[Cholesterol embolism in the brain: a clinicopathological analysis of 13 autopsy cases].
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Biomedical subjects
Publications and source records attributed to C Yutani.
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Forty-five relatives of 4 families with hereditary angioneurotic edema (HANE) were studied. Twenty-five, including 11 asymptomatic kindreds with the disposition, showed typical changes in complement system compatible with HANE. Follow-up study of HANE patients showed that, even in remission period, complement, coagulation and fibrinolytic systems can be activated. During edema attacks, moderate haemoconcentration and neutrophilia were encountered and kallikrein-kinin system was found to be also activated. Replacement therapy with C 1-inhibitor preparation for an edema attack revealed that clinical improvement paralleled the increase in blood levels of high molecular weight kininogen. Thus, HANE attack is considered to be elicited in kindreds with the hereditary disposition by activation of plasma protease systems, particularly by that of kallikrein-kinin system. On the other hand, exogenous triggers that can initiate activation of the protease systems can be classified into 2, neuro-humoral (sympathetic nerve response) and physico-chemical, categories. Hence, the edema attack of kindreds with the hereditary disposition can at least be modified by the biosynthesis of plasma factors and the individual susceptibility to the liberated catecholamines. These two different reaction processes are considered to be linked by the release of plasminogen activator and/or Hageman factor activating enzyme.
A 75-year-old man complaining of dyspnea and having sings of postcapillary pulmonary hypertension was diagnosed as pulmonary veno-occlusive disease and confirmed at autopsy. This is the oldest case ever reported. Almost all the small veins 2 mm or less in external diameter were partially or nearly completely occluded by intimal fibrous tissue, and the obstructive changes in the pulmonary arteries were much more limited. Pulmonary veno-occlusive disease is a rare, almost inevitably fatal disease of unknown etiology which has only recently been separated clearly from primary pulmonary hypertension as a distinct entity. Chest roentgenogram finding suggesting postcapillary pulmonary hypertension is a clue to a diagnosis and differentiates this from two other causes of clinical primary pulmonary hypertension, that is, recurrent pulmonary embolism and plexogenic pulmonary arteriopathy.
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In this study, the method of preserving isolated canine hearts for transplantation was established. The newly-developed method is named retrograde coronary sinus microperfusion technique. Canine hearts were arrested and cooled to 4 degrees C by means of normograde coronary perfusion with Collins' solution containing 10% fluorocarbon (FC) in order to avoid myocardial damage during warm ischemic time. Then, the hearts, immersed in Collins' solution, were retrogradely perfused through a coronary sinus at a rate of 60 ml/hr or 30 ml/hr with above-mentioned solution. After preservation for 36 hours, heterotopic heart transplantation was performed in an abdominal cavity of a recipient dog. The 36 hour-preserved hearts restored their cardiac beat after reperfusion with recipient blood. Histological observation did not show any abnormal findings in these preserved hearts. In order to evaluate the cardiac functions of preserved hearts for 24 to 36 hours, left ventricular end-systolic pressure-volume ratio (Emax) and end-diastolic pressure-volume relation were measured. Both were well restored in 24 hour-preserved hearts. The 36 hour-preserved hearts resulted in Emax within a satisfactory range, while end-diastolic pressure was elevated in all cases. This was markedly impaired with infusion of isoproterenol. These results strongly suggested that transplantation using 24 hour-preserved hearts could be performed well. On the other hand, the 36 hour-preserved hearts were considered to function satisfactorily under careful management after grafting. The retrograde coronary sinus microperfusion method developed here was found to be useful for a distant heart procurement.
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A case of moderately differentiated adenocarcinoma with argyrophilia arising in the endometrium of a 58-year-old Japanese woman is reported. Although she showed no endocrine symptoms, neuro-secretory granules were documented in the cytoplasm of some tumor cells by electron microscopy. Moreover, dopamine production by the tumor was demonstrated by biochemical determination. The possible mechanism accounting for such a lesion is discussed.
Eight children with myocarditis underwent a cardiac catheterization study and right ventricular endomyocardial biopsy. The degree of histological changes of the biopsy specimens correlated well with the severity of the clinical manifestations. Patients who had evidence of residual functional impairment had biopsy findings compatible with their clinical course.
We examined the inactive to active renin ratio in the renin granules of the cadaver kidneys and the plasma in patients with diabetic nephropathy. The inactive renin in the break-through fraction when the plasma or the renin from the renin granules was put into a pepstatin column was determined. The inactive renin in this fraction was activated by trypsin. Concerning plasma, the inactive to active renin ratio was 90 in the patients and 9 in the normal subjects. On the other hand, this ratio was 0.29 in the patients' kidneys and 0.28 in the control kidneys. These results suggest that the increase of the inactive to active renin ratio in plasma of diabetic nephropathy does not result from the change of the renin storage in the kidneys.
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An 8 year-old boy died suddenly 4 years after the onset of Kawasaki disease. He was examined by a cardiac mass screening for school children one and a half year before the death, and was evaluated as having no sequela of Kawasaki disease. The autopsy showed coronary arterial aneurysms and obstruction with fresh and old myocardial infarction. One of the problems of the present method for cardiac mass screening for school children is that it is performed only with history taking, physical findings and electrocardiograms. This method is completely insufficient to find out coronary involvement as a sequela of Kawasaki disease. All the children with history of Kawasaki disease should be examined by two dimensional echocardiography, which is the most sensitive and specific noninvasive method to detect the coronary involvement in our experience.
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Two hundred one patients (138 boys, 63 girls), 1 month to 11 years old, with Kawasaki disease underwent coronary angiography, ultrasonic tomography, myocardial imaging, and biopsy of right ventricular myocardium. Aneurysms of epicardial coronary arteries, mostly left, occurred in 26 cases (12.9%). Degeneration and proliferation of endothelium, edema, scarring, and fibrosis, slight to moderate, involved the tunicae of intramural small vessels in all cases. These changes tended to abate with time. The basic lesion of Kawasaki disease, round-cell myocarditis and fibrosis, involved all cases and persisted. Disarrangement, abnormal branching, disarray, and hypertrophy of myocytes correlated significantly with myocarditis, but not with epimural or intramural coronary angitis. As a consequence of the myocardial changes, it is suggested that some cases may terminate as myocardiopathy. Scoring criteria for vascular and myocardial changes in biopsy specimens are included.