Progressive lupus glomerulonephritis. Treatment with prednisone and combined prednisone and cyclophosphamide

We report a prospective randomized study of 39 patients with systemic lupus erythematosus and progressive glomerulonephritis who were assigned to treatment groups that received either prednisone alone or prednisone and cyclophosphamide combined. They received treatment for 6 months and were then followed up for an additional 18 months. No difference in outcome was seen in the two groups at the end of 6 months. Among patients followed up for an average of 24 months, fewer individuals showed later renal progression among those treated with cyclophosphamide and prednisone than among the group treated with prednisone alone.     

Goodpasture syndrome involving overlap with Wegener's granulomatosis and anti-glomerular basement membrane disease

A 68-year-old Caucasian woman presented to the hospital with nodular pulmonary infiltrates and acute renal failure. Wegener's granulomatosis was initially considered to be most likely because of the presence of increased serum levels of c-anti-neutrophil cytoplasmic antibodies (c-ANCA). A consultation through the Internet after a renal biopsy demonstrated crescentic, necrotizing glomerulonephritis and linear deposits of immunoglobulin G (IgG) and complement C3, typical of anti-glomerular basement membrane (GBM) disease. Hemodialysis was instituted; however, the patient suddenly developed a massive cerebral hemorrhage and died before full therapy could take effect. Postmortem analysis of the patient's sera revealed high titers of IgG against the alpha 3 NC1 domain of type IV collagen. Serologic evidence of both p-ANCA and anti-GBM antibodies are becoming more frequently recognized in the setting of rapidly progressive glomerulonephritis. The patient reported here had the unusual combination of c-ANCA antibodies with anti-GBM disease, and this association raises complex questions regarding the pathogenesis of this type of renal injury.     

Pilot trial of immunoglobulin versus plasma exchange in patients with Guillain-Barré syndrome

We compared intravenous immunoglobulin (IVIG) and plasma exchange (PLEX) in the treatment of 50 patients with Guillain-Barré syndrome (GBS). Standard outcome measures did not differ for the two groups. Sixty-one percent of the PLEX-treated group and 69% of the IVIG-treated group improved by one disability grade at 1 month. The complication rate was higher in the PLEX-treated group. We conclude that the efficacy of IVIG in the treatment of GBS is comparable with that of PLEX and that it can be used safely, although we had a small number of patients. We did not observe a higher relapse rate with IVIG. The usefulness of combination therapy is unknown at this time.     

Adenine metabolism during and after exchange transfusions in newborn infants with CPD-adenine blood

CPD-adenine blood (adenine in a final concentration of 0.25 mmol/1) was used in exchange transfusions in four newborn infants. The amount of adenine in one exchange transfusion ranged from 27 to 29 mumol per kg bodyweight. The maximum P-adenine concentration during the exchange transfusions ranged from 4 to 8 mumol/1 but decreased to pretransfusion levels 20 minutes after the exchange transfusions. During a 24-hour period following the exchange transfusions, the total urine excretion of adenine and 2,8-dihydroxyadenine corresponded to 0.5 to 1.3 per cent of the given adenine dose calculated on a molar basis. Accumulated data indicate that CPD-adenine blood can be used even in repeated exchange transfusions in newborn infants.     

An adult case of Reye syndrome induced by diclofenac sodium, and recovered by plasma exchange

This study was designed to measure circulating hepatocyte growth factor (HGF) in a rat model of arterial thrombosis. The carotid endothelial surface of rats was denuded with a balloon catheter and partially occluded. The amount of thrombus formation was expressed as the difference between the wet weights of paired treated and nontreated carotid arteries. Thrombus weight increased gradually up to 60 min after denudation. Plasma HGF did not increase before 30 min, but had significantly increased by 60 min compared with all previous measurements (p<0.05). Measurements of circulating HGF may be useful in the early diagnosis of arterial thrombosis.     

Combinaed chemotherapy with cyclophosphamide, vinblastine, procarbazine, and prednisone (CVPP) for patients with advanced Hodgkin''s disease. An alternative program to MOPP

When electroshcok therapy was administered to male psychiatric patients without anticonvulsive premedication, serum growth hormone (GH) increased; the increase was not prevented by an infusion of 20% glucose (5 ml per min) 20 min prior to electroshock. Therefore, the GH rise is not caused by muscle exercise during convulsions. Infusing 30 mg of phentolamine 40 min prior to electroshcok inhibited the GH response. Phentolamine's effect shows that the stress-induced GH release that follows electroconvulsive therapy is mediated by alpha-adrenergic neurons.     

Immunosuppressive treatment of a spontaneous inhibitor hemophilia A using cyclophosphamide, vincristine and prednisone following prior Factor VIII stimulation

A 59-year-old patient who presented with hematuria and recurrent soft tissue bleeding was found to have a factor VIII inhibitor level of 52 Bethesda units (BU)/ml and acquired hemophilia was diagnosed. After treatment with immunoglobulins (0.4 g IgG/kg per day for one week) the factor VIII inhibitor titer decreased to 12 BU/ml. Because of another episode of retroperitoneal hemorrhage, the patient was put on an immunosuppressive combination therapy which was first described by Lian et al. (1988). Our patient was infused with a factor VIII concentrate followed by cyclophosphamide, vincristine and prednisone. This regimen was repeated every 3-4 weeks. After 6 courses a further decline in the factor VIII inhibitor concentration, but no complete eradication of the autoantibody, was achieved. The factor VIII inhibitor level has remained at 2.5 BU/ml for more than 7 weeks without further bleeding episodes. The pathophysiology and treatment of acquired hemophilia are discussed.     

Complications of membrane-filtration plasma exchange

Guided by the Roy Adaptation Model of Nursing, the relationship of children's age, gender, exposure to past painful experiences, temperament, fears, and child-rearing practices to their pain responses to a venipuncture was examined. A sample of 94 children aged 8 to 12 years and their female caregivers were recruited from three outpatient clinics. During the venipuncture, children's behavioral and heart rate responses were monitored; immediately after, their subjective responses were recorded. Canonical correlation revealed two variates. In the first, age and threshold (temperamental dimension) correlated with pain quality, behavioral responses, and heart rate responses, explaining 12% of the variance. In the second, age, the temperamental dimensions of distractibility and threshold, and medical fears explained only 5.7% of the variance in pain quality and heart rate magnitude. Significant correlations between pain intensity, quality, behavioral responses, and heart rate responses support the multidimensionality of pain.     

Successful treatment with plasmapheresis, cyclophosphamide, and cyclosporin A in type B syndrome of insulin resistance. Case report

CASE HISTORY: A woman born in 1949 was diagnosed in 1990 with systemic lupus erythematosus. She was treated with prednisolone, and < 1 year later she presented with marked hyperglycemia. Large doses of insulin were given four times per day. Even though the patient was thin (BMI 17.4 kg/m2), very little improvement was seen. INVESTIGATIONS AND TREATMENT: Serum insulin levels were high, and a euglycemic clamp investigation confirmed severe insulin resistance. The patient's serum contained insulin receptor antibodies inhibiting insulin binding, and thus the patient had a type B syndrome of insulin resistance. After diet and exercise, glycemic control stabilized and insulin treatment was withdrawn. However, in late 1993 she was in a catabolic and hyperglycemic state even though prednisolone doses were increased and azathioprin was added. In early 1994 she was treated with plasmapheresis and cyclophosphamide i.v. Subsequently, cyclosporin A was started as a maintenance therapy in addition to azathioprin. There was a rapid and sustained clinical improvement. Since late 1994 and onward, there is no sign of diabetes or glucose intolerance and there are no demonstrable insulin receptor antibodies in the patient's serum. DISCUSSION: Severe type B insulin resistance may respond favorably to treatment with plasmapheresis and cyclophosphamide followed by cyclosporin A in combination with azathioprin.     

Long-term plasma exchange. Analysis of indications, outcome and side effects

In order to determine the characteristics and the course of diseases treated with long-term plasmapheresis (e.g., more than 25 plasma exchanges), we retrospectively studied 850 patients who underwent plasmapheresis. Long-term plasma exchange was prescribed to 38 patients who failed to respond to conventional therapy; cryoglobulinemias, peripheral neuropathies and monoclonal gammopathies were their most frequent underlying diseases. Improvement was noted in 65.8% cases. Only minor side effects were observed and the risk/benefit ratio for such therapy was excellent.