bluuebunny/crossref_metadata_2025_split huggingface dataset:
The arthropathy of fabry disease
Authors: Sheth Kumudchandra J. , Bernhard Gerson C. 
Published: February 2007

AbstractFabry disease, an X‐linked recessively inherited glycosphingolipidosis, results from defective activity of the lysosomal enzyme ceramide trihexosidase termed α‐galactosidase A (1). The cumulative deposition of the substrate ceramide trihexose (CTH) in various tissues accounts for the clinical manifestations of the disease (2). Although excruciating pains in the extremities are frequently present, pains localized in joints are uncommon. There is a characteristic arthropathy of the distal interphalangeal joints of the fingers with limitation of extension and degenerative changes which are evident on radiologic examination (3). Because patients with Fabry disease have attacks of fever, pains in extremities, and elevated ESR, rheumatic fever or rheumatoid arthritis is frequently the initial diagnosis (3). We wish to report synovial and joint capsule biopsies from a male who was initially diagnosed as having rheumatoid arthritis and was later confirmed to have Fabry disease by low plasma α‐galactosidase level.
Elastofibroma: A rare cause of periscapsular pain
Authors: Prete Pamela , Thorne Roger P. 
Published: February 2007

AbstractIn 1961 Järvi and Säxen reported 4 cases of a previously undescribed connective tissue tumor containing elastinophilic fibers which they termed elastofibroma dorsi (1). Their patients were elderly, and the tumors were all located in the subscapular space adjacent to the apex of the scapula and beneath the rhomboid and latissimus dorsi muscles. One of the four had bilateral tumors.Since the original description, over 60 cases have been reported (1–30). The true incidence of this unique soft tissue tumor is probably underestimated because of its obscure location and benign and usually silent course. Elastofibroma has not been recognized as a cause of periscapular complaints despite its characteristic scapular location.The purpose of this report is to present a case of bilaterally occurring elastofibromas that caused periscapular pain in a 61‐year‐old man and review the salient features of this rare lesion.
Resolution of the identity of certain antigen‐antibody systems in systemic lupus erythematosus and sjögren's syndrome: An interlaboratory collaboration
Authors: Alspaugh Margaret , Maddison Peter 
Published: February 2007

AbstractThe Ouchterlony technique of double immunodiffusion, combined with some physicochemical characterization of the antigens involved, has been used to characterize several antigen‐antibody systems of importance in certain connective tissue diseases, notably systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD), Sjögren's syndrome, rheumatoid arthritis, scleroderma, and poly‐ and dermatomyositis (1–12). These systems appear to have not only considerable diagnostic significance but also importance in determining prognosis and disease course.
Ophthalmologic safety of long‐term hydroxychloroquine treatment
Authors: Rynes Richard I. , Krohel Gregory , Falbo Anthony , Reinecke Robert D. , Wolfe Bruce , Bartholomew Lee E. 
Published: February 2007

AbstractOcular toxicity of long‐term hydroxychloroquine treatment was assessed by regular ophthalmologic examinations in 99 patients. No patient developed significant loss of vision or visual field constriction to a white test object. Three patients had evidence of toxicity, but the medication had to be permanently discontinued in only one who subsequently had regression of all abnormalities except visual field constriction to a red test object. Neither duration of treatment nor the diagnosis of systemic lupus erythematosus predisposed patients to toxicity.
Simultaneous immunologic studies of skin and kidney in systemic lupus erythematosus
Authors: Morris Richard J. , Guggenheim Stephen J. , Mcintosh Rawle M. , Rubin Robert L. , Kohler Peter F. 
Published: February 2007

AbstractThirty‐two biopsies of kidneys and of normal skin were performed simultaneously on patients with systemic lupus erythematosus (SLE) to determine whether the deposition of immune reactants in skin was correlated with the severity of renal injury or with several serologic measures of systemic disease activity. Immunofluorescent deposits at the dermal‐epidermal junction (lupus band test) did not correlate with any clinical or histologic measures of glomerulonephritis or with serologic abnormalities. Immune deposits in dermal venules were found in 56% of the skin biopsies and were correlated with hypocomplementemia and higher levels of immune complexes in serum, and possibly with glomerular subendothelial electron dense deposits. Azathioprine therapy was correlated with absence of both subepidermal and vascular immune deposits. Immunofluorescent findings on serial skin biopsies on 13 patients were highly inconsistent. It is concluded that the lupus band test is clinically useful only as a diagnostic aid but not helpful in assessing renal or serologic activity of lupus, and that dermal vascular deposits of immunoglobulin or complement are more frequent than previously recognized and correlate with measures of circulating immune complexes.
A tissue culture model of cartilage breakdown in rheumatoid arthritis
Authors: Steinberg James , Tsukamoto Soichiro , Sledge Clement B. 
Published: February 2007

AbstractThe effects of hydrocortisone, indomethacin, and gold thiomalate on proteoglycan release were assessed in bovine nasal cartilage‐rheumatoid synovium cocultures. Of the three agents, only hydrocortisone consistently inhibited both basal and synovium‐stimulated cartilage breakdown. Hydrocortisone responsivity was a direct function of the degradative capacity of synovial specimens, and this was equally well demonstrated both in patients receiving long‐term therapy and those given perioperative glucocorticoid therapy. The data are consistent with significant hydrocortisone inhibition of lysosomal enzyme‐mediated degradation of cartilage.
Quantification of human plasma inorganic pyrophosphate
Authors: Ryan Lawrence M. , Kozin Franklin , Mccarty Daniel J. 
Published: February 2007

AbstractPlasma inorganic pyrophosphate (PPi) levels rose predictably (28%) after vigorous systemic exercise and returned to baseline values after 30 minutes of rest. Plasma PPi levels were greater (36%) in femoral venous blood than in femoral arterial blood. Forearm muscular exercise, however, did not result in a detectable rise in plasma PPi levels in antecubital veins. The physiologic reasons for both the systemic exercise effect and the arteriovenous differences remain unknown. Measurement of plasma PPi for 10 consecutive days in a single normal subject under basal conditions, where these and other previously identified biologic variables (fasting and diurnal variation) were controlled, showed a coefficient of variation of 12.7%. Thus biologic variability contributed less to observed variation in plasma PPi than did methodologic errors (coefficient of variation of method = 8%). The striking reproducibility of plasma PPi in this subject suggests that this important metabolite is under rather tight homeostatic control.
A review of the use, evaluations, and criticisms of the preliminary criteria for the classification of systemic lupus erythematosus
Authors: Canoso Juan J. , Cohen Alan S. 
Published: February 2007

AbstractThe use of the Preliminary Criteria for the Classification of Systemic Lupus Erythematosus (SLE) has been reviewed. Most authors describing 10 or more patients have used the criteria. Although originally designed for classification of patients, the criteria have often been used for the diagnosis of SLE. The sensitivity of the criteria was evaluated and found to be similar to the 90% reported in the original ARA trial. Studies on specificity yielded conflicting results. Various suggestions to improve the criteria have been made, including a more detailed description of some of the organ involvements and the adoption of laboratory tests that have been improved since or were unavailable at the time of original publication of the criteria.
Serum complement abnormalities in the antinuclear antibody‐positive relatives of children with systemic lupus erythematosus
Authors: Lehman Thomas J. A. , Hanson Virgil , Singsen Bernhard H. , Kornreich Helen K. , Bernstein Bram , King Karen K. 
Published: February 2007

AbstractSerum C3, C4, and total hemolytic complement (CH50) levels were measured for 21 children with systemic lupus erythematosus (SLE) and 81 first degree relatives. The mean serum C4 and CH50 levels of the 12 relatives with antinuclear antibodies (ANA) were depressed to levels equal to those of the index cases. A similar depression was not found for C3, nor was there a depression of C3, C4, or CH50 in the relatives without ANA. If preexistent depression of C4 levels can be documented in the ANA‐positive relatives of index cases, it may provide an explanation for the inherited predisposition to SLE in some families.
Cardiac involvement in polymyositis
Authors: Denbow Charles E. , Lie J. T. , Tancredi Robert G. , Bunch Thomas W. 
Published: February 2007

AbstractCardiac involvement in polymyositis was investigated in 20 autopsied cases. Clinically, 13 of 18 patients had abnormal electrocardiograms, and 9 of the 20 patients had previous evidence of congestive heart failure. Histologically documented myocarditis was detected in 6 patients (4 with congestive heart failure and 2 without), 4 of whom also had small vessel disease of the myocardium. Patients with polymyositis may have a cardiopathy in the absence of overt myocardial inflammatory disease.
Collagenase production by synovial fibroblasts treated with phorbol myristate acetate
Authors: Brinckerhoff Constance E. , Mcmillan Rodger M. , Fahey John V. , Harris Edward D. 
Published: February 2007

AbstractA series of intracellular events occurring after treatment of rabbit synovial fibroblasts with 0.01 μg/ml phorbol myristate acetate (PMA) were measured. Ten minutes after addition of PMA, there was a temporary increase in intracellular cyclic AMP levels, followed by a transient decrease in incorporation of 3H‐thymidine into DNA. Approximately 500 ng/mg cell protein of PGE2 were found in culture medium from the 12‐ to 24‐ hour incubation period, but significant collagenase was not detectable until 24 to 36 hours. Treatment with aspirin or indomethacin abolished PGE2 production but did not affect collagenase levels. Production of enzyme was associated with a cessation of cell proliferation, measured by protein content/culture and cell number. No enzyme was detectable in untreated cultures. Synovial fibroblasts treated with phorbol myristate acetate may provide a good model for studies on the mechanism of induction of collagenase production.
Sex hormone modulation of autoimmunity in NZB/NZW mice
Authors: Roubinian Jirayr , Talal Norman , Siiteri Pentti K. , Sadakian Jacqueline A. 
Published: February 2007

AbstractPrepubertal castration of female NZB/NZW (B/W) hybrid mice did not influence mortality, whereas prepubertal castration of male B/W mice caused premature death and enhanced autoantibody formation. Prepubertal castration combined with the administration of sustained estradiol‐17‐β (E‐2) enhanced mortality and autoantibody development and promoted immune complex nephritis in both sexes. The enhanced mortality observed in castrated males was significantly reduced if they were treated with sustained progesterone (P). Mice of both sexes receiving P had the highest levels of autoantibodies. By contrast, sustained 5‐α‐dihydrotestosterone (DHT) and testosterone (T) suppressed these autoimmune parameters. The suppressive effect of androgens was not a nonspecific anabolic property, since danazol (a compound with attenuated masculinizing but intact anabolic properties) was ineffective in suppressing disease. AntiDNA antibody formation developed prematurely in males given cyproterone acetate, an antiandrogen. However, this metabolic blocker did not influence mortality or proteinuria. Unexpected early enhancement of mortality was observed in female and male mice castrated and given both E‐2 and DHT (when compared to sham‐operated controls or mice given E‐2 alone). When castration was combined with the administration of both E‐2 and P, mortality was enhanced in males but was similar to E‐2 alone in females. Androgen therapy was also suppressive when it was delayed and given to female mice with more advanced disease. DHT given to intact 3‐month‐old or castrated 6‐month‐old B/W females significantly improved survival without affecting the serum levels of anti‐DNA antibodies. These observations demonstrate that sex hormones significantly modify disease in B/W mice and may have therapeutic potential.
Approach to the study of the role of sex hormones in autoimmunity
Authors: Steinberg Alfred D. , Melez Kathleen A. , Raveche Elizabeth S. , Reeves J. Patton , Boegel William A. , Smathers Patricia A. , Taurog Joel D. , Weinlein Lisa , Duvic Madeleine 
Published: February 2007

AbstractInvestigators from this laboratory have been studying sex hormones in normal and autoimmune mice for the past 10 years. We have found that immune responses to DNA are influenced by sex hormones. Androgens reduce and estrogens increase both spontaneous and immunization‐induced antibodies to single‐stranded DNA in NZB x NZW, NZB x C3H, NZB x CBA, NZB x DBA mice. Treatment of female NZB/W mice with testosterone or 5 α dihydrotestosterone retards the progress of autoimmunity. Castration is not necessary for this effect. In contrast, danazol has no favorable effect on the disease process. Estrogens cause a marked acceleration of autoimmunity and a reduction in thymus weight. During the course of these studies, we found that a number of problems or variables arise in studying sex hormone effects, including: 1) X‐linked genes, 2) metabolism of testosterone to estrogens, 3) dose of hormone, 4) age at which administration is initiated, 5) differential effects of sex hormones on different autoantibodies and various immune responses.
Alterations of estrogen metabolism in systemic lupus erythematosus
Authors: Lahita Robert G. , Leon Bradlow H. , Kunkel Henry G. , Fishman Jack 
Published: February 2007

AbstractEstradiol metabolism in 10 patients with systemic lupus erythematosus (SLE) and 29 normal controls was studied by measurement of urinary metabolites after injection of labeled 3H‐estradiol. Patients with SLE manifested increased 16‐hydroxylation of estrone. Diseased men differed from diseased women to the extent that only 16α‐hydroxyestrone was elevated in men, whereas women had elevations of both 16α‐hydroxyestrone and estriol. These data suggest that patients with SLE have abnormal patterns of estradiol metabolism, leading to increased estrogenic activity.
Glucocorticoids and immune responses
Authors: Crabtree Gerald R. , Gillis Steven , Smith Kendall A. , Munck Allan 
Published: February 2007

AbstractWe describe a series of investigations on mechanisms of antiinflammatory and immunosuppressive actions of glucocorticoids. Glucocorticoid receptors and primary mechanisms of action have been studied with isolated rat thymus cells. When added to these cells glucocorticoids immediately form cytoplasmic hormone‐receptor complexes which after activation bind to the nuclei where they apparently induce mRNA for specific proteins that rapidly inhibit glucose transport and acetate incorporation into lipids. Protein and RNA metabolism are inhibited more slowly and eventually the cells die. With normal peripheral human lymphocytes, similar but slower effects are produced. A dramatic increase in receptor sites per cell is seen in lymphocytes stimulated with concanavalin A or antigen. This increase is probably associated with preparation for mitosis. We do not find, contrary to widespread belief, that these stimulated cells are insensitive to glucocorticoids. Such insensitivity has been invoked to explain the insensitivity to glucocorticoid suppression of secondary compared to primary immune responses. An alternative explanation emerges from our experiments with T‐cell growth factor. T‐cell growth factor, produced by mitogen or antigen stimulated spleen cells, is necessary for the proliferation of T‐lymphocytes in culture and may be responsible for clonal expansion of antigen‐responsive T‐cells. Treatment of Con A‐stimulated rat spleen cells or human peripheral mononuclear cells with 100 nMdexamethasone inhibits the production of the growth factor by 95%. This effect is specific for glucocorticoids. Addition of T‐cell growth factor completely reverses the inhibition by glucocorticoid of mitogen‐induced blastogenesis. We propose that the inhibitory actions of glucocorticoids on antigen‐ or mitogen‐induced T‐cell blastogenesis are related to inhibition of production of T‐cell growth factor and that the reason the primary immune response is more sensitive to glucocorticoids is that by inhibiting production of T‐cell growth factor, glucocorticoids block the clonal expansion necessary to amplify the primary response. We have also studied effects of glucocorticoids on Fc receptors, which play important roles in phagocytosis and other aspects of immune responses. Treatment of the human progranulocytic cell line HL‐60 with dexamethasone for 72 hours reduces by 35‐50% the Fc receptors per cell with no effect on cell viability or proliferation and slight increase in leucine incorporation. The effect is specific for glucocorticoids. These findings indicate that an important component in glucocorticoid‐induced immunosuppression may be a reduction in Fc receptors, and with the results on T‐cell growth factor, suggest that glucocorticoids regulate immune processes via effects on lymphokines and other immunologically important proteins.
Sex differences in response to hepatitis b virus
Authors: Blumberg Baruch S. 
Published: February 2007

AbstractSex differences related to responses to hepatitis B infection are reviewed. In most human populations there is a higher prevalence of chronic carriers of hepatitis B virus (persistently HBsAg+) among males than females. Females are more likely than males to produce anti‐HBs in response to infection. Diseases associated with increased frequencies of carriers are more prevalent among males. The response of parents to hepatitis B virus (HBV) infection appears to affect the sex ratio at birth of their offspring. Couples in which either parent is a carrier have higher sex ratios (higher proportion of males) compared with couples in which neither parent is HBsAg+. Couples in which the mother is anti‐HBs+ have children with lower sex ratios than either carriers or uninfected couples.
Infection in patients with systemic lupus erythematosus
Authors: Perez H Daniel , Andron Richard I , Goldstein Ira M 
Published: February 2007

AbstractWe have found subnormal amounts of chemotactic activity in zymosan‐treated sera from 13 of 29 patients with systemic lupus erythematosus (SLE). As an explanation for this abnormality, the presence of a uniquely specific, heat‐stable inhibitor of complement (C5)‐derived chemotactic activity has been documented in sera from 11 of these patients. Sera from 2 other patients contained elevated levels of nonspecific, heat‐labile chemotactic factor inactivator (CFI) activity. The serum from 1 patient contained the heat‐stable inhibitor as well as elevated levels of CFI. Patients with SLE whose sera contained the heat‐stable inhibitor had more active disease clinically, but otherwise they were indistinguishable from patients without the inhibitor. When patients with the heat‐stable inhibitor improved clinically, this usually was accompanied by a decrease in serum inhibitory activity. Only one episode of bacterial infection was observed among 16 patients with SLE whose sera yielded normal amounts of chemotactic activity after treatment with zymosan. In contrast, 7 of 11 patients with SLE whose sera contained the heat‐stable inhibitor suffered serious bacterial infections. The presence of this heat‐stable inhibitor in sera from some patients with SLE may contribute, in part, to their increased susceptibility to infection.
Collagen‐induced arthritis in rats
Authors: Stuart John M , Cremer Michael A , Kang Andrew H , Townes Alexander S 
Published: February 2007

AbstractWhen rats were injected intradermally with an oil emulsion of native type II collagen, they developed an inflammatory polyarthritis. The incidence and severity of arthritis increased as the amount of collagen injected was increased. Rats 4 1/2 weeks old were the most susceptible to the development of arthritis, whereas weanling and older animals were relatively resistant. There was no difference in incidence between males and females. Mononuclear cells from peripheral blood, lymph nodes, and spleen were cultured with type II collagen and responded maximally to a collagen concentration of 25 μg/ml. The earliest detectable response was in peripheral blood mononuclear cell cultures obtained 6 to 8 days after immunization. The response of lymph node and spleen cells tended to lag behind that of peripheral blood cells at the earlier time intervals. Antibodies were detected in sera by hemagglutination at 8 days postimmunization. Quantitation of IgM and IgG antibodies by radioimmunoassay showed good correlation with hemagglutination titers and increased binding of collagen by both classes of antibody in arthritic as compared to nonarthritic animals. It is clear that the development of both humoral and cellular immunity to type II collagen is associated with the development of arthritis and may be important in the pathogenesis of this disease.
Reaction of superficial bursae in response to specific disease stimuli
Authors: Canoso Juan J , Yood Robert A 
Published: February 2007

AbstractAlthough microscopic studies have shown similarities between bursal and joint membranes, little is known about bursae and their response to disease states. Eighty‐six cases of superficial bursitis due to trauma, bacterial infection, or gout were reviewed and compared with cases of joint inflammation due to the same etiologies. In traumatic bursitis the bursal fluid mucin test was more abnormal and the viscosity lower than that of joint fluid in traumatic arthritis. The bursal fluid total leukocyte count of septic bursitis was less than 20,000/mm3 in 8 of 13 cases but in only 1 of 21 synovial fluids from cases of septic arthritis (P = 0.005). In gouty bursitis the mean total leukocyte count of bursal fluid was 2800/mm3, compared with a mean synovial fluid total leukocyte count of 28,700 in gouty arthritis (P < 0.02). These findings indicate that superficial bursae react less intensely than diarthrodial joints to specific disease stimuli and that a relatively low bursal fluid leukocyte count is often present in cases of septic and gouty bursitis.