Spontaneous rupture of the spleen revealing primary human immunodeficiency virus infection.
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Biomedical subjects
Publications and source records attributed to J H Barrier.
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In apparently localized amyloidosis, there is no appropriate test to determine whether systemic deposits exist. We studied the value of serum amyloid P component (SAP) scintigraphy and labial salivary gland (LSG) biopsy on patients with apparently localized amyloidosis in 12 patients who had neither clinical nor biological evidence of systemic amyloidosis. All patients had an LSG biopsy and echocardiography. Iodine-123-labelled serum amyloid P component (123I-SAP) scintigraphy was performed in all patients. Whole-body scintigraphy was done, and tissue retention was evaluated at 24 h and 48 h. Of these 12 patients, three had amyloidosis in their LSG and had abnormal 123I-SAP scintigraphy; these three had a secondary clinical history of systemic amyloidosis. Three other patients had abnormal 123I-SAP scintigraphy without detectable systemic amyloid deposits, but one had a previous history of bilateral carpal tunnel syndrome treated with infiltration. 123I-SAP scintigraphy in association with LSG biopsy may be helpful in determining the localized or systemic character of amyloid disease.
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In order to evaluate the efficacy of interferon alpha (IFNa) in mixed cryoglobulinemia (MC), a prospective multicenter clinical trial was conduced in April 1992. It consisted of treating 20 clinically symptomatic MC patients with IFNa for 26 weeks. Hepatitis C virus (HCV) infection was detected in 16 patients. A complete or partial clinical remission was obtained in 12 patients (60%). Eleven of these 12 responders (91.6%) experienced a clinical relapse less than 12 months after the end of therapy. Side effects were noted in 10 patients (50%). It was concluded that subcutaneously administered IFNa does not provide long-term remission.
Three men and one woman (mean age 52 years) were admitted to hospital for septicemia (2 cases), sudden partial loss of visual acuity (1 case) and suspected conjunctivitis (1 case). Three of the patients showed risk factors (diabetes, alcohol intoxication, immunosuppression). Panophthalmitis (affecting all tunics of the eye) was apparent from the initial examination in all 4 cases (2 bilateral and 2 unilateral). Ocular involvement was associated with endocarditis and meningitis (pneumococcus) in 1 case, with nocardiosis (pulmonary, cerebral and nodal) in 1 case, and with septicemia with bacterial arthritis (Escherichia coli, streptococcus A) in 2 cases. Hemocultures were positive in 3/4 cases. The micro-organism was also detected in the joint (n = 2), urine (n = 1) and cerebrospinal fluid (n = 1), during pulmonary transparietal puncture (n = 1) and in intraocular biopsy tissue (n = 1). All patients received appropriate antibiotic therapy intravenously and intraocularly. The infection was cured in all cases, but with severe functional sequelae: blindness in 2 cases, and unilateral enucleation in the other 2 cases.
The authors report a giant cell arteritis case associating trismus and hemifacial oedema in a febrile context. After spontaneous regression of other manifestations, the apparition of more typical signs allowed to associate the diagnosis of temporal arteritis, later confirmed histologically. Thus, when facing a trismus case, even more when fever is present, it seems important to associate with the Horton's disease, no matter what the antecedents found at the interrogatory be, whether initial or isolated. The Doppler reveals flux abnormalities of the superficial branches of the external carotid. The examination of facial, temporal and internal maxillary arteries has a good negative predictive value in this pathology. It would be useful in therapeutic supervision.
OBJECTIVES: To estimate the incidence of adult Still's disease (ASD) and to specify, if possible, associated factors. METHODS: A retrospective study of the populations of the Brittany and Loire regions in west France was made from 1 January 1982 to 31 December 1991. All internal medicine and rheumatology practitioners of these regions were consulted. RESULTS: Sixty-two (62) cases were reported (93% response). The disease incidence calculated over five years was 0.16 per 100,000 inhabitants in the study population. There was no sex bias (sex ratio 1.06 in ASD v 1.05 in the overall population. The mean age of the study population was 36 years, with two peaks of distribution at 15-25 and 36-45 years. A history of allergy was present in 23% of patients (n = 14). In two patients, it was possible to correlate an environmental allergen to exacerbation of ASD. CONCLUSION: The yearly incidence of ASD was estimated to be 0.16 per 100,000 inhabitants. However, it was not possible to incriminate any infectious, toxic, or genetic factors in exacerbation of the disease.
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A prospective study, including 86 patients with giant cell arteritis proven by temporal artery biopsies, was performed to determine the prevalence of anticardiolipin antibodies, the antibody isotype, the relationship of antibodies to ischemic involvement and their evolution during corticosteroid treatment. Controls were obtained from 50 subjects without vasculitis or autoimmune disorders. Before treatment, 50% of patients' sera contained anti-cardiolipin antibodies (vs 8% in controls, p < 0.0001). These antibodies were IgG-ACL in 36% of patients and IgM-ACL in 17% of patients (vs respectively, 4% and 2% in controls). No significant association was observed between the incidence of ischemic ocular complications and the levels of anti-cardiolipin antibodies. During corticosteroid treatment, the level of anti-cardiolipin antibodies, mostly IgG class, decreased to become negative in 2 months. An increase of these antibodies was observed with the occurrence of relapses in four patients, after the end of corticosteroid therapy. The significance of IgG and IgM antibodies is different in giant cell arteritis. IgG antibodies may be markers of the course of this vasculitis.
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OBJECTIVE: To determine the prevalence of antineutrophil cytoplasmic antibodies (ANCA) in patients with active giant cell arteritis (GCA). METHODS: 23 patients with GCA were selected according to ACR 1990 criteria. Sera were harvested in all patients at an active stage of the disease and during followup (1 to 3 sera/patient for a total of 50 sera). ANCA positivity was searched for by indirect immunofluorescence (IIF) and enzyme linked immunosorbent assay (ELISA) using a neutrophil extract, and antigen specificity was determined by proteinase 3 (PR3), lactoferrin (LF) and myeloperoxidase (MPO) ELISA: RESULTS: Only 1/23 patients exhibited reactivity in IgG ANCA ELISA and IIF, with borderline anti-MPO reactivity in ELISA which was not inhibited by preincubation with MPO in the liquid phase, and no reactivity in Western blot analysis. Specificity could not be demonstrated in another patient who had positive IgG ANCA ELISA but negative ANCA IIF and negative antigen specific ELISA: All other patients were ANCA negative. CONCLUSION: As our patients with GCA did not exhibit typical ANCA when validated antigen specific assays were used, careful laboratory controls and clinical evaluation would seem essential in cases of apparent ANCA positivity.
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Neisseria elongata subsp. nitroreducens, an usual commensal of the nasopharynx, can cause serious infections, usually endocarditis. A case of Neisseria elongata endocarditis complicating mitral valve prolapse in a patient allergic to penicillin is reported. Cure was obtained with a combination of ceftriaxone and gentamicin, there being no requirement for surgery during the acute phase of the disease.
We carried out a prospective study on stopping steroid use in 22 cases of giant cell arteritis. 30% had transitory insufficient adrenal function. CD8 cells monitoring may be useful in order to decide withdrawing steroids.
Alpha 1-antitrypsin (alpha 1-AT) is the major inhibitor of proteinase 3 (PR3), the main target antigen of antineutrophil cytoplasm antibodies (ANCA) in Wegener's granulomatosis. alpha 1-AT is encoded by a polymorphic gene, with over 75 alleles, defining severely, medium and non-deficient protease inhibitor (PI) phenotypes. We describe the association of severely and medium deficient PI phenotypes with anti-PR3 positive systemic vasculitis, and postulate a pathogenetic role for alpha 1-AT deficiency and the occurrence of ANCA, with specificity for PR3 in a subgroup of patients with Wegener's granulomatosis.