Biomedical subjects
Publications and source records attributed to A Sobel.
In a rare but severe case of hypovolaemic shock related to monoclonal gammapathy, intravenous infusion of Ginkgo biloba extract resulted in dramatic recovery. Treatment was pursued with the oral form.
Since 1977, 22 patients with hereditary angioneurotic oedema have been treated with androgenic-anabolic steroids (danazol or stanozolol). The most frequent adverse reactions were myalgia, weight increase, changes in libido, and menstrual disorders in women. In view of these side-effects, it is suggested that patients about to undergo surgery (chiefly E.N.T. or stomatology) should be put, one week before the operation, on a 10-day high dosage treatment. Long-term treatment should be reserved to severe forms of the disease, with frequent attacks and visceral involvement, using the minimum effective dosage (which is always low) without trying to correct biochemical abnormalities. Stanozolol should be given by preference to men and danazol to women. Treatment is always more difficult in pre-menopausal women. With this method, the complications, notably hepatic disorders, are rare, and long-term treatment, when absolutely necessary, is well tolerated.
Explore the source record for details and available documents.
The in vivo function of the reticulo-endothelial system in humans is currently studied by the blood clearance of 51Cr labeled RBC sensitized with IgG anti-Rh. By replacing 51Cr by 111In we developed a method which allows imaging and assessment of clearances of circulating immune complexes (CIC) by individual organs. Nine normal subjects were studied successively with 51Cr labelled CIC and 111In-labelled CIC by blood sampling and sequential imaging. Time activity curves were obtained over spleen, liver, heart lungs and kidneys. The blood clearance values were similar with 111In (T1/2 +/- SD = 23.4 min +/- 6.4 min) and 51 Cr (T1/2 = 22.9 min +/- 5.8 min). The blood clearance derived from the time activity curve obtained over the cardiac area (T1/2 = 26.6 min +/- 8.4 min) was not significantly different from that obtained by blood sampling. Most of the CIC were taken up by the spleen with a T1/2 of 20.3 min +/- 4.1 min. Although hepatic uptake was observed in all subjects, it was lower than that of the spleen and CIC were subsequently released from the liver in five of the nine patients. The spleen/liver ratio increased with time, reaching a plateau averaging 24.0 +/- 5.8 within 80 min in all subjects. CIC imaging allows, by a non-invasive procedure, the computation of CIC blood clearance and the estimation of the respective role of spleen and liver in phagocytic function.
Explore the source record for details and available documents.
Thyrotropin releasing hormone (TRH) stimulates the synthesis and release of prolactin in mammotropic cells of the anterior pituitary and in GH4C1 cells. TRH simultaneously enhances the phosphorylation of a small number of proteins revealed in GH4C1 cells by two dimensional gel electrophoresis. The same phosphoproteins could be detected in normal rat pituitary cells in primary culture and were phosphorylated with the same pharmacology as in GH4C1 cells. Moreover, these proteins may also be related to hormonal regulations of the other cell types of the anterior pituitary.
The coincidence of fragile X syndrome (fra(X] and systemic lupus erythematosus (SLE) in the same family is reported here for the first time. A 16-year-old boy with typical fra(X) had a severe SLE with multiple organ involvement. His 12-year-old sister of normal intelligence had circulating antinuclear antibodies and proliferative glomerulonephritis. The fra(X) was not found in her karyotype. Except for abnormalities due to immunosuppressive treatment during pregnancy, the association of SLE and chromosome abnormalities has been only reported in Klinefelter's syndrome. The possible pathogenic role of sex hormonal abnormalities due to an extra X chromosome has been suggested in the occurrence of SLE.
Explore the source record for details and available documents.
7 patients were treated by plasma exchanges and antiplatelet drugs for thrombotic thrombocytopenic purpura (TTP). The effectiveness of therapy was reviewed daily and retrospectively estimated on day 3, d6, d9, and d12. 6 of these 7 patients were cured. The low predictive value of the initial response to therapy is underlined. The clinical and biological status on d12 seems to have the best predictive value for the final outcome. These data encourage us to continue the plasma exchanges until d12, whatever the initial response to therapy in TTP.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
A remarkably severe spontaneous amyloidosis involving multiple organs has characterized the inbred PS mouse strain since the 25th generation. The amyloid substance was extracted with H2O and purified by successive gel filtrations on Sephadex G100, Sephadex G10 and Biogel P4. It was submitted to biochemical, immunochemical and histochemical analysis in order to determine its origin. Potassium permanganate resistance of the affinity for Congo Red dye, aminoacid composition, cross-reactivity with anti-mouse light chains antisera suggested an amyloidogenic process comparable to that described for the AL substance in man. However, even if abnormal lymphoid infiltrates were present in several organs, the presence of M component could not be demonstrated in serum or urine of these mice. This indicated either a limited tumoral mass or a tumor poorly secreting a precursor that would be strongly amyloidogenic. Alternatively, the existence of a so far unidentified precursor could not be excluded.
The actions of thyrotropin-releasing hormone (TRH) on protein phosphorylation in the GH4C1 clonal strain of rat pituitary cells were examined by two-dimensional polyacrylamide gel electrophoresis and related to the ability of this tripeptide to stimulate prolactin (PRL) synthesis and release, respectively. Between 2 and 10 min after addition of the hormone, TRH increased the rate of phosphorylation of a small number of specific cytoplasmic proteins. The dose-response characteristics for TRH-induced protein phosphorylation were closely similar to those for its biological actions (ED50 congruent to 2 nM). TRH did not stimulate protein phosphorylation in the related GH12C1 cell strain which lacks TRH receptors. The proteins modified by TRH treatment were present in low amounts in the cell; they were not detectable by silver staining of the gels, except for protein 1 (Mr = 80,000) whose TRH-induced conversion from its unphosphorylated to its more acidic phosphorylated form was observed directly on silver-stained gels. Two distinct sets of phosphoproteins were identified among the seven affected by TRH: set I, five proteins whose phosphorylation was increased only by agents which stimulated PRL synthesis; and set II, two proteins whose phosphorylation was also increased by vasoactive intestinal peptide, which stimulates PRL release but not its synthesis in GH4C1 cells. This distinction between phosphoproteins related either to the release or synthesis of PRL was substantiated further by the use of an analog of TRH, A42658, which stimulated PRL synthesis and phosphorylation of proteins of set I at a lower concentration (0.2-0.5 microM) than was required to stimulate PRL release and phosphorylation of proteins of set II (greater than 0.5 microM). Finally, pretreatment of the cells with 1 mM ethylene glycol bis(beta-aminoethyl ether)-N,N,N',N'-tetraacetic acid, which prevents TRH-induced PRL release but not synthesis, also suppressed TRH-stimulated phosphorylation of proteins of set II, but not of set I. These results relate stimulation by TRH of the phosphorylation of two distinct sets of proteins to the two major biological action of TRH in GH4C1 cells; proteins of set I appear to be related to the regulation of PRL synthesis, whereas those of set II appear related to modulation of PRL release.
A retrospective study of 258 reports of Goodpasture's syndrome published from 1955 to 1982 has made it possible to follow changes in prognosis and to determine the present prognostic factors. In these 27 years, the mortality rate has dropped from 86% to 13%, and 51% of the patients now survive without dialysis. Death from respiratory failure virtually disappeared as soon as plasmapheresis was introduced. However 36% of patients are still threatened with chronic renal failure, the risk being higher in patients with initial oliguria or anuria and with extra-capillary crescents involving more than 50% of glomerules, and still higher when these two factors coexist. In such a case, only 21% of patients may hope to survive in renal autonomy.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.