Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0851184 (thinning)
 11,252 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

To investigate the effect of Kawasaki syndrome on myocardial function, as well as the influence of high-dose intravenous gamma-globulin therapy on resolution of functional abnormalities, we studied 98 patients with Kawasaki syndrome during five time intervals from onset of illness: 1) 10 days or less, 2) 11-31 days, 3) 1-3 months, 4) 3-12 months, and 5) 1-3 years. Normal controls included 48 children under age 8 years, without known cardiovascular disease. Using two-dimensional directed M-mode echocardiograms, we obtained chamber dimensions, fractional shortening, rate-corrected velocity of shortening (Vcfc) adjusted for end-systolic wall stress, and early diastolic function parameters that included adjusted peak rates of left ventricular dimension change, wall thinning, and their respective timing. Left ventricular systolic and diastolic dimensions were larger (both p less than 0.01) in patients than in normal subjects in period 1. Stress-adjusted Vcfc was much lower in patients in the 3 months after disease onset; by period 5, contractility was comparable in patients and normal subjects. Adjusted indexes of early diastolic function did not differ significantly between patients and normal subjects. To investigate the effect of gamma-globulin, we analyzed data on 47 patients prospectively randomized to therapy with aspirin alone (n = 19, 40%) or to aspirin plus gamma-globulin, 400 mg/kg/day for 4 consecutive days (n = 28, 60%). In period 1, before treatment, the two groups had mean fractional shortening and stress-adjusted Vcfc comparable to each other but much lower than those of normal subjects (p less than or equal to 0.001). Patients treated with aspirin alone continued to have diminished fractional shortening and Vcfc compared with normal subjects in periods 2, 3, and 4 (all p less than or equal to 0.05). In contrast, fractional shortening and Vcfc in gamma-globulin-treated patients in these periods were comparable to those of normal subjects. By period 5, no difference was detected in systolic function or contractility between either treatment group and normal subjects. We conclude that early abnormalities of left ventricular contractility and myocardial function, as assessed by echocardiography, generally resolve by 1-3 years after disease onset and that recovery is accelerated by administration of IVGG in the acute phase.
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PMID:Left ventricular contractility and function in Kawasaki syndrome. Effect of intravenous gamma-globulin. 272 Sep 25

The long-term effects of Kawasaki disease on the cardiac function were evaluated in 67 patients. Serial M-mode echocardiograms were obtained at the time of the initial diagnosis, 1 to 3 months, at 3 to 12 months, and at greater than 12 months following the diagnosis. Left ventricular and left atrial dimensions, shortening fraction, left and right ventricular systolic time interval ratios, and computer analysis of digitized echoes of the left ventricular chamber and posterior wall were obtained. The left atrial and left ventricular dimensions were abnormal in half of the patients throughout the study periods. The shortening fraction was abnormal initially but became normal by the end of 3 months. The peak rates of emptying of the left ventricle and thickening of the posterior wall were significantly reduced in all evaluation periods. In addition, the peak rate of diastolic thinning of the posterior wall was reduced, although the peak rate of filling remained normal. Finally, more than 30% of patients studied beyond 12 months had a prolonged major filling and thinning period. There was no difference between patients with or without coronary artery aneurysms. All other systolic and diastolic phase intervals and rates of changes were normal. Contrary to previously published reports, we conclude that patients with Kawasaki disease who do not have demonstrable coronary artery disease, exhibit abnormalities of cardiac chamber size and function long after their acute illness.
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PMID:Long-term echocardiographic evaluation of cardiac size and function in patients with Kawasaki disease. 392 39

The involvement of the cardiovascular system in rabbits with serum sickness is similar of that in Kawasaki disease, both with respect to the histologic changes of the myocardium, the valves, the coronary arteries, and the aorta and to the sequence with which they appear. Although coronary arteritis appears the same histologically, aneurysms did not occur in mature rabbits, even in case of necrosis of an arterial segment with deletion of muscle and elastic tissue. On the other hand, weanling rabbits with serum sickness had a tendency to develop aneurysms of coronary arteries, despite little cellular infiltration and reactive fibrocellular hyperplasia. The age factor thus appears to play an important role in the development of coronary aneurysms in Kawasaki disease, irrespective of the underlying mechanism, be it a markedly increased permeability to mediators through degenerated endothelial cells, or primary degenerated muscle cells with marked thinning of the media without much inflammatory cellular reaction. Furthermore, this study provides an insight into the long-term prognosis of transient dilatation of coronary arteries in the acute stage of Kawasaki disease.
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PMID:Coronary artery aneurysms develop in weanling rabbits with serum sickness but not in mature rabbits. An experimental model for Kawasaki disease in humans. 763 14

A case of diffuse mesangial sclerosis (DMS) associated with Kawasaki disease is reported. A previously healthy Japanese girl, aged 4 months, presented with clinical features of Kawasaki disease. At week 10 of the illness, she developed the nephrotic syndrome, which was refractory to steroid therapy. Renal biopsy demonstrated a diffuse mesangial proliferative glomerulonephritis with microcystic tubular dilatation and, ultrastructurally, marked thinning of the lamina densa in the glomerular basement membrane (GBM) and the tubular basement membrane (TBM) of the proximal tubule. She went into chronic renal failure and died at the age of 11 months. At autopsy, the kidney revealed DMS. Histologically, we found Finnish microcystic disease in its early stages in the biopsy. Using a newly developed monoclonal antibody, we analysed the alpha chains (alpha 1-alpha 6) of type IV collagen in the GBM and TBM. There was no defective constitution of alpha chains on the thin GBM, but the thin TBM of the microcystic proximal tubule showed a weak or discontinuous reactivity for alpha 1 and alpha 2 chains, suggesting faulty formation of the basement membrane. The sclerosing glomeruli of the DMS did not depend on collapse of the GBM, which was positive for alpha 3-alpha 5 chains, but mainly on the proliferation of mesangial matrix, which was positive for alpha 1 and alpha 2 chains.
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PMID:Diffuse mesangial sclerosis associated with Kawasaki disease: an analysis of alpha chains (alpha 1-alpha 6) of human type IV collagen in the renal basement membrane. 923 Sep 14

Coronary aneurysm in Kawasaki's disease (Acute febril infantile mucocutaneous lymph node syndrome, MCLS) may cause sudden death in childhood and ischemic heart disease in adults. We encountered two adult autopsy cases of Kawasaki's disease with multiple coronary aneurysms. The first case was a 56-year-old man who hospitalized due to recurrent syncope since 51 years of age. At age 55 coronary angiography (CAG) had shown multiple aneurysms in the left and right coronary artery. In September 1991, he developed chest pain and was brought to the hospital, almost dead on arrival (DOA). The patient died later the same day despite cardiopulmonary resuscitation. Autopsy findings showed cardiomegaly (470 g) with multiple coronary aneurysms of three coronary arteries. Microscopically, intimal thickening and medial thinning were found in the aneurysmal wall with calcification and disruption of the internal elastic lamina. The second case, a 28-year-old man had been diagnosed with rheumatic fever and mitral regurgitation at 4 years of age. Coronary aneurysms were noted on CAG at 26 years of age. In April 1992, he developed fever and was admitted to a local hospital where he was diagnosed with infectious endocarditis. After his being transferred to our hospital, disturbance of consciousness suddenly developed and he died in September 1992. Autopsy findings showed cardiomegaly (430 g) with left ventricular hypertrophy and multiple coronary aneurysms in left anterior descending coronary artery and left circumflex coronary artery. The aneurysmal wall showed intimal thickening and medial thinning with multiple recanalizations of occlusive lumina and fibrous intimal thickening. The mitral valve showed mild fibrosis and calcification without valvular deformity. There was no evidence of bacterial endocarditis. Both cases were finally diagnosed as Kawasaki's disease. Ischemic heart disease or lesions related to coronary aneurysm in Kawasaki's disease may show an increased incidence in the near future. Kawasaki's disease should have been followed even in adulthood after treatment in childhood.
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PMID:[Adult multiple coronary aneurysms of Kawasaki's disease's sequelae; two autopsy cases]. 952 43

We present a case of a young woman with Kawasaki disease who was comprehensively evaluated by electrocardiograph-gated cardiac multidetector computed tomography (MDCT). MDCT disclosed 3-vessel giant coronary arterial aneurysms and associated focal apical septal myocardial pathology. This was characterized by an early enhancement defect, myocardial thinning, and hypokinesia. These findings are likely due to focal ischemic insult resulting from distal embolization of thrombus material from the giant proximal right coronary arterial aneurysms. This case illustrates the full capabilities of MDCT in the comprehensive evaluation of Kawasaki disease.
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PMID:Comprehensive evaluation of Kawasaki disease with electrocardiogram-gated cardiac multidetector computed tomography. 2053 Dec 28

Coronary artery aneurysm is a rare disorder, which occurs in 0.3%-4.9% of patients undergoing coronary angiography. Atherosclerosis accounts for >90% of coronary artery aneurysms in adults, whereas Kawasaki disease is responsible for most cases in children. Recently, with the advent of implantation of drug-eluting stents, there are increasing reports suggesting stents causing coronary aneurysms, months or years after the procedure. The pathophysiology of coronary artery aneurysm is not completely understood but is thought to be similar to that for aneurysms of larger vessels, with the destruction of arterial media, thinning of the arterial wall, increased wall stress, and progressive dilatation of the coronary artery segment. Coronary angiography remains the gold standard tool, providing information about the size, shape, and location and is also useful for planning the strategy of surgical resection. The natural history and prognosis remain unclear. Despite the important anatomical abnormality of the coronary artery, the treatment options of coronary artery aneuryms are still poorly defined and present a therapeutic challenge. We describe four cases, which were managed differently followed by a review of the current literature and propose some treatment strategies.
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PMID:Coronary Artery Aneurysm: Evaluation, Prognosis, and Proposed Treatment Strategies. 3162 Feb 55