Bilateral adrenal hypertrophy in an AIDS patient with multivisceral human cytomegalovirus infection.
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Biomedical subjects
Publications and source records attributed to M Gayraud.
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Fifteen patients suffering from severe systemic diseases were treated with monthly pulses administration of cyclophosphamide (0.7 g/m2 of body surface): 8 acute systemic lupus erythematosus, 2 Wegener's granulomatous, 1 polyarteritis nodosa, 1 rheumatoid vasculitis, 1 progressive systemic sclerosis, 1 relapsing uveitis and 1 dermatopolymyositis. The indications for cyclophosphamide were: glomerulonephritis (6 cases), resistance to previous treatments (7 cases) and undesirable side effects of corticosteroid therapy (2 cases). After 3 pulses, the disease was controlled in 12 patients (80%) and corticosteroids could be decreased in all 12 cases without an evolutive relapse of the disease. Five patients developed infections (2 septicemia, 1 zona, 1 herpes gingival stomatitis and 1 viral meningitis) which were treated without sequelae. One patient developed cystitis with hematuria after the 3rd pulse; association of mesna, a urinary tract protective agent, enabled the continuation of treatment without a cystitis relapse. At the end of our retrospective study, the efficacy of pulse cyclophosphamide administration seems to be satisfactory but the risk of undesirable side effects should limit its use to severe systemic diseases or those resistant to conventional therapies.
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Sensory neuropathy of trigeminal nerve is one of the neurological complications of systemic diseases, particularly scleroderma. Three cases are reported in which onset occurred at different stages of the disease, the main symptom being painful dysesthesias. All three branches of the trigeminal nerve may be affected but a preference is apparent for V2 and V3. Signs are usually limited to hypoesthesia and the chronic course is influenced little by treatment.
Muscle biopsies from 57 patients with dermatomyositis or polymyositis were histologically evaluated and compared with the disease's clinical course. Perifascicular atrophy, perivascular infiltrates and tubular inclusions in endothelial cells were significantly more frequent in young patients with dermatomyositis. On the other hand, in adult polymyositis, which evolves more slowly, necrosis with slight muscular atrophy and perinecrotic infiltrates was observed. This division into two groups was clear when the clinical evolution and histological patterns were compared. The mean age of each group was different, but there was a large overlap. Two different pathogenetic mechanisms can be envisaged: primary involvement of muscle capillaries with muscle ischemia in young patients with dermatomyositis and primary involvement of muscle fibers in adults afflicted with polymyositis.
The treatment and prognosis of periarteritis nodosa associated with hepatitis B virus were reconsidered from a series of 13 patients representing 32.5 per cent of the 40 patients with periarteritis nodosa admitted during the same period. HBs and HBe antigens were present in every case, and hepatitis B virus replication was demonstrated by the finding of viral DNA in serum. One patient had anti-HBc IgM's. Five patients were treated with corticosteroids, cyclophosphamide and occasional plasma exchanges. All were cured or achieved complete remission. Eight patients were treated with plasma exchanges and vidarabine, either as first-line therapy (3 cases) or after failure of corticosteroids and/or immunosuppressants (5 cases). This treatment was clinically effective in 5/8 cases, including 3 with seroconversion. The 2 patients in whom the combined treatment failed were given corticosteroids; one of them also had plasma exchanges. The 8th patient died after a few days of treatment. Eleven of the 13 patients are still alive and either cured or in complete remission. Two patients who developed severe chronic hepatitis after steroids were discontinued received vidarabine alone: arrest of viral replication was obtained in both cases, with emergence of an anti-HBe (but not anti-HBs) antibody. The overall positive virological response rate to vidarabine alone or combined with plasma exchanges was 50 per cent. When vidarabine was prescribed as treatment of acute periarteritis nodosa (the 2 cases where it was used for chronic hepatitis being excluded), this response rate was 37.5 per cent. This, in patients with periarteritis nodosa associated with hepatitis B virus immunosuppressive drugs should be withdrawn and replaced by plasma exchanges and antiviral agents. This would be the first-line treatment to be replaced by corticosteroid therapy if it fails.
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Recurrent thromboembolic accidents are frequent complications of Behçet's disease. Ten patients were investigated, 5 of them during an acute exacerbation of the disease. Abnormal responses to the venostasis test and to DDAVP (i.v.) were demonstrated in acute cases. Factor VIII activities after DDAVP were increased in all 10 patients, which suggests that the response of the vascular endothelium to the stimulus was normal and that a fibrinolytic system inhibitor may be present in the plasma of some patients.
The renal biopsies of 13 patients presenting with a predominantly renal form of angiitis were reviewed. The principal lesions were glomerular with a segmental and focal extracapillary glomerular nephritis in all cases. Arteriolar lesions with necrosis or granuloma were inconstant (6/13) but necrosis of the tuft reflecting capillary involvement were common (11/13). Interstitial tubular disease and immunofluorescence were not specific. Renal biopsy is a good indication of diagnostic when performed early before the development of sclerosis: arteriolar necrosis and suggestive changes: extracapillary segmental and focal glomerulonephritis. In the latter case, a careful search for extrarenal involvement with guided biopsy studies usually allow a diagnosis to be confirmed. The renal and extrarenal clinical signs, the biochemical changes, treatment and outcome were analysed. Two of the 13 patients had Wegener's disease, one patient had angiitis and linear fixation of IgG along the glomerular and tubular basal membranes.
Seven patients, 4 women and 3 men afflicted with severe progressive systemic sclerosis (PSS) were treated with Plasma Exchange after failure of different other treatment. All patients presented Raynaud phenomenon and arthritis, 6 patients presented extensive skin lesions, 5 of them digestive manifestations, 3 pulmonary fibrosis. In one case PSS was associated with polymyositis, one patient presented bilateral recurrent cornea ulcerations, (Sjögren Syndrome++) and one patient numerous skin ulcerations. In 5 patients adjuvant corticosteroid therapy was given during the course of PE. In 3 patients PE must be stopped after one or two sessions because of insufficient venous access. Among the 4 other patients 8 to 20 PE were performed: the patient with cornea ulcerations became blind during the treatment, skin ulcerations and severe Raynaud phenomenon did not improved in two other patients. Benefit of PE was noted in only one patient with regressive myositis, and improvement of articular and cutaneous symptoms. Therefore, PE are not useful in most patients afflicted with PSS, they are difficult to realize in numerous patients and did not improve clinical symptoms in most cases.
The effects of estradiol, which are requisite for the normal occurrence of events related to reproduction, are not restricted to that field. They play a part in the overall trophicity of the female organism. On the other hand, relative or absolute estrogen hyperactivity results in harmful clinical effects and in disastrous cellular consequences, i.e. initiation or promotion of genital carcinomas. After a review of the different steps of the synthesis and action of estradiol, the various compounds that are active on these steps are considered: antigonadotropes (norsteroid progestogens, androgens, danazol, prolactin, corticosteroids, LHRH), inhibitors of estrogen synthesis (aminogluthetimide and testolactone), compounds that increase catabolism (progestogens), and antagonists of the cellular effect of estradiol (tamoxifen, nafoxidin, clomifen, progesterone, progestogens and androgens). The putative or established site of action and the main aspects of therapeutic use are given for each substance.
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