[Hydatidosis involving the heart and liver simultaneously. A case report with successful surgery].
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Biomedical subjects
Publications and source records attributed to P Godeau.
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BACKGROUND: D-penicillamine-induced muscle disorders are well-known, tiopronine-induced disorders are less often reported. CASE REPORT: A 62-year-old patient, given tiopronine for rheumatoid arthritis, developed severe polymyositis with characteristic clinical and pathology features. The course was favorable after tiopronine withdrawal and substitution with methotrexate. DISCUSSION: Clinicians should be aware of the side-effects of tiopronine, particularly muscle disorders, and implement careful surveillance to achieve early diagnosis and appropriate therapy.
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A previously healthy 34-year-old woman, was diagnosed as having systemic lupus erythematosus (SLE), with membranous glomerulopathy which improved rapidly. Neither lupus anticoagulant nor anticardiolipin antibodies were detected in her plasma. After three months of total remission, she developed a severe pulmonary thromboembolism for which no specific biological cause was found. Her plasma was analysed for different antiphospholipid antibodies: lupus anticoagulant and anticardiolipin antibodies were again negative. Using an ELISA prepared with either five different anionic phospholipids or zwitterionic phosphatidylethanolamine, solely an anti-phosphatidylethanolamine IgG was discovered in her plasma. In lupus patients, the presence of antiphospholipid antibodies is now well recognized as a high risk factor for repeated thrombosis and/or recurrent abortions. This case suggests that the presence of antiphosphatidylethanolamine antibody should be investigated in cases of unexplained thrombosis in SLE, where the usual clinical and biological investigations have failed to shed light.
Cardiac complications are exceptional in long-term chloroquine therapy; congestive heart failure and restrictive cardiomyopathy may develop, but disorders of conduction are more frequent. The characteristics of these disorders emerge from 12 cases in the literature and from 2 personal cases. The usual disorder is fascicular block which may become a complete, syncopal, atrioventricular block, as in one of our 2 patients. The time elapsed between the beginning of treatment and the occurrence of these disorders (2 to 30 years) and the total dose of chloroquine received (100 to 2,500 g) are extremely variable. Retinopathy or neuromyopathy is present in 64 and 35 percent of the cases respectively. The diagnosis is confirmed by endomyocardial biopsy with electron microscopic study which shows vaculoar myopathy with numerous large secondary lysosomes containing a dense material of lamellar structure (myelinic figures, curvilinear bodies). Regression of heart conduction disorders after withdrawal of chloroquine seems to be inconstant and incomplete. The rare occurrence of this complication raises the question of genetic predisposition. We believe that chloroquine therapy should be contra-indicated in patients with a history of conduction disorders and that a 6-monthly electrocardiographic control of these patients would be justified.
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New insights about the following aspects of lupus erythematosus are developed: (1) thrombotic and neurological manifestations associated with the presence of antiphospholipid antibodies; (2) cardiac complications, especially coronary ischemia, conduction abnormalities and valvular involvement; (3) pregnancy, with particular attention to fetal and maternal prognoses, Soulier-Boffa syndrome and congenital atrioventricular heart blocks; (4) subacute lupus erythematosus, bullous lupus and cutaneous manifestations associated with the lupus anticoagulant.
Acebutolol whose hypotensive properties are well established, appears as a most interesting beta-adrenergic blocking agent for simplification of treatment of moderate hypertension in man. Hypertensive patients likely to benefit from treatment with acebutolol can be identified within 5 days after administration of a single initial dose of the product. Long-term treatment with acebutolol can be considered in those patients identified as receptive. In non-responders, acebutolol can be used in combination with a diuretic agent. Such simplification of the therapeutic schedule in hypertension will certainly be helpfull both to the physician and his patient.
Blood chloroquine concentrations were measured in 5 patients having complications related to this treatment. No significant differences were noted when compared to controls. Further studies are required however before drawing any definite conclusions. The persistence of fairly high residual levels, several months after stopping treatment, is evidence of chloroquine retention in these patients.
A case is reported of autochthonous leprosy in a French patient who had never lived in an endemic zone. The disease was of the lepromatous leprosy type and the liver, spleen, skin nerves and muscles were affected. Characteristic findings were Hansen's bacillus in nasal scrapings, a deficiency in cellular immunity, and the presence of cryoglobulins and circulating immune complexes. Autochthonous leprosy which has not been imported, and without signs of direct contagion, appears to be exceptionally rare. The authors review the clinical forms of leprosy and immunological findings.
Two cases are reported of popliteal cysts simulating sural phlebitis, one in a patient with lupus, the other during the course of rheumatoid arthritis. Diagnosis is based upon the following signs: sudden onset of pain, sometimes after effort, and in the knee rather than in the calf, and more especially ecchymosis occurring in the internal supramalleolar region. The decision as to whether anticoagulant treatment should be instituted must be based on the certitude of the diagnosis, and this can be obtained in an atraumatic manner by ultrasonography of the popliteal fossa as shown by iconography.
Highly selective angiography was used to study eleven cases of Horton's arteritis. The results showed the presence of segmental vascular abnormalities which, though mainly affecting the temporal artery region, were also present in that of the internal maxillary artery. This examination procedure can be of great value by assisting the surgeon in his choice of biopsy sample, which is essential for diagnostic purposes.
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We have followed 45 patients affected with Wegener's granulomatosis and conducted a retrospective analysis of its evolutive aspects and prognostic factors. The study concerned 25 men and 20 women, with an age-range of 50 +/- 15 years at the onset of the disease. The time between occurrence of the first manifestations and the diagnosis was 16 +/- 21 months, the follow-up was 6.3 +/- 8 years. All patients except 3 were given steroids, associated in 39 cases with an immunosuppressor and in 9 cases with plasma exchanges. In 2 localized forms in the ENT area, remission was possible with cotrimoxazole alone. 15 deaths occurred, the causes of which are infection and renal insufficiency when death occurs early, and cardiovascular disorders and neoplasms when death occurs late. The survival probability is 93 p. cent at one year, 83 p. cent at 3 years and 75 p. cent at 5 years from the time of the first manifestations and 75 p. cent at one year, 53 p. cent at 3 years and 50 p. cent at 5 years from the time of the diagnosis. Relapses have been reported in 11 patients, some of which occurred several years after discontinuation of the treatment: they emphasize the need for prolonged monitoring of these patients. Presence of one of the poor prognostic factors: age 50 years (p less than 0.05), presence of renal insufficiency (p less than 0.05) or hemoglobinemia less than or equal to 10 g/cc (p less than 0.05), should be an indication for a more intensive treatment of Wegener's granulomatosis.
A new case of lymphocytic interstitial pneumonitis developed in the course of a persistent generalized lymphadenopathy syndrome is reported. The patient was a 30-year old Haitian woman with only her ethnic risk factor. Broncho-alveolar lavage showed high cellularity with mostly major lymphocytosis (76%) and a fall of the OK T4/OK T8 ratio to 0.23. The LAV was isolated from the lavage fluid lymphocytes on the same day and within the same culture time as from blood, using lymphocyte culture and measurement of reverse transcriptase activity in the supernatant fluid of cell cultures. This, together with the strongly positive (1/80) LAV serology in fluid as compared with blood (1/640), suggested that the LAV virus was directly or indirectly involved in the pneumonitis, being responsible for lymphocyte proliferation as it is in lymph nodes. No superinfection with a bacterial, fungal or other than LAV viral agent was found in blood or in lavage fluid. Lymphocytic interstitial pneumonitis is uncommon in AIDS or ARC (13 cases reported), but its incidence no doubt is underestimated, as it may be latent. It certainly accounts for the high lymphocyte count observed in broncho-alveolar lavage fluid in the absence of superinfection and, most probably, for many cases of so-called "non-specific pneumonia". In 1986, patients with apparently primary lymphocytic interstitial pneumonitis should be investigated for AIDS or ARC.
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Much that has been written in the last few years concerning temporal arteritis has allowed us to better clarify the clinical picture, and for example frequency of hepatic involvement. The atypical forms of the disease such as those that involves large arteries, or those in which sedimentation rate is normal, are better known. It is in such atypical cases, particularly in a young patient, that an external carotid arteriography is most useful. Though the pathophysiology of the disease is not well understood, serologic and histologic arguments exist in favor of an autoimmune etiology, in which the antigen would be the alestin, found in the internal elastic lamina of the artery. Temporal arteritis is theorically well classified amongst the giant cell granulomatous arterities but there are some atypical forms difficult to distinguish from Takayasu's arteritis or even periarteritis nodosa.