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Biomedical subjects

X Bosch

Publications and source records attributed to X Bosch.

At least 217 records · Page 12Linked to original sources

Ki-1+ anaplastic large-cell lymphoma of T-cell origin in an HIV-infected patient.

Non-Hodgkin's lymphomas (NHL) are part of the spectrum of disease associated with HIV infection. However, there are only occasional reports of NHL of T-cell origin in HIV-infected patients. A previously asymptomatic HIV-infected man, who was seronegative for human T-lymphotropic virus type I antibodies, developed a high-grade peripheral T-cell lymphoma of anaplastic large-cell type which was Ki-1 + (CD30 +), HLA-DR+, epithelial membrane antigen +, CD25 +, CD71 +, CD2 + and CD5 +. Pan-B markers CD19 and CD22 and histiocytic marker CD68 were negative. At diagnosis the patient had 0.3 x 10(9)/l T-helper lymphocytes. The response to chemotherapy was dramatic and the patient is alive and disease-free 18 months after treatment. A review of previously described peripheral T-cell lymphomas in HIV-positive individuals is performed, and we conclude that the spectrum of neoplasms in such cases is probably broader than originally thought.

Adult↗

Systemic lupus erythematosus in the elderly: clinical and immunological characteristics.

Systemic lupus erythematosus (SLE) predominantly affects young women in their 20s. In 40 out of 250 (16%) patients with SLE seen in our hospital disease onset occurred after the age of 50. The interval between the time of onset and diagnosis was five years in this older group compared with three years in the younger group. Arthritis, as a first symptom, was less common in the older onset group. During the follow up a lower incidence of arthritis, malar rash, photosensitivity, and nephropathy was found in the older onset group. In contrast, this group showed an increased incidence of myositis. High titres of anti-dsDNA tended to occur less often and the incidence of anti-Ro antibodies was lower in the older onset group. These features seem to distinguish patients with older onset SLE as a particular subset.

Adolescent↗

The 'primary' antiphospholipid syndrome: antiphospholipid antibody pattern and clinical features of a series of 23 patients.

Twenty-three patients with the 'primary' antiphospholipid syndrome were studied over 2-6 years. Twenty-two (96%) had antiphospholipid antibodies detected by ELISA (87% had antibodies to thromboplastin and 70% to cardiolipin), and 18 out of the 21 tested patients (86%) had lupus anticoagulant activity by coagulative assays. Mean age of the cohort was 29.9 years and the sex ratio (female:male) 4.75:1. Eleven patients presented 18 venous and/or arterial thrombosis and 13 had 25 foetal losses (84% occurred during the second and third trimester). Other clinical features were migraine, livedo reticularis, and epilepsy. Three patients had relatives with systemic lupus erythematosus. Thrombocytopaenia was seen in 33%, antinuclear antibodies in low or moderate titre in 30%, and haemolytic anaemia in 13%. During the follow-up, two patients presented recurrent thrombosis despite anticoagulant therapy, one of them dying because of recurrent pulmonary thromboembolism. Four patients achieved successful term pregnancies after treatment with aspirin and a further patient after treatment with aspirin and low dose prednisolone. No patient developed systemic lupus erythematosus or any other definable connective tissue disease. The 'primary' antiphospholipid syndrome may exist as a distinct clinical entity and all younger patients presenting with thrombotic events, foetal losses and/or thrombocytopaenia, without any evidence of a well defined disease, should be tested for antiphospholipid antibodies in order to rule out this syndrome.

Adult↗

Prognostic implication of anti-neutrophil cytoplasmic autoantibodies with myeloperoxidase specificity in anti-glomerular basement membrane disease.

Anti-neutrophil cytoplasmic autoantibodies (ANCA) were detected in 12 out of 37 (32%) serum samples from patients with anti-glomerular basement membrane (GMB) disease by an indirect immunofluorescence assay. In 11 cases, ANCA were directed against myeloperoxidase, as revealed employing neutrophils devoid of this enzyme as the test substrate. Patients having both ANCA and anti-GBM antibodies (AGBMA) were considerably older (mean age 59 years) than patients with AGBMA alone (mean age 33 years). In addition, patients with both antibodies had some clinical and pathologic data that suggested an associated systemic vasculitis. This was supported by the fact that among these patients, those with highest ANCA titres recovered renal function despite being initially on hemodialysis, as opposed to those with lowest ANCA titres or AGBMA alone. In patients with both antibodies, there was an inverse relationship between AGBMA and ANCA values (p = 0.02). Moreover, the mean AGBMA level tended to be higher for patients with AGBMA alone than for those with both ANCA and AGBMA. These results suggest that, at least in some cases, there may be a contribution of an ANCA-related mechanism in the pathogenesis of anti-GBM disease. Although the exact role of ANCA in this and other diseases remains to be clarified, there is important clinical evidence that in anti-GBM disease ANCA may represent a serologic marker of good prognosis identifying a subset of patients who may recover renal function.

Adolescent↗

[An elevated prevalence of subclinical pulmonary involvement in systemic lupus erythematosus].

Lung function was prospectively studied in a group of non-selected systemic lupus erythematosus (SLE) female patients who consecutively came to our Lung Function Laboratory. There was no previous history of smoking in any patient nor there was clinical and/or radiographic evidence of lung affectation prior to the diagnosis of SLE. Some respiratory lung function abnormalities were observed in 17 patients (65%). The most frequent functional anomaly was the alteration in carbon monoxide transfer (DLCO), which was present in 10 cases (38%). On the other hand, a restrictive ventilatory failure was observed in 8 patients (31%) and a obstructive ventilatory failure in 6 other (23%). Out of these, 3 patients (12% of the total number of cases) presented bronchial hyperreactivity, which could also be confirmed in a patient presenting a mixed ventilatory failure. Isolated air entrapment could be observed in 2 patients, which could indicate disfunction of small airways in these cases. There was no correlation between functional respiratory failure and clinical activity of SLE, duration of the disease, other organ involvement, treatment and/or serum antibodies. Given that a great number of these functional lung anomalies (42%) exist in the absence of any clinical and radiological anomalies and that an important subgroup of patients are responsive to bronchodilators, we consider that the systemic evaluation of lung function must be obliged in the clinical treatment of SLE patients.

Adolescent↗

[Hemoptysis: a prospective study of 108 cases in an emergency service].

A prospective study is performed in 108 patients presenting hemoptysis who were attended in the emergency department. Final diagnosis was achieved in 89 cases (82.4%), being lung infections of tuberculous origin or not, neoplasias, and chronic obstructive lung emphysema the main observed etiologies. A low percentage of severe hemoptysis is found (5.6%). Diagnosis was obtained in 79.6% of patients by clinical history, physical exam, chest x-ray and ORL exam. Only 13.6% of patients who were not diagnosed in the Emergency department were later diagnosed by follow up and performance of complementary tests. Similarly, the lack of an initial etiological diagnosis had no repercussion, by itself, in a worse prognosis. Therefore, it is recommended to perform a single strict evolution control in patients with hemoptysis of unknown origin who present risk factors of lung neoplasia (male sex, age greater than 40 years, smoking greater than 45 packs/year).

Age Factors↗

Hemodynamic, platelet and clinical responses to prostacyclin in unstable angina pectoris.

The hemodynamic and platelet effects of prostacyclin (PGI2) were investigated in 27 patients with unstable angina (14 treated patients; 13 control subjects) given a 72-hour infusion (5 ng/kg/min) or placebo. This randomized study was double-blind and conducted as a substudy of a multicenter trial testing the clinical efficacy of PGI2. The clinical and angiographic features were identical in the 2 groups. Blood pressure and heart rate were not modified significantly by PGI2. A recurrence of angina during infusion occurred in 8 treated patients (57.1%) and in 8 control subjects (61.5%). Two patients receiving PGI2 and none in the control group developed a myocardial infarction. Levels of 6-keto-prostaglandin F1 alpha, a stable metabolite of PGI2, increased from baseline values (less than 20 pg/ml) to 605 +/- 41 pg/ml during infusion. Levels of fibrinopeptide A, beta-thromboglobulin, platelet factor 4, thromboxane B2 and the platelet aggregates ratio in blood were similar between the 2 groups before, during and after PGI2 infusion. Prostacyclin reduced ex vivo platelet aggregation to adenosine diphosphate and thromboxane B2 generation by approximately 50% during the infusion period with return of aggregation to baseline and platelet thromboxane B2 production to above baseline after the discontinuation of PGI2. Thus, despite favorable effects of PGI2 upon platelet aggregation and systemic hemodynamics, the prostanoid failed to improve the clinical evolution of unstable angina.

6-Ketoprostaglandin F1 alpha↗

[Subacute kidney insufficiency in exclusively vascular kidney amyloidosis].

Two patients are reported with secondary amyloidosis in whom renal biopsy disclosed massive amyloid deposition with exclusively extravascular localization. In one case, autopsy showed selective amyloid deposition in the vessel walls of most examined organs. In both patients, the clinical presentation consisted of subacute renal failure without proteinuria. A possible reduction of renal perfusion, secondary to a depleted blood volume in one case and to pharmacological inhibition of prostaglandin synthesis in the other, could enhance renal dysfunction in both patients, who, on the other hand, previously had mild renal failure. The clinical presentation of renal amyloidosis basically depends on the distribution and severity of amyloid deposits. Vascular localization represents an uncommon pattern of renal amyloidosis, generally associated with chronic renal failure with minimal or no proteinuria.

Acute Kidney Injury↗

Systemic lupus erythematosus in a patient with partial lipodystrophy.

Systemic lupus erythematosus developed in a 35-year-old woman who had partial lipodystrophy since she was 7 years old. To our knowledge, this association has been reported only once. She also had hypocomplementemia, mesangiocapillary glomerulonephritis type II, and a serum assay with positive results for C3 nephritic factor. The association of partial lipodystrophy with other autoimmune disorders suggests an immunologic pathogenesis for this disease.

Adult↗

Primary intrapulmonary benign schwannoma. A case with ultrastructural and immunohistochemical confirmation.

Neurogenic pulmonary tumours are rare. Neurofibromas are the most common variety and tend to occur in patients with generalized neurofibromatosis, whereas schwannomas arise sporadically in patients who have no evidence of this disorder. A case of solitary benign schwannoma arising in a segmental bronchus is presented, and the published cases reviewed. Microscopically, the tumour had a spindle-shaped cell proliferation with palisade formation in several fields and low cellularity in other areas. The diagnosis was supported by positive immunostaining for S-100 protein and by ultrastructural examination which showed elongated cells surrounded by well-preserved basal lamina and numerous cytoplasmic processes also possessing a basal lamina.

Aged↗