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Biomedical subjects

D Danoff

Publications and source records attributed to D Danoff.

36 records · Page 2Linked to original sources

Can experienced clinicians predict the outcome of lupus nephritis?

The ability of four experienced clinicians to predict short-term outcome (serum creatinine level at 1 year) and long-term outcome (renal insufficiency) was evaluated in 87 patients with lupus nephritis. The correlational agreement and the accuracy of their predictions were contrasted with the actual outcomes observed and with statistically generated prognostic regression models. In contrast to previously published data, all four clinicians predicted both short-term outcomes (P < 0.001) and long-term outcomes (P < 0.02) well. The clinicians' predictions approximated that of a statistically generated computer model for both agreement and accuracy for renal function at 1 year. The four clinicians identified nearly identical clinical variables as important in determining prognosis. Provision of biopsy data to the clinicians improved short-term and long-term prediction slightly. The value of the statistical models was 'validated' by demonstrating that three of the four clinical variables identified by the models, but not by the clinicians, could enhance clinical prediction (P < 0.05). In addition, the extent of tubulo-interstitial involvement on biopsy, a predictor that has recently received increased attention, could improve the long-term clinical predictions of all four clinicians (P < 0.05).

Adolescent↗

Association of anti-DNA idiotype markers with clinical and serological manifestations in patients with systemic lupus erythematosus.

Serum levels of 6 anti-DNA antibody idiotypes were measured in 65 consecutive patients with systemic lupus erythematosus (SLE) and 45 healthy subjects. Five of the 6 idiotypes were elevated in SLE sera compared to the normal controls (p less than 0.005). Analysis of the associations of the idiotypes with clinical, hematological, and serological characteristics revealed that significantly decreased serum levels of 3 idiotypes (103.1, 100, and 1305) were associated with nephritis and that one of these idiotypes (103.1) was also associated with discoid rash. An association of lowered levels of 3 idiotype markers (604, 1305, and 1400) was also observed with the presence of lupus anticoagulant and anticardiolipin antibodies. Serial studies in individual patients with SLE nephritis failed to show a close correlation of serum idiotype levels with the degree of proteinuria, creatinine clearance, anti-DNA antibody, or complement values. The association of decreased levels of specific idiotypes with the presence of nephritis, discoid rash, and antiphospholipid antibodies suggests the participation of these antibodies in the pathogenesis of disease.

Adolescent↗

The relationship of socioeconomic status to subsequent health status in systemic lupus erythematosus.

We examined the relationship of socioeconomic status to health status, as determined by the Arthritis Impact Measurement Scales, in 78 systemic lupus erythematosus patients who had been entered into a prospective study. After controlling for age, disease duration, and disease severity, a significant relationship between socioeconomic status and outcome was not demonstrated. All study subjects had health insurance for medical services. The results have potential implications for health care policy.

Depression↗

Antiphospholipid antibodies in systemic lupus erythematosus: evidence of an association with positive Coombs' and hypocomplementemia.

Anticardiolipin antibodies (aCL) were evaluated in 65 consecutive patients with systemic lupus erythematosus using an enzyme linked immunoassay (ELISA) and were detected in 14 (22%). There was no association of aCL with active disease, specific clinical manifestations, high DNA binding or circulating immune complex levels. The presence of aCL was associated with lupus anticoagulant activity (p less than 0.001), positive VDRL (p less than 0.05), and lower mean platelet counts (p less than 0.05) and C3 levels (p less than 0.05). Both aCL and lupus anticoagulant activity were associated with positive Coombs' (p less than 0.01) and low C4 (p less than 0.01 and p less than 0.05, respectively). Analysis of red blood cell (RBC) eluates and absorption studies using fixed RBC suggested that some aCL may act as anti-RBC antibodies, likely directed at membrane phospholipid epitopes. Furthermore, we hypothesize that complement may participate as a cofactor maximizing lupus anticoagulant activity.

Adult↗

Reticuloendothelial system Fc receptor function in systemic lupus erythematosus: effect of decreased sensitization on clearance of autologous erythrocytes.

Recent reports of reticuloendothelial system Fc receptor function using anti-Rh(D) coated red blood cells (RBC) provide conflicting data regarding the degree of abnormality in systemic lupus erythematosus (SLE). The effect of different sensitizing doses of anti-Rh(D) on Fc receptor function was evaluated in 10 controls and 9 patients with SLE (5 with renal disease, 4 without) using 1 microgram anti-Rh(D) (6,700 molecules anti-Rh(D)/RBC by saturation analysis) and 0.125 microgram anti-Rh(D) (1,100 molecules/RBC). At the lower sensitizing dose the sensitivity of the assay was significantly increased. To correctly interpret the significance of studies of Fc receptor function from different centers, it is necessary to know the exact sensitizing doses used for each study.

Adult↗

Wegener's granulomatosis. Clinical features and outcome in 13 patients.

Thirteen patients with Wegener's granulomatosis were seen over 10.5 years. The clinical features resembled those in previously reported series, except for the increased frequency of inflammatory arthritis, which was a prominent early feature in ten patients (77%). Four (31%) of the 13 had fulminant vasculitis and died before receiving an adequate course of cytotoxic drug therapy. Two of these four had a pulmonary-renal syndrome that mimicked Goodpasture's syndrome. All of the remaining nine patients (69%) achieved an initial remission with cytotoxic agents (azathioprine or cyclophosphamide), but four died in less than one year with no evidence of vasculitis at autopsy. The 56% survival rate to one year in these nine patients contrasts with an 86% to 100% survival in other series. Chronic renal failure was a prominent sequela in those who survived one year.

Adolescent↗

Peripheral neuropathy with IgM kappa monoclonal immunoglobulin directed against myelin-associated glycoprotein.

Three patients with a monoclonal IgM kappa paraproteinemia had a slowly progressive segmental demyelinating peripheral neuropathy. Sural nerve biopsies showed predominantly large fiber loss, with widening of the intraperiod line of myelin associated with active demyelination. Immunoblot analysis showed reactivity of IgM to myelin-associated glycoprotein (MAG) of human myelin. An enzyme-linked immunosorbent assay (ELISA) was developed to provide a simple quantitative technique for detection of antibodies to MAG. Treatment with corticosteroids, immunosuppressive agents, and plasmapheresis produced no significant improvement.

Aged↗

Deforming arthritis in systemic lupus erythematosus.

Of 45 patients in the McGill Lupus Registry 6 were found to have Jaccoud's deformities. One of these 6 and 4 others had fixed flexion contractures of the elbows. Those with Jaccoud's deformities were similar to the other patients with systemic lupus erythematosus in all respects except that those with Jaccoud's syndrome had had their disease and their arthritis significantly longer. It is concluded that Jaccoud's deformities are the result of longstanding arthritis and that elbow contractures occur via a different mechanism.

Adult↗

Disseminated coccidioidomycosis: clinical, immunologic and therapeutic aspects.

A patient with disseminated coccidioidomycosis initially had pulmonary and skin manifestations and survived for 14 years before dying of meningitis due to Coccidioides immitis. In addition to several courses of amphotericin B therapy the patient received injections of transfer factor derived from appropriate donors and miconazole nitrate therapy. The immunologic defence mechanisms of the patient during the course of his disease were studied and the possibility of a cell-mediated immunologic defect, potentially reversible by transfer factor, was demonstrated.

Adult↗

Sleep attacks--apparent relationship to atlantoaxial dislocation.

Sleep attacks, while being characteristic of narcolepsy, have been reported in several metabolic and central nervous system disorders--most of which are refractory to treatment. We have recently treated a 47-year-old man with disabling sleep attacks who was found to have atlantoaxial dislocation. Despite narrowing of the sagittal cervical canal diameter to 12 mm (behind the dens), symptoms of spinal cord compression were mild and intermittent, while signs were absent. Posterior fusion of the C-1 and C-2 vertebrae resulted in relief of symptoms. We suggest that this sleep disorder was a function of impaired efferent control of respiration.

Axons↗

Vasculitis as a presenting manifestation of primary biliary cirrhosis: a case report.

The association of vasculitis and asymptomatic primary biliary cirrhosis (PBC) was documented in a 39 year old female. Cyclophosphamide was used with good results. The Raji cell assay for circulating immune complexes (CIC) was positive initially with further values changing in parallel with disease activity. The C1q binding assay failed to reveal significant levels of CIC at any time. Lymphocytotoxic antibodies were not detected in the serum. Sucrose density ultra-centrifugation experiments revealed 9-17S material in sera that were positive for CIC by the Raji cell assay. We conclude that intermediate size CIC may be important in the pathogenesis of the vasculitis associated with PBC.

Adult↗

Reticuloendothelial system Fc receptor function in systemic lupus erythematosus.

Reticuloendothelial system Fc receptor function was measured in 10 patients with systemic lupus erythematosus (SLE) taking corticosteroids, 10 SLE patients not receiving corticosteroids and in 19 controls, 9 of whom were corticosteroid-dependent asthmatics and 10 of whom were healthy. Clearance studies were performed using autologous 51 Cr-labeled erythrocytes that had been sensitized with human IgG anti-Rh(D) [6,700 molecules/cell]. The clearance curves could be split into a fast (t1/2 fast) and a slow (t1/2 slow for any of the groups of patients. The results were correlated with serum C3, C4, DNA-binding, fluid phase 125I-Clq binding, a disease activity index, corticosteroid dose and duration of therapy with corticosteroids. The only significant correlation was an inverse correlation of C4 with the t1/2 slow in SLE patients not on corticosteroids (r=- 0.71, p less than 0.05). The t1/2 slow of the 3 SLE patients with active nephritis (86 +/- 40 min) was significantly different from the 17 SLE patients with inactive nephritis or normal renal function (37 +/- 5 min) (P less than 0.05). We conclude that there is no overall defect of Fc receptor function in our patients with SLE although there is decreased clearance in patients with active lupus nephritis.

Asthma↗