Lupus vasculitis

Anatomical studies have demonstrated the high incidence of vasculitis in SLE, the appearances of which are variable and non-specific, ranging from necrotizing angiitis which is undistinguishable from periarteritis nodosa, to scarring lesions. Micro-angiitis is easily demonstrated in skin lesions and is also encountered to varying degrees in CNS, renal, cardiac, pulmonary and gastrointestinal localisations. Disease of large vessels is more rare and sometimes causes gangrene of the limbs. In SLE, vasculitis should be distinguished from thrombosis related to lupus anticoagulant and from atherosclerosis favoured by chronic steroid therapy but perhaps initiated by vascular deposits of immune complexes during the acute inflammatory stage. The treatment of lupic angiitis is mainly based on steroid therapy. The results are variable, probably due to the fibrous nature of some of the vascular lesions.     

Shunt nephritis with crescent formation

The case of a 17-year-old girl is reported, who presented shunt nephritis, 8 years after the ventriculo-atrial shunt insertion was made. Renal biopsy showed membranoproliferative glomerulonephritis. The symptoms of uremia mingled with those of chronic septic state. Persistent hypocomplementemia and cryoglobulinemia dominated the picture of the disease. At the beginning of the disease Staphylococcus aureus abunded in the blood culture. In spite of the therapy the general status of the patient declined steadily.     

Disseminated Candida infection syndrome in heroin addicts--dominance of a single Candida albicans biotype

Among 21 intravenous heroin abusers with cutaneous and ocular manifestations of disseminated Candida infection, a single C. albicans strain type (serotype A, biotype 153/7) was isolated from skin lesions in 14 cases. This suggests that central contamination of the heroin with C. albicans is less likely to be the source of infection than an endogenous source, and that one particular strain type is either better adapted than others to grow in the lemon juice used as a heroin solvent, or more likely than others to cause the specific pathology seen in these patients.     

Myoelectrical activity and intraluminal flow in human sigmoid colon

Myoelectric spike bursts were recorded in the sigmoid colon by means of an intraluminal silastic tube equipped with 3 Ag–AgCl ring electrodes fixed 15 cm apart on the tube that was introduced by flexible sigmoidoscopy. In six subjects, the tube was also equipped with three catheters whose tip opened 1 cm aborad from each electrode, for pressure recordings. In six other subjects, the tube was equipped with both electrodes and a catheter opening at the tip of the probe for infusing fluids at a rate of 12 ml/min into the colonic lumen. The fluid was collected with another tube inserted in the rectum and the volume was measured at 1-min intervals. Colonic spiking activity was made of rhythmic stationary bursts (RSB) and of sporadic bursts that were either propagating (SPB) or not propagating (SNPB). All sporadic bursts were associated with intraluminal pressure waves whose amplitude was significantly higher than that associated with rhythmic bursts. In the infusion experiments, the volume of fluid collected did not change significantly whether rhythmic bursts were present or not (3.9±1.7 ml/min and 3.3±1.9 ml/min respectively) (mean±sd). However, the volume was significantly higher when sporadic nonpropagating bursts were present (9.4±4.1 ml/min), and even higher when the sporadic bursts were propagating (21.6±8.8 ml/min). These results indicate that (1) the occurrence of sporadic bursts, particularly when propagating, is associated with intraluminal pressure waves that lead to significant propulsive movements; and (2) rhythmic bursts do not seem to be involved in colonic propulsive activity.This work was supported by the grant DG282 from the Medical Research Council of Canada.     

Elevated levels of the rapid inhibitor of plasminogen activator (t-PAI) in acute myocardial infarction

Myocardial infarction is frequently caused by acute coronary thrombosis. A previous study in patients three years after myocardial infarction has shown twice as high concentrations of the rapid inhibitor of plasminogen activator (t-PAI) as in healthy controls. The present study involves 29 patients with acute onset of myocardial infarction. Already on admission the mean concentration of t-PAI was 16.5 +/- 7.4 units/ml as compared to 7.5 +/- 2.3 in healthy controls. It is presently unknown if moderately elevated t-PAI levels contribute to a delay of the spontaneous thrombolysis of the coronary occlusion, thus promoting the development of myocardial infarction.     

Forme fruste of Hunter's disease

We report on a 19-year-old girl with hepatosplenomegaly and possible hematological disease. We suspected Gaucher's disease on account of histological and biochemical evidence found in specimens from the liver, spleen, and bone marrow. 18 months later, pebbled skin developed on her neck and upper back. Histological examination revealed large amounts of mucous material between the collagen bundles deep in the dermis, which proved to be dermatan sulfate. The clinical and histological symptoms are characteristic for Hunter's disease.     

Pharmacokinetics of intravenously administered 125I-labelled human alpha 1-acid glycoprotein

The volumes of distribution of many acidic drugs have been shown to be close to that of their binding protein, i.e. serum albumin. The distribution of basic drugs mainly bound to alpha 1-acid glycoprotein (AAG) can be questioned with respect to its dependency upon the distribution of this plasma protein. So, a pharmacokinetic study was performed in 7 subjects with human 125I-labelled alpha 1-acid glycoprotein. The steady-state volume of distribution was found to be 5.37 +/- 0.82L. The central volume was 3.23 +/- 0.33L, close to that of plasma volume and the peripheral volume was 2.14 +/- 0.63L. These data allowed the establishment of an equation giving access to the volume of distribution of a basic drug by relating its unbound fraction to physiological distribution of alpha 1-acid glycoprotein. The values yielded by this equation show that the actual and calculated volumes of distribution of basic drugs mainly bound to AAG are discrepant. This protein is thus not the main factor controlling the distribution of basic drugs within the body.     

Concomitant association of radiotherapy and chemotherapy in inflammatory breast cancer. Initial results of phase II trial

Between 01/1986 and 12/1987, 15 patients displaying inflammatory breast carcinoma, were included in a phase II trial. The aim of the treatment was to increase the local response by the potentiation of radiotherapy by concomitant chemotherapy (continuous infusion 5 FU, vindésine, Cyclophosphamide). This treatment consisted of four series of radiotherapy: 18 Gy/10 fractions/12 days spaced by a 2-week rest period between series, to a total dose of 72 GY to the breast tumor. Chemotherapy was undertaken over the first 5 days of radiotherapy in each series. Two patients had metastatic disease (bone-liver). Seven patients had not responded to an initial standard chemotherapy treatment. Six patients were initially treated with the concomitant association. The treatment was very well tolerated by all 15 patients. Inflammation disappeared in all patients within 6 weeks after the beginning of the association. A tumor decrease was observed in all patients, complete in 60%. All 7 patients who had failed initial chemotherapy recurred 8 to 19 months after the association, despite a good response; 3 died of disease and 4 were in local or metastatic evolution on last follow up. The 6 patients treated initially with the association Radiotherapy/Chemotherapy were all alive with a 27 months median follow up (24-40). 2/6 mastectomies were performed: one for breast recurrence and one for persistent residual mass. In this latter patient histologic examination showed no residual active tumor. 4/6 patients have their breast preserved without sequelae. High local response rates were observed with the concomitant combination of radiotherapy and chemotherapy, specially when administered as initial treatment.