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)

A case of Takayasu's disease in a young Caucasian female is described. The major complications which developed over the ten year course of the disease include nephrotic syndrome, severe refractory hypertension, aortic valve regurgitation associated with aneurysmal dilatation of the ascending aorta, and recurrent congestive heart failure. Amyloid deposits have been demonstrated in the aorta, atrial appendage, aortic valve, and renal cortex. The association of amyloidosis and Takayasu's disease is discussed.
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PMID:Takayasu's disease associated with generalised amyloidosis. 286 47

The surface electrocardiogram remains an insensitive method for detection of ventricular hypertrophy. Technical problems related to body size and habitus and distance from the heart cannot be overcome. Coronary arterty disease and amyloidosis, although frequently associated with hypertrophy, tend to obscure the electrocardiographic changes because of the attendant loss of voltage. The progress made in the last 20 years is due primarily to re-evaluation of traditional criteria in terms of careful anatomic correlation. The studies cited have the advantage of using specific clinical diagnoses in a defined population, specific chamber weights, and a 97.5 percentile confidence level for distinguishing normal pathologic and electrocardiographic data from abnormal. They are limited because the results may not apply to females or patients with mitral stenosis and congenital heart disease. In general, the electrocardiogram can be expected to detect left ventricular hypertrophy in six out of ten patients with the disease, and will misdiagnose the problem in about one out of every ten without the disease. Methodology using multiple criteria will achieve the best sensitivity and specificity. Several methods are available and of comparable accuracy. Simplicity of these methods varies widely and will be a factor in the choice of the method selected. The electrocardiogram will perform best in the population of patients with hypertension and aortic stenosis or regurgitation and have its greatest limitation in patients with coronary artery disease and myocardial infarctions. Echocardiography is proven to be more sensitive than the electrocardiogram for detection of left ventricular hypertrophy. Sensitivity is around 90 per cent with 95 per cent specificity. Its major limitations lie in the expense as compared to the electrocardiogram and in inadequate image resolution in a small proportion of patients. In order to achieve the results reported by centers proficient in this technique, careful attention must be paid to precise standardization of measurements and selection of images to be measured. When this is done the echocardiogram certainly offers a distinct advantage over the electrocardiogram in detecting left ventricular hypertrophy. We recommend the use of left atrial abnormality as a criterion to diagnose left ventricular hypertrophy when there is right bundle branch block. When left bundle branch block is present on the electrocardiogram, traditional criteria are probably no more accurate than the bundle branch block itself.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Recent progress in the electrocardiographic diagnosis of ventricular hypertrophy. 296 47

Small vessel disease has been described in various cardiac conditions including diabetes mellitus, amyloidosis, and connective tissue disease. Less well understood is the incidence and morphological features of small vessel disease in patients with myocardial disease of unknown etiology. This study examines the incidence, clinical presentation, and pathological changes of small vessel disease in patients with normal epicardial coronary arteries undergoing endomyocardial biopsy. Biopsy specimens in 110 consecutive patients were analyzed by light and electron microscopy. Small vessel abnormalities were present in 16 patients (14.6 percent) of whom five patients had associated hypertension and 11 patients had idiopathic small vessel disease. There were six males and 10 females with a mean age of 53 (26 to 76) years. Clinical presentations were arrhythmias, heart failure, or chest pain. The left ventricular ejection fraction was reduced (less than 50 percent) in 12 of these 16 patients. The morphological features of small vessel disease included marked thickening of the arterial wall owing to subendothelial deposits of heterogeneous electron dense materials consisting of microfibrils, collagen and elastic fibers, cellular debris, and other amorphous substances. Subendothelial deposits comprised a mean 60 percent (40 to 76 percent) of the arterial wall thickness.
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PMID:Morphological changes in small vessels on endomyocardial biopsy. 371 82

DMSO is a clear odorless liquid, inexpensively produced as a by-product of the paper industry. It is widely available in the USA as a solvent but its medical use is currently restricted by the FDA to the palliative treatment of interstitial cystitis and to certain experimental applications. Cutaneous manifestations of scleroderma appear to resolve (albeit equivocally) following topical applications of high concentrations of DMSO. A limited number of small clinical trials indicate that intravenous DMSO may be of benefit in the treatment of amyloidosis, possibly by mobilizing amyloid deposits out of tissues into urine. Dermal application of DMSO seems to provide rapid, temporary, relief of pain in patients with arthritis and connective tissue injuries. However, claims for antiinflammatory effects or acceleration of healing are currently unwarranted. There is no evidence that DMSO can alter progression of degenerative joint disease, and, for this reason, DMSO may be considered for palliative treatment only and not to the exclusion of standard antiinflammatory agents. The safety of DMSO in combination with other drugs has not been established; neurotoxic interactions with sulindac have been reported. In experimental animals, intravenous DMSO is as effective as mannitol and dexamethasone in reversing cerebral edema and intracranial hypertension. An initial clinical trial in 11 patients tends to support this latter application. DMSO enhances diffusion of other chemicals through the skin, and, for this reason, mixtures of idoxuridine and DMSO are used for topical treatment of herpes zoster in the UK. Adverse reactions to DMSO are common, but are usually minor and related to the concentration of DMSO in the medication solution. Consequently, the most frequent side effects, such as skin rash and pruritus after dermal application, intravascular hemolysis after intravenous infusion and gastrointestinal discomfort after oral administration, can be avoided in large part by employing more dilute solutions. Most clinical trials of DMSO have not incorporated the components of experimental design necessary for objective, statistical evaluation of efficacy. Randomized comparisons between DMSO, placebo and known active treatments were rarely completed. Final approval of topical DMSO for treatment of rheumatic diseases in particular will require a multi-center, randomized comparison between high and low concentrations of DMSO and an orally-active, nonsteroidal antiinflammatory agent.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Medical use of dimethyl sulfoxide (DMSO). 391 2

We present 11 patients with immunotactoid glomerulopathy, a new syndrome characterized clinically by proteinuria (11/11), microscopic hematuria (9/11) and hypertension (9/11). The patients consisted of six females and five males, aged 25 to 59 years (mean, 44.6). Proteinuria was the presenting feature and the reason for renal biopsy in all patients. The diagnosis of immunotactoid glomerulopathy was established at renal biopsy by the presence of glomerular extracellular microtubules composed of immune reactants. All the biopsies studied by immunofluorescence (10 cases) had glomerular deposits of IgG and C3. In three biopsies studied with IgG subclass specific antisera, only one patient had monoclonal immunoglobulin deposits (IgG3 kappa). In six cases the glomerular deposits were analyzed for light chains. In three the deposits contained kappa only, and three consisted of both kappa and lambda. In two cases the immune aggregates were confined to the mesangium, and in the remaining eight cases, the deposits were present in the mesangium and the glomerular basement membranes. Electron-dense deposits composed of microtubules were present in the same distribution within the glomerulus as the immune reactants. The microtubules had a uniform diameter in each biopsy, but they varied in size from case to case. They were approximately the same size in eight cases (mean, 22.3 +/- 3 [SD] nm). Three cases had much larger microtubules: 34.2 nm, 35.4 nm, and 48.9 nm in diameter. Although the 22.3-nm microtubules resembled amyloid in their appearance, glomerular distribution and random orientation in the tissue, they were more than twice the diameter of amyloid (8.9 nm), and Congo red and thioflavin T stains for amyloid were negative. Similar microtubular structures have been described in patients with cryoglobulinemia, SLE and paraproteinemia, but these diseases were excluded in our patients on clinical, serologic and in some cases histologic grounds. More important, none of our patients had clinical or histochemical evidence of amyloidosis, an entity which may be confused with immunotactoid glomerulopathy on a morphologic basis. Follow-up, from 22 to 94 months (mean, 52.6) was obtained in all 11 patients, and 2 clinical courses were noted. Six patients had progressive deterioration of renal function, with five requiring dialysis. This group had severe hypertension (4/6) and nephrotic-range proteinuria (5/6) at some point in their course. The remaining five patients with stable renal function had proteinuria of less than 2.0 g/24 hr in most cases (4/5), and none had severe hypertension. This dichotomy correlated with the distribution of immunotactoids.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Immunotactoid glomerulopathy. 401 May

Authors review their own experience in PAN, Lupus ery thematosus and renal Amyloidosis. Two patients with PAN, both with arterial hypertension: one of them of macrosco pic type, presenting great aneurysms localized in brain and in renal arteries; the other patient had microscopic type, with good response to corticotherapy after three years of follow-up. Four patients with lupus erythematosus nephritis; kidney biopsy was performed in three of them: two cases with membranoproliferative glomerulonephritis, and the last one with extramembranos glomerulonephritis. All of them had nephrotic syndrome, and arterial hypertension. Seven patients with renal amyloidosis, four related to reumatoid artritis, two related to mucoviscidosis and the las case was a patient with recurrent mediterranean fever.
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PMID:[Collagenosis nephropathies]. 611 54

The arteriolar changes in renal biopsy samples were studied by light and electron microscopy and immunohistologic observations. Arteriolar hyaline thickening was found to occur in virtually all renal diseases, regardless of whether these were accompanied by hypertension or not. Only amyloidosis and dense deposit glomerulonephritis were accompanied by specific ultrastructural arteriolar changes. The nonspecific "hyalin" was shown ultrastructurally to contain various components: accumulated basement membrane material, fine granular deposit (with filamentous or lipid details), and granulovesicular and threadlike membrane structures. Presumably the material constituting these structures originates partly from the blood and partly from elements of the vascular wall itself.
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PMID:Arteriolar lesions in human renal biopsy material with special regard to the ultrastructural changes in the basal lamina network of the vascular wall. 620 94

Fibrillary renal deposits and nephritis. The authors have studied 8 patients whose glomeruli contain abundant fibrils in their mesangial matrix and basement membranes. Although the location of these fibrils is very similar to that of amyloid, they are about twice the size of amyloid fibrils, averaging 20 nm in width, and fail to react as amyloid does with special stains. Immunofluorescence-microscopic studies are usually positive with antiserums to IgG, often IgM, and in some cases IgA, and also kappa and lambda light chains, C3, and C4. The fibrils are associated with diffuse mesangial widening and increased mesangial matrix strands. Although peripheral glomerular capillary walls appear to be spared initially, their eventual involvement leads to glomerular capillary collapse and glomerular obsolescence. Crescent formation occurred in 5 cases, focally in 3 and diffusely in 2. Tubular basement membrane involvement was seen in 1 case. These patients exhibit hematuria, and proteinuria, and often hypertension and renal insufficiency. Proteinuria was in the nephrotic range in 3 patients in whom involvement of glomerular capillary basement membranes was extensive. Unless electron microscopy is applied to renal biopsies, these cases may be considered to represent mesangiocapillary or rapidly progressive glomerulonephritis, or amyloidosis. The nature of these fibrils is as yet not determined. It is likely that they have been called "atypical amyloidosis" in the past.
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PMID:Fibrillary renal deposits and nephritis. 635 91

The clinical presentation and spectrum of renal histopathology is described in 143 patients aged 60 years or more, with renal disease. In 82 patients renal biopsy revealed primary renal disease. In the remainder, changes associated with systemic conditions were found. These included amyloidosis, polyarteritis nodosa and hypertension. Fifty patients present with the nephrotic syndrome, one third of whom had a membranous glomerulonephritis on the renal biopsy. Three patients had a carcinoma associated with this renal histology. Two patients had a minimal change lesion and their nephrotic syndrome responded to corticosteroids. Renal biopsies from the 45 patients present with renal failure revealed a variety of histopathology which included idiopathic crescentic nephritis and antiglomerular basement membrane disease. Percutaneous renal biopsy is a valuable diagnostic aid in elderly patients with renal disease.
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PMID:Renal biopsy in the elderly: clinicopathological correlations in 143 patients. 650 3

In the present study we report the renal pathological findings from autopsy material along with relevant clinical data on 21 spinal cord injury patients with end-stage renal disease (SCI-ESRD) treated with maintenance haemodialysis. These data are compared with the relevant clinical and post-mortem findings on 43 ambulatory dialysis patients who expired during the same time period. The SCI-ESRD patients exhibited markedly different clinical and renal histopathological data when compared to the ambulatory--ESRD group. Chronic pyelonephritis and amyloidosis dominated the findings and were the major causes of renal insufficiency. Acute pyelonephritis, papillary necrosis, calculous disease, pyonephrosis and perinephric abscess formation were also more frequently present in the SCI-ESRD patients. Hypertension and nephrosclerosis, which were common findings in the ambulatory--ESRD patients were comparatively rare in the SCI-ESRD patients. In addition, the incidence of acquired cystic disease (ACD) was considerably less in the SCI-ESRD group. Although the reasons for these findings are not entirely clear several possible explanations are given. Infection with gram negative sepsis was the predominant cause of death in the SCI-ESRD patients, while death secondary to cardiovascular disease predominated in the ambulatory-ESRD group. Furthermore, the urinary tract and infected decubitus ulcers were determined to be the major source for sepsis in the SCI patients. From these findings it would follow that more effective prevention and control of these infections would result in not only a lower incidence of renal failure but also a substantially reduced morbidity and mortality in chronic SCI.
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PMID:Renal pathology in end-stage renal disease associated with paraplegia. 671 46


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