Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hereditary hemorrhagic telangiectasia (HHT) or Rendu-Osler-Weber disease is a genetic disorder with autosomal dominance and variable penetrance, characterized by epistaxis, telangiectasia and visceral manifestations of the disease. The estimated minimal prevalence is 1/10,000 inhabitants. The diagnosis is established on clinical criteria, and may be further confirmed by the identification of causative mutations in either the ENG or the ACVRL1 gene coding for endoglin and ALK1, respectively. Pulmonary vascular manifestations of HHT include pulmonary arteriovenous malformations (PAVMs; especially in patients with ENG mutations) and less frequently pulmonary hypertension (especially in patients with ACVRL1 mutations). In 15-33% of patients with HHT, PAVMs consist of abnormal communications between pulmonary arteries and pulmonary veins, causing right-to-left shunting, and thus, frequently hypoxemia and dyspnea on exertion, although PAVMs may remain asymptomatic and frequently undiagnosed unless complications occur. PAVMs result in severe and frequent complications often at a young age, which may reveal the diagnosis, e.g. transient ischemic attack and cerebral stroke (10-19% of patients), systemic severe infections and abscesses (including cerebral abscess in 5-19% of patients), and rarely massive hemoptysis or hemothorax. Infections in HHT are related to the right-to-left shunting that bypasses the pulmonary capillaries and facilitates the passage of septic or aseptic emboli into the systemic and especially cerebral circulation, and potentially to minor defects in innate immunity. Treatment of PAVMs based on transcatheter coil vaso-occlusion of the feeding artery significantly decreases right-to-left shunting, hypoxemia and dyspnea on exertion, and reduces the risk of systemic complications. Long-term follow-up is warranted after transcatheter vaso-occlusion of PAVMs due to frequent recanalization of treated PAVMs and development or growth of untreated PAVMs. Patients with HHT should be informed of the risk of PAVM and potentially severe complications occurring in heretofore asymptomatic subjects. All adult patients with HHT should be proposed systematic screening for PAVM, by contrast echocardiography (preceded by anteroposterior chest radiograph) or computed tomography of the chest. Pulmonary hypertension is rare in HHT, and may be due either to systemic arteriovenous shunting in the liver increasing cardiac output or be clinically and histologically indistinguishable from idiopathic pulmonary arterial hypertension. Pulmonary hypertension is detected by systematic examination of right cardiac cavities and tricuspid regurgitation flow at echocardiography, and the diagnosis is established by right heart catheterization.
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PMID:Pulmonary vascular manifestations of hereditary hemorrhagic telangiectasia (rendu-osler disease). 1764 82

Atorvastatin has been extensively studied in the primary and secondary prevention of cardiovascular events, and may have some clinical advantages over various other statins in these respects. The principal primary prevention study of atorvastatin, ASCOT-LLA (Anglo-Scandinavian Cardiac Outcomes Trial-Lipid Lowering Arm), revealed that atorvastatin reduced the relative risk of primary coronary heart disease (CHD) events by 36% (p = 0.0005) compared with placebo in patients with hypertension. Much published data confirm the secondary preventive benefits of atorvastatin in various clinical settings. The IDEAL (Incremental Decrease in End Points Through Aggressive Lipid Lowering) and TNT (Treating to New Targets) trials demonstrate the preventive efficacy of atorvastatin in patients with stable CHD. Relative to simvastatin (in the IDEAL trial) and low-dosage atorvastatin (in the TNT trial), intensive atorvastatin therapy (80 mg/day) reduced the risk of nonfatal myocardial infarction (MI) by 17-22% (p < or = 0.02). Furthermore, the ALLIANCE (Aggressive Lipid-Lowering Initiation Abates New Cardiac Events) and GREACE (GREek Atorvastatin and Coronary-heart-disease Evaluation) trials highlight the benefits of atorvastatin in the 'real world' setting in patients with stable CHD. Compared with 'usual' care, atorvastatin reduced the risk of nonfatal MI by 47-59% (p < or = 0.0002).Moreover, the MIRACL (Myocardial Ischemia Reduction with Aggressive Cholesterol Lowering), PROVE-IT (PRavastatin Or atorVastatin Evaluation and Infection Therapy) and IDEAL-ACS (Acute Coronary Syndromes) studies outline the benefits of high-dosage atorvastatin therapy started within 24-96 hours, 10 days or 2 months, respectively, of an acute coronary syndrome. Relative to placebo, pravastatin and simvastatin, atorvastatin reduced the risk of death or major cardiovascular events by 16-18% (p < or = 0.048). In patients undergoing revascularisation procedures, the AVERT (Atorvastatin VErsus Revascularisation Treatment) study revealed that 18 months' administration of atorvastatin 80 mg/day was at least as effective as angioplasty plus usual care in reducing the risk of ischaemic events in low-risk patients with stable coronary artery disease. Furthermore, the ARMYDA (Atorvastatin for Reduction in MYocardial DAmage during angioplasty) and ARMYDA-3 trials showed that 7 days' administration of atorvastatin 40 mg/day before coronary intervention significantly reduced the risks of periprocedural myocardial damage (ARMYDA), postprocedural MI (p = 0.025; ARMYDA) and atrial fibrillation (p = 0.003; ARMYDA-3) versus placebo. In addition, it has been reported that C-reactive protein levels and the combined incidence of cardiovascular events (death, MI and target segment revascularisation during the 6-month follow-up) were significantly higher in coronaropathic patients undergoing non-surgical revascularisation procedures (stent implantation) not receiving statin therapy compared with those treated with atorvastatin (80mg). Overall, therefore, the marked efficacy of atorvastatin in the primary and secondary prevention of cardiovascular events underscores the pivotal place that this statin has in general cardiovascular disease management, and suggests even greater potential clinical utility for the drug in some clinical settings.
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PMID:Atorvastatin efficacy in the primary and secondary prevention of cardiovascular events. 1791 May 19

We describe a 66-year-old woman hospitalized with fever, fatigue and hepatopathy. In her medical history arterial hypertension (treated with propranolol and lisinopril), diabetes mellitus type 2 (no treatment before admission) and a gout arthropathy were noted wherefore a therapy with allopurinol 300 mg per day has been started 4 months before. Liver biopsy revealed fibrin-ring granulomas, compatible with allopurinol-induced hepatitis. Because of persistence of high fever after stopping allopurinol, steroids (1 mg/kg) were started. Under this treatment, she developed pancytopenia and fever. The bone marrow aspiration revealed Leishmania infantum. A second liver biopsy showed amastigotes and a disappearance of the granulomas. The history revealed a travel to Malta 2 years earlier. Despite adequate treatment with liposomal amphotericin B the patient deteriorated and finally died in septic shock.
Infection 2008 Aug
PMID:Hepatitis with fibrin-ring granulomas. 1792

HCV infection may be related to many extrahepatic manifestations including mixed cryoglobulinemia (MC). Clinical manifestations commonly associated to MC include arthralgia, purpura, vasculitis, peripheral neuropathy and renal function abnormalities. Treatment with interferon often leads to remission, especially in virological responders, or to disappearance of MC-related clinical manifestations. We report on a patient with chronic hepatitis C, deficit of G6P-DH, type II MC, who developed a cryoglobulinemic vasculitis with purpura, renal impairment and arterial hypertension, during treatment with PEG-interferon a-2b plus amantadine. The occurrence of purpuric lesions and MC-related nephropathy with increased cryocrit despite negative viremia, in a patient previously asymptomatic, during interferon treatment, is unusual.
Infection 2008 Jun
PMID:Cryoglobulinemia-related vasculitis during effective anti-HCV treatment with PEG-interferon alfa-2b. 1796 5

A 97-year-old lady was hospitalized for left leg cellulitis. Comorbidity included hypertension and congestive heart failure. While in hospital, she developed a painless vesicular rash localized to the territory of the left trigeminal nerve (third branch), which evolved to pustules and crusts (Figure 1). A chickenpox-like disseminated eruption of vesicles followed within 4 days, with the same evolution pattern (Figure 2).The diagnosis of disseminated zoster was suspected. A PCR analysis confirmed the presence of varicella-zoster-virus (VZV) in an abdominal vesicle. The patient was treated with oral valacyclovir for 7 days. Clinical examination, laboratory tests (including HIV serology), and a chest radiograph revealed no evidence of underlying immunodeficiency or malignancy.
Infection 2009 Apr
PMID:Disseminated zoster in an elderly patient. 1916 30

Given the increasing numbers of patients requiring long-term hemodialysis, there is an inevitably increasing population of patients with occluded central venous inflow (subclavian, innominate, and caval) despite access or access possibilities in the arm. In an effort to avoid sternotomy, we have attempted to treat these patients with a substernally tunneled subclavian to right atrial bypass. Patients treated in this fashion have an existing fistula with symptomatic venous hypertension or good fistula options but complete central vein obstruction, a patent subclavian/axillary vein to the costoclavicular junction, and no other options in the contralateral arm. Claviculectomy is performed and the subclavian vein isolated. Through a third intercostal space "minipericardiotomy," the right atrial appendage is exposed. A retrosternal tunnel is fashioned, and bypass is performed from the subclavian vein to atrial appendage. Eleven patients aged 20-70 (mean 46) years underwent surgery at our institution between February 2004 and March 2007. Three bypasses were performed with autogenous vein (two femoral and one saphenous), while eight were performed with polytetrafluoroethylene in an effort to preserve the superficial femoral vein for later leg bypass. There was one early mortality due to sepsis, and early morbidity was limited to one patient with a symptomatic pericardial effusion. Mean follow-up was 16 (range 3-43) months. Sixty-seven percent and 33% of arteriovenous fistulas remained functional at 6 and 10 months, respectively; and one patient's fistula remained functional at 21 months. Four patients (36%) developed central bypass stenosis or occlusion, one requiring a redo bypass and three angioplasty. Infection occurred in two patients (18%), with removal of autogenous vein graft in one. While a significant number of these bypasses fail, upper extremity access is maintained in a reasonable number of patients (67% at 6 months) who are not candidates for local repair or stenting and would thus have no other upper extremity access options. This technique offers an alternative to sternotomy and brachiocephalic vein reconstruction, although the superiority of one method over the other will require direct comparison.
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PMID:Subclavian vein to right atrial appendage bypass without sternotomy to maintain arteriovenous access in patients with complete central vein occlusion, a new approach. 1935 37

It is a retrospective study of descriptive type on a 4 years period, from April 1, 1999 to March 31, 2003. The aim was to determine factors bound to morbidity and mortality of renal affections in the Conakry University Hospital Center Nephrology Unit. The study was based on 606 hospitalized patients of whom 21 dialysed. The study's references were age, sex, renal affections frequency, mortality, associated pathologies, hospitalization period, death hours and other factors of cardio-vascular risks (tobacco, alcohol). Patients having answered to the selection criteria were 365 men (60.23%) and 241 women (39.77%) with a sex ratio of 1.51. The average age was 44 +/- 17 years old with extremes of 15 and 95 years old; 16.34% of the patients were aged less than 25 years and 14.03% were more than 65 years old. According to the charge taking, 462 (76.24%) were at their neighbors' charge, only 144 (23.76%) could take themselves in charge for their medical care. According to the received treatment before hospitalization, 357 had consumed decoctions of leaves and roots, 86 consulted a health center. The average period of hospitalization was 13 +/- 9 days with extremes of 1 and 80 days. Nicotine addiction was observed with 183 patients of whom 181 were men and alcoholism with 134 patients of whom 122 were men. Renal affections were chronic renal failure (51%), arterial hypertension (30.36%), chronic kidney disease (8.09%), intense renal failure (7.59%), urinary infections (1.65%), intense kidney disease (0.99%) and kidney cancer (0.33%). Among them, 130 deaths were observed (21.45%). According to the period going on before the medical check up, 24 death occurred 2 weeks after the first symptom, and 106 after more than a month. Considering the hours, 33 death (25.38%) occurred between 8 a.m. and 4 p.m. and 63 deaths (48.47%) between 4 p.m. and 8 a.m.; in 34 cases, the hour was not specified. Mortality was due to chronic renal failure in 97 cases (74.61%), to arterial hypertension in 19 cases (14.62%) and to other affections in 14 cases (0.77%). Infections, diabetes, arterial hypertension and anemia sickle cells were renal risk factors. Morbidity and mortality factors were numerous and varied: medical check up delay, traditional cure, patients 'weak turnover, lack of medical care, lack of required equipment and the absence of popular health education.
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PMID:[Renal diseases--morbidity and mortality in Nephrology Service, National Hospital Donka]. 1943 46

Infection is the second most common cause of mortality in patients with end stage re-nal disease (ESRD). Following strict aseptic precautions during a hemodialysis (HD) session could reduce dialysis-related infection, thereby reducing mortality and morbidity rates. This retrospective study was undertaken to identify the prevalence of dialysis-related bacteremia, sepsis, and catheter infections during HD at Bahrain Specialist Hospital, Bahrain, after following rigid infection control procedures. All HD sessions performed between January 2004 and December 2007 were included. Strict aseptic precautions were observed for every patient in our dialysis unit. The patients' demographic characteristics as well as presence of hypertension (HTN), diabetes mellitus (DM) and use of immunosuppressive drugs were recorded. Results of culture of dialysis catheter tip were collected for all catheters removed or changed during the study period. Catheter surface culture yielding more than 15 colonies and catheter lumen culture yielding more than 1000 CFU/mL were considered positive. All episodes of rigors, chills, bacteremia, and sepsis were recorded. Overall, a total of 1084 HD sessions performed on 46 patients were studied. The mean age of the study patients was 55.2 years (SE 2.5). Fifty four percent were male, 50% had DM, 85% had HTN and 11% were immunosuppressed. With implementation of strict aseptic precautions no catheter-related infection, bacteremia or sepsis was found. Culture of 50 dialysis catheters showed Diptheroid in three patients, MRSE in two patients and MSSE, Enterobacter, and Klebsiella in one patient each. None of the study patients had signs or symptoms of infection or bacteremia. Our study further indicates that following strict aseptic precautions during HD sessions can reduce, if not eliminate, infection as a major cause of mortality and morbidity.
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PMID:Infection-free hemodialysis: can it be achieved? 1958 20

The haemolytic uraemic syndrome (HUS) includes the triad of haemolytic anaemia, thrombocytopenia, and acute renal failure. The classical form [D(+) HUS] is caused by infectious agents, and it is a common cause of acute renal failure in children. The enterohaemorrhagic Escherichia coli-producing Shiga toxin (Stx) is the most common infectious agent causing HUS. Other infectious agents are Shigella and Streptococcus pneumoniae. Infections by S. pneumoniae can be particularly severe and has a higher acute mortality and a higher long-term morbidity compared to HUS by Stx. Atypical HUS [D(-)Stx(-)HUS] are often used by paediatricians to indicate a presentation of HUS without preceding diarrhoea. Almost all patients with D(-)Stx(-)HUS have a defect in the alternative pathway, for example, mutations in the genes for complement factor H, factor I, and membrane co-factor protein. Mutations in the factor H gene are described more often. The majority of children with D(+) HUS develop some degree of renal insufficiency, and approximately two thirds of children with HUS will require dialysis therapy, while about one third will have milder renal involvement without the need for dialysis therapy. General management of acute renal failure includes appropriate fluid and electrolyte management, antihypertensive therapy, and the initiation of renal replacement therapy when appropriate. Specific management issues in HUS include management of the haematological complications of HUS, monitoring for extra-renal involvement, avoiding antidiarrhoeal drugs, and possibly avoiding of antibiotic therapy. In addition to the obligatory supportive treatment and tight control of hypertension, there is anecdotal evidence that plasma therapy may induce remission and, in some cases, maintain it. Fresh frozen plasma contains factor H at physiological concentrations. A new therapy for D(-)Stx(-)HUS is a humanised monoclonal antibody (Eculizumab) that blocks complement activity by cleavage of the complement protein C5. It prevents the generation of the inflammatory peptide C5a and the cytotoxic membrane-attack complex C5b-9. We have first positive results, but it is still not approved for HUS.
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PMID:Clinical practice. Today's understanding of the haemolytic uraemic syndrome. 1970 87

Chronic kidney disease (CKD) is a major public health problem and can result in end-stage renal disease with need for dialysis or transplantation. In Europe up to 12% of the adult population had some renal impairment, while in the United States the end stage of CKD has increased dramatically from 209.000 in 1991 to 472.000 in 2004. Diabetes and hypertension are major causes of kidney pathology. Infection, particularly ascending infection, is more common with increasing age, as both immune function declines and associated pathology predisposing to infection, such as obstructive uropathy, becomes more common. Most pathological changes in the kidney appear to be initiated by oxidative stress, followed by an inflammatory reaction. Oxidative stress results from an imbalance between free radicals and their detoxification by endogenous and exogenous scavengers, including polyunsaturated fatty acids (PUFA). Recent studies showed that PUFA supplementation slowed the rate of loss of renal function in patients with IgA nephropathy. Then, studies of omega-3 supplementation in dialysis patients describe salutary effects on triglyceride levels and dialysis access patency. We examined the relationship between total plasma PUFA levels and change in creatinine clearance over a three-year follow-up in the older persons enrolled in the InCHIANTI study, a population-based epidemiology study conducted in Tuscany, Italy. This study showed that older adults with low total plasma PUFA levels have a greater decline in creatinine clearance over three years of follow-up. These findings suggest that a higher dietary intake of PUFA may be protective against progression to chronic kidney disease.
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PMID:Omega-3 and renal function in older adults. 2004 16


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