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Biomedical subjects

A D Askari

Publications and source records attributed to A D Askari.

At least 19 recordsLinked to original sources
PubMed1985

Small-cell lymphoma and Sjögren's syndrome. Lymphoplasmacytic subvariant of small-cell lymphoma with IgA/kappa immunoglobulin surface markers.

To our knowledge, the association of the plasmacytoid variety of small-cell lymphoma with primary Sjögren's syndrome has not been reported. We describe a patient with SS who developed plasmacytoid small-cell lymphoma. As opposed to the commonly detected IgM, the lymphocyte surface immunoglobulins contained monoclonal IgA/kappa light chains. In addition to the unique immunopathologic features, the impressive response to chemotherapy and the importance of surface immunoglobulin markers in the diagnosis of malignancy in Sjögren's syndrome are discussed.

Female↗
PubMed1984

Colchicine for treatment of relapsing polychondritis.

This report describes the effectiveness of oral colchicine, 0.6 mg twice daily, in abating three attacks of chondritis in two patients who fulfilled the diagnostic criteria for relapsing polychondritis (RP). Chondritis of the pinnae of the ears was utilized as a therapeutic guideline toward improvement. Collagen antibodies and urinary mucopolysaccharides were negative during flares and after improvement; antinuclear antibodies (ANA) and anti-deoxyribonucleic acid (DNA) were present in one patient. Both patients experienced a continued chondritis for at least 10 days before colchicine was started. Marked improvement was observed in 4 days, and complete resolution occurred after 7 days' treatment with colchicine. These observations indicate that the effectiveness of colchicine, as a relatively safer medicine, should be evaluated further in the treatment of RP.

Adult↗
PubMed1983

Pancreatitis with arthropathy and subcutaneous fat necrosis. Evidence for the pathogenicity of lipolytic enzymes.

The occurrence of peripheral fat necrosis in exceptional cases of pancreatic disease is not well understood. We report studies on such a patient with arthropathy and subcutaneous nodules. Examination of serial serum samples demonstrated striking elevations of the pancreatic enzymes phospholipase A, 3-3.4 units/ml (normal 0.17-0.41); lipase, 7-39 Sigma-Tietz units/ml (normal less than 1); immunoreactive trypsin, 912-3,207 ng/ml (normal 12-41). The distinguishing characteristic of the patient's synovial fluid was a marked elevation of hydrolized fatty acids (680 mg/dl versus 19 +/- 19 in control inflammatory joint fluids). Synovial fluid fatty acid distribution was identical to values for tissue fat. In contrast, serum fatty acid levels and distribution were normal. No associated proteinase inhibitor or significant immunologic abnormality was detected. Certain properties of adipose cells and lipolytic enzymes may help explain the characteristically selective necrosis of fat cells observed in this syndrome.

Fat Necrosis↗
PubMed1983

Arthritis of hemochromatosis. Clinical spectrum, relation to histocompatibility antigens, and effectiveness of early phlebotomy.

Five patients who presented with arthritis as the sole manifestation of hereditary hemochromatosis and 51 family members were studied. Studies included clinical evaluation for the presence of arthritis and hemochromatosis, roentgenography of hands, knees, and pelvis, serum iron and serum ferritin measurements, complete HLA typing for 50 of the A and B loci, and, when indicated, liver biopsy. Arthritis occurred in 45 percent of persons with hemochromatosis. Although typical involvement of second and third metacarpophalangeal joints was observed in all five patients and some family members, two with typical arthritis did not have characteristic radiographic changes, two had constitutional symptoms without arthropathy, and one had unilateral hand changes. A specific HLA haplotype (A2/B17 in Family 1 and A29/B15 in Family 2) correlated with hereditary hemochromatosis but not with the arthropathy. Phlebotomy alleviated the early constitutional symptoms but did not help advanced arthritis. Anti-inflammatory drugs, intraarticular injections of glucocorticoids, and resection osteotomies of metacarpal heads were other treatment modalities.

Anti-Inflammatory Agents↗
PubMed1981

Arthropathy, hypouricemia and normal serum iron studies in hereditary hemochromatosis.

A patient manifesting the arthropathy of hemochromatosis without abnormal serum iron studies is described. Hemochromatosis was confirmed by liver biopsy. This case serves to emphasize the diagnostic value of the characteristic arthropathy of hemochromatosis. Our observations in this patient support the hypothesis that the pathogenesis of hereditary hemochromatosis differs from that of acquired iron overload states. The concurrent presence of hypouricemia is explored in this patient and in 18 other patients with hereditary hemochromatosis. Men with hereditary hemochromatosis were found to have lower serum uric acid levels than expected. In our patient, a renal defect in tubular reabsorption of uric acid appears responsible for hypouricemia. The apparent association of hemochromatosis and hypouricemia deserves further investigation.

Arthritis↗
PubMed1981

Rheumatoid nodulosis. A relatively benign rheumatoid variant.

Subcutaneous nodules and rheumatoid factor (RF) are criteria used to diagnose rheumatoid disease. Their presence correlates with disease severity and poorer prognosis. They have been reported, however, in patients with little arthritis and no systemic disease. We studied four such patients, in whom (1) RF was present in high titer; (2) nodules were often extensive (nodulosis) and involved elbows, hands, and feet, with a predilection for tendons; and (3) roentgenograms showed large, subchondral bone cysts without cortical erosion of correlation with nodule location. The conditions of three of these patients had been previously misdiagnosed as gout or xanthoma. Our findings were similar to those in seven other patients described in earlier reports. We suggest that nodulosis, bone cysts, and elevated RF with little active arthritis constitute a relatively benign variant of rheumatoid disease.

Diagnosis, Differential↗
PubMed1981

Colchicine in the treatment of Paget disease of bone: a new therapeutic approach.

Paget disease of the bone (PDB) has been treated effectively with various agents including calcitonins, diphosphonates, and mithramycin. Each agent has relatively serious toxic side effects or practical inconveniences associated with its use. An effective agent with fewer adverse reactions and a more convenient route of administration would be preferable. The purpose of this study was to evaluate the effect of colchicine for the treatment of PDB in five patients. All patients were symptomatic and had typical changes on roentgenograms and bone scans consistent with the disease. Serum alkaline phosphatase ranged from 408 to 1,311 mU/ml (normal, 30 to 115 mU/ml), and urinary excretion of total hydroxyproline ranged from 68 to 205 mg/24 hr (normal, 30 to 65 mg/24 hr). Colchicine, 0.6 mg, was given orally three times a day to each patient, who had subsequent follow-up with clinical and laboratory determinations evaluated at each visit. The duration of follow-up was eight to 28 weeks, with a mean of 20 weeks. Pain was relieved in all patients, and two became asymptomatic. Serum alkaline phosphatase decreased 18% to 38%, and urinary hydroxyproline decreased 26% to 53% from the pretreatment values. The biochemical values and and clinical symptoms changed markedly in two patients, correlating with withdrawal and reinstitution of colchicine. These results indicate that colchicine may be effective in the treatment of PDB. Although the mode of action and long-term efficacy of colchicine in this disorder remains to be evaluated, the antimitotic effect on osteoprogenitor cells, the adherence of colchicine to the microtubular structures in preexisting osteoblasts, and the nonspecific anti-inflammatory effect of this agent may explain the therapeutic response noted in this study.

Aged↗
PubMed1980

Granulocytopenia with marked lymphocytosis manifesting Sjogren syndrome.

Sjogren syndrome is a multi-system disease leading to diverse organ involvement during its course [1, 2]. Hematologic abnormalities described in Sjogren syndrome include anemia, mild leukopenia [3, 4], eosinophilia, elevated erythrocyte sedimentation rate, hypergammaglobulinemia, mixed cryoglobulinemia, and a variety of autoantibodies [5]. Marked lymphocytosis with granulocytopenia is distinctly unusual and has not been previously reported. We report a case of Sjogren syndrome who presented with constellation of the latter problems without prominent sicca manifestations.

Agranulocytosis↗
PubMed1980

Behçet's disease and treatment with colchicine.

A case of Behçet's disease characterized by anterior uveitis, arthritis, oral, genital, and cutaneous lesions, as well as gastrointestinal involvement, all documented over a 10-month period, is presented. Less commonly appreciated complications, such as pyoderma gangrenosum, hidradenitis suppurativa, perianal fistula, and persisting leukocytosis, were among striking clinical features of the patient's history. Remission of cutaneous lesions and no recurrence of ocular or gastrointestinal manifestations occurred with 0.6 mg oral colchicine twice daily in a period of 5 weeks.

Adult↗
PubMed1980

Pulmonary hypertension and systemic lupus erythematosus.

A combination of systemic lupus erythematosus (SLE) and fatal pulmonary hypertension occurred in a patient who, to our knowledge, had the highest pulmonary artery pressure (120/65 mm Hg) reported without any clinical or autopsy findings of pulmonary interstitial disease or vasculitis. The gradual development of pulmonary hypertension over years is a rare complication in patients with SLE.

Adult↗
PubMedDate not supplied

Wegener granulomatosis simulating bacterial endocarditis.

Cardiac involvement in Wegener granulomatosis is uncommon. We report a case of Wegener granulomatosis that presented as culture-negative endocarditis with aortic valvular vegetation. The clinical manifestations included gingival hyperplasia, gangrenous digital infarcts, mononeuritis multiplex, high fever, inflammatory arthritis, pansinusitis, splenic infarct, and aortic valvular vegetation, which underscore the difficulty of distinguishing systemic vasculitis from bacterial endocarditis. Contrary to the common notion that valvular vegetation is invariably associated with bacterial endocarditis, this case proves that such findings can occur in Wegener granulomatosis as well. Clinicians are guided toward early treatment with corticosteroids and cyclophosphamide to prevent fatal complications.

Aortic Valve↗