Antiphospholipid Syndrome (APS) is characterized by the presence of circulating antiphospholipid antibodies in patients with thrombosis or pregnancy morbidity. Antibodies involved in these disorders ...are mainly those directed against β
2-Glycoprotein I (β
2GPI) with the major epitope apparently located on discontinuous antigen with several parts of Domain I (DmI) involved. The relation between anti-DmI antibodies and patients’ risk categories is unknown.
The synthetic full-length and correctly-folded DmI (1–64) to set up a competitive inhibition enzyme-linked immunoadsorbent assays (ELISA) was used. Plasma of 22 patients with APS and triple positivity Lupus Anticoagulant positive (LAC+), IgG anti-cardiolipin positive (aCL+), IgG anti-β
2GPI positive (a β
2GPI +), 15 with double positivity (IgG aCL+, IgG aβ
2GPI+), 9 with single positivity (IgG aβ
2GPI+) and 20 controls were evaluated.
Median of percentage inhibition was 25.5% interquartile range (IQR)17.2-33.0 in triple positive patients. Significantly lower inhibition was observed in patients with double positivity, median inhibition 5.0% (IQR 0.0-27.0) and in patients with single positivity median inhibition was 2.0% (IQR 0.5-8.0) (p
<
0.0001). No inhibition was detected in control subjects or using β
2GPI peptides (40–52 and 57–70), or when antithrombin, an insignificant control protein was used.
High risk patients with APS and triple laboratory positivity as compared with double and single positivity patients have significantly higher titre of anti-DmI antibodies as evaluated by an inhibition test.
Vertebral artery dissection is one of the more frequent cerebral-vascular disorders in the young adult. The initial symptoms rarely consist of vertigo with clinical characteristics of Selective ...Monolateral Acute Vestibular Deficit Syndrome. The case is described of a patient, who arrived with intense rotatory vertigo associated with neurovegetative symptoms and spontaneous nystagmus, which we initially diagnosed as right neuronitis. About 48 hours later, the symptoms of vertigo disappeared spontaneously, and prevalently nuchal cephalea appeared. Since the symptoms were atypical and the otoneurologic study revealed normal canalar and otholithic function, a cerebral nuclear magnetic resonance, with contrast, was carried out which showed the presence of multiple areas of cerebellar ischaemia, prevalently on the left, and at the level of the right occipital lobe. Study of the patient was completed with an intracranial angio-nuclear magnetic resonance of the neck arteries and cerebral angiography the findings of which were compatible with left vertebral artery dissection. It is important to emphasize, as reported in the literature, that in cases in which atypical evolution of the pathology appears, or instrumental data do not confirm initial suspicions, a more scrupulous study is always necessary in order to find a possible central cause. Among the central causes, it should not be forgotten that multiple small cerebellar strokes (more frequent in elderly patients) and even more rarely also vertebral artery dissection (which is typical of younger patients) may become evident in a clinical picture that is almost identical to that seen in selective monolateral acute vestibular deficit syndrome.
Synovial sarcoma (SS) is a malignant mesenchymal neoplasm usually involving the lower limbs of young adults. Localization in head-neck district is rare. Histologically, these are characterised by a ...biphasic or monophasic variant, the latter being more rare and difficult to identify. Immunohistochemistry plays a crucial role in the diagnosis. Cytogenetics also play an important role since both the monophasic and the biphasic forms are characterised by a reciprocal translocation (x;18) (p 11.2;q 11.2). Treatment options include an aggressive surgical approach and radiotherapy, whereas the role of chemotherapy remains to be defined. The case is described of monophasic synovial sarcoma located in the hypopharynx and a review is made of the literature concerning this rare neoplasm.
Beta2-glycoprotein I (β2GPI), a relevant antigen in Antiphospholipid Syndrome (APS), binds anionic macromolecules including heparin (Hep). A possible formation of ternary complexes between β2GPI, ...antibodies and Hep in APS is thus possible. The aim of this study was to evaluate Hep-β2GPI interaction in patients with APS. The affinity of Heps of different length, including unfractionated Hep (UFH), low-molecular weight Hep (enoxaparin) and pentasaccharide (fondaparinux), to human β2GPI was estimated by fluorescence spectroscopy, yielding dissociation constant (Kd) values of 1.1, 24.0 and 89.4µM, demonstrating that the longer UFH binds to β2GPI far more tightly than the shorter ones. Plasma and protein G-purified IgGs from eight patients with APS (i.e. five with thromboembolic disease and three with catastrophic APS), were fractionated by affinity chromatography using a Hep (UFH)-bound column, eluted with a linear NaCl gradient. For each chromatographic analysis, fractions were collected in the whole NaCl gradient and tested by ELISA for the presence of β2GPI and anti-β2GPI IgG. The results of Hep-affinity chromatography and ELISAs concurrently indicate that either β2GPI and anti-β2GPI IgG elute from the Hep column in the same chromatographic peak, at a retention time identical to that of the purified, isolated β2GPI, thus suggesting that circulating immunocomplexes containing β2GPI are present in patients with APS. PUBLICATION ABSTRACT
It has been reported that IgG to oxidized LDL/beta2-glycoprotein I (oxLDL/beta2GPI) complexes are associated with arterial thromboembolism (TE) in patients with antiphospholipid syndrome (APS). How ...these antibodies behave in arterial as compared to venous TE in APS is unknown. The aim of the present study was to evaluate the association of IgG anti-oxLDL/beta2GPI with clinical manifestations in category I APS patients. Fifty-seven APS patients with triple positivity (Lupus Anticoagulant (LAC), anti cardiolipin (aCL) and anti-beta2-glycoprotein I (abeta2GPI) antibodies), 28 with arterial and 29 with venous thromboembolism, were included in the study. There were no differences in the dRVVT ratio, IgG/IgM aCL and IgG/IgM abeta2GPI titers in the two patient groups. There were no differences in the IgG (78.5 U+/-59.8 vs. 112.2 U+/-92.3) and IgM (16.3 U+/-15.9 vs. 21.1 U+/-14.3) anti-oxLDL/beta2GPI mean values. A significant correlation was found between IgG anti-oxLDL/beta2GPI and IgG anti-beta2GPI titers in the whole group of APS patients. Patients in the arterial group were older and had more risk factors for atherosclerosis. Data from this study do not support the hypothesis that IgG anti-oxLDL/beta2GPI are specifically associated to arterial TE in Category I APS patients.
Necrotizing external otitis (NEO), or malignant external otitis, a severe penetrating infection of the ear canal and the temporal bone, which occurs almost exclusively in diabetic or immunodepressed ...elderly patients, is caused in most cases by Pseudomonas aeruginosa while Staphylococcus epidermidis and Aspergillus fumigatus have been described as other possible agents. Recently bone scintigraphy, carried out above all employing SPECT, has been considered an important tool in diagnosis and follow-up of NEO. Many Authors confirm the role taken by Technetium99m-methylene-diphosphonate (MDP) and Gallium67-citrate bone scanning. Benecke suggested that response to therapy could be monitored with Ga67 or In111 scans. Two cases of NEO in which diagnosis and follow-up were made using Tc99m-nanocolloid (NC) SPECT are presented in this paper. The Authors discuss the advantages of this latter radiotracer, compared to those obtained with Ga67-citrate, in the follow-up evaluation of therapy efficacy. Tc99m-NC, in fact, is a better inflammation index in that its fixation on bone tissue is determined by the permeability of the basal membrane of vessels. Furthermore, with Tc99m-NC antibiotic therapy may be continued as long as necessary. Bone scintigraphy with other radiotracers may infact result positive for a long period after disease remission in that their fixation is linked to the one remodelling process.
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