergo during refrigerated storage [2-9].(ABSTRACT TRUNCATED AT 250 WORDS)

Blood Preservation↗
PubMed2002

Standardization of PCR amplification for fragile X trinucleotide repeat measurements.

To provide the clinical diagnostics community with accurate protocols and measurements for the detection of genetic disorders, we have established a quantitative measurement program for trinucleotide repeats associated with human disease. In this study, we have focused on the triplet repeat associated with fragile X syndrome. Five cell lines obtained from the Coriell Cell Repository were analyzed after polymerase chain reaction (PCR) amplification and size separation. These cell lines were reported to contain CGG repeat elements (ranging from 29 to 110 repeats). Our initial measurements focused on measurement variability: (a) between slab-PAGE and capillary (CE) separation systems (b) interlane variability (slab-PAGE) (c) intergel variability, and (d) variability associated with amplification. Samples were run in triplicate for all measurements, and the analysis performed using Gene Scan analysis software. The repeat sizes were verified by DNA sequence analyzes. The standard deviations for interlane measurements in slab-gels ranged from 0.05 to 0.35. There was also little variation in size measurements performed on different gels and among PCR amplifications. The CGG repeat measurements performed by capillary electrophoresis were more precise, with standard deviations ranging from 0.02 to 0.29. The slab-PAGE and CE size measurements were in agreement except for the pre-mutation alleles, which yielded significantly smaller sizes by CE.

Alleles↗
PubMed2000

Emergency repair of type A aortic dissection in type IV Ehlers-Danlos syndrome.

Ehlers-Danlos syndrome type IV is a distinctive syndrome in which thin and fragile skin, premature ageing, bruising and scarring are combined with lethal or life-threatening arterial weakness. Aortic rupture either at the aortic root and arch, or sometimes lower down the artery, are particularly characteristic. Even quite minor injury can produce dangerous vascular tearing and damage. Technical difficulties encountered in arterial repair or venous ligature are particularly worrying. The authors report the treatment of a ruptured type A aortic dissection associated with Ehlers-Danlos syndrome where the extreme fragility of the tissues and tendency to bleed posed a difficult task for the surgeon.

Adult↗
PubMed2000

Rapid thawing increases the fragility of the cryopreserved arterial wall.

OBJECTIVE: To extend present knowledge of the biomechanical and structural changes which occur in the cryopreserved, rapidly thawed arterial wall. MATERIALS AND METHODS: Minipig iliac arterial segments were cryopreserved at -196 degrees C in either minimum essential medium or Wisconsin solution. Fresh segments served as the control group. After 1 month, the specimens were rapidly thawed (37 degrees C) and processed for biomechanical, ultrastructural, morphological and immunohistochemical (MMP-1, MMP-2, MMP-3 and MMP-9) analysis. Visualisation of apoptotic cells was performed by TUNEL method. For the mechanical distension analysis, an in vitro circuit was designed. RESULTS: The cryopreserved segments showed a 42% incidence of spontaneous fracture and the appearance of microfractures which affected the endoluminal third of the vessel. An accumulation of liquid in the subelastica was observed. An increased expression of wall-degradative enzymes (mainly MMP-2) was also observed following cryopreservation. No significant differences were detected in the proportional elasticity module or tensile strength of the specimen groups. No differences in mechanical distension were observed between groups after the vessel segments were subjected to the pulsatile circuit flow for 72 h. Cell damage was most intense in the specimens cryopreserved in Wisconsin solution. CONCLUSIONS: Cryopreservation in both the solutions employed, followed by rapid thawing, induce changes in the permeability which increase the fragility of the cryopreserved arterial wall. Both increased expression of wall-degradative enzymes and accumulation of liquid may contribute to graft failure after implantation.

Adenosine↗
PubMed2000

Polycystin 1 is required for the structural integrity of blood vessels.

Autosomal dominant polycystic kidney disease (ADPKD), often caused by mutations in the PKD1 gene, is associated with life-threatening vascular abnormalities that are commonly attributed to the frequent occurrence of hypertension. A previously reported targeted mutation of the mouse homologue of PKD1 was not associated with vascular fragility, leading to the suggestion that the vascular lesion may be of a secondary nature. Here we demonstrate a primary role of PKD1 mutations in vascular fragility. Mouse embryos homozygous for the mutant allele (Pkd1(L)) exhibit s.c. edema, vascular leaks, and rupture of blood vessels, culminating in embryonic lethality at embryonic day 15.5. Kidney and pancreatic ductal cysts are present. The Pkd1-encoded protein, mouse polycystin 1, was detected in normal endothelium and the surrounding vascular smooth muscle cells. These data reveal a requisite role for polycystin 1 in maintaining the structural integrity of the vasculature as well as epithelium and suggest that the nature of the PKD1 mutation contributes to the phenotypic variance in ADPKD.

Animals↗
PubMed1981

Inhibition of thrombocytopenic petechiae by exogenous serotonin administration.

The effect of serotonin on vascular fragility, visible as petechiae, was examined in antiplatelet serum-induced thrombocytopenic hamsters. Serotonin was administered intravenously or intraperitoneally, and following a single injection of 0.5 mg/100 g body weight, a temporary inhibition of petechial formation for approximately 1 h was observed. Repeated serotonin injections maintained animals free of petechiae for the 4-hour period of administration. Plasma levels of serotonin and the effect of serotonin on cutaneous perfusion were not significant factors in the observed inhibition of petechiae. The data suggest that in normal animals platelet serotonin may contribute more to maintaining microvascular structural integrity than platelet adhesion.

Animals↗
PubMedDate not supplied

[Vascular hyperfragility in systemic lupus erythematosus treated with low doses of cortisone].

We report an unusual cutaneous manifestation of systemic lupus erythematosus (SLE) in a 15-year old female. The diagnosis was made on the basis of clinical symptoms, cutaneous hystology (positive "lupus band test") and on laboratory findings (hypocomplementemia, positive antinuclear antibodies and rheumatoid factor). Treatment with methylprednisolone (0.5 mg/kg/die) improved the clinical symptoms but, after 2 months, large ecchymotic lesions appeared on the lower legs below the knee extending as far as the ankles, likely triggered by minor local traumas. Coagulative function was normal, the lupic anti-coagulant factor (LAF) was negative, anticardiolipin antibodies were absent and there was no thrombocytopenia. There was only a slight increase in clotting times in vitro, in presence of ADP. The amount of cortisone was reduced and the type of treatment modified; satisfactory control of the disease was attained with deflazacort (0.3 mg/kg/die). The ecchymosis on the lower limbs never disappeared even though they became slightly smaller. Ecchymotic lesions are not usually included in the wide range of cutaneous manifestations associated with SLE. Moreover vascular fragility resulting from pressure and minor traumas is known to be a cutaneous complication of hypercorticism; nevertheless the doses of cortisone administered to this patient were rather low and other clinical signs of steroid hyper-dosing were absent although cortisolemia assay at base and after stimulus with ACTH was not performed. We would suggest that the negligible platelet binding defect (whether primary or SLE-associated) together with the low amounts of cortisone administered caused ecchymotic lesions to appear in this patient suffering from a disease (SLE), in which the small cutaneous vessels are favourite targets.

Adolescent↗
PubMed1997

Pathogenesis, early diagnosis, and prophylaxis for chronic hemophilic synovitis.

The most common clinical manifestation of hemophilia is intraarticular bleeding (hemarthrosis). Although any joint can be involved, the articulations most frequently affected are the knees, elbows, and ankles. Hemorrhages may occur spontaneously, or as a result of trauma. Because hemophilia is a genetically determined coagulation disorder, the bleeding incidences may start early in a child's life. After several hemarthroses, hemophilic synovitis will develop and, because of the profusion and fragility of vessels within the tissue, will lead to a vicious cycle of bleeding-synovitis-bleeding. If the synovitis is not controlled, cartilage damage will occur. hemophilic arthropathy then will become apparent clinically and radiographically, causing functional impairment of the affected joint. Prophylaxis and early diagnosis of acute hemarthrosis are essential to prevent the development of hemophilic synovitis. Hemarthroses must be treated aggressively if hemophilic synovitis is to be prevented. Continuous prophylaxis from age 2 to 18 years has been claimed to reduce the incidence of chronic hemophilic synovitis and joint damage, which remain the main causes of disability in the life of the patient who has hemophilia.

Adolescent↗
PubMed2000

Clinical implications of disturbances of uterine vascular morphology and function.

Menstrual disturbances are one of the most common problems presenting to the gynaecologist. In order for the endometrium to bleed, vessels must break down. Disruption in the regulation of endometrial vascular growth and function has been found in association with spontaneous and sex steroid-induced disturbances of menstrual bleeding. Although circulating oestrogens and progestogens influence the endometrial vessels, this effect appears to be indirect, and regulation is primarily via local factors. Deficient vasoconstriction and haemostasis with excessive fibrinolysis is seen in menorrhagia. Breakthrough bleeding in users of progestogen-only contraceptives is associated with increased superficial vascular fragility and disruptions in the supporting basement membrane. Blood vessels in uterine fibroids are abnormal in distribution and appearance. Adenomyosis is also commonly associated with menstrual disturbance, and alterations in vascular distribution suggest altered angiogenesis. Successful human embryo implantation requires endometrial vascular breakdown. Excessive thrombosis associated with the antiphospholipid syndrome may interfere with this re-modelling and compromise implantation. Arteriovenous malformations are a rare but important cause of excessive or irregular vaginal bleeding. Abundant vessels with abnormal morphology, associated with aberrant angiogenesis can be seen, and embolization of these vessels may be an effective conservative treatment. Improved understanding of the regulation of the uterine vasculature is likely to lead to targeted therapies to prevent unscheduled vascular breakdown and to control menstrual disturbance at an endometrial level.

Arteriovenous Malformations↗
PubMed2001

Plastic microchip electrophoresis for genetic screening: the analysis of polymerase chain reactions products of fragile X (CGG)n alleles.

Clinical screening of abnormal chromosomes associated with fragile X syndrome (FXS) demands a high-throughput method including DNA sizing and detection of the amplified products. This study is to explore the use of polymer microchip electrophoresis for the analysis of polymerase chain reaction (PCR) products of fragile X (CGG)n alleles to facilitate a fast exclusion test of FXS. The sequences flanking the CGG-repeat of FMR1 gene was amplified by betaine-PCR and the amplified products were desalted and then analyzed by microchips which were fabricated on poly(methyl methacrylate) (PMMA) substrate. The PCR bands with more than six CGG-repeats in difference could be clearly distinguished in less than 3 min by microchip electrophoresis with a separation length of 6 cm. It was found that the signal was greatly enhanced with the use of both covalent (Cy5) and intercalating dye (TORRO-3), which has never been demonstrated before. We tested the method by reanalysis of twelve samples from males and six samples from females. For female samples with less than six repeat differences, Southern blotting method was performed to confirm or exclude the findings from microchips. It was found that the test results from all male and female samples show a 100% correlation between the microchip electrophoresis and the existing methods.

Electrophoresis, Capillary↗
PubMed2005

Dotlike hemosiderin spots are associated with past hemorrhagic strokes in patients with lacunar infarcts.

BACKGROUND AND PURPOSE: Dotlike hemosiderin spots ongradient-echo T2(*)-weighted magnetic resonance imaging of the brain have been histologically diagnosed as old microbleeds associated with small vessel disease (SVD). The authors hypothesize that the presence of many dotHSs may be correlated with the fragility of small vessels and the recurrence of SVD, including lacunar infarction and deep intracerebral hemorrhage (ICH). METHODS: To investigate how dotHSs are related to past history of SVD, the number of subcortical or deep dotHSs was investigated in 146 patients with lacunar infarctions (95 men, 51 women, age 38 to 90 [66.6+/-9.4] years). They were divided into 2 subgroups according to history of deep ICHs or lacunar infarctions. The odds ratio (OR) for past history was estimated from logistic regression analyses with the number of subcortical or deep dotHSs as well as other factors. RESULTS: Of 146 patients with lacunar infarctions, 11 had past symptomatic ICHs and 19 had past symptomatic lacunar infarctions. An elevated rate of history of ICH was found for lacunar infarction patients with many deep dotHSs (>or=3; OR, 9.1; 95% confidence interval, 1.6-51, P=.015). However, history of lacunar infarction was not significantly associated with the number of subcortical or deep dotHSs. CONCLUSIONS: Our findings suggest that many deep dotHSs on T2(*)-weighted magnetic resonance imaging may be correlated with deep ICH-lacunar infarction type of SVD recurrence but not lacunar infarction-lacunar infarction type.

Adult↗
PubMed2005

Clinical study on the effect of Shengxueling on idiopathic thrombocytopenic purpura.

OBJECTIVE: To observe the clinical effect of Shengxueling (SXL) on idiopathic thrombocytopenic purpura (ITP), and study the possible mechanism. METHODS: Eighty-six cases of ITP were randomly divided into two groups. The SXL group, 56 patients treated with SXL, a traditinal Chinese medicine and 30 patients administered with prednisone were taken as control. Each group took drugs for 3 months and was under follow-up observation. RESULTS: In the SXL group, the total effective rate was 85.71%, similar to prednisone 83.33% (P > 0.05) for 3 months, but the total effective rate of SXL (91.07%) were obviously better than that of the control group (53.33%) (P < 0.01) for 6 months and had no obvious adverse reaction. The patients bleeding was alleviated or stopped, the general condition was improved. At the same time, blood platelet count (PLT) was increased, platelet associated immunoglobulin (PAIg) and interleukin-4 (IL-4) were markedly dropped, the level of natural killers cells activity (NKa) increased, the rate of T lymphocyte subsets gradually returned to normal level. Megakaryocyte tended to maturation on bone marrow smear after treatment. All differences above were statistically significant. CONCLUSION: SXL is an effective and safe medicine for ITP. Its mechanism could regulate cytoimmune, inhibit platelet antibody to reduce the destruction of platelet, increase the number of platelet, promote the division and maturation of megakaryocyte, facilitate the production and release of platelet, lower the fragility of capillary, prevent and cure hemorrhagic tendency.

Adolescent↗