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Biomedical subjects

J Font

Publications and source records attributed to J Font.

At least 217 records · Page 12Linked to original sources

[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↗

Association of antibodies against phospholipids with heart valve disease in systemic lupus erythematosus.

A prospective echocardiographic study was carried out on 132 consecutive patients with systemic lupus erythematosus (SLE) derived from three European university medical centres. The prevalence of valvular lesions in patients with SLE was 22.7% compared with 2.9% in a control group of 68 healthy volunteers. 50 SLE patients had antibodies against phospholipids. The prevalence of valve vegetations (8/50 [16%]) and of mitral regurgitation (19/50 [38%]) was significantly higher among the SLE patients with antiphospholipids than among those without (1 and 10/82 [1.2% and 12%], respectively). During follow-up of the patients with valvular lesions, haemodynamically significant clinical valve disease developed in 6 but surgery was required in only 1; 9 had cerebrovascular occlusions; and 7 died, although no death was due directly to the cardiac involvement. Thus, valvular heart disease, particularly affecting the mitral valve, is common in patients with SLE, and the presence of antibodies against phospholipids is associated with a higher prevalence of valvular abnormalities in these patients.

Adolescent↗

Normal proprioceptive trigeminal afferents in patients with Sjögren's syndrome and sensory neuronopathy.

A pure sensory neuropathy due to neuronal damage in the gasserian and spinal ganglia has been described in patients with Sjögren's syndrome. Conventional electromyographic (EMG) studies can demonstrate the selective involvement of the sensory pathways but they do not provide definite evidence for the site of the lesion. Noting that the trigeminal sensory neurons carrying cutaneous and muscular afferents are differentially located in the gasserian and mesencephalic nuclei, respectively, we carried out an electrophysiological study of the trigeminofacial and trigeminotrigeminal reflexes in 5 patients with Sjögren's syndrome and pure sensory neuropathy, in 10 patients with sensory-motor neuropathies of other causes, and in 10 healthy subjects. Our results show that patients with Sjögren's syndrome and pure sensory neuropathy who exhibited abnormal blink reflexes and an abnormal, cutaneous-induced masseter silent period had normal jaw jerks, whereas patients with sensory-motor neuropathies who exhibited abnormal cutaneous responses had abnormal jaw jerks. These findings suggest that the lesion in pure sensory neuropathy involves damage to the neurons of the gasserian ganglia and not to the trigeminal axons, since an axonal lesion would be expected to involve the large axons from muscle spindle receptors.

Adult↗

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↗

WGA binding to the surface of two autologous human melanoma cell lines: different expression of sialyl and N-acetylglucosaminyl residues.

Two autologous human melanoma cell lines were studied to determine their capacities to bind wheat germ agglutinin (WGA). Both cell lines were derived from the same patient, the first, IGR 39, originated from the primary tumor, the second, IGR 37, was established from a metastatic lymph node. WGA binding sites on the surface of these cell lines were compared before and after sialidase and/or tunicamycin treatments. IGR 39 cells exhibited two classes of WGA binding sites with high and low affinities, whereas IGR 37 cells had only one class of high affinity binding sites. After tunicamycin treatment, the capacity of IGR 39 cells to bind WGA was markedly altered, since only one class of WGA binding sites with high affinity was observed under these conditions, whereas tunicamycin did not induce significant changes in the lectin binding of IGR 37 cells. The low affinity WGA binding sites, which were only found on IGR 39 cells, corresponded to sialyl residues present in N-linked glycoproteins. The high affinity binding sites present on both cell lines probably involved sialyl and N-acetyl-glucosaminyl residues associated with O-linked glycoproteins and/or glycolipids. No direct correlation could be drawn between the number of WGA binding sites and the overall sialic acid levels exposed to sialidase treatment. The 3-fold increase in the amount of cell surface glycopeptides obtained after pronase digestion and specifically binding to WGA-Sepharose was in good agreement with the overall higher number of WGA binding sites on IGR 39 compared to IGR 37 cells. Thus, subtle carbohydrate changes of cell surface glycoconjugates might account for the differences between the biological properties of human melanoma cell lines of low and high tumorigenicity.

Acetylglucosamine↗

Antiphospholipid antibodies in unselected patients with repeated abortion.

We have studied, prospectively, the incidence of several antiphospholipid antibodies (lupus anticoagulant, anticardiolipin, anti-phosphatidylserine, anti-phosphatidic acid, anti-phosphatidylinositol and anti-thromboplastin antibodies) in 65 consecutive patients with two or more (range 2-8, mean 3.1) abortions. Lupus anticoagulant activity was detected in seven (10.7%) patients and all of them exhibited other antiphospholipid antibodies. Of the previous pregnancies in these seven women, 88% had ended in spontaneous abortion. Four of them achieved pregnancy after low-dose aspirin therapy was started, and carried successfully to term. It is concluded that antiphospholipid antibodies, namely lupus anticoagulant, should be routinely screened in the recurrent spontaneous aborter.

Abortion, Habitual↗

Pure sensory neuropathy in patients with primary Sjögren's syndrome: clinical, immunological, and electromyographic findings.

A pure sensory neuropathy caused by lymphocytic infiltration of the dorsal root ganglia has been reported in a few patients with Sjögren's syndrome. The clinical, immunological, and electromyographic findings of five patients with this type of neuropathy and primary Sjögren's syndrome were reviewed. Typical clinical indications were the presence of a chronic asymmetrical sensory deficit, initial disease in the hands with a predominant loss of the vibratory and joint position senses, and an association with Adie's pupil syndrome or trigeminal sensory neuropathy. The simultaneous impairment of the central and peripheral evoked cortical potentials suggested that there was a lesion of the neuronal cell body. The neuropathy preceded the diagnosis of Sjögren's syndrome in four patients. Four patients were positive for Ro antibodies, but systemic vasculitis or malignancy was not found after a mean follow up of six years. These findings indicate that in patients with a sensory neuropathy the diagnosis of Sjögren's syndrome has to be considered, even if the patient denies the presence of sicca symptoms, and that appropriate tests must be carried out.

Adie Syndrome↗

Isotype distribution of anticardiolipin antibodies in systemic lupus erythematosus: prospective analysis of a series of 100 patients.

A prospective study of IgG and IgM isotypes of anticardiolipin antibodies (aCL) in a series of 100 patients with systemic lupus erythematosus was carried out. To determine the normal range of both isotype titres a group of 100 normal control serum samples was studied and a log-normal distribution of IgG and IgM isotypes was found. The IgG anticardiolipin antibody serum was regarded as positive if a binding index greater than 2.85 (SD 3.77) was detected and a binding index greater than 4.07 (3.90) was defined as positive for IgM anticardiolipin antibody. Twenty four patients were positive for IgG aCL, 20 for IgM aCL, and 36 for IgG or IgM aCL, or both. IgG aCL were found to have a significant association with thrombosis and thrombocytopenia, and IgM aCL with haemolytic anaemia and neutropenia. Specificity and predictive value for these clinical manifestations increased at moderate and high anticardiolipin antibody titres. In addition, a significant association was found between aCL and the presence of lupus anticoagulant. Identification of these differences in the anticardiolipin antibody isotype associations may improve the clinical usefulness of these tests, and this study confirms the good specificity and predictive value of the anticardiolipin antibody titre for these clinical manifestations.

Anemia, Hemolytic↗

Calcium-activated, phospholipid-dependent protein kinase activity in calf platelets.

Protein kinase C (PKC) has been widely studied from different tissues of mammals. Human platelets display higher levels of PKC activity, if compared with other sources. The PKC activity from calf platelets crude extract was determined in the presence of various protease inhibitors such as PMSF, Leupeptin or Trypsin inhibitro, and the Ca(2+)-chelators EGTA and EDTA. The free calcium requirement was 0.25 mM, calculated with the help of the Solgas-water computer program, which represents 1 mM CaCl2, in these assay conditions. Optimum PKC activity was obtained at 4 min in the presence of PS plus DAG or TPA, using H1 type III-S histone as substrate. Phospholipid-interacting drugs, such as trifluoperazine, chlorpromazine and tetracaine, inhibited the PKC activity in a dose-dependent manner. Triton X-100, a non-ionic detergent, which is usually employed to solubilize the membrane fraction, in different translocation assays, inhibited PKC activity at concentrations higher than 0.01%. In these conditions, non-proteolytic PKC activity from calf platelets was easily determined, and shares similar activity levels with those described in human platelets.

Animals↗

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↗