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O Blétry

Publications and source records attributed to O Blétry.

At least 91 records · Page 5Linked to original sources
PubMed1993

[Trigeminal neuropathy and connective tissue diseases].

Connective tissue diseases are a rare and poorly understood etiology of trigeminal neuropathy. In a retrospective multicentric study covering 12 years. 12 cases were identified in the archives of the Departments of Neurology, Rheumatology and Internal Medicine: 4 were associated with progressive systemic sclerosis. 4 with mixed connective tissue diseases and 4 with Sjögren's syndrome. In 9 of the cases, the neuropathy led to the diagnosis of connective tissue disease. Trigeminal neuropathy usually developed during the evolution of the connective-tissue disease but, in 3 cases, it preceded other clinical signs. As a rule, the connective tissue disease was relatively inactive when the trigeminal nerve was involved. The neuropathy was usually unilateral (9 times), localized to the inferior branch(es) of the trigeminal and manifested itself by an isolated sensation of cutaneous numbness associated with paresthesias. Only one patient had mixed motor and sensory involvement. The topography of the lesions (peripheral, truncal, radicular or central nuclear) remains unknown in most cases, however, electromyographic study of the blinking reflex in 2 patients confirmed peripheral neuropathy in both of them. Corticotherapy had only a minor effect: neuralgia was rare and carbamazepine was ineffective. The pathogenicity of trigeminal involvement is discussed and these observations are compared to the 151 cases reported in the literature.

Adult↗
PubMed1993

[Interferons. Interferons alpha and gamma: indications in systemic diseases].

Interferon alpha (INF-alpha)--In systemic diseases, most indications for INF-alpha result from its effect on haematological or hepatological manifestations. The spectacular effect of INF-alpha in chronic myeloid leukemia has led to its use for the treatment of hypereosinophilia syndrome and systemic mastocytosis. Over the last 6 years, we have treated 7 patients with the hypereosinophilia syndrome who were resistant to corticotherapy and had markers of myeloproliferation. Although both hydroxyurea and INF-alpha can be effective alone, their combination led to a decrease in the eosinophilia count to 1,000/ml, a decrease which was long-lasting in most cases. INF-alpha is also used in histiocytosis X alone or in combination with retinoids or with etoposide and has been found effective in several observations. In carcinoid syndromes whether treated priorly or not with a 5-fluoro-uracil-streptozoticin combination, INF-alpha leads to an objective response in two-thirds of the patients. Several multicentric protocols are currently assessing the efficacity of INF-alpha in mixed cryoglobulinaemias. In most observations these cryoglobulinaemias are seen in patients with markers of hepatitis C (mainly HCV) and the early results are encouraging. Temporary improvement has been reported in discoid or subacute lupus in 8 out of 10 cases. Haemangiomas of the infant, when life-threatening and corticoresistant, may be a good indication for INF-alpha. Thus 20 newborns or infants (including 4 with Kasabach-Merrit syndrome) have been treated with good results in 18. Interferon gamma (INF-gamma).(ABSTRACT TRUNCATED AT 250 WORDS)

Hematologic Diseases↗
PubMed1993

[Treatment of hypereosinophilic syndromes of myeloproliferative expression with the combination of hydroxyurea and interferon alpha. Apropos of 7 cases].

Seven patients suffering from hypereosinophilic syndrome with clinical and/or hematological symptoms of a myeloproliferative syndrome were treated with a combination of hydroxyurea and interferon-alpha. To date, 6 of them have been followed for more than 1 year. In all cases, this therapy decreased circulating hypereosinophilia to under 1,500/mm3 and obtained normal eosinophil levels in 5 patients with a parallel regression of visceral complications. None of the patients experienced treatment-associated side effects, particularly hematological ones. Hypereosinophilic syndrome has a poor prognosis due, for the most part, to the development of visceral, cardiac and neurological complications, and, more secondarily, to the risk of progression towards acute leukemia. Conventional treatments, corticosteroids and hydroxyurea, have greatly improved the prognosis, but failure of these therapeutics remains common in the myeloproliferative form of the disease. Because interferon-alpha was proven to effectively treat chronic myeloid leukemia, it has been proposed for the treatment of hypereosinophilic syndromes, and encouraging results have been obtained despite the high daily doses required to control the disease. In the future, its association with hydroxyurea could represent an alternative therapy capable of controlling the disease at low doses thereby limiting the risk of side effects.

Adult↗
PubMed1992

[Role of antiphospholipid antibodies in fetal loss].

A review of the literature and our own experience have shown that the three types of antibodies to phospholipids (APL) usually looked for (i.e. dissociated treponema serology, antiprothrombinase and anticardiolipin) increase the risk of foetal loss, irrespective of whether the mothers have or do not have systemic lupus erythematosus. The prevalence of APL may exceed 40 percent in some series of women who suffered at least 3 foetal losses. Conversely, the incidence of foetal loss (often late in pregnancy) in women with APL has been estimated at 25 to 75 percent, depending on the studies. The presence of lupus seems to increase the risk. There is no consensus on the best factor predictive of foetal loss (antiprothrombinase or one of the anticardiolipin isotypes). Foetal loss seems to be caused by thrombosis of the placenta, the origin of which remains controverted. The therapeutic escalade consists of abstention (in the first pregnancy), aspirin (about 100 mg/day), aspirin-corticosteroid combination or subcutaneous heparin, high-dose intravenous immunoglobulins and plasmapheresis. With these various methods, the birth of a normal child can be expected in almost every case.

Abortion, Spontaneous↗
PubMed1992

[Stauffer's syndrome caused by a benign intracystic renal hematoma].

We report a case of Stauffer's syndrome, characterized mainly by cholectasis, that occurred in a patient with benign intracystic renal haematoma and subsided after nephrectomy. Stauffer's syndrome was recognized, in 1961, as a paraneoplastic manifestation, usually associated with hypernephroma. This syndrome has been reported in only 2 cases of benign renal disease, which was a pseudotumoral xanthogranulomatous pyelonephritis.

Cholestasis, Intrahepatic↗
PubMed1992

Cerebral venous thrombosis in Behçet's disease: clinical study and long-term follow-up of 25 cases.

Among 250 patients with Behcet's disease, we describe 25 cases of angiographically proven cerebral venous thrombosis. Intracranial hypertension was the most frequent manifestation. Two initially untreated patients relapsed. Treatment of cerebral venous thrombosis consisted of combined heparin and steroids in 19 patients, steroids alone in three, and heparin alone in three others. Neurologic symptoms improved rapidly in all. Nineteen patients received long-term anticoagulation, and two received aspirin. Relapse of cerebral venous thrombosis or development of optic atrophy did not occur in treated patients. Partial or total recanalization of the occluded sinus was frequent. After more than 3 years of follow-up, the prognosis of dural sinus thrombosis is satisfactory.

Adolescent↗
PubMed1992

[Objective evaluation of sclerodermic skin using the slip-test. Results and validation with 30 patients and 60 controls].

The slip test is a non-invasive way to objectively evaluate the extent of scleroderma by measuring the "slipping" of the skin over an underlying bone. The results obtained in 30 scleroderma patients and 60 controls (178 tests due to multiple measurements) were analyzed to determine which test sites show a significant difference between patients and controls (Student's t-test) and which sites are the most discriminating for the diagnosis of the disease (multivariate analysis). Patient values were clearly distinct from those of controls. The best sites (defined by p < 0.05) were: the mandible, the second and first phalanges of the middle finger, the hands, the forearms, the epicondyles, the sternum, the iliac crests, the patellae, the tibias (the upper and lower fourths) and the feet; in all, 23 sites to which can be added the opening of the mouth (measurement of the maximal interlabial distance), which proved to be a reliable parameter. Multiple regression analysis yielded an equation that identified patients with scleroderma based on 4 variables (right forearm, right iliac crest, sternum and right rib). The slip test is thus the first reproducible quantitative test able to confirm and quantify clinical impressions in scleroderma. It is entirely able to objectively monitor the evolution of the disease under treatment. Nevertheless, complementary studies are needed to verify whether the slip test, which can be performed in 20 minutes during an office visit, can be used advantageously in the routine clinical evaluation of scleroderma.

Adult↗
PubMed1992

[Etiological aspects of orthostatic hypotension].

Orthostatic hypotension (OH) must be distinguished from supine hypotension made worse by standing up and, in particular, from vasovagal syncope. At first approximation, asympathicotonic invariable pulse OH virtually always related to an organic lesion of the baroreflex arch must be distinguished from variable pulse OH which is usually functional and may also be due to organic lesions with exclusive or predominant sympathetic system disorders. In case of doubt, it may be useful to measure palmar and plantar sympathetic potentials. The principal causes of variable pulse OH are therapeutic drugs, absolute or relative hypovolaemia, endocrine diseases (adrenal insufficiency, phaeochromocytoma), spinal quadriplegia and two congenital diseases including dopamine beta-hydroxylase deficiency. In Guillain-Barré syndrome, diabetes and alcoholism, the OH pulse may be variable or invariable. The main causes of asympathicotonic OH are ageing, post-prandial period, certain infections (e.g. tabetic neurosyphilis, botulism, EBV and HIV infections), a few systemic diseases and isolated neurological diseases. Among the systemic diseases responsible for OH are diabetes, alcoholism and chronic liver diseases of other causes, porphyria, lead poisoning, Biermer's disease, amyloidosis, several connective tissue diseases, including systemic lupus erythematosus, and some cancers associated or not with Lambert-Eaton syndrome. Among isolated neurological diseases are the familial diseases described by Riley and Day, multisystem atrophies (first described by Shy and Dager) and pure peripheral dysautonomia. To differentiate the latter from an incipient Shy-Dager syndrome, it may be helpful to use pharmacological tests: plasma catecholamine levels measurements in supine position, and clonidine test with repeated growth hormone assays in upright position.

Blood Pressure Determination↗
PubMed1992

Expression of HLA class II antigens on skin fibroblasts in scleroderma.

Skin biopsies were taken from 11 patients with morphoea, nine with acrosclerosis and 10 with diffuse systemic sclerosis and processed for immunohistochemical studies using a panel of monoclonal antibodies including antibodies to MHC class II antigens. A significantly higher percentage of HLA-DR positive dermal cells were observed in the reticular dermis in biopsies from patients with morphoea (44.1 +/- 16.2%), acrosclerosis (15.9 +/- 5.4%) and systemic sclerosis (39.5 +/- 2.3%) when compared with the controls (6.6 +/- 2%). A smaller percentage of dermal cells also expressed HLA-DP and -DQ. The degree of monocnuclear cell infiltrate in the biopsies, however, did not correlate with the percentage of HLA class II positive fibroblasts. In organ culture, the expression of the HLA class II antigens was almost totally lost after 3 days and was no longer detected on fibroblasts after 3 weeks of culture.

Cell Count↗
PubMed1991

[Chronic constrictive pericarditis. 27 cases].

Twenty-seven cases of chronic constrictive pericarditis seen between 1975 and 1990 in an internal medicine department were analyzed retrospectively. The chronic pericarditis was consecutive to one (n = 5) or several (n = 7) episodes of acute pericarditis. Echography demonstrated the presence of pericardial effusion in 74 percent of the cases, pericardial thickening in 41 percent and/or compression of right heart cavities in 55 percent. Computerized tomography of the chest, performed in 16 cases, showed pericardial effusion in 63 percent of the cases, pericardial thickening in 37 percent and lymph node enlargement in 19 percent. Magnetic resonance imaging of the chest was carried out in 2 patients but showed no abnormality. All 11 patients who underwent cardiac catheterization were found to be adiastolic. The cause of constrictive pericarditis, elicited in 13 patients was neoplasia in 4, sequelae of radiotherapy in 2, injuries in 2, mediastinal and retroperitoneal fibrosis in 2, myocardial infarction in 1, purulent pericarditis in 1 and bacteriologically proven tuberculosis in 1. Medical treatment with corticosteroids (n = 16) and/or antituberculous therapy (n = 15) was successful in 2 patients; 25 patients had to undergo surgery 7 +/- 11 months after constriction was diagnosed. Pericardial drainage (through a pericardiopleural window in 4 cases) proved to be sufficient in 10/15 patients but failed in 5. Pericardectomy was performed initially in 3 cases and after failure of medical treatment and/or drainage in 11 cases. The 4 patients with neoplastic constrictive pericarditis died 10 months on average after the diagnosis, but the remaining 23 patients were alive after à 9 to 48 months (mean: 19 +/- 15) follow-up. These results suggest that the data provided by echocardiography and computerized tomography of the chest usually point to the relevant therapeutic measures without a need for invasive haemodynamic exploration. Idiopathic constrictive pericarditis now accounts for 50 percent of the cases; tuberculosis has become exceptional, but the other, previously exceptional causes (neoplasia, heart surgery, radiotherapy, connective tissue diseases) are more frequent. Corticosteroids should be used in chronic constrictive pericarditis occurring after cardiac surgery or in the course of a connective tissue disease, but they are effective only in highly inflammatory forms of the disease. Modern treatment relies on early surgery, since functional results and patient's survival are closely related to the date of pericardectomy which must be carried out before very important myocardial repercussions develop.

Adrenal Cortex Hormones↗
PubMed1991

[Relapse of Wegener's granulomatosis. Retrospective study of 18 cases].

During a mean therapeutic follow-up of 4 years, 25 out of 61 patients with Wegener's granulomatosis had one or several relapses. As in 7 of them the initial diagnosis had proved erroneous, this study concerns the remaining 18 patients who together totalled 31 relapses after the correct diagnosis was made. Twenty relapses occurred early on, within 4 months of a change in treatment, and they corresponded to recurrent activity of the disease. Ten relapses occurred later on, after treatment had been discontinued or kept at maintenance dose level. The relapses appeared as early as the first year in 41 percent of the cases. The ENT region was affected in 61 percent of the patients. Nine relapses occurred in the absence of treatment, including 6 which took place 1 to 8 years after treatment. Three relapses occurred less than 3 months after a prednisone and oral cyclophosphamide therapy was initiated. Compared with the group of patients without relapse, those in the relapse group were younger, more often treated with azathioprine and cyclophosphamide administered intravenously, and followed up for a longer period; their probability of survival was also higher. In case of early relapse after withdrawal of treatment the mean duration of that treatment had been shorter than in the absence of relapse. None of the patients whose treatment had been stopped more than 20 months after its onset had an early relapse. These data prompted us to make the following proposals: initially, cyclophosphamide should be administered as bolus injections (the time required to obtain results is then shorter than with the oral route), and maintenance therapy should last at least 20 months. Azathioprine should be used only when cyclophosphamide is contra-indicated. The relative indications for intravenous and oral cyclophosphamide remain to be determined. Discontinuing all treatments is a difficult decision to make, but even then the patients should be under close supervision in view of the possibility of late relapses.

Adolescent↗