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Biomedical subjects

A Fischer

Publications and source records attributed to A Fischer.

At least 757 records · Page 42Linked to original sources
PubMed1985

[St Jude's Medical prosthesis in patients over 65].

Between September 1979 and December 1982, 56 St Jude Medical valvular prostheses were implanted in 54 patients over 65 years of age. Surgery consisted in simple aortic valve replacement (35 cases), simple mitral valve replacement (12 cases), double aortic and mitral valve replacement (2 cases), valve replacement and coronary artery bypass surgery (3 cases), aortic valve replacement and replacement of the ascending aorta (1 case) and mitral valve replacement and tricuspid annuloplasty (1 case). The operative mortality (within 30 days of surgery) was 3.5% (2 cases). Patients were assessed by clinical examination, ECG, chest X-ray, echocardiogram and laboratory investigations on average 19 months after surgery. There were 3 late deaths (1 endocarditis, 1 cardiac failure and 1 subdural haematoma). No cases of significant haemolysis were observed. There were no cases of thrombosis of the valve or any deaths directly related to the valve. Four patients had cerebral embolism (4.9% per patient/year). None were fatal and only 1 patient had sequellae. Clinical improvement was very significant; 96% of the patients are now in the NYHA Classes I and II whilst 80% were in Class III or IV before surgery. The cardiothoracic ratio decreased significantly from 0.56 to 0.51 (p less than 0.01). The authors conclude that elderly patients may derive great benefits from valvular cardiac surgery and that age in itself is not a contraindication to this type of surgery. The St Jude Medical prosthesis is an excellent prosthesis but thromboembolism remains a major problem as with other mechanical prostheses. Anticoagulation for life is essential.

Age Factors↗
PubMed1985

Enhanced plasminogen-activator production by leukocytes in the human and murine Chediak-Higashi syndrome.

We have studied the coagulation status of eight patients with the Chediak-Higashi syndrome (CHS), both in the chronic and the accelerated phase of the disease. It has been shown that during the accelerated phase there are coagulation abnormalities. These abnormalities include a peripheral thrombocytopenia, minor alterations of liver clotting factors, and mainly a profound hypofibrinogenemia and hypoplasminogenemia, which cause life-threatening bleedings. These disorders are of complex origin, but a fibrinolytic process, possibly primary, appears to play a significant role, since the present evidence for intravascular coagulation is not definitive. The accelerated phase of the CHS is characterized by a visceral infiltration by macrophages and lymphocytes. Therefore, we have investigated the possible role of the macrophages in the fibrinolytic process. We have found an excessive plasminogen activator (PA) production by CHS mononuclear cells in the accelerated phase and to a lesser extent in the chronic phase, except in one patient in whom no anomaly was found. Single-cell studies revealed an increased number of PA-producing cells among the monocyte-macrophage lineage rather than a higher level of production per cell. Polymorphonuclear cells (PMN) from patients with CHS were also shown to contain more PA. Slight but significant abnormalities in PA production were observed in obligatory heterozygotes (five out of nine), indicating the inherited nature of the excessive PA production. Finally, an enhanced PA production was similarly demonstrated using beige mice macrophages. The exacerbated production of PA by macrophages in the accelerated phase of the CHS can account to some extent for the coagulation abnormalities that have been observed.

Acute Disease↗
PubMed1984

Isolation of new lymphotropic retrovirus from two siblings with haemophilia B, one with AIDS.

A human T-lymphotropic retrovirus was isolated from cultured T lymphocytes from two siblings with haemophilia B. Patient 2 was healthy, but patient 1 had acquired immunodeficiency syndrome. The retrovirus differed from human T-cell leukaemia virus (HTLV) but it was similar to the lymphadenopathy-associated retrovirus (LAV) in its morphology and its major core protein (P25). Both patients had antibodies against LAV and patient 1's retrovirus, detected by an enzyme-linked immunosorbent assay or a radioimmunoprecipitation assay. Seroepidemiological data indicated the transmission of this retrovirus by plasma products.

Acquired Immunodeficiency Syndrome↗
PubMed1984

Impaired natural killer activity in lymphohistiocytosis syndrome.

In six patients with well-documented lymphohistiocytosis syndrome, natural killer activity was found to be profoundly impaired and could not be increased by incubation in vitro with interferon. This abnormality was not found in parents of the affected children. A clear correlation with the activity of the disease was observed, although a delay of a few weeks (possibly reflecting the life span of NK cells in blood) was seen in the disappearance of NK activity after the onset of the disease and its reappearance after remission. No absolute correlation was observed between NK activity and percentage of leukocytes detected by the Leu-7 monoclonal antibody. Our findings indicate that testing NK activity is useful for the diagnosis of lymphohistiocytosis syndrome and can be used as an index of activity of the disease, among other major clinical and biologic signs of this syndrome. Reversal of the NK activity defect (rather than detection of Leu-7 positive cells) appears to be a good criterion of complete remission.

Antibodies, Monoclonal↗
PubMed1984

[AIDS in the infant].

Recently, we and others have encountered a group of infants who developed AIDS. They were born from parents who belonged to identified risk factors for AIDS. The clinical and immunologic features were described. The existence of a pediatric form of AIDS transmitted other than by transfusion suggests the possibility of transplacental perinatal or postnatal transmission.

Acquired Immunodeficiency Syndrome↗
PubMed1984

Familial nasopharyngeal carcinoma.

We report 3 cases of nasopharyngeal carcinoma occurring in a single family group. All 3 patients are male, caucasian, born in Australia and of Greek origin. Two of the patients are brothers, the third their first cousin.

Adult↗
PubMed1984

[Treatment of the Wiskott-Aldrich syndrome by a graft of allogeneic bone marrow].

We herein describe the first French case of successful bone marrow transplantation (BMT) in a patient with the Wiskott-Aldrich syndrome. Although the patient required hospitalization for a total of one year during his first 4 years of life for bleeding, eczema, protracted diarrhea and multiple infections, the bone marrow transplantation has permitted a complete and stable correction of the thrombocytopenia, the eczema and the immunodeficiency. The patient was prepared by a total body irradiation (850 rads) with a partial lung shielding and anti-lymphocyte globulins. The BMT was immediately followed by a severe but transient herpetic infection and acute graft versus host reaction (grade II) which resolved after steroid therapy. The thrombocytopenia disappeared 3 months after the BMT. The infections and the eczema did not reappear. Immune functions are entirely normal and all blood cells have been shown to be of donor origin (the sister of the recipient). The boy is growing normally and is doing well 3 1/2 years thereafter. He only suffered from bilateral cataracts secondary to the irradiation requiring lens extraction. One can now expect a success rate of 75% in bone marrow transplantation in patients with Wiskott-Aldrich syndrome as evaluated from a world review. In contrast, symptomatic treatment of the disease leads to a mean survival of 7 years, survival rarely exceeding 18 years.

Bone Marrow Transplantation↗
PubMed1984

[Generalized BCG infection, expression of multifactorial deficiency of intramacrophage bactericidal action. Anatomo-clinical study of 11 cases].

In France, because of routine vaccination, BCG infection is the rule and not the exception. It may thus incidentally reveal a latent immunodeficiency. Eleven cases (10 fatal) of generalized BCG infection observed from 1967 to 1981 at the Hôpital des Enfants-Malades (Paris) are reviewed. The clinico-pathologic analysis of these 11 cases showed that the underlying immunodeficient states were of various natures. They could be grouped under 3 different headings: (1) Cellular immunodeficiency, as part of a severe combined immunodeficiency (5 cases) and Di George's syndrome (1 case); (2) Deficiency in the bactericidal activity of the macrophages, either isolated (2 cases) or associated with fatal granulomatous disease (1 case); (3) unclassified conditions, probably original, associated with chronic Salmonella infection. The heterogeneity of theses cases emphasizes the complexity and diversity of the mechanisms which lead to the elimination of the intracellular agents. Because they provide useful insight into the poorly understood mechanisms of bactericidal activity of macrophages, further investigations of infantile disseminated BCG infection are necessary.

Child, Preschool↗
PubMed1984

[Autoimmune hemolytic anemia in children. Apropos of 14 cases].

Autoimmune hemolytic anemia is a rare disorder in childhood. The therapeutical difficulties encountered are described in a series of 14 patients aged 6 weeks to 10 years, 8 of them being under the age of 1 year at time of diagnosis. A remission was observed spontaneously in 1 patient and was obtained with steroids in 8 others. One patient died from acute irreversible hemolysis. Four patients were splenectomized because of an immediate or delayed resistance to steroids. After splenectomy, two patients died from infection, one was cured and the last patient is still in remission after one year. Steroid therapy is the primary treatment of autoimmune hemolytic anemia. The initial dose of 2 mg/kg/day has to be maintained until the remission (normal hemoglobin level and reticulocyte count) is achieved, then progressively decreased leading to alternate day therapy for several months. Resistance to steroid requires splenectomy, although it is an hazardous treatment in young children. The indication for immunosuppressive agents in childhood is difficult to define. It can be proposed in patients with steroid dependency in order to reduce the dose of steroid and/or to avoid long term steroid therapy.

Acute Disease↗
PubMed1983

[Left ventricular function following revascularization for occlusion of a coronary artery lasting 1 to 2 hours].

The time-lag between coronary occlusion and irreversible damage to the myocardium is ill-defined in man. In 10 patients the changes in left ventricular function have been studied after coronary occlusion during diagnostic or therapeutic cardiac catheterization of 1-2 hours' duration. Revascularization was achieved either surgically or through intracoronary streptokinase infusion. The interval between occlusion and onset of extracorporal circulation or reopening was 61 to 119 minutes. Despite enzyme elevation (CPK, CK-MB, SGOT) and appearance of Q-waves in 5 patients, no significant alteration of left ventricular function was noted on repeat cardiac catheterization 10 to 230 days after the accident. These observations, suggest that coronary occlusion of 1-2 hours' duration fails to produce significant irreversible damage to the myocardium despite electrocardiographic and enzymatic signs of myocardial infarction.

Aspartate Aminotransferases↗
PubMed1983

[Intracoronary thrombolysis as a treatment for evolving myocardial infarction].

Myocardial infarction is almost always the consequence of a thrombotic obstruction of one or more coronary arteries. We report our experience with the first 24 cases of intracoronary thrombolysis for recanalization of obstructed coronary arteries. 19 cases were successful, 1 case was partially successful and in 4 instances no reopening was observed. The amount of streptokinase used was 206 000 +/- 107 000 units, and reperfusion was achieved after 37 +/- 27.5 minutes. Recanalization of the vessel was accompanied by cessation of precordial pain and partial or complete normalization of the electrocardiogram. In one case bypass surgery was necessary because of reocclusion. Left ventricular function improvement after thrombolysis was dependent on the time-lag between occlusion and recanalization. These observations confirm others' experience that intracoronary thrombolysis appears to have favorable effects in patients with evolving myocardial infarction.

Coronary Disease↗
PubMed1983

Synthesis of intrathecal interferon in systemic lupus erythematosus with neurological complications.

Intrathecal synthesis of interferon in the absence of viral or bacterial infection was detected during the occurrence of neurological complications in two patients with systemic lupus erythematosus. The interferons displayed characteristics similar to those observed in the sera of patients with the disease. No interferon inducing activity was detected in the cerebrospinal fluid or serum of the two patients. These observations support the hypothesis of a localised mechanism of interferon induction in systemic lupus erythematosus which includes the interaction of lymphocytes with damaged tissues.

Adolescent↗
PubMed1983

Bone-marrow transplantation for inborn error of phagocytic cells associated with defective adherence, chemotaxis, and oxidative response during opsonised particle phagocytosis.

Two girls had delayed umbilical cord detachment, recurrent bacterial infection, inability to form pus, and marked leucocytosis. Their phagocytes were defective in tests of adherence, random migration, chemotaxis, and oxidative burst. NK activity was virtually absent. This rare disorder, due to an inherited absence of a 180 kilodalton membrane glycoprotein on polymorphonuclear cells, is usually lethal within 2 years. Allogeneic HLA-matched bone-marrow transplantation done at ages 4 months in one patient and 2 years in the other after intensive conditioning was successful and resulted in nearly complete correction of phagocytic cell function and NK activity within two months. One patient died 9 months after transplantation from severe chronic graft-versus-host disease with obstructive bronchopneumopathy. The other is doing well 1 year after transplantation and showing stable chimerism and normal phagocytic function.

Bone Marrow Transplantation↗
PubMed1983

Computer-aided manufacture of individual endoprostheses. Preliminary communication.

Long-term anchorage of foreign material in vital bone has proven to be the main problem in endoprosthetics. In the authors' opinion, modelling an endoprosthetic anchorage component to the individual anatomic needs is, at present, the best way to transmit stresses harmoniously from the prosthesis to the sensitive bone, in order to utilize functional adaptation, and to guarantee long-term function. The production of a true-to-scale joint model is possible with computer-assisted tomography. This model of the joint or bone canal can be used to construct an individual endoprosthesis prior to surgery. A procedure described.

Biomechanical Phenomena↗