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)

Using indirect immunofluorescence, cryostat sections from renal biopsy specimens of 14 adult patients showing marked diffuse thinning of the glomerular basement membrane (GBM) on ultrastructural analysis were examined for the presence of the Goodpasture (GP) epitope M2 using anti-M2 antiserum. In no case was a total absence of M2 noted. The fluorescence pattern was fine but homogeneously linear along the GBM in 12 cases, intensity varying from +-++, as compared with for the control specimen GBM. A faint, broken line of stain, intensity+, was observed in biopsy specimens of two patients, one of whom had family members with progressive hereditary nephritis, type Alport's syndrome. Clinical presentation was dominated by hematuria (10/14 patients) but also included three patients with isolated proteinuria. Two patients had nephrotic range proteinuria. Other than the GBM changes, histological findings were sparse, with either no abnormalities or only slight mesangial increase in most. One case of focal segmental sclerosis and hyalinosis was also found. The findings from this study suggest that the abnormally thin GBM does not lack the GP epitope, but it may be reduced.
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PMID:Abnormally thin glomerular basement membrane and the Goodpasture epitope. 218 29

Five patients with right ventricular dysplasia (RVD) and 28 patients with predominantly left sided dilated cardiomyopathy (DCM) were studied. RVD was characterised by syncope, recurrent sustained ventricular tachycardia, which typically had a left bundle branch block pattern on the surface electrocardiogram, right heart failure, and faint or absence of the right ventricular free wall on the myocardial scintigraphy. Two-dimensional echocardiographic, radionuclide angiographic, and contrast ventriculographic studies were typical of the right ventricular abnormalities in both Uhl's anomaly and arrhythmogenic right ventricular dysplasia. Two of them were died suddenly. Pathologic examinations showed "parchment-like" thinning of portions of the right ventricular free wall and its replacement by adipose tissue, although such degenerations were not found in the septum and the left ventricle. By contrast, DCM was characterised by exertional dyspnea and short run ventricular tachycardia, most of which had a right bundle branch block pattern. Ten of them had left ventricular myocardial defects on the scintigraphy, of whom five died suddenly. Hemodynamic data showed that left ventricular dysfunction was predominant more than right ventricle. Thus we postulate that these two syndromes are manifestations of a congenital, pathophysiologic process - the "involved right or left ventricle" syndrome.
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PMID:[Right ventricular dysplasia: clinical features compared with dilated cardiomyopathy]. 401 15

A family is described with corectopia, nystagmus, absent foveal reflexes, and corneal changes inherited in an autosomal dominant pattern. Other abnormalities found in this family include microcornea, Bitot's spots, and iris colobomas. Faint superficial opacities and fine superficial vessels were found in the peripheral cornea of younger family members. Older family members showed an increase in the density of the superficial opacities and areas of stromal thinning were found in the 60-year-old proband. Corneal vessels and progression centrally. Full-thickness stromal opacities and changes have been associated with familial and sporadic aniridia and iris colobomas. The present report describes similar changes in association with corectopia.
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PMID:Corectopia with nystagmus, absent foveal reflexes and corneal changes. 721 Dec 87

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

An 8-month-old Labrador retriever bitch was evaluated for sudden-onset, progressive abdominal distension. Physical examination revealed an exaggerated inspiratory effort, severe ascites, bilateral jugular vein distension, and hypokinetic femoral arterial pulses. Thoracic auscultation detected tachycardia with muffled heart sounds, without audible cardiac murmurs. Thoracic radiographs identified severe right ventricular enlargement and pleural effusion. The electrocardiogram was consistent with incomplete right bundle branch block or right ventricular enlargement. Echocardiography demonstrated severe right ventricular and atrial dilation, secondary tricuspid regurgitation, and thinning and hypocontractility of the right ventricular myocardium. Left heart chamber sizes were slightly decreased, with normal left ventricular contractility. A diagnosis of arrhythmogenic right ventricular cardiomyopathy was reached, based on the characteristic clinical, electrocardiographic, radiographic and echocardiographic findings, and the exclusion of other causes of isolated right ventricular failure. Treatment effected good control of clinical signs, until acutely decompensated congestive right heart failure led to euthanasia after 4 months. Arrhythmogenic right ventricular cardiomyopathy is a well-described clinical entity in humans, and has previously been documented in 3 male dogs. The condition is characterised by progressive fibro-adipose replacement of right ventricular myocardium, while the left ventricle usually remains unaffected. It should be considered a differential diagnosis in any young dog presented with isolated right heart failure, syncope, or unexplained ventricular tachyarrhythmias. This article reports the 1st case of arrhythmogenic right ventricular cardiomyopathy in a female dog, and highlights its echocardiographic features.
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PMID:Arrhythmogenic right ventricular cardiomyopathy in a dog. 1103 Mar 67

Hypereosinophilic syndrome was diagnosed in a 14 year old girl who presented with recurrent syncope. An ambulatory electrocardiogram revealed intermittent type 2 second degree left atrioventricular (AV) block. Focal thinning of the interventricular septum was noted on echocardiography, contrary to the commoner finding of regional ventricular wall thickening among patients with hypereosinophilic syndrome. High grade AV block as a manifestation of hypereosinophilic syndrome is rare and focal thinning of the left ventricle as a manifestation of the syndrome has not been described earlier.
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PMID:Unusual cardiac manifestation of hypereosinophilic syndrome. 1218 26

Arrhythmogenic right ventricular dysplasia (ARVD) is a complex arrhythmogenic cardiomyopathy, characterized by a partial or total replacement of the right ventricular myocytes by fatty and fibrous tissue. In this study, we present a case of ARVD in 17 year old girl, who was admitted to the hospital after syncope with ventricular arrhythmia. The echocardiography did not demonstrate structural cardiac abnormalities but the magnetic resonance recently showed thinning of the right ventricular wall. The girl was treated with the lidocaine, amiodarone and next, after radiofrequency catheter ablation she was receiving metoprolol. The girl has remained asymptomatic for four years of follow-up.
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PMID:Arrhythmogenic right ventricular dysplasia:clinical study. 1759 88

ABSTRAST: A 3-year-old boxer bitch was evaluated for syncope and exercise intolerance. Physical examination revealed tachypnea, tachyarrhythmia and pulse deficit. Echocardiography demonstrated right atrial dilatation, thinning and hypokinesis of the right ventricular wall and normal contractility of the left ventricle. Three months after the first onset of clinical symptoms, the dog developed signs of congestive right heart failure despite treatment, and euthanasia was performed because of refractory ascites. The diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) was reached based of the clinical evolution of rapid progressive right ventricular heart failure and the particular form of isolated right atrial and right ventricular alterations without any echographic abnormalities of the pulmonic and tricuspid valves. Definitive confirmation was made by necropsy. In contrast to boxer type dilated cardiomyopathy (BDCM), dilatation occurred primary on the right atrium and ventricle and the right ventricular wall was thinner in subtricuspidal, apical and infundibular locations, similar to the "triangle of dysplasia" of human patient with ARVC. Histological lesions showed myocyte atrophy and replacement with adipose and fibrous tissue extended from the epicardium toward the endocardium in the right ventricle (RV) and right atrium (RA). In contrast to BDCM, where the lesions are multifocal, they take here the form of waves, with a large base against the epicardium and a spike towards the endocardium, known as lesions with a wave-front pattern, which are typical of ARVC. This report with supraventricular arrhythmias and echographic location in the RA and RV supports the hypothesis that ARVC is a progressive disease which may also have left ventricular free wall involvement if the patient lives long enough.
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PMID:Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) in a Boxer. 1908 56

Sarcoidosis is a multisystem granulomatous disease of unknown atiology and cardiac involvement can occur. Echocardiographic abnormalities, such as left ventricular dysfunction, segmental wall thinning, ventricular aneurysm, or valvular abnormalities are often subtle until the later stages of the disease. However, sarcoid has a predilection to cause ventricular arrhythmias or conduction system abnormalities in the early stages and hence may develop palpitations, syncope, or sudden death before structural abnormalities are detected. If patients with early cardiac sarcoid are identified, they respond well to corticosteroid therapy, and defibrillator implantation may reduce the risk of sudden death from malignant arrhythmias. We present a case of a patient with cardiac sarcoid, manifesting as conduction system disease. Cardiac magnetic resonance imaging demonstrated a sarcoid granuloma within the LV septum. Although the standard echocardiographic evaluation was unremarkable, this area corresponded to abnormal strain imaging on echo. Similarly, while conventional echocardiographic measurements failed to demonstrate a response to steroid therapy, strain imaging showed improved regional myocardial function. This coincided with improvement in the conduction abnormalities. This case study showed the potential of strain imaging in demonstrating cardiac involvement from sarcoidosis and in assessing the therapeutic response to corticosteroid therapy.
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PMID:Response to steroid therapy in cardiac sarcoidosis: insights from myocardial strain. 2199 Feb 78

Congenital coronary artery fistula complicated with giant coronary artery aneurysm is a very rare condition. In this case report, we present a 65-year-old woman, referred to us with a continuous heart murmur, occasional atypical chest pain and few episodes of fainting. A giant aneurysm and a coronary-pulmonary fistula were diagnosed using multiple cardiovascular imaging modalities to provide a sufficient anatomical picture. The patient was considered at high risk of sudden death from aneurysm rupture and received surgical treatment. Subsequent histopathological examination revealed a true aneurysm with severe wall calcifications, ulcerations and large areas with marked thinning of the wall. The postoperative course was uneventful.
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PMID:Giant aneurysm in a left coronary artery fistula: diagnostic cardiovascular imaging and treatment considerations. 2366 53


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