[Malignant histiocytosis in AIDS].
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Acquired Immunodeficiency Syndrome↗
Biomedical subjects
D Storck
Publications and source records attributed to D Storck.
[Magnetic resonance imaging in the diagnosis of agenesis of the pericardium].
The authors report a case where the diagnosis of complete pericardial agenesis was made by magnetic resonance imaging (MRI). This anomaly is rare, often asymptomatic (two-thirds of the cases) and its diagnosis may be evoked on a chest X-Ray and sonocardiography. MRI confirms the initial clinical impression and, besides, enables to completely rule out associated cardiac or pulmonary pathologies.
Heart↗
[Renal manifestations in dermatomyositis and polymyositis].
Renal involvement is usually considered to be rare in dermatomyositis and polymyositis. The aim of this prospective study was to reevaluate the renal changes which may occur in these two conditions. Twenty patients, 12 with dermatomyositis and 8 with polymyositis of the adult type, were investigated to determine the incidence, the severity and course of renal disease. The 24 hour proteinuria was over 0.1 g in 14 cases and greater than 1 g/24 hours in 6 cases, including one patient with the nephrotic syndrome. Microscopic haematuria was noted in 3 cases and pyuria in 8 cases. Endogenous creatinine clearance was significantly decreased (p less than 0.001) in patients with dermatomyositis compared with a control group of subjects paired for age, sex, weight and serum creatinine concentration. Renal biopsy (5 cases) showed minimal glomerular lesions (2 cases), endocapillary proliferation and extramembranous glomerulonephritis (1 cases) and amyloid deposits (1 case). Although the proteinuria, haematuria and pyuria responded to steroid therapy (except in 1 case), the creatinine clearance remained decreased during the course of steroids.
Adult↗
Monoclonal antibodies defining a minor and a private idiotope on human rheumatoid factors.
Two mouse monoclonal antibodies (mAb A216-5 and L 49-3) with antiidiotypic activity against two human monoclonal IgM rheumatoid factors (IgM RFs) were defined. Each of these monoclonal antibodies (two mouse IgG 1K) reacted with an idiotope located on the heavy chain of the immunizing monoclonal IgM RF and was able to inhibit RF fixation to the antigen. These monoclonal antibodies did not react with other monoclonal IgM RFs from patients with macroglobulinemias or cryoglobulinemias and, therefore, did not recognize the known cross-reactive idiotopes of human monoclonal RFs. The presence of both 216-5 and 49-3 idiotopes on polyclonal IgM RFs from unrelated patients was undetectable by the inhibition assays. However, using a four-stage solid-phase radioimmunoassay, the 216-5 idiotope (minor), but not the 49-3 idiotope (private), was frequently present at a low concentration on polyclonal IgM RFs from patients suffering from rheumatoid arthritis, primary Sjögren syndromes, various infectious diseases, systemic vasculitis, and sarcoidosis and during aging. Interestingly, the 216-5 idiotope was undetectable among polyclonal IgM RFs of 12 normal adults. The main conclusions of these data are the following. The definition of minor and private idiotopes of human RFs requires the use of assays able to detect low amounts of antibodies among polyclonal Ig. The anti-IgG B cells which are sometimes clonally expanded during Waldenström diseases and cryoglobulinemias can also be activated during nonneoplastic diseases, among the other RF-secreting B cells.(ABSTRACT TRUNCATED AT 250 WORDS)
Antibodies, Monoclonal↗
[Malignant diffuse peritoneal mesothelioma: clinical and therapeutic aspects. Apropos of a case with gelatinous ascites].
Peritoneal mesothelioma ranks second in frequency after pleural mesothelioma. A well-established clinical entity since 1960, mesothelioma is increasingly frequent due to improvements in diagnostic methods and to the fairly wide industrial use of asbestos, a well-known carcinogenic substance for mesothelial cells. The diagnosis of malignant peritoneal mesothelioma rests on a corpus of convergent data. Treatment consists of surgery combined with radiotherapy and chemotherapy. The mean survival from the time of diagnosis is 8-10 months.
Ascites↗
[Regression of IgA kappa monoclonal gammopathy in IgA lambda Kahler's disease].
The authors report the case of a patient with a stable IgA kappa gammapathy over several years which finally evolved to an IgA lambda myeloma during which the initial gammapathy regressed. As the two gammapathies probably arose from two different cellular populations, the regression of the IgA kappa dysglobulinaemia is an additional argument in favour of the hypothesis that benign monoclonal gammapathies are the result of cellular hyperplasia.
Humans↗
[Bacteriologic surveillance of patients with infectious endocarditis. Value and limitations of the determination of minimal inhibitory concentration and serum bactericidal activity].
This is a retrospective study of the records of 17 patients who suffered from infectious endocarditis with positive blood cultures, and concerns the possibilities of predicting immediate infectious signs in patients:--by testing each of the antibiotics used for the minimal inhibitory concentration;--by determining the peak of the serum bactericidal activity of patients. All the patients were under observation, as outpatients, for more than a year and proved completely cured of their infectious endocarditis. A satisfactory minimal inhibitory concentration does not necessarily prevent an infectious evolution in the immediate future. The determination of the peak serum bactericidal activity does not reflect faithfully and instantaneously the anti-infectious action of the treatment; this is shown by the excellent and rapid evolution 13 times out 20 although serum bactericidal activity was considered insufficient (less than 1/8). This level of 1/8 is not a guarantee of therapeutic efficiency. Finally the antiinfectious treatment has been, in the majority of cases, determined by clinical signs and in very few cases by serum bactericidal activity results.
Adult↗
Abnormalities of in vitro responses to polyclonal activation of peripheral blood lymphocytes in patients with active sarcoidosis.
A variety of immunological abnormalities have been described in patients with sarcoïdosis. At the blood level, both hypo and hyper immune responsiveness seem to coexist and were related to abnormal T cell and macrophage functions by using allogenic cocultures and/or lymphocyte fractionation. We tested several components of cell mediated responses with two in vitro models: (a) the pokeweed mitogen activation of B cells which is T cells and macrophage dependent; (b) the Epstein-Barr virus (EBV) activation of B cells which is T cell- and macrophage-independent. We confirm previous data showing that PWM induced Ig Production of peripheral blood lymphocytes (PBL) of patients with sarcoïdosis is significantly reduced compared with normal PBL. However, this is associated with an increase of IgG, IgM synthesis of EBV-infected PBL in these sixteen patients. Thus, there is no evidence for a complete B cell defect in sarcoïd PBL. Furthermore, by using limiting dilution analysis of antibody secreting cells, there is an increase of precursor B cells EBV infectable in PBL of sarcoïd patients but T cells are effective in reducing EBV-induced B cell proliferation. Finally, these abnormalities are concomitant to the disease, disappear with it, and are apparently not correlated with the stage or the activity of sarcoïdosis.
Adult↗
Inhibition by DL-alpha-difluoromethylornithine of the pokeweed mitogen-induced immunoglobulin production in cultured human lymphocytes.
The effects of DL-alpha-difluoromethylornithine (DFMeOrn), an irreversible inhibitor of L-ornithine decarboxylase, on immunoglobulin production were studied in vitro using human peripheral blood lymphocytes stimulated with pokeweed mitogen. DFMeOrn inhibits in a concentration-dependent manner the usual pokeweed mitogen-induced increases of polyamine contents (putrescine, spermidine, spermine) and of [3H]thymidine incorporation. In parallel with the reduction of polyamine content and of thymidine incorporation, IgG and IgM productions are diminished, a 70% decrease being observed at 5 mM DFMeOrn concentration. Therefore, it appears that inhibition of polyamine biosynthesis may ultimately interfere with the cellular immunologic response by blocking cell proliferation. These findings certainly deserve further consideration both under in vitro and in vivo conditions.
Cells, Cultured↗
First case of acquired functional C1(-) INH deficiency: association with angioedema during Churg and Strauss vasculitis.
This report deals with the first case of acquired functional C1(-) INH deficiency with normal antigenic C1(-) INH level which was detected in a young girl with angioedema and Churg and Strauss vasculitis. This complement abnormality was associated with slightly depressed levels of CH50, C4 and C2, but a normal level of C3, and high levels of total IgE and IgM rheumatoid factors. Finally, most of these abnormalities disappeared after corticosteroid therapy and clinical improvement.
Adolescent↗
[Scleroderma and pregnancy. New case and review of the literature].
A new case of the rare association of scleroderma and pregnancy is reported. The pregnancy was complicated by renal failure in the last month of gestation which was initially well controlled by anti-hypertensive therapy but then suddenly progressed to pre eclampsia with signs of foetal distress necessitating emergency caesarian section. A moderately hypotrophic child was delivered. The mother progressively recovered; diuresis and blood pressure returned to normal and the proteinuria disappeared. In the light of previously reported cases and of recent advances in our knowledge of scleroderma, especially scleroderma renal disease, the authors discuss their attitude to the management of women with scleroderma wishing to become pregnant. They review the role and place of renal biopsy in the detection of subclinical renal lesions due to the scleroderma, the presence of which would be a decisive factor in assessing the risks of pregnancy. Scleroderma renal disease is, in fact, a major contraindication to pregnancy because of the very poor foetal prognosis and the risk of maternal death due to a lethal progression of renal failure.
Abortion, Spontaneous↗
Idiotypic network: possible explanation of seronegativity in a patient with rheumatoid arthritis.
In order to explain long term seronegativity in certain patients with rheumatoid arthritis (RA), we looked for the presence of anti-idiotypic antibodies against rheumatoid factors (RFs). From a patient's serum with classical but seronegative RA, we isolated a low quantity of the IgG fraction which partially recognized polyclonal RF idiotypes. The purified Fab'2 anti-idiotypic antibodies were able to inhibit up to 48% of in vitro RF production by pokeweed mitogen stimulated lymphocytes from a patient's peripheral blood with seropositive RA. The two main conclusions of this study are: (1) this single patient with seronegative RA has serum antibodies directed against RF idiotypes and (2) these anti-idiotypic antibodies could be implicated in the generation of seronegativity.
Adult↗
[Value of blood lipase determination in pancreatic diseases. Comparison with blood amylase assay].
The comparative diagnostic value of plasma lipase and amylase levels was evaluated in 90 patients with various symptoms of pancreatic disease. Comparison with healthy subjects showed that the lipase assay was more sensitive than the amylase assay in patients with acute pancreatitis or post-traumatic pancreatic injury, but not in patients with chronic pancreatitis. On the other hand, acute pancreatitis can be detected earlier by measuring plasma amylase levels, owing to the rapid release of this enzyme. The relative importance of these two laboratory tests is discussed.
Amylases↗
A highly conserved determinant on human rheumatoid factor idiotypes defined by a mouse monoclonal antibody.
Different human IgM rheumatoid factor (IgM RF) idiotypes have been described defined by polyclonal rabbit anti-idiotypic antibodies. These antisera do not allow clear genetic analysis of the idiotypic determinants, be they cross-reactive or private. Therefore, we tried to obtain a set of monoclonal anti-idiotypic antibodies directed against RF idiotypes. Purified IgM RF serum from a patient with classical rheumatoid arthritis was used to immunize BALB/c mice. The spleen cells were fused with Sp 2/0 Ag 14, a nonsecreting mouse myeloma cell line, and a hybrid producing monoclonal anti-idiotypic antibody was selected. The mouse antibody, an IgG1 kappa, reacts with an identical or similar determinant located on (or close to) the binding site of all tested monoclonal or polyclonal IgM RF from totally unrelated patients with Waldenströms's macroglobulinemias or rheumatoid arthritis. The monoclonal antibody also reacts with 2 rheumatoid arthritis patients' IgG RF and with a low proportion of normal polyclonal IgM without detectable RF activity. An hypothesis is proposed to explain the existence of a such highly conserved determinant on RF idiotypes.
Animals↗
Plasma carnitine in women. Effects of the menstrual cycle and of oral contraceptives.
The plasma concentrations of carnitine were determined in a group of 35 women and 35 men admitted to a clinic, and in another group of 18 women during their menstrual cycle. The values found for the women (45.1 +/- 2.6 nmol/ml of free carnitine and 59.1 +/- 2.8 nmol/ml of total carnitine) were not significantly different from the values obtained in men (respectively 42.4 +/- 1.7 and 55.5 +/- 1.9 nmol/ml). No direct relationship between the free or total carnitine concentrations and the concentrations of circulating lipids could be demonstrated. During the menstrual cycle the plasma concentrations of free and total carnitine remained unchanged. Intake of oral contraceptives caused an elevation in blood triacylglycerols and decreases in the levels of luteinizing hormone, follicle-stimulating hormone, and free and total carnitine.
Adult↗
[In vitro lymphocyte stimulation by the pokeweed mitogen in normal subjects and undernourished patients. Influence of ornithine salts].
The influence of different concentrations of ornithine phosphate and ornithine alpha ketoglutarate on pokeweed mitogen stimulation of immunoglobulin production in vitro was tested. Lymphocyte response of normal subjects was compared with lymphocyte response of undernourished patients. Our results indicate that IgG and IgM synthesis, under usual culture conditions of PWM stimulation, can be optimized in certain normal subjects by adding low concentrations (10(-5)M/l) of ornithine in culture medium. Moreover, in this culture system, the deficit observed in undernourished patients can be partially corrected by adding physiological (close to serum) concentrations of ornithine. More data are necessary in order to find out in vivo the effect of administration of ornithine on the immune system which is influenced by many (specially hormonal) factors.
Humans↗
[Radiological features of small bowel involvement in Henoch-Schönlein syndrome of the adult. Report of 6 cases].
Henoch's purpura involve often the small intestine. These involvement are rarely visualised by radiology. They consist of submucosal edema with thickening of the folds and hematoma with intramural thumbprints on the mesenteric border. The lesions are segmental. The disease process is reversible and healing is complete without stenosis or fibrosis.
Adolescent↗
[Insensitivity of some rats to intraperitoneal injections of collagenase and trypsin].
In the rat peritoneal injections of collagenase or trypsin give rise to severe lesions. In our experience 20% of the animals remain intact. The frequency of lesions increases with older and heavier subjects. Moreover 25% of the rats who remained free of lesions after a first injection of collagenase resist to a second one. This shows that they are strongly protected against the enzyme. The exact nature and location of this protective mechanism are not known.
Animals↗