Robbins and Cotran pathologic basis of disease / , Robbins and Cotran pathologic basis of disease / , کتابخانه دیجیتال جندی شاپور اهواز

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Robbins and Cotran pathologic basis of disease

The goal of this review is to present a comprehensive survey of the many intriguing facets of creatine (Cr) and creatinine metabolism, encompassing the pathways and regulation of Cr biosynthesis an...

http://www.afboard.com/library/creatinemetabolism.pdf

Creatine and Creatinine Metabolism

ContextThroughout the past 40 years, a vast and sometimes contradictory literature
has accumulated regarding hypertrophic cardiomyopathy (HCM), a genetic cardiac
disease caused by a variety of mutations in genes encoding sarcomeric proteins
and characterized by a broad and expanding clinical spectrum.ObjectivesTo clarify and summarize the relevant clinical issues and to profile
rapidly evolving concepts regarding HCM.Data SourcesSystematic analysis of the relevant HCM literature, accessed through
MEDLINE (1966-2000), bibliographies, and interactions with investigators.Study Selection and Data ExtractionDiverse information was assimilated into a rigorous and objective contemporary
description of HCM, affording greatest weight to prospective, controlled,
and evidence-based studies.Data SynthesisHypertrophic cardiomyopathy is a relatively common genetic cardiac disease
(1:500 in the general population) that is heterogeneous with respect to disease-causing
mutations, presentation, prognosis, and treatment strategies. Visibility attached
to HCM relates largely to its recognition as the most common cause of sudden
death in the young (including competitive athletes). Clinical diagnosis is
by 2-dimensional echocardiographic identification of otherwise unexplained
left ventricular wall thickening in the presence of a nondilated cavity. Overall,
HCM confers an annual mortality rate of about 1% and in most patients is compatible
with little or no disability and normal life expectancy. Subsets with higher
mortality or morbidity are linked to the complications of sudden death, progressive
heart failure, and atrial fibrillation with embolic stroke. Treatment strategies
depend on appropriate patient selection, including drug treatment for exertional
dyspnea (β-blockers, verapamil, disopyramide) and the septal myotomy-myectomy
operation, which is the standard of care for severe refractory symptoms associated
with marked outflow obstruction; alcohol septal ablation and pacing are alternatives
to surgery for selected patients. High-risk patients may be treated effectively
for sudden death prevention with the implantable cardioverter-defibrillator.ConclusionsSubstantial understanding has evolved regarding the epidemiology and
clinical course of HCM, as well as novel treatment strategies that may alter
its natural history. An appreciation that HCM, although an important cause
of death and disability at all ages, does not invariably convey ominous prognosis
and is compatible with normal longevity should dictate a large measure of
reassurance for many patients.

https://jamanetwork.com/journals/jama/articlepdf/194713/jcc10012.pdf

Hypertrophic cardiomyopathy: A Systematic review

Background: Kawasaki disease is an acute vasculitis of childhood that leads to coronary artery aneurysms in ≈25% of untreated cases. It has been reported worldwide and is the leading cause of acqui...

Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association.

Indirect immunofluorescence of certain human sera demonstrated an antigen(s) in the cytoplasm of 1--5% of the cells of a T-cell line, MT-1, from a patient with adult T-cell leukemia (ATL), which is endemic in southwestern Japan. The antigen was not detected in other human lymphoid cell lines, including six T-cell lines, seven B-cell lines, and four non-T non-B cell lines. The antigen did not show cross antigenicity with that of herpesviruses, including Epstein--Barr virus, herpes simplex virus, cytomegalovirus, varicella-zoster virus, herpesvirus saimiri, and Marek disease virus. The proportion of antigen-bearing cells was increased by a factor of approximately 5 on culture in the presence of 5-iodo-2'-deoxyuridine. Antibodies against the antigen in MT-1 cells were found in all 44 patients with ATL examined and in 32 of 40 patients with malignant T-cell lymphomas (most of them had diseases similar to ATL except that leukemic cells were not found in the peripheral blood). The antibodies were also detected in 26% of the healthy adults examined from ATL-endemic areas but in only a few of those examined from ATL-non-endemic areas. On electron microscopy, extracellular type C virus particles were detected in pelleted MT-1 cells cultured in the presence of 5-iodo-2'-deoxyuridine.

https://www.pnas.org/content/pnas/78/10/6476.full.pdf

Adult T-cell leukemia : antigen in an ATL cell line and detection of antibodies to the antigen in human sera

Pathologic studies were done on 20 hearts of patients who had typical clinical signs and symptoms of Kawasaki disease. The cardiac lesions were classified according to the duration of illness at the time of death. Stage I (zero to nine days) was characterized by acute perivasculitis and vasculitis of the microvessels (arterioles, capillaries, and venules) and small arteries, and acute perivasculitis and endarteritis of the three major coronary arteries (MCAs). Pericarditis, myocarditis, inflammation of the atrioventricular conduction system, and endocarditis with valvulitis were also present. Stage II (12 to 25 days) was characterized by panvasculitis of the MCAs and aneurysm with thrombus in the stems. Myocarditis, coagulation necrosis, lesion of the conduction system, pericarditis, and endocarditis with valvulitis were also present. In stage III (28 to 31 days), granulation of the MCAs and disappearance of inflammation in the microvessels were noted. Patients in stage IV (40 days to 4 years) had scarring with severe stenosis in the MCAs. Fibrosis of the myocardium, coagulation necrosis, lesions of the conduction system, and endocardial fibroelastosis were also present. The features observed revealed Kawasaki disease to be acute and inflammatory. The angiitis begins in the microvessels and fibrinoid necrosis of the media is rare. The disease is one with a pathologic pattern previously unknown.

Pathology of the heart in Kawasaki disease.

To clarify the pathophysiologic role of intramyocardial small artery (IMSA) diseases in hypertrophied hearts, narrowings of the IMSA were quantitatively evaluated in 39 autopsied hearts, 10 from patients with typical hypertrophic cardiomyopathy (HCM), four from patients with HCM showing features mimicking dilated cardiomyopathy (DCM-like HCM), 10 from patients with hypertension, and 15 from normal adults. The relations of narrowings of the IMSA to myocytic hypertrophy, myocardial fiber disarray, and fibrosis were also examined. The external caliber and the ratio of the luminal area to the total vascular area (percent luminal area, % lumen) were calculated by an image analyzer in 85 to 203 IMSAs from each patient. The external calibers of the IMSAs were similar among groups of hearts with HCM, hypertensive hearts, and normal hearts but were greater in those with DCM-like HCM. The mean % lumen of the IMSAs was similarly reduced in the hearts with HCM (29 +/- 5% in the ventricular septum and 31 +/- 5% in the left ventricular free wall) and in hypertensive hearts (30 +/- 8% and 31 +/- 7%) compared with that in normal hearts (40 +/- 5% and 38 +/- 5%) and was the lowest in the ventricular septum of hearts with DCM-like HCM (17 +/- 3%). The mean % lumen of the IMSA was inversely correlated with heart weight (r = -.59), the mean size of myocytes (r = -.66 in the ventricular septum, r = -.63 in the free wall), and percent fibrotic area in the septum (r = -.68). The mean % lumen values of the IMSAs in the tissues with and without disarray in the hearts with HCM were similar. Thus IMSA disease is of pathophysiologic importance in patients with HCM, DCM-like HCM in particular, or with hypertension.

Quantitative analysis of narrowings of intramyocardial small arteries in normal hearts, hypertensive hearts, and hearts with hypertrophic cardiomyopathy.

To elucidate the expression of the atrial natriuretic polypeptide (ANP) gene in the ventricle of the human failing heart, we have measured ANP and ANP messenger RNA (ANPmRNA) levels in left ventricular aneurysm obtained at operation, biopsy specimens of left ventricles from dilated cardiomyopathy (DCM) and autopsy samples of old myocardial infarction (OMI) and DCM hearts, and compared the levels with those in the normal ventricle. The ANP level (mean +/- SE) was 17.5 +/- 6.9 ng/g in the normal ventricle, and increased to 660.3 +/- 122.2 ng/g in the left ventricular aneurysm tissues and to 3,138.6 +/- 1,642.1 ng/g in the biopsy specimens of the DCM ventricle. These levels were approximately 40 and 200 times higher than in the normal ventricle. The increase of ANP levels was observed in both infarcted and noninfarcted regions of the OMI heart, and in the entire ventricle of the DCM heart. A significant positive correlation was found between the ANP level in aneurysm tissues and pulmonary capillary wedge pressure (r = 0.85). The ANPmRNA level in the left ventricular aneurysm showed about a 10-fold increase compared with that in the normal heart and reached 23% of that in the atrium of the same heart. A similar increase in the ANPmRNA level was observed in the entire ventricle of DCM. These data clearly indicate that the expression of the ANP gene in the ventricle is augmented in the failing heart in accordance with the severity of heart failure. In the atrium of the failing heart, ANP and ANPmRNA levels were only two times higher than those in the normal atrium. Thus, the augmentation in the expression of the ANP gene was more prominent in the ventricle than in the atrium. Taking tissue weight into account, the total content of ANPmRNA in the ventricle of the failing heart is much the same as that in the normal atrium. The ratio of the ANP level to the ANPmRNA level in the ventricle is much smaller than that in the atrium. These results suggest more rapid secretion of ANP after synthesis in the ventricle. These findings demonstrate that the expression of the ANP gene is augmented in the human ventricle of the failing heart and suggest that the ventricle becomes a substantial source of circulating ANP in congestive heart failure.

/pdf/augmented-expression-of-atrial-natriuretic-polypeptide-gene-1bn5mypc08.pdf

Augmented expression of atrial natriuretic polypeptide gene in ventricle of human failing heart.

BACKGROUND Brain natriuretic peptide (BNP), as a cardiac hormone, is expressed together with atrial natriuretic peptide (ANP) in the ventricles in congestive heart failure However, the ventricular expression of BNP in hypertrophic cardiomyopathy (HCM) with normal systolic function is still unclear

METHODS AND RESULTS The study population consisted of 39 HCM patients with asymmetric septal hypertrophy and 10 control subjects without any specific cardiac disease Eleven cases of HCM were obstructive (HOCM), and the other 28 cases were nonobstructive (HNCM) All of these patients had a normal ejection fraction Immunohistochemical analysis of endomyocardial biopsy specimens with specific monoclonal antibodies showed BNP immunoreactivity in the HOCM group (5/10, 50%) but not in the HNCM group (0/22) or in control subjects (0/5) In HOCM, left ventricular end-diastolic pressure was significantly higher in the BNP-positive patients than the BNP-negative patients Histological changes such as myocardial fiber disarray, hypertrophy of myocytes, and fibrosis were greater in BNP-positive patients than BNP-negative patients in HCM However, the expression had no significant relation with other clinical parameters The elevation of the BNP plasma level versus control subjects was marked in both HOCM (85-fold) and HNCM (23-fold) By contrast, the elevation of the ANP plasma level versus control subjects was mild in HOCM (57-fold) and HNCM (42-fold) The ratio of BNP level to ANP level was higher in HOCM (416) than in HNCM (146) and control subjects (028), and it was higher than the ratio previously reported for severe congestive heart failure (172)

CONCLUSIONS These findings suggest that BNP is expressed in the ventricular myocytes of HCM with normal systolic function In HOCM, ventricular expression of BNP may be augmented in response to both obstruction and diastolic dysfunction

Ventricular expression of brain natriuretic peptide in hypertrophic cardiomyopathy.

BACKGROUNDIschemic preconditioning slows ATP depletion and ultrastructural damage during the final episode of ischemia. To define the influence of creatine phosphate (CP) and intracellular pH (pHi) on this effect, CP and pHi were serially measured in porcine hearts without collateral circulation by using 31P-NMR spectroscopy and ultrastructural examination.METHODS AND RESULTSFarm pigs weighing 12-15 kg were anesthetized with Fluothane. The control group underwent a single occlusion (20 minutes or 60 minutes); the preconditioned group underwent four episodes of 5-minute occlusion and 5-minute reperfusion followed by a sustained occlusion (20 minutes or 60 minutes). After ischemic preconditioning, CP increased to 115 +/- 11% (p less than 0.05) of preischemic value and ATP decreased to 84 +/- 8% (p less than 0.05) of preischemic value, but pHi returned to preischemic value. At 5 and 10 minutes of sustained ischemia, CP was significantly preserved in the preconditioned group (control group, 19 +/- 3% versus p...

Hisayoshi Fujiwara

Papers

Pathology of the heart in Kawasaki disease.

Quantitative analysis of narrowings of intramyocardial small arteries in normal hearts, hypertensive hearts, and hearts with hypertrophic cardiomyopathy.

Augmented expression of atrial natriuretic polypeptide gene in ventricle of human failing heart.

Ventricular expression of brain natriuretic peptide in hypertrophic cardiomyopathy.

Ischemic preconditioning preserves creatine phosphate and intracellular pH.