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Biomedical subjects

A Gabrielli

Publications and source records attributed to A Gabrielli.

At least 91 records · Page 5Linked to original sources

[Correlation between the radiologic study of deglutition and the clinical picture in systemic sclerosis (scleroderma)].

Fifty-one patients with systemic sclerosis (scleroderma) were studied by means of videofluoroscopy in order to evaluate the abnormalities in the oropharyngeal and esophageal phases of deglutition and to correlate the radiological patterns with the clinical features of the disease. Thirteen patients (25.5%) exhibited swallowing disorders such as oral leakage, retention, penetration, mild or moderate aspiration and abnormal upper esophageal sphincter behavior. These dysfunctions were more evident in patients with esophageal motility abnormalities. A normal radiological pattern in the esophagus was not associated with swallowing alterations. Remarkably, patients with oral-pharyngeal disorders had a higher incidence of lung diseases. Forty-five patients (88%) exhibited disorders of the esophageal phase of deglutition, such as mild or severe motility abnormalities or hiatal hernia, gastro-esophageal reflux, reflux esophagitis, and stricture. Radiological findings in the esophagus can be abnormal in the early stages of the disease. On the other hand, the radiological pattern of esophageal motility can be occasionally negative in advanced or extensive disease. This indicates a discrepancy between clinical symptoms and radiological picture of the esophagus. The radiological examination of the oral-pharyngeal and esophageal phases of deglutition is important in patients with scleroderma in order to evaluate visceral involvement, motility disorders, and risk of aspiration. Such radiological information can be useful in preventing esophagitis and pulmonary complications.

Adult↗

A retrospective study of antibodies against basement membrane antigens (type IV collagen and laminin) in patients with primary and secondary Raynaud's phenomenon.

Antibodies against type IV collagen were detected in the sera of 21% of patients with primary Raynaud's phenomenon (PRP) and 68% of patients with systemic sclerosis. Sera from 25% of the PRP patients and 48% of systemic sclerosis patients displayed antibodies to laminin. Eighty percent of the patients with PRP and antibodies to basement membrane antigens developed additional serologic or clinical manifestations, whereas only 16% of patients with PRP and no anti-basement membrane antibodies developed such manifestations.

Adolescent↗

Assessment of disease activity in essential cryoglobulinemia by serum levels of a basement membrane antigen, laminin.

When compared with 40 normal controls, 46 patients with essential cryoglobulinemia had higher serum concentrations of a basement membrane antigen, laminin fragment P1 (LP1). Serum LP1 values were more pronounced in patients with visceral involvement, compared with those with skin vasculitis only. Asymptomatic patients or those with arthralgia usually had normal values. Increased disease activity was associated with a significant increase in LP1 (P less than 0.01). For evaluation of disease activity, determination of serum LP1 was a more sensitive measure than any complement factor assay.

Adolescent↗

Depression by Fc gamma receptor ligands of SRBC-induced IgM-PFC generation in human blood mononuclear cell cultures.

A tissue-culture system to stimulate human peripheral blood mononuclear cells (PBMC) has been employed in which IgM plaque-forming cell (IgM PFC) generation in response to sheep erythrocytes (SRBC) is dependent on macrophages and T suppressor and helper lymphocytes. In this system PBMC from normal subjects give IgM PFC responses ranging from 26 to 938 PFC/culture. Heat-aggregated human IgG or immune complexes present for the duration of culture induce a significant depression of PFC. Unaggregated IgG has no effect on the response or only a moderate stimulatory effect at the highest dose. The results of these experiments are compatible with previous results in a murine system, which indicated that Fc gamma receptor-positive (Fc gamma R+) cells in the suppressor subset are the target for aggregated IgG and induce depression of the PFC response. A similar mechanism may be operating in the human system described here, although the target cell has not been identified. These results may reflect a mechanism of immunomodulation dependent on interaction of Fc receptor (FcR) ligands with FcR, which may play a role in the pathogenesis of immune complex disorders.

Antibody-Producing Cells↗

Mononuclear cells from rheumatoid arthritis patients exhibit a depressed IgM response to sheep erythrocyte stimulation in vitro.

Human peripheral blood mononuclear cells when cultured for the short term (11 days) produce an optimal IgM plaque-forming cell (PFC) response to sheep erythrocytes, which is dependent on macrophages and T suppressor and helper lymphocytes. With this system we have investigated the PFC response of individuals with rheumatoid arthritis and compared this to the response of normal sex- and age-matched controls and of patients with osteoarthritis. The response of rheumatoid arthritis patients was significantly lower than that of subjects in the other two categories.

Animals↗

Levels of type IV collagen and laminin fragments in serum from patients with progressive systemic sclerosis.

Sera from patients with progressive systemic sclerosis (PSS) were studied using immuno-assays for laminin P1 fragment and the carboxyterminal NC1 domain from type IV collagen. Compared to healthy controls, patients with PSS showed elevated serum levels of both fragments derived from basement membrane proteins. However, there was no difference when patients with and without Raynaud's phenomenon were compared and no correlation could be established with the activity of the disease or clinically defined types of scleroderma. Our data indicate that the metabolism of basement membrane proteins is involved in the course of scleroderma; however, it remains questionable whether these assays could become useful as diagnostic or prognostic tools.

Collagen↗

Evidence of immune deposits and of basement membrane alterations in dermal vessels of normal skin of patients with essential mixed cryoglobulinemia.

To gain insight into the pathogenic mechanisms of immune complex-mediated vasculitis, clinically normal skin specimens from the forearms of 12 patients with essential mixed cryoglobulinemia were investigated by light microscopy, immunofluorescence microscopy, and electron microscopy. Basement membrane alterations were documented in 9 patients. Eleven patients had deposits in vessel walls, but only in 1 was there evidence of inflammation. The same immunoglobulins of the cryoproteins could be demonstrated in the tissue deposits by immunofluorescence analysis and by immunoelectron microscopy. These findings suggest that, in essential mixed cryoglobulinemia, immune reactants in dermal vessels of normal skin are more common than is evidenced by clinical examination. Moreover, it seems that cryoglobulins, as such, are not sufficient to trigger an inflammatory process: Additional local or plasma factors are required. Deposits were absent in 7 patients with cryoglobulinemia that was associated with a primary disorder. This could be ascribed to the lower cryocrit levels documented in this group of patients, or to the shorter duration of their disease.

Adult↗

HLA class I- like antigen expression on human leukemic cells.

The expression of HLA class I- like molecules was analyzed on human acute and chronic leukemic cells. The presence on leukemic cells of class I- like molecules, absent on the patient's normal lymphocytes, was examined by complement- dependent lymphocytotoxicity using platelet absorbed alloantisera that recognize HLA-linked, 45-12 kd, beta-2-microglobulin associated molecules, selectively expressed on PHA-activated cells. A positive reactivity of the anti- class I- like alloantisera was found in 50% of the acute leukemias (cALL, T-ALL and AML), independently of the lineage of differentiation, while chronic lymphocytic leukemias (B-CLL) were constantly negative. It is suggested that beta-2-microglobulin associated HLA molecules may represent markers of leukemic blast activation and/or maturation state.

Adult↗

Immune deposits in normal skin of patients with systemic lupus erythematosus: relationship to the serum capacity to solubilize immune complexes.

Immunofluorescent deposits at the dermal-epidermal junction (DEJ) of the skin (lupus band test or LBT) were evaluated in 134 patients with various connective tissue diseases. LBT was found positive in 23 of 32 (71.8%) patients with systemic lupus erythematosus (SLE), in 3 of 53 (5.6%) patients with rheumatoid arthritis (RA), and in 1 of 5 cases of mixed connective tissue disease (MCTD). No deposits were found in patients with systemic sclerosis and with vasculitis. In patients with SLE a positive LBT showed a direct correlation with serum Clq binding activity (ClqBA) and with hypocomplementemia. The mean ClqBA was 13.49 +/- 12.85 and 2.38 +/- 2.27% in SLE patients with positive and negative band test, respectively (P less than 0.005). Likewise depressed mean serum levels of C3 and C4 were detected in patients with skin deposits (P less than 0.005). Sera from SLE patients showed an overall decreased capacity to solubilize preformed immune complexes when compared to normal sera. Furthermore 10 band-positive patients were less able to solubilize immune complexes than sera from LBT-negative lupus patients (45 +/- 16 and 62 +/- 11%, respectively; P less than 0.01). Also the capacity to inhibit the precipitation of immune complexes was decreased in SLE patients with negative LBT (P less than 0.05). In conclusion our data suggest that in SLE patients a decreased complement-mediated solubilization of immune complexes is involved in the persistence of high levels of circulating immune aggregates and, considered its correlation with positive LBT, may be responsible for the deposits of immunoglobulins at the dermal-epidermal junction of the skin.

Adolescent↗

[Cyclosporin A].

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Bone Marrow Transplantation↗

Depression of direct plaque-forming cell response in mouse spleen cell cultures by aggregated IgG2b-induced factors.

Mouse spleen cells were treated with concanavalin A (Con A) or aggregated mouse IgG2b for 48 h in culture. When cells thus treated were added to fresh mouse spleen cell cultures immunized with SRBC they depressed the response of B lymphocytes as measured by enumerating plaque forming cells (PFC) on the fourth day of culture. When supernatant from cells cultured with IgG2b was added to immunized cultures this resulted in depression of PFC generation similar to that observed by addition of treated cells. The depression observed was essentially in the same range as that observed by addition of Con A treated cells or their supernatant. These observations extend previous work suggesting that IgG2b-induced PFC depression may result from activation of suppressor T cells with elaboration of soluble suppressor factors. This mechanism of immunomodulation may be important in the pathogenesis of immune complex disorders.

Animals↗