[Sneddon's syndrome and antiphospholipid antibodies].
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Biomedical subjects
Publications and source records attributed to F Bermejo.
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Sneddon syndrome is know as the association of idiopathic livedo reticularis and cerebrovascular lesions. The most characteristic trait of this syndromes is a non-inflammatory arteriopathy in medium caliber vessels. The pathogenic role of antiphospholipid antibodies in this disease is not clear. Clinical characteristics and etiopathogenic features of eight patients with Sneddon's syndrome are reviewed, specially regarding its relationship with primary antiphospholipid syndrome. A female predominance was found (3:1) as well as a relationship with hypertension (five patients suffered hypertension), but no relation was found with contraceptive use. Three patients showed evidence of antiphospholipid antibodies, present as anticardiolipin antibodies with significative titers in three cases and lupus anticoagulant in one. Digital artery biopsy performed in four patients showed in all of them the pathologic features characteristic of this disease. Seven patients were treated with platelet activity inhibitors and one with oral anticoagulants. Six of them have had a year and half follow-up without showing any new ischemic stroke. The main etiopathogenic factor on Sneddon's syndrome is the presence of a non-inflammatory arteriopathy in medium caliber vessels. Blood hypertension and antiphospholipid antibodies could play a role in the development of cerebrovascular lesions in some cases. No relationship has been found with oral contraceptives in this series of patients. Medium term prognosis with platelet activity inhibitors therapy seems benign.
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We have reviewed the clinical and pathological records of 40 aged patients who showed only vascular lesions on histological examination. They were followed up for 3.5 +/- 6.3 years before death, and in 28 cases the diagnosis of dementia was done during life. Demographic data, vascular and systemic illnesses, psychiatric neurological and neuropsychological disturbances, and pathological findings were compared between demented and non-demented patients. The number of strokes, several neurological and almost all neuropsychological disturbances, the volume of macroscopic cerebral infarct, especially in frontal, occipital and basal regions, the lacunar state and the white matter lesions, were significantly greater in demented patients. However most of them had less than 100 ml3 of brain infarct. The relative influence of each type of cerebral vascular lesion upon the dementia syndrome was determined by means of multivariate analysis. The volume of macroscopic cerebral infarct, the white matter lesion and the lacunar state showed quite similar contributions to mental deterioration.
A group of patients with transient ischemic attacks (65 cases), reversible ischemic neurologic deficits (37 cases) and infarctions with minimum residuum (41 cases) was included in a prospective follow-up for a mean period of 5.2 years. Most of the patients were treated with acetylsalicylic acid or other antiplatelet agents, or with anticoagulants. Principal complications during follow-up were: cerebral infarction, 15 cases (10.4%); cerebral hematoma, 2 (1.3%); transient ischemic attacks, 17, (11.8%); reversible ischemic neurologic deficits, 9 (6.2%); myocardial infarction, 4 (2.7%); and death, 11 (7.6%). The cumulative incidence for stroke or death was 6.2% for the first year and 14% for the fifth year. There were no significant risk factors for the occurrence of stroke or vascular death on time-related multivariate analysis (Cox). Disabling stroke was less frequent after infarctions with minimum residuum than after transient ischemic attacks or reversible ischemic neurologic deficits.
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Three patients with myoclonic encephalopathy caused by bismuth salts are reported, one with the severe and another with the mild form; dimercaprol was effective for the rapid improvement of the symptoms in both. In one of the patients in whom the clearance of bismuth was calculated it was found to be increased after dimercaprol therapy. We think that dimercaprol is an effective drug for the therapy of myoclonic encephalopathy caused by bismuth salts.
Two cases of myoclonic encephalopathy due to bismuth salts intoxication are reported. In both, treatment with dimercaprol led to clinical recovery. This therapy was shown to enhance bismuth clearance. We also present data on the CSF metabolites dopamine, norepinephrine and serotonin of one patient.
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Different penicillins (phenylacetyl, 2-hydroxyphenylacetyl, 4-hydroxyphenylacetyl, phenoxyacetyl and 2-thiopheneacetylpenicillin) have been synthesized "in vitro" by direct N-acylation of 6-aminopenicillanic acid (6-APA) with the acyl group of several acyl-CoA derivatives. The enzyme that catalyzes these reactions, acyl-CoA: 6-APA acyltransferase of Penicillium chrysogenum, was purified to homogeneity (374-fold) and its amino acid composition is given. This protein accepts as substrates several aliphatic acids and different aromatic acids with the only requirement that an acetyl-CoA moiety must be present in the substrate molecule. Shortening or lengthening of the acyl moiety prevents the 6-APA-N-acylation reaction. The presence of an amino group in the alpha-position of the acetyl group does not allow this molecule to be used as substrate. However, different substitutions in the phenyl group (hydroxylation of the carbons 2 and 4) or its replacement by another aromatic ring (thiophene) were accepted with varying reactions rates in the acylation reaction when a 176-fold purified acyltransferase was employed. The homogeneity pure enzyme accepts as substrate thiophene acetyl-CoA but it did not 2-hydroxyphenyl and 4-hydroxyphenylacetyl-CoA. The presence of an oxygen atom between the aromatic and the acetyl moieties did not affect the catalysis.
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