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Biomedical subjects

V J Ferrans

Publications and source records attributed to V J Ferrans.

At least 361 records · Page 20Linked to original sources
PubMed1977

Histologic and ultrastructural features of primary and secondary endocardial fibroelastosis.

The average size of elastic fibers in thickened left ventricular endocardium was much larger in four patients with congenital endocardial fibroelastosis (EFE) than in six patients with acquired EFE (secondary to ischemic heart disease in two patients, to prosthetic cardiac valves in three, and to irradiation of the chest in one). Both components of normal elastic tissue (central, amorphous cores, and peripheral microfibrils) were present in endocardial elastic fibers of each patient. Ultrastructural identification of elastic fibers was greatly facilitated by staining with silver tetraphenylporphin sulfonate.

Adult↗
PubMed1977

Ultrastructure of hyaline microthrombi in myocardial capillaries of pigs with spontaneous "mulverry heart disease".

Three 5-week-old pigs from an Indiana farm died of the cardiac form ("mulberry heart disease") of selenium-vitamin E deficiency. Grossly, the hearts had prominent hydropericardium and extensive serosal and myocardial hemorrhage. Histopathologic study demonstrated marked myocardial edema, congestion, and hemorrhage, with numerous capillary hyaline microthrombi that stained red with the periodic-acid Schiff rocedure. Ultrastructural study revealed that these thrombi were composed of elongated, dense masses of intertwined fibrin strands with occasional entrapped erythrocytes.

Animals↗
PubMed1977

Ultrastructural alterations in nutritional cardiomyopathy of selenium-vitamin E deficient swine. I. Fiber lesions.

Cardiomyopathy was produced in 38 weanling swine by feeding a semisynthetic diet deficient in selenium and vitamin E for 13 to 59 days. Pigs were killed for morphologic studies of the cardiac lesions at sequential times after development of the deficiency disease. Gross examination disclosed hydropericardium and scattered pale streaks and patches of necrosis in the myocardium, especially the left ventricle. Histopathologically, the lesions were scattered throughout the heart but were most severe in the atria. Ultrastructurally, the damaged fibers had many features of myofibrillar degeneration with hypercontraction bands, myofibrillar lysis, and mitochondrial swelling, disruption, and mineralization. Numerous macrophages appeared to have passed through focal disruptions in the external laminae of the muscle cells and engulfed sarcoplasmic and nuclear debris. Stromal collapse and mild fibrosis persisted as residual lesions in scattered areas of myocardium in pigs with long term deficiency. Although vascular lesions were present in the hearts of selenium and vitamin E deficient pigs, it was concluded that the fiber alterations developed independently of the vascular changes. The pathogenesis of this cardiomyopathy induced by nutritional deficiency is thought to be related to lack of protection by the selenoenzyme, glutathione peroxidase, and the antioxidant, vitamin E, from lipoperoxidative damage.

Animals↗
PubMed1977

Ultrastructural alterations in nutritional cardiomyopathy of selenium-vitamin E deficient swine. II. Vascular lesions.

Selenium-vitamin E deficiency was produced in weanling swine by feeding a semisynthetic basal diet for 13 to 59 days. Pigs were killed sequentially for morphologic studies of the cardiac lesions. In hearts with vascular damage, gross hemorrhages were scattered in the myocardium and serosal surfaces. Light and electron microscopic study revealed myocardial arteriolar damage characterized by segmental fibrinoid accumulation in vessel walls and by scattered fibrin thrombi. Ultrastructural study disclosed extensive subendothelial and inner wall accumulations of dense granular deposits of serum proteins and masses of fibrin in arterioles in which dense deposits of fibrinoid were identified by light microscopy. Endothelial cells of these arterioles were loosely attached to each other. In arterioles with fibrin thrombi, the endothelium was disrupted. In mildly injured arterioles, increased endothelial permeability resulted in insudation of blood proteins into the vessel wall to produce accumulation of fibrinoid. In severely injured vessels, endothelial integrity was destroyed, smooth muscle cells were necrotic and thrombosis had developed. Initiation of these arteriolar lesions was apparently the result of lipoperoxidative damage to endothelial cell membranes that lacked protection by selenium-vitamin E.

Animals↗
PubMed1977

Ultrastructural alterations in skeletal muscle of ducklings fed selenium-vitamin E-deficient diet.

Newly-hatched ducklings were fed a selenium-vitamin E-deficient diet for 13 to 21 days. Ducklings were killed sequentially and the skeletal muscle alterations were studied by light and electron microscopy. An early alteration in skeletal muscle fibers was lysis of myofibrils, at first focal and later widespread. The sarcoplasm of damaged fibers appeared hyalinized by light microscopy. Degenerated skeletal muscle fibers with extensive lysis of myofibrils also had marked mitochondrial alterations, including swelling, formation of matrical densities, disruption of cristal membranes, and persistence of thick, dense, wavy, disrupted membranes. A sheath of external lamina persisted over degenerated segments of fibers which were invaded by macrophages that phagocytosed and removed the sarcoplasmic debris. Regeneration in damaged fibers was thought to proceed from thin subsarcolemmal satellite cells that survived the degenerative process. These satellite cells apparently developed into myoblasts, which fused to form myotubes that extended through the degenerated segments of fibers. Fibrillogenesis and sarcomerogenesis were apparent in myotubes. These structural alterations are discussed in terms of current knowledge of the combined biochemical role of selenium and vitamin E in maintaining cellular integrity.

Animals↗
PubMed1976

Thrombosis in association with atherosclerosis induced by dietary perturbations in dogs.

The distribution, severity, and complications of diet-induced atherosclerosis in dogs can be altered by changing the source of fat in the diet. Thrombosis and thromboembolic disease associated with atherosclerosis occurred with diets containing beef tallow and lard of coconut oil but were absent in dogs fed cottonseed oil as a source of fat. Experiemtnal animals with and without thrombosis are of value as models in elucidating the role of platelets and thrombostatic mechanisms in atherosclerosis.

Animals↗
PubMed1976

Acute lethal carditis caused by high-dose combination chemotherapy. A unique clinical and pathological entity.

An acute lethal myopericarditis has been observed in four out of fifteen patients receiving high-dose combination chemotherapy which includes cyclophosphamide 45 mg/kg/day for four days. In all cases the myopericarditis occurred 5-9 days after the initiation of chemotherapy, with dyspnoea, tachycardia, orthostatic hypotension, fluid retention, decreased voltage on electrocardiography, and pericardial effusion documented by echocardiogram, and progressed in 2 to 6 days to a fatal low-output state despite vigorous treatment. In three of the four patients, necropsy was permitted and revealed the unique pathological finding of fibrin microthrombi in capillaries, fibrin strands in the interstitium, and fibrin strands within the heart-muscle cells.

Acute Disease↗
PubMed1976

Cardiac rhabdomyoma: a clinicopathologic and electron microscopic study.

Cardiac rhabdomyomas are rare tumors of infancy. In a series of 36 patients 78 percent were under 1 year of age, and only one patient was over age 15 years. Ninety percent of the rhabdomyomas were multiple and occurred with nearly equal frequency in the right and left ventricles. Although reportedly infrequent in the atria, rhabdomyomas involved either one or both atria in 30 percent of patients. In 50 percent of patients at least one of the tumor masses was intracavitary and obstructed 50 percent or more of one of the cardiac chambers or valve orifices. Symptoms referable to obstruction of intracardiac blood flow were present in nine patients, none of whom had tuberous sclerosis, and all of whom would appear to have been good surgical candidates. Histologically the rhabdomyomas were composed of classic "spider cells". Electron microscopic studies revealed scattered bundles of myofibrils ringing these cells and radiating toward the center; glycogen was present both free in the cytoplasm and within mitochondria. Distinct intercellular junctions resembling intercalated discs with well defined desmosomes and nexuses were present. Many of the cells contained leptofibrils, arranged either peripherally or in spiraled clusters in the center of the cell. Rhabdomyomas derive from cardiac muscle cells and appear to represent hamartomas rather than true tumors.

Adult↗
PubMed1976

The heart in the Hurler syndrome: gross, histologic and ultrastructural observations in five necropsy cases.

Clinical and morphologic features of the cardiovascular system are described in five necropsy patients with the Hurler syndrome. In all five patients the coronary arteries, four cardiac valves, mural endocardium of all four chambers, myocardial walls and aorta were affected in a characteristic manner. All of these sites contained large clear cells known as Hurler cells (readily visible by light microscopy). In addition, granular cells were observed in semi-thin (1 mu) sections and by electron microscopy in the coronary arteries, atrioventricular (A-V) valves and in myocardial interstitium. These latter cells appear to produce collagen in an abnormal way and are probably responsible for the heavy deposits of collagen in the cardiovascular system of patients with the Hurler syndrome. In the cardiac muscle cells, in smooth muscle cells of the coronary arteries and in fibroblasts, wherever located, deposits of acid mucopolysaccharides and glycolipids usually were also observed. The acid mucopolysaccharide deposits were observed easily with light microscopy except in the cardiac muscle cells where they were seen only with electron microscopy. The glycolipid depositis, observed only on examination of 1 mu thick sections or with electron microscopy, have not previously been observed in coronary arteries or in myocardial cells. The infiltration into the heart by these cells and deposits in all five patients resulted in severe narrowing of the extramural coronary arteries, considerable thickening of the cardiac valves (the left-sided more than the right-sided valves), generalized thickening of mural endocardium and "stiffening" of the myocardial walls. Thus, the cardiovascular lesions in the Hurler syndrome are specific and life-threatening.

Adolescent↗
PubMed1976

Cardiac pathologic findings in patients treated with bone marrow transplantation.

Cardiac pathologic findings were analyzed in 22 necropsy cases from a series of 29 patients with leukemia, aplastic anemia, or metastatic cancer who had been treated with ablative therapy followed by bone marrow transplantation. Some cardiac alterations were similar to those that occur in patients with hematologic and neoplastic diseases not treated with bone marrow transplantation, and consisted of cardiomegaly, cardiac atrophy, hemorrhage, foci of necrosis due to shock associated with sepsis or hepatic failure, myocardial abscesses secondary to systemic candidiasis or staphylococcal infection, fibrinous pericarditis, and hemosiderosis. Other cardiac alterations were more specifically related to factors associated with transplantation procedure. Six patients exhibited a distinctive interstitial reactive change characterized by the presence of (1) moderate to large numbers of Anitschkow cells, occurring alone or in small cellular aggregates and histiocytes, histiocytic cells with nuclei of the Anitschkow type, lymphoid cells, and plasma cells, and (2) nuclei of the Anitschkow type in cardiac vascular and endocardial smooth muscle, endothelial and Schwann cells, and occasional cardiac muscle cells. This alteration may have been induced by abnormal immune mechanisms, as suggested by the observation that five of the six patients with interstitial change had clinical evidence of graft-versus-host disease. Two patients developed fatal congestive cardiac failure in the early post-transplant period and exhibited myocardial damage with histologic and post-transplant period features indicative of severe acute injury. Findings in these two patients consisted of necrotic muscle cells, which exhibited multiple contraction bands, diastase-resistant PAS staining, and intracellular fibrin deposits; microthrombi, which were composed of fibrin and occasionally of fibrin and platelets; and extravasated erythrocytes and fibrin strands in the interstitium. One of the two patients also exhibited unusual nuclear alterations, which were characterized by replacement of normal chromatin by palely stained fibrous and filamentous material. Clinicopathologic analysis strongly suggested that the fatal cardiotoxicity in both patients resulted primarily from effects of high doses of cyclophosphamide, which were administered as part of a four drug regimen that provided tumor ablation and immunosuppression for bone marrow transplantation. Our findings emphasize the need for less toxic antineoplastic and immunosuppressive therapy for use in bone marrow transplantation procedures.

Abscess↗
PubMed1976

The carcinoid endocardial plaque; an ultrastructural study.

Ultrastructural studies disclosed that the plaque-like endocardial thickenings in three patients with the carcinoid syndrome were composed of smooth muscle cells embedded in a stroma that was rich in acid mucopolysaccharides, collagen, and microfibrils, but devoid of elastic fibers. The smooth muscle cells contained variable numbers of myofilaments and cisterns of rough surfaced endoplasmic reticulum, and their basement membranes were greatly thickened, reduplicated, and arranged in layers. The endocardial plaques appeared histologically and ultrastructurally similar regardless of their location in the heart. The smooth muscle cells in these plaques appear to have been derived from primitive mesenchymal cells, which normally are present in the subendocardial endothelial space. These observations are interpreted as indicating that the plaques develop as a result of healing of a superficial endocardial injury, which may be initiated by release of bradykinin from hepatic metastases of a carcinoid tumor.

Endocardium↗
PubMed1976

Infantile cardiomyopathy with histiocytoid change in cardiac muscle cells. Report of six patients.

Clinical and pathologic findings are presented in 14 patients (six newly reported, eight described previously), all children ranging in age from 6 to 24 months, with a clinicopathologic syndrome termed "infantile cardiomyopathy with histiocytoid change in cardiac muscle cells." This syndrome is manifested clinically by severe, eventually fatal cardiac arrhythmias, and is characterized pathologically by cardiac hypertrophy and by a distinctive type of focal degeneration of the muscle cells, which lose their myofibrils, undergo marked mitochondrial hyperplasia, become rounded in shape and enlarged, and resemble histiocytes. Evidence is presented to support the conclusions that these manifestations are those of a cardiomyopathy, that cardiac hypertrophy precedes the onset of the clinical features, that the focal degeneration is likely to be a cause rather than a consequence of the arrhythmias, and that the latter develop only in the late stages of the disorder. The etiology of this cardiomyopathy remains unclear.

Cardiomyopathies↗
PubMed1976

Ultrastructure of sarcoplasmic reticulum in atrial myocardium of patients with mitral valvular disease.

Alterations observed in the sarcoplasmic reticulum of muscle cells in left and right atrial myocardium from 10 patients with mitral valvular disease consisted of: a) proliferation of rough-surfaced endoplasmic reticulum, which formed large cisterns in perinuclear areas of hypertrophied cells and was considered indicative of increased protein synthesis; b) proliferation of free sarcoplasmic reticulum, a change that occurred in degenerated cells and appeared to be related to loss of contractile elements; c) two types of aggregates of tubules of free SR--one type was associated wtih abnormal Z-band material and was found only in cells showing loss of myofibrils and proliferation of free SR, whereas the other was not associated with either of these changes and occurred in less severely altered cells; and d) proliferation and enlargement of cisterns of extended junctional sarcoplasmic reticulum, which formed two distinct types of complexes: the first of these consisted of large, convoluted (Type A) cisterns that were wide (550 to 650 A in thickness) and did not have a central dense lamina; the second was composed of stacks of concentric or parallel (Type B) cisterns that were narrower (220 to 300 A in thickness), had a central dense lamina, and were separated from one another by layers of glycogen granules. The formation of these complexes of cisterns was regarded as an extreme form of overdevelopment of extended junctional sarcoplasmic reticulum in atrial muscle cells.

Adult↗
PubMed1976

Intracellular collagen fibrils in cardiac valves of patients with the Hurler syndrome.

Ultrastructural study of thickened mitral and tricuspid valves from three patients with the Hurler syndrome disclosed collagen fibrils located within membrane-bound cytoplasmic dense bodies in small granular cells. These cells, which differ morphologically from the clear cells containing mucopolysaccharide deposits, are considered to be fibroblasts in advanced stages of degeneration. It is postulated that the elevated concentrations of dermatan sulfate in Hurler's disease lead to an abnormally high synthesis of collagen and to its polymerization in intracellular loci.

Child↗
PubMed1976

Sarcolemmal alterations in cardiac hypertrophy and degeneration.

Sarcolemmal alterations were studied in hypertrophied and/or degenerated ventricular muscle cells from: 1) patients with hypertrophic cardiomyopathy, congestive cardiomyopathy of various causes, aortic valvular disease, or congenital heart diseases associated with obstruction to right ventricular outflow; 2) patients with various neoplasms treated with anthracycline drugs; 3) rabbits given large doses of anthracyclines, and 4) dogs subjected to anoxic cardiac arrest during total cardiopulmonary bypass. Sarcolemmal changes in hypertrophied, nondegenerated cardiac muscle cells consisted of: 1) increase in the area and in the degree of irregularity of cell surfaces, including intercalated discs and T tubules; 2) dilation of T tubules, and 3) formation of large numbers of multiple intercalated discs. Moderately degenerated cardiac muscle cells, with or without associated hypertrophy, showed sarcolemmal changes characterized by: 1) dilation and decreased numbers of T tubules; 2) marked surface irregularity; and 3) dissociation of intercalated discs, associated with development of vesicles in widened disc interspaces, and of variably complex junctional structures formed between two parts of the plasma membrane of the same muscle cell (intracytoplasmic junctions). Sarcolemmal alterations in cells with end-stage degeneration were associated with loss of myofibrils and consisted of: 1) disappearance of T tubules; 2) loss of junctional contacts wtih adjacent cells; 3) marked surface irregularity; 4) formation of intracytoplasmic junctions, and 5) thickening of basement membranes. These observations show that remodeling of the cell surfaces is an important feature of the cellular responses to the stimuli of hypertrophy or degeneration.

Adolescent↗