Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038454 (stroke)
 147,016 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We conducted a retrospective analysis of 37 children with Escherichia coli O157:H7-associated hemolytic-uremic syndrome. The infection was traced to contaminated hamburgers at a fast-food restaurant chain. Within 5 days of the first confirmed case, the Washington State Department of Health identified the source and interrupted transmission of infection. Ninety-five percent of the children initially had severe hemorrhagic colitis. Nineteen patients (51%) had significant extrarenal abnormalities, including pancreatitis, colonic necrosis, glucose intolerance, coma, stroke, seizures, myocardial dysfunction, pericardial effusions, adult respiratory disease syndrome, and pleural effusions. Three deaths occurred, each in children with severe multisystem disease. At follow-up two children have significant impairment of renal function (glomerular filtration rate < 80 ml/min/per 1.73 Hm2); both of these children have a normal serum creatinine concentration. Hemolytic-uremic syndrome is the most common cause of acute renal failure in children, and this experience emphasizes the systemic nature of this disease. Clinicians should anticipate that multisystem involvement may occur in these patients, necessitating acute intervention or chronic follow-up. This outbreak of Hemolytic-uremic syndrome also highlights the microbiologic hazards of inadequately prepared food and emphasizes the importance of public health intervention in controlling Hemolytic-uremic syndrome.
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PMID:Escherichia coli O 157:H7-associated hemolytic-uremic syndrome after ingestion of contaminated hamburgers. 793 69

Hemolytic uremic syndrome (HUS) can be clinically classified into two types: typical cases with a diarrheal prodrome of association with E. coli O157, and atypical cases without antecedent diarrhea. However, HUS is not common in Taiwan. To evaluate the clinical course, complications and outcome of HUS in children, and to identify the risk factors for mortality, retrospectively, seven cases of HUS in our hospital in the past 6 years were studied. Six of them were boys, and one was a girl. Their ages ranged from 0.67 to 3 years. None of them were preceded by diarrheal prodrome. Acute renal failure, hypertension and liver involvement were noted in all cases. Stroke and seizure developed in three of the cases with sequelae. Two cases progressed into end-stage renal disease (ESRD). One case developed acute respiratory distress syndrome (ARDS). Two cases (28.5%) expired. ESRD especially associated with ARDS was highly related to mortality.
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PMID:Clinical aspects of the hemolytic uremic syndrome. 982 77

Results of different studies conducted over the past 2 decades on the relationship between oral contraceptive (OC) use and venous thromboembolism, stroke, and myocardial infarct are summarized. The possibility that OCs would increase the risk of venous thrombosis was 1st raised by a case reported in 1961, and has been confirmed by at least 9 retrospective and 4 prospective studies in the UK, US, and Scandinavia. An increased risk of venous thrombosis has been confirmed only among women currently using OCs and possibly among those stopping use within 1-2 weeks. The risk is unrelated to duration of use, although few data are available on women using OCs continuously for more than 3 years. The proof of a relationship between risk of venous thrombosis and estrogen content is convincing, although an association with progestin content has also been suggested. Evidence is beginning to accumulate for an association between smoking and venous thrombosis in OC users and nonusers alike. British prospective studies have indicated a risk of death from venous thrombosis in OC users of 2-3/100,000 users/year during the 1970s, but modern low-dose formulations, better patient selection, and better surveillance have probably reduced the risk further. The evidence of a relationship between OC use and cerebral hemorrhage is only moderately convincing, with any increased risk unlikely to be more than 2-fold. The data regarding cerebral thrombosis are more consistent and convincing; they demonstrate a positive association of risk of cerebral thrombosis with both the estrogen and progestin content of OCs. Past as well as current users may be at increased risk, but data on the effect of duration of use are lacking. At least 9 retrospective and 2 prospective studies have established the significance of the risk of myocardial infarct in OC users. Risk of myocardial infarct may be related to both estrogen and progestin content, and appears to be limited to current users. Little evidence has been found of a relationship to duration of use. Strong evidence exists of a relationship between OC use and other risk factors for myocardial infarct, including smoking and hypertension. Very few deaths were observed from this cause in women under 35 in the 1970s. As with venous thrombosis, the mortality risks of stroke and myocardial infarct have probably declined appreciably in the past few years. OCs have been implicated in blood pressure elevations as well as a series of cardiovascular problems such as Budd-Chiari syndrome, occlusion of arteries in the intestines and extremities, and hemolytic uremic syndrome. The few available published studies suggest that administration of estrogens to peri- or postmenopausal women does not entail a cardiovascular risk.
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PMID:[Vascular disease and hormonal treatment--epidemiology]. 1228 Jan 97

We report a patient with relapsing hereditary hemolytic uremic syndrome (HUS) that began in the neonatal period with life-threatening jaundice and hemolytic anemia. He progressed to end-stage renal failure at 14 years of age and had a cerebrovascular accident while on dialysis. The cause of HUS was a constitutional deficiency in the von Willebrand factor cleaving protease. Hematological features of HUS significantly improved following bilateral nephrectomy. After renal transplantation, he had an early recurrence of HUS associated with two episodes of retinal and cerebral ischemia. Long-term treatment with fresh-frozen plasma exchanges prevented recurrence of HUS, cerebrovascular attacks, and early loss of the graft.
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PMID:Plasma therapy in von Willebrand factor protease deficiency. 1237 19

Despite chemotherapy, median survival of patients with advanced pancreatic cancer (APC) remains poor. Gemcitabine (GEM) remains standard treatment. Numerous phase II studies have suggested that combination therapies may improve response rates. Mitomycin C (MMC) when used as a single agent may have response rates comparable to other cytotoxic drugs. Therefore, MMC could be an interesting drug to be combined with GEM. This study aimed to assess the feasibility, toxicity and efficacy of GEM combined with MMC in patients with APC. Between April 1997 and January 2002, 55 consecutive patients were treated with GEM 800 mg/m2 i.v., days 1, 8 and 15, and MMC 8 mg/m2 i.v., day 1, every 4 weeks in an outpatient setting. Patient characteristics included: M/F 34/21, median age of 58 years, ECOG PS 0-2. A median of 3 cycles was administered. The most frequent toxicity was thrombocytopenia grade III/IV in 54% of patients. Ten patients experienced dyspnea+/-X-ray-proven pneumonitis (n=2). One of these patients developed a hemolytic uremic syndrome after the sixth application of MMC. There was one early death as a consequence of a stroke. The objective response rate was 29% (95% confidence interval: 17-43). Eighteen patients had stable disease resulting in an overall tumor growth control of 62%. Time to progression was 4.7 months and median overall survival was 7.25 months. We conclude that, except for thrombocytopenia, the combination of GEM and MMC is well tolerated. These results compare favorably to single-agent chemotherapy with GEM or the combination of 5-fluorouracil plus MMC. Furthermore, this regimen is cost-effective and, since it can be given on an outpatient basis, contributes to the quality of life.
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PMID:Gemcitabine and mitomycin C in advanced pancreatic cancer: a single-institution experience. 1520 99

The aim of the study was to analyze the etiology, the factors for progression of chronic renal failure to end-stage-renal disease (ESRD), and the influence of ESRD on the survival rate among a cohort of 59 heart transplant patients (HTP) referred for the management of chronic renal failure (CRF). At the time of the first nephrology consultation (6 +/- 4.25 years after cardiac transplantation) the mean creatininemia was 261.5 +/- 99 micromol/L and mean creatinine clearance (Cockcroft formula) was 32 +/- 15 mL/min. The cause of CRF were calcineurin inhibitor toxicity in 38.9% of patients, vascular events in 15.2%, hemolytic uremic syndrome in 5%, membranous glomerulopathy in 3.3%, diabetes in two patients, focal/segmental glomerulosclerosis in 3.3%, renal hypoplasia in 1.7%, and unknown in 27%. Evolution to ESRD occurred in 38.9% of patients: 17 patients started hemodialysis, three peritoneal dialysis, and two received a preemptive kidney transplantation. Creatininemia (micromol/L) at the time of nephrology referral was 229.2 +/- 72.6 versus 315.8 +/- 113.4 (P < .001) and creatinine clearance (mL/min) was 34.9 +/- 15.1 versus 27.3 +/- 13.7 (P = .049) for patients with CRF versus ESRD, respectively. Both proteinuria (g/24 hours) of 1 +/- 2.2 versus 2.3 +/- 1.8 (P = .02) and tobacco use in 35.1% versus 54.4% (P = .045) were significantly associated with progression of CRF, while age at the time of heart transplantation, cause of cardiac failure and renal failure, high blood pressure, type 2 diabetes, dyslipidemia, alcoholism, cirrhosis, and cerebral vascular accident were not. Death occurred in 18 HTP: 50% of patients with ESRD and 18.5% of patients with CRF-a 2.6 relative risk of of death in HTP patients with ESRD compared with HTP with CRF only (P < .01).
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PMID:Chronic renal failure and end-stage renal disease are associated with a high rate of mortality after heart transplantation. 1584 18

Arterial hypertension is a major risk factor for atherosclerotic ischemic heart disease and cerebrovascular stroke. Blood pressure measurements were taken in the supine position in 6,282 healthy adolescents (3073 boys, 3209 girls) aged 13-17 years of both sexes. Forty-eight subjects were found to have hypertension: 35 idopathic and 13 secondary to reflux nephropathy, chronic glomerulonephritis, coarctation of the aorta, and hemolytic uremic syndrome. Systolic blood pressure was significantly higher among males than females at ages 15-17 years (p<0.001); diastolic blood pressure was higher among males at age 17 years only (p<0.05). Males with BMI>17 exhibited higher systolic blood pressure than females. Prevalence of hypertension, mostly primary, in a large cohort of students was 0.76%. Early screening is important for early interventions and reducing cardiovascular morbidity and mortality in adulthood.
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PMID:Screening for hypertension in high school. 1621 Nov 96

Pathogenesis of thrombotic thrombocytopenic purpura (TTP) was a mystery for over half a century until the discovery of ADAMTS13. ADAMTS13 is primarily synthesized in the liver, and its main function is to cleave von Willebrand factor (VWF) anchored on the endothelial surface, in circulation, and at the sites of vascular injury. Deficiency of plasma ADAMTS13 activity (<10%) resulting from mutations of the ADAMTS13 gene or autoantibodies against ADAMTS13 causes hereditary or acquired (idiopathic) TTP. ADAMTS13 activity is usually normal or modestly reduced (>20%) in other forms of thrombotic microangiopathy secondary to hematopoietic progenitor cell transplantation, infection, and disseminated malignancy or in hemolytic uremic syndrome. Plasma infusion or exchange remains the initial treatment of choice to date, but novel therapeutics such as recombinant ADAMTS13 and gene therapy are under development. Moreover, ADAMTS13 deficiency has been shown to be a risk factor for the development of myocardial infarction, stroke, cerebral malaria, and preeclampsia.
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PMID:ADAMTS13 and von Willebrand factor in thrombotic thrombocytopenic purpura. 2558 50

Complement plays a key role in host defense, but its dysregulation can cause autologous tissue injury. Complement activation is normally controlled by regulatory proteins, including factor H (FH) in plasma and membrane cofactor protein (MCP) on the cell surface. Mutations in FH and MCP are linked to atypical hemolytic uremic syndrome, a type of thrombotic microangiopathy (TMA) that causes renal failure. We describe here that disruption of FH function on the cell surface can also lead to disseminated complement-dependent macrovascular thrombosis. By gene targeting, we introduced a point mutation (W1206R) into murine FH that impaired its interaction with host cells but did not affect its plasma complement-regulating activity. Homozygous mutant mice carrying this mutation developed renal TMA as well as systemic thrombophilia involving large blood vessels in multiple organs, including liver, lung, spleen, and kidney. Approximately 30% of mutant mice displayed symptoms of stroke and ischemic retinopathy, and 48% died prematurely. Genetic deficiency of complement C3 and factor D prevented both the systemic thrombophilia and renal TMA phenotypes. These results demonstrate a causal relationship between complement dysregulation and systemic angiopathy and suggest that complement activation may contribute to various human thrombotic disorders involving both the micro- and macrovasculature.
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PMID:Murine systemic thrombophilia and hemolytic uremic syndrome from a factor H point mutation. 2825 23

Hemolytic uremic syndrome (HUS) is often encountered in children with acute kidney injury. Besides the well-known shiga toxin-producing Escherichia coli-associated HUS, atypical HUS (aHUS) caused by genetic complement dysregulation has been studied recently. aHUS is a rare, chronic, and devastating disorder that progressively damages systemic organs, resulting in stroke, end-stage renal disease, and death. The traditional treatment for aHUS is mainly plasmapheresis or plasma infusion; however, many children with aHUS will progress to chronic kidney disease despite plasma therapy. Eculizumab is a newly developed biologic that blocks the terminal complement pathway and has been successfully used in the treatment of aHUS. Currently, several guidelines for aHUS, including the Korean guideline, recommend eculizumab as the first-line therapy in children with aHUS. Moreover, life-long eculizumab therapy is generally recommended. Further studies on discontinuation of eculizumab are needed.
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PMID:Atypical hemolytic uremic syndrome and eculizumab therapy in children. 2956 42


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