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J Ordi

Publications and source records attributed to J Ordi.

At least 19 recordsLinked to original sources

[Hepatic mesenchymatous hamartoma presenting in an adult patient].

Mesenchymatous hamartoma is a benign liver tumor characteristically found in childhood, particularly during the first two years of life. A case of mesenchymatous hamartoma detected in a 23-year-old female during a study for non specific abdominal disturbances is presented. Imaging techniques showed a large mass in the right hepatic lobe with hypodense areas of cystic appearance. Pathologic study demonstrated the characteristic mixoid mesenchymatous component with areas of cyst formation and rests of liver tissue inside the tumor. Despite its rarity in adults this case demonstrates the need to consider mesenchymatous hamartoma in the differential diagnosis of hepatic masses in adults, particularly when radiologic examination demonstrates cystic components.

Adult

The value of complement activation products in the assessment of systemic lupus erythematosus flares.

Complement activation products (CAP) have been reported as sensitive markers of disease activity in systemic lupus erythematosus (SLE). We have measured seven parameters of the complement system (C3, C4, factor B, C3a, C4a, iC3b, and the terminal complement complex-TCC-) in 61 SLE patients to study their interrelationship and relative efficacy as diagnostic indicators of lupus activity. Disease activity was judged according to a clinical index (SLEDAI) to be active in 22 and inactive in 39 patients. Subjects with active SLE showed increased levels of C3a, C4a, and TCC compared with those of stable lupus and normal controls, and plasma concentrations of these CAP manifested a positive correlation with disease activity scores. However, values of factor B and iC3b did not correlate with lupus flares. Serum C3 levels were a better reflection of the degree of SLE activity than were C4 levels. The anaphylatoxins were extremely sensitive markers of disease activity but they lacked enough specificity, and iC3b was not at all informative for this purpose. On the whole, TCC concentration was the most useful parameter (77% sensitivity, 80% specificity) to monitor lupus activity, correlating the best with the activity scoring system, and thus offers a better laboratory marker of lupus severity than conventional measurements of complement.

Adolescent

High prevalence of platelet autoantibodies in patients with systemic lupus erythematosus.

The aim of this study was to determine the prevalence of platelet autoantibodies (PAA) in patients with systemic lupus erythematosus (SLE) and its correlation with clinical and other laboratory manifestations of the disease, as well as to evaluate the influence of platelet count and disease activity on the result of the test for PAA. Ninety SLE patients, 29 with thrombocytopenia, were evaluated. The presence of PAA was determined using the direct and indirect platelet suspension immunofluorescence test. A total of 166 PAA determinations were performed in the 90 patients upon entry into the study. Fifty-six of the 90 patients (62%) with SLE were positive for PAA. There were no statistically significant correlations between the presence of PAA and the different disease manifestations except for thrombocytopenia (P = 0.0005). The presence of PAA in the same patient was significantly associated with current thrombocytopenia and disease activity. It was concluded that the prevalence of PAA in SLE patients is high. All SLE patients with thrombocytopenia had PAA, although some patients with PAA had a normal platelet count. The presence of PAA in SLE patients is not synonymous with thrombocytopenia but should instead be regarded as one of the multiple serological abnormalities which may develop in these patients.

Adolescent

Signaling through CD50 (ICAM-3) stimulates T lymphocyte binding to human umbilical vein endothelial cells and extracellular matrix proteins via an increase in beta 1 and beta 2 integrin function.

Regulated adhesion of T lymphocytes to antigen-presenting cells, endothelial cells and extracellular matrix proteins is crucial in T lymphocyte activation and migration to the sites of injury. In this study, we show that three monoclonal antibodies (mAb) recognizing different epitopes on the CD50 (ICAM-3) molecule increase T lymphocyte adhesion to tumor necrosis factor (TNF)-stimulated human umbilical vein endothelial cells and extracellular matrix proteins. These phenomena are mediated by an increase in beta 1 and beta 2 integrin avidity since (a) CD50-induced adhesion to endothelial cells was abrogated by simultaneous blocking of beta 1- and beta 2-mediated adhesion pathways but not by interfering with either one individually, (b) CD50 mAb increased beta 1 integrin-mediated adhesion to extracellular matrix proteins and to fibronectin-derived synthetic peptides, (c) CD50 mAb enhanced T lymphocyte binding to ICAM-1 transfectants, and (d) CD50 mAb did not modify surface expression patterns of beta 1 or beta 2 integrins on T lymphocytes. Our data suggest that constitutively expressed CD50 (ICAM-3) can play a pivotal role in initiating a cascade of adhesion events which may be crucial in immune activation and in the development of inflammatory lesions.

Antigens, CD

Pyoderma-gangraenosum-like ulcers associated with lupus anticoagulant.

A 45-year-old women presented with a deep ulcerative lesion of the left breast. Two months later, a new cutaneous necrotic lesion located in the right pretibial areas was observed. The only serum abnormality was the presence of circulating lupus anticoagulant. Clinical features suggested the diagnosis of pyoderma gangraenosum and histologic examination showed microthrombosis in the dermal capillary vessels adjacent to a dense, mixed inflammatory infiltrate with some degree of vascular damage. Although thrombosis may be the result of vasculitis, it is likely that in our patient the presence of a lupus anticoagulant activity played a role in the pathogenesis of the cutaneous ulcers.

Breast

[Extrinsic compression of the pulmonary artery by a mediastinal lymphoma. Diagnosis by Doppler echocardiography].

A patient with an anterior mediastinal tumor and cardiomegaly observed by plain chest roentgenogram is presented. The echocardiographic-Doppler examination showed massive pericardial efussion with hemodynamic compromise, and external compression of the right atrium and pulmonary artery. A pericardiocentesis was performed, and a mediastinoscopy with biopsy was diagnostic of lymphoblastic lymphoma.

Adolescent

[Hodgkin's disease and non-Hodgkin's lymphoma occurring simultaneously].

The simultaneous appearance of Hodgkin's disease and a non Hodgkin's lymphoma is very infrequent and has classically been considered as coincidental. Nevertheless, recent clinical observations suggest that neoplastic cells which lead to Hodgkin's disease are of lymphoid origin. The case of a "discordant" lymphoma formed by a laterocervical Hodgkin's disease and a large cell gastric lymphoma is presented. The coexistence of these two diseases, as herein presented, supports the lymphoid origin of Hodgkin's disease.

Female

Secondary (AA) amyloidosis associated with Castleman's disease. Report of two cases and review of the literature.

This article describes two patients with localized Castleman's disease (CD) of the mixed hyaline vascular and plasma cell type located at the mesentery of the small bowel, associated with systemic symptoms, anemia, an increased plasma level of acute phase reactants, and systemic amyloidosis. There were amyloid deposits on the vascular walls of the liver, spleen, and mesenteric lymphoid mass. On immunohistochemical studies, amyloid was shown to be of the AA type in both cases. After surgical resection of the mesenteric mass, clinical and laboratory manifestations improved, and the amyloidosis showed no progression in these patients 23 years and 1 year later. The authors' review of the literature shows a striking similarity of CD cases associated with amyloidosis in terms of the abdominal localization of the lymphoid mass, secondary pattern (AA type) of amyloidosis, and improvement of clinical manifestations after removal of the tumor, without progression of amyloid deposition.

Adult