Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0085584 (encephalopathy)
 18,178 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

4 patients with hypertensive crisis (glomerulonephritis [n = 2], phaeochromocytoma [n = 1], reno-vascular hypertension [n = 1] combined with encephalopathy, showed a normalisation of blood-pressure up to 18 days during angiotensin-II-blockade with saralasin. Prior, blood pressure was treated insufficiently by intravenous diazoxide and Na-nitroprusside. Increased plasma-renin-activity and plasma levels of catecholamines pointed to an activation of the renin-angiotensin- and sympathico-adrenergic system. A trial of therapy with saralasin--especially, if blood-pressure response to diazoxide and sodium-nitroprusside is insufficient--could be indicated. Side-effects like pressor-reactions are excluded by very low priming doses (0,1 microgram/kg/min); rebound-hypertension at the end of the therapy is avoided by an overlapping therapy with renin suppressing drugs (beta-receptor blockers, clonidine, guanfacinum).
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PMID:[Saralasin in resistant hypertensive crisis (author's transl)]. 3 82

Pediatric cases of systemic lupus erythematosus with an unfavorable outcome (terminal renal failure requiring chronic hemodialysis, or death) assembled during a retrospective multicenter study of pediatric SLE in the Paris metropolitan area were analyzed. Seven patients (6 girls, 1 boy) were entered into a chronic hemodialysis program. Four had diffuse proliferative glomerulonephritis, the pattern of glomerular disease classically responsible for end-stage renal failure. The other three patients had membranous glomerulonephritis with active segmental lesions, a form of glomerulopathy whose severe prognosis deserves to be emphasized. Nine other patients (8 girls, 1 boy) died. In six patients, death occurred as a result of a flare with malignant hypertension and progressive renal failure (1 case), pancreatitis (1 case), encephalopathy (2 cases) or cardiomyopathy (2 cases). An infectious disease (tuberculosis, mumps) was apparently the cause of the two cases of encephalopathy. One girl died as a result of a hemorrhagic syndrome with a cerebral hematoma. Two other girls died at home. Overall, among 111 children with SLE 14% had an unfavorable outcome. Sex and age at onset seemed to have no bearing on prognosis. Patients with renal involvement were apparently more likely to have an unfavorable outcome. Lastly, although the influence of ethnic origin is unproven, children living in foreign countries of French overseas territories, but treated in France have an increased risk for unfavorable outcomes.
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PMID:[Unfavorable outcomes in disseminated lupus erythematosus in children. Cooperative study in the Paris region]. 192 11

We describe 11 cases of Systemic Lupus Erythematosus (SLE) with pediatric onset (10 females and 1 male). Mean age at onset was 10.9 years (range 3 to 16 years). Initial manifestations: cutaneous involvement in 7 cases, articular symptoms in 7 cases, renal involvement in 5 cases (proteinuria and/or microhematuria, or renal failure), pancytopenia in 3 cases. In 3 cases the onset of the disease was extremely sudden and severe: one patient had an intestinal infarct following mesenteric thrombosis associated with glomerulonephritis; another started with encephalopathy (deep coma, stage III); a third patient presented renal failure due to acute glomerulonephritis. At diagnosis all patients received systemic steroid therapy with the exception of one who had only a cutaneous involvement. The course of the disease is described. We underline that, in our series, it was rare for organs and systems, apart from the central nervous system, to be involved in exacerbations after initial onset of the disease. Six patients are presently asymptomatic or have only minor cutaneous and/or articular manifestations which are well controlled with low-dose cortisone therapy. Laboratory indices did not return to normal in any of the patients. In fact, in our series the disease doesn't appear to reach a complete remission, even many years after onset and no patient seems to be able to withdrawal the therapy at all. Our data confirm, according to other Authors, that the course of LES with paediatric onset is more severe than in adults.
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PMID:[Systemic lupus erythematosus: description of a pediatric series]. 868 1

The substitution of guanine for adenine at position 3243 of the leucine tRNA gene of mitochondrial DNA was originally described in association with MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes). Diabetes mellitus associated with the mutation (mitochondrial diabetes) is a different phenotype from MELAS. We identified 11 patients with the mutation among 385 Japanese diabetic patients: two had MELAS and nine had mitochondrial diabetes. We present data on a male patient with mitochondrial diabetes who developed the nephrotic syndrome at the age of 23. Light microscopy revealed mesangial expansion, PAS-positive deposits and segmental sclerosis in the glomeruli. Scattered mesangial electron-dense deposits and thickening of the basement membrane were found on electron microscopy, suggesting that diabetic glomerulosclerosis accompanied by focal glomerulosclerosis (FGS). Mitochondrial diabetes may pre-dispose patients to renal complications, including forms of glomerulonephritis, such as FGS.
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PMID:Renal complications in patients with diabetes mellitus associated with an A to G mutation of mitochondrial DNA at the 3243 position of leucine tRNA. 1046 41

The objective of the study was to determine the current epidemiology of infective endocarditis (IE) and to evaluate correlation of blood and valve cultures with complication rates of the disease. During 1999-2001 138 patients, 91 (65.9%) males and 47 (34.1%) females with mean age of 50 +/- 16, were observed. The incidence of IE makes 0.0042% per year in Kaunas district, in-hospital mortality rate was 24.6%. Forty five patients had positive blood culture, 54.8%--negative blood cultures, 16.9% of valve tissue cultures were positive. Patients with positive blood culture had higher incidence of acute IE, vascular phenomena, peripheral abscess and toxic encephalopathy. Culture negative endocarditis presented itself with worse left ventricular function, more frequent immunologic phenomena, myocarditis, glomerulonephritis, cardiac operation and perivalvular leak. Positive valve culture was associated with more frequent perivalvular leak. Mortality rate did not differ in groups.
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PMID:[Effect of blood and valve cultures on complication rate and outcome of infective endocarditis (analysis of data of patients treated at Kaunas district hospitals, 1999-2001)]. 1253 8

The case of inflammatory vasculopathy and encephalopathy caused by treatment with tacrolimus is reported. This 49-year-old woman developed progressive gait ataxia and right-sided hemiparesis after 7 years of tacrolimus therapy for focal sclerosing glomerulonephritis. MRI presented multifocal cerebral lesions with contrast enhancement. Oligoclonal banding was positive. When the treatment with tacrolimus was stopped, the clinical symptoms resolved completely and the MRI findings improved with corticoid monotherapy.
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PMID:[Case of inflammatory vasculopathy and encephalopathy caused by treatment with tacrolimus]. 1522 Oct 68

We describe a 15-year-old boy with acute transient encephalopathy complicating poststreptococcal glomerulonephritis. Based on advanced magnetic resonance imaging, cerebral alterations were related to cerebrovascular autoregulatory dysfunction (ie, a vasogenic edema) and vasculitis was excluded. These insights into the pathophysiology improve patient management and argue against the therapeutic immunosuppression postulated by some authors.
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PMID:Reversible encephalopathy complicating post-streptococcal glomerulonephritis. 1639 14

A case of acute encephalopathy with posterior corticosubcortical vasogenic edema on magnetic resonance imaging is reported. Angiography showed cerebral arterial vasospasm. A diagnosis of acute post-streptococcal glomerulonephritis was made 2 days after admission. This report highlights the fact that acute post-streptococcal glomerulonephritis can be revealed by a posterior reversible encephalopathy syndrome and that cerebral vasospasm can concur with vasogenic edema in this condition.
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PMID:Posterior reversible encephalopathy syndrome revealing acute post-streptococcal glomerulonephritis. 1690 29

We report the case of a young woman who had transient encephalopathy with nausea, cognitive impairment, 2 generalized seizures accompanied by visual impairment, and stenotic alterations of cerebral vessels lasting for weeks until complete resolution. These findings were associated with an elevated antideoxyribonuclease B level and biopsy-proven poststreptococcal glomerulonephritis. At the time of the encephalopathy, the patient had no electrolyte level disturbances, an only mildly elevated urea level, and moderate arterial hypertension and was on methylprednisolone therapy. For a couple of days, cranial magnetic resonance imaging showed multiple disseminated asymmetric hyperintensities on T(2)-weighted and fluid-attenuated inversion recovery, suggesting vasogenic edema. However, Doppler ultrasound examinations showed stenoses of extracerebral and multiple intracerebral arteries that persisted for several weeks, lasting considerably longer than the cerebral edema. This finding does not fit the context of hypertensive or steroid-induced encephalopathy, but is consistent with diagnosis of an accompanying vasculitis. Treatment with methylprednisolone for several weeks was associated with resolution of arterial stenoses and neurological symptoms, complete reversibility of Doppler sonographic findings, and significant improvement in renal function.
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PMID:Transient encephalopathy complicating poststreptococcal glomerulonephritis in an adult with diagnostic findings consistent with cerebral vasculitis. 1693 Dec 24

The aim of this study was to define the current demographic, clinical and prognostic characteristics of acute post-streptococcal glomerulonephritis (APSGN) in French Polynesia and to compare these features with those of other populations. Fifty children, all of whom were <15 years old and had been admitted to the Territorial Hospital of Papeete for APSGN between January 2005 and December 2007, were retrospectively enrolled in the study. Diagnostic criteria were microscopic or macroscopic haematuria, decreased C3 fraction of the complement and evidence of recent streptococcal infection. The annual incidence was 18 cases per 100,000 children <15 years of age in 2007. Most of the children (98%) enrolled in the study were of Polynesian ethnic origin, 27 were male (54%), and the average age at presentation was 6.7 years. Signs of previous respiratory infections were clearly evident in 40% of the children. Most of the patients presented during the rainy season, correlating with the relatively high incidence of skin infections at this time. The majority of patients had proteinuria (98%), with 25% having proteinuria in the nephrotic range (proteinuria/urinary creatinine >3 g/g). The presentation was severe in 22% of the children (congestive cardiac failure, severe hypertension and/or encephalopathy), and renal failure was an initial presenting symptom in 43.7%. The C3 fraction was lower in severe presentations, but the type of haematuria, level of proteinuria and inflammatory syndrome were not correlated with immediate severe forms or with initial renal failure. Haematuria resolved in a mean of 7.7 months and proteinuria in a mean of 3.9 months. None of the children had hypocomplementemia for more than 8 weeks. Acute post-streptococcal glomerulonephritis is endemic among French Polynesians, and they can be considered to be a high-risk population. Despite a high incidence of skin infections, however, the predominance of respiratory infections potentially indicates that French Polynesia is on the way to become a low-incidence area. Systematic detection and treatment of group A Streptococcus should be intensified.
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PMID:Acute post-streptococcal glomerulonephritis in children of French Polynesia: a 3-year retrospective study. 1987 55


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