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Biomedical subjects

M H Ornstein

Publications and source records attributed to M H Ornstein.

At least 19 recordsLinked to original sources

Dramatic improvement of left ventricular function after cytotoxic therapy in lupus patients with acute cardiomyopathy: report of 6 cases.

Although lupus cardiomyopathy is thought to be clinically uncommon, we encountered 6 patients with systemic lupus erythematosus (SLE) over a 10 year period who had severe left ventricular dysfunction and showed remarkable improvement in their cardiac function after cytotoxic therapy. All patients met the American College of Rheumatology criteria for classification of SLE and presented with signs of severe biventricular failure relatively early in their disease. Concurrent manifestations of SLE at the time of cardiomyopathy included rash, arthritis, myalgias, pleuritis, pericarditis, and nephritis. Four of the 6 patients were taking prednisone 20 mg/day at the time heart failure developed. In all cases the CPK were normal. Evaluation of cardiac function by echocardiogram and/or radionuclide gated blood pool scintigraphy revealed a severe depression of ventricular function with initial left ventricular ejection fraction (LVEF) ranging from 11 to 34% (mean 19%). Within 6 months of initiation of cytotoxic treatment all patients showed a dramatic response: the post-treatment LVEF ranged from 25 to 55%. This series of patients suggests that cardiomyopathy may be a more common complication of SLE than previously reported. Cardiomyopathy occurs relatively early in the course of SLE, may lead to severe cardiac dysfunction despite corticosteroid therapy, and appears to be responsive to cytotoxic therapy.

Acute Disease↗

The antiinflammatory and antiviral effects of hydroxychloroquine in two patients with acquired immunodeficiency syndrome and active inflammatory arthritis.

OBJECTIVE: To report the antiinflammatory and antiviral effects of hydroxychloroquine (HCQ) treatment in 2 patients with AIDS and inflammatory arthritis. METHODS: Two patients with AIDS and inflammatory arthritis were treated with HCQ, which was given in a loading dose of 600 mg/day. The maintenance dosage was calculated to remain below 6.5 mg/kg/day. Both patients had initial T cell subset studies; 1 patient, had serum and plasma collected before and after 1 year of HCQ treatment. Assays were performed for T cell subsets, recoverable human immunodeficiency virus type 1 (HIV-1) RNA, mitogen- and antigen-specific proliferation, and interleukin-6 (IL-6) levels. New studies on the use of HCQ as an anti-HIV-1 agent are reviewed. RESULTS: Both patients had a dramatic decrease in their arthritis activity. Neither patient required immunosuppressive therapy or developed an opportunistic infection. In the patient who was studied after 1 year of therapy, there was a 1-log decrease in recoverable HIV-1 RNA, improved mitogen- and antigen-specific immune responses, and a large decrease in the IL-6 level while taking HCQ. Recent in vitro and in vivo assays in patients with HIV infection have shown similar antiviral and antiinflammatory effects from HCQ. CONCLUSION: HCQ may exert simultaneous anti-inflammatory and antiviral effects in patients with HIV infection and inflammatory arthritis. If larger studies confirm this observation, it may be the drug of choice in this population of patients.

Acquired Immunodeficiency Syndrome↗

A reexamination of the relationship between active rheumatoid arthritis and the acquired immunodeficiency syndrome.

Three patients with rheumatoid arthritis (RA) that remitted with the development of the human immunodeficiency virus (HIV) infection have been described in the literature, and this has contributed to the belief that RA and HIV infection or the acquired immunodeficiency syndrome (AIDS) cannot coexist. However, a computerized MEDLINE literature search revealed reports of 4 patients who did have active RA and AIDS or HIV infection, as well as other case reports of symmetric polyarthritis compatible with RA in patients with HIV infection. Each of the patients whose RA remitted had received standard disease-modifying antirheumatic drug therapy, and 1 of the 3 had a normal T helper:T suppressor ratio at the time of remission. Of the 4 previously reported patients with active RA and AIDS or HIV infection, all had decreased numbers of T helper lymphocytes. The present report describes a fifth patient with both RA and AIDS and reviews the data concerning the coexistence of these 2 diseases. It appears that active RA may indeed coexist with AIDS. It remains to be seen under what settings HIV may have a disease-modifying effect in RA. These issues have important implications regarding the pathogenesis and therapy of RA, especially in terms of the role of CD4+ lymphocytes and anti-CD4 monoclonal antibody therapy.

Acquired Immunodeficiency Syndrome↗

A patient with antineutrophil cytoplasmic antibody-negative pulmonary capillaritis and circulating primed neutrophils.

We describe an antineutrophil cytoplasmic antibody-negative patient with capillaritis, acute respiratory failure, and primed neutrophils circulating systemically. Thoracoscopic lung biopsy showed capillary destruction, with neutrophils infiltrating the lung interstitium and air spaces but no alveolar damage or hemorrhage. We suggest that this atypical, limited form of pulmonary injury may occur when circulating neutrophils are primed, but, in the absence of an activating factor, not fully activated.

Antibodies, Antineutrophil Cytoplasmic↗

Correlation of liver and skin histopathology with serology in a patient with cutaneous vasculitis and hepatitis C infection.

A strong body of evidence supports a viral etiology for many forms of vasculitis. Recent literature supports the association of the hepatitis C virus (HCV) with essential mixed cryoglobulinemia. We describe a case of cutaneous vasculitis associated with simultaneous active hepatic inflammation demonstrated by skin and liver biopsies. Immunofluorescence, hepatitis C serology, and study of the cryoprecipitate all confirmed HCV infection and deposition of immune complexes. Treatment with interferon alpha-2b was extremely effective as compared with prednisone and colchicine. This case provides further evidence that active hepatic infection with HCV may be a cause of cryoglobulinemia and cutaneous vasculitis. It also suggests that further studies of antiviral therapy for vasculitis should be explored.

Female↗

An association between refractory HELLP syndrome and antiphospholipid antibodies during pregnancy; a report of 2 cases.

The HELLP syndrome (hemolysis, elevated liver enzymes and low platelets) is a thrombotic microangiopathic vasculopathy that presents in pregnancy. HELLP syndrome usually resolves with delivery of the fetus without sequelae and is often managed successfully with conservative care. To our knowledge, there has been no prior report of an association between HELLP syndrome and elevated titers of antiphospholipid antibodies (aPL). We describe 2 patients who each presented with HELLP syndrome that was refractory despite delivery of the fetus, corticosteroids, and anticoagulation. Both patients had elevated levels of anticardiolipin antibodies. Examination of skin and placental pathology revealed diffuse deposition of fibrin with small vessel thrombi, without evidence of vasculitis. Coincident with plasmapheresis, HELLP resolved in both patients. We conclude that there may be an association between the presence of aPL and a protracted HELLP syndrome in pregnancy; this may be a new manifestation of the antiphospholipid syndrome. Plasmapheresis appears to benefit these patients. Further investigation into this association is warranted.

Adrenal Cortex Hormones↗

Are fibre supplements really necessary in diverticular disease of the colon? A controlled clinical trial.

Fifty-eight patients with uncomplicated diverticular disease of the colon took bran crispbread, ispaghula drink, and placebo for four months each in a randomised, cross-over, double-blind controlled trial. Assessments were made subjectively, using a monthly self-administered questionnaire, and objectively, by examining a seven-day stool collection at the end of each treatment period. In terms of a pain score, lower bowel symptom score (the pain score and sensation of incomplete emptying, straining, stool consistency, flatus, and aperients taken), and total symptom score (belching, nausea, vomiting, dyspepsia, and abdominal distension) fibre supplementation conferred no benefit. Symptoms of constipation, however, when assessed alone, were significantly relieved. Both fibre regimens produced the expected changes in stool weight, consistency, and frequency. It is concluded that dietary fibre supplements in the commonly used doses do no more than relieve constipation. Perhaps the impression that fibre helps diverticular disease is simply a manifestation of Western civilisation's obsession with the need for regular frequent defecation.

Adult↗

High fibre diet.

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Cellulose↗