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Biomedical subjects

O Bletry

Publications and source records attributed to O Bletry.

At least 109 records · Page 6Linked to original sources

Treatment of progressive systemic sclerosis with plasma exchange. Seven cases.

Seven patients, 4 women and 3 men afflicted with severe progressive systemic sclerosis (PSS) were treated with Plasma Exchange after failure of different other treatment. All patients presented Raynaud phenomenon and arthritis, 6 patients presented extensive skin lesions, 5 of them digestive manifestations, 3 pulmonary fibrosis. In one case PSS was associated with polymyositis, one patient presented bilateral recurrent cornea ulcerations, (Sjögren Syndrome++) and one patient numerous skin ulcerations. In 5 patients adjuvant corticosteroid therapy was given during the course of PE. In 3 patients PE must be stopped after one or two sessions because of insufficient venous access. Among the 4 other patients 8 to 20 PE were performed: the patient with cornea ulcerations became blind during the treatment, skin ulcerations and severe Raynaud phenomenon did not improved in two other patients. Benefit of PE was noted in only one patient with regressive myositis, and improvement of articular and cutaneous symptoms. Therefore, PE are not useful in most patients afflicted with PSS, they are difficult to realize in numerous patients and did not improve clinical symptoms in most cases.

Adolescent↗

[Value of plasma exchange in necrotizing angeitis. 11 cases].

Plasma exchanges proved useful in a series of 11 patients with necrotizing angeitis resistant to conventional treatment (corticosteroids alone in 6 cases and combined with cyclophosphamide in 5 cases). Among the 9 patients alive after 18 months' follow-up, 5 were in remission without treatment. The only disturbing complication of plasma exchanges was hepatitis B in 3 of the 4 survivors who received frozen fresh plasma as substitute. It is suggested that these immuno-compromized patients should receive diluted albumin instead of fresh frozen plasma.

Adolescent↗

[The renin-angiotensin system in hypertensive patients with scleroderma and polyarteritis nodosa (author's transl)].

The renin-angiotensin system was studied in 14 normotensive and hypertensive patients with scleroderma and polyarteritis nodosa (PAN). The variations in blood pressure, plasma renin activity (PRA) and serum aldosterone were compared after the Captopril test. Mean arterial pressure fell 15,7 +/- 2,6 p. 100 (p less than 0,01) in hypertensive patients, but no change was observed in normotensive subjects. PRA and serum aldosterone did not change significantly in hypertensives with scleroderma. Large variations in blood pressure, PRA and serum aldosterone were observed in 2 out of 3 patients with PAN, the third one presenting a fall in blood pressure without significant changes in PRA or serum aldosterone. These results suggest that other mechanisms are responsible for hypertension in these patients and that the Captopril acted on other factors than the renin-angiotensin system.

Blood Pressure↗

[Complete retention of urine of sudden onset secondary to herpes genitalis infection (author's transl)].

The authors report three cases of complete urinary retention of sudden onset during a herpes genitalis infection secondary to traumatic sexual relations. In practice, the diagnosis was made in the absence of any urological cause, the presence of local signs indicative of herpetic infection of the genital organs and lumbar puncture. Mictional disturbances disappeared in 2 to 3 weeks without leaving any sequelae. The possibility of nervous system complications in genital herpes infections has been know since 1904. By contrast, disturbances in micturition have been reported much more recently, in 1953. Complete urinary retention of sudden onset is not rare: 17 cases out of a series of 486 cases of genital infection. This may be the only clinical sign of nervous system involvement. Urinary retention develops between a few days and 3 weeks after the onset of genital herpes. Neurological examination of the lower limbs is normal. There may be transient impotence in the male and loss of the bulbo-cavernosus reflexes and impaired sensation in an S2-S3 distribution. Bladder tone is spontaneously hypoactive and increases following the injection of Urecholine. The herpes virus (HSV) is found only during the acute phase of the genital infection but almost never a few days later, when urinary retention develops.

Antibodies, Viral↗

Bullous dermatosis associated with dysglobulinemia (two cases). Relationships with epidermolysis bullosa acquisita.

Two cases of bullous dermatosis with dysglobulinemia are described. The first one was associated with renal and neuromuscular amyloidosis and production of a monoclonal lambda IgG. Optical and electron microscopy showed amyloid deposits beneath basal lamina of the dermis. Results for direct and indirect immunofluorescence (IF) were negative. This bullous dermatosis is not an epidermolysis bullosa acquisita (EBA), in the strict sense, because the amorphous material deposited is amyloid. In the second case, associated with Waldenström's disease, there was no cutaneous or systemic amyloidosis. Direct IF was positive; linear IgM deposits were seen along the basal membrane of the bulla and the healthy skin. Indirect IF showed the presence of circulating antibodies against basal membrane zone. This bullous dermatosis is probably an EBA, despite the absence of IgG deposits. The absence of electron microscopy does not permit the confirmation of this diagnosis.

Aged↗

[Cardiac manifestations of Takayasu's disease (excluding hypertensive heart disease) (author's transl)].

The literature is reviewed in the light of 6 cases of Takayasu's disease with cardiac manifestations. Whilst the cardiac manifestations of Takayasu's disease are most often due to the effects of hypertension, there nevertheless exist specifically cardiac lesions: coronary, valvular and myocardial. Coronary lesions, typically ostial, a veritable extension of aortic disease, may also effect the vessels themselves. The course is generally grave but surgery has been performed in a number of cases. Aortic insufficiency is usually moderate and without haemodynamic consequences and is the most frequent valve lesion, others being rare. Direct myocardial involvement with the possibility of arrhythmias or conduction disturbances is also rare, as are pericardial and endocardial lesions.

Adolescent↗

[Neurologic manifestations of Behcet's disease. 24 cases].

Dominant symptoms in 24 patients with neurological manifestations of Behçet's disease were meningo-encephalitis (13 cases), febrile meningeal syndrome (3 cases), intracranial hypertension (6 cases), polyneuritis of the lower limbs (1 case), and isolated retrobulbar ocular neuritis (1 case). Several classical notions concerning the "Neuro-Behçet" meningo-encephalitis were confirmed: the frequency of central motor lesions (33 p. cent), cerebrospinal fluid alterations (anomalies in 100 p. 100 of cases in the initial stage), and the often unfavorable course (40 p. cent). An unusual finding in this series was the frequency of cerebral veins thrombosis (4 certain and 2 probable). These could be differentiated from meningo-encephalitis by their symptomatology, predominantly that of intracranial hypertension, and by their course: often the first disorder to appear in the course of Behçet's disease, they followed a favorable neurological course but were constantly complicated by other vascular manifestations, usually phlebitis. It is suggested that a long-term anticoagulant treatment should be associated with corticoid therapy in these forms.

Adult↗

[Clinical aspects, outcome and prognosis of Horton's disease. Retrospective study of 47 cases].

The authors report their experience of Horton's disease in 47 histologically confirmed cases treated between 1966 and 1979. The symptomatology is recalled with the incidence of the various clinical and biochemical signs. The actuarial survival curve shows a 71.2 p. cent three year and a 61 p. cent five year survival rate. There was no statistically significant difference at three years with a control population. The duration of maintenance therapy was, on average, of 24.8 months, never less than 15 months and sometimes reaching 60 months. The average maintenance dose was 12 mg. Specific and non-specific complications are discussed. The incidence of refractory and cortico-dependent forms shows that the optimal treatment for this disease has yet to be found; the often brilliant initial results of corticotherapy do not reflect its long-term efficacy.

Adult↗

[Neuropathy associated with ichthyosis and splenomegaly in an adult patient. Possible relationship with altered glycosphingolipid metabolism (author's transl)].

The authors report on the case of a 40-year-old woman presenting with ichtyosiform erythroderma, central and peripheral neuropathy, splenomegaly and low serum haptoglobin levels. The patient's blood and urine contained large amounts of various ceramides polyhexoside including, in particular, a ceramide tetrahexoside containing fucose. The possibility of a new form of sphingolipidosis is discussed.

Adult↗

[Efficacy of captopril in periarteritis nodosa with arterial hypertension and renal failure (author's transl)].

In a patient with periarteritis nodosa failure of antihypertensive treatment with beta-blockers and diuretics encouraged the authors to use captopril in doses of 150 mg per day. Blood pressure rapidly returned to normal levels. The hypotensive effect of captopril is ascribable to its action on the renin-angiotensin system, since plasma renin activity was particularly high in this patients.

Angiotensin I↗

[Periarteritis nodosa associated with hepatitis B virus. 42 cases (author's transl)].

HBs antigen was detected by immunodiffusion or radioimmune assay in the blood of 19 out of 42 patients with periarteritis nodosa examined between 1970 and 1978. Histological or biochemical signs of hepatitis were present in 6% of HBs--positive patients and 1% of HBs-negative patients. The incidence of clinical symptoms was similar in both groups, except for multiple neuritis, which was much more frequent in HBs-positive patients (89%) than in HBs-negative patients (52%; p less than 0.001), suggesting a close relationship between neuritis and HBs and HBs antigen. There was a negative correlation between myalgias and skin lesions and the presence of the antigen. The prognosis of periarteritis nodosa was similar in both groups. The course of the disease seemed to be more prolonged in HBs-positive patients, but there was no significant difference in mortality rate.

Adult↗

Multiple pulmonary arterial aneurysms in Behcet's disease and Hughes-Stovin syndrome.

Four case studies of patients with angiographically documented pulmonary arterial aneurysms are presented. In two cases, Behcet's disease was diagnosed; one case corresponded to the syndrome described by Hughes and Stovin, that is, venous thrombosis especially of the vena cava accompanied by singular or multiple pulmonary arterial aneurysms in young patients; and the last case could best be described as an association of the two. Our observations lead us to question the existing notions concerning the relationship between Behcet's disease and Hughes-Stovin syndrome-the clinical, angiographic and histologic aspects of the vascular manifestations are comparable. Typically the two diseases run similar courses with death resulting frm the rupture of the aneurysms and massive hemoptysis. These case studies cast certain doubts as to the effectiveness of the corticosteroid treatment usually prescribed. Finally, we suggest that Hughes-Stovin syndrome might be, in fact, a manifestation of Behcet's disease.

Adult↗

Pulmonary involvement in Behcet disease.

The radiologic appearance of pulmonary involvement in six cases of Behcet disease is described. Chest radiographs in five patients showed infiltrates and/or rounded opacities followed by excavation in two cases and by pleural rupture in one. Repeat chest films on four of these five patients 3 weeks to 9 months after diagnosis showed resolution of the infiltrates and the subpleural opacities. The other findings from chest radiography in three patients were rounded or lobulated opacities near the hila. Four of the six patients underwent pulmonary angiography, which in all cases showed wide-spread occlusions of pulmonary arteries, accompanied in three cases by segmental or lobular pulmonary artery aneurysms corresponding to the proximal opacities visible on the plain films. Two of the three patients who displayed pulmonary artery aneurysms died of massive hemoptysis 3 and 13 months after angiography. In the third patient, progress under medical treatment was favorable; chest film 10 months after treatment started showed complete resolution of the aneurysms. Repeat angiogram also showed partial recanalization of the occluded arteries.

Adult↗

Polycythemia vera and primary hyperparathyroidism.

A patient with the clinical manifestations of polycythemia and hypercalcemia underwent an extensive examination for occult neoplasm. No malignancy was found; however, a parathyroid adenoma was excised. After its removal, the hypercalcemia and polycythemia disappeared during a two-year follow-up period.

Adenoma↗

[Disorders of conduction in lupus erythematosus : frequency and incidence in a group of 112 patients (author's transl)].

A retrospective study of 112 cases of lupus erythematosus, 103 acute disseminated lupus erythematosus (ADLE) and 9 chronic discoid lupus (CDL), was conducted to determine the incidence of disorders of conduction (DC), and to study their prognosis and discuss their pathogenicity. The mean age of the group was 38 +/- 16 years, and the mean follow-up period after discovery of the DC was 53 months. Cardiac lesions were present in 49.5 p. cent of the 103 patients with ADLE : pericarditis in 27 p. cent, murmur from lupus endocarditis or cardiomyopathy in 23 p. cent, heart failure in 4.8 p. cent, and hypertension in 17 p. cent. Disorders of conduction were present in 18 (17.5 p. cent) of the 112 patients studied. These included 5 partial right bundle-branch blocks (no complete right bundle-branch block), 2 complete and 3 partial left bundle-branch blocks, 5 complete, 2 first degree, and 1 second degree atrioventricular blocks (AVB). The atrioventricular blocks were usually located in the truncal or fascicular regions, but in 2 cases they were nodal in origin. The 5 complete AVB were associated with ADLE in two cases and CDL in the three other cases. The AVB in the ADLE cases appeared 9 to 20 years after the onset of the lupus, these two patients developing pericardiomyocarditis unaccompanied by disorders of conduction. The three complete AVB occurring during CDL were detected 9 to 18 months after the diagnosis. A fatal outcome was noted in 13 (12.5 p. cent) of the ADLE patients and one of the 9 cases of CDL. Ten-year survival curves showed no difference in prognosis for the groups with or without disorders of conduction, but mortality increased in patients with DC after 10 years. As disorders of conduction were more frequently observed in patients with lupus than in a control population, they can be attributed to either a lupus myocarditis or prolonged administration of synthetic antimalarial agents. Disorders of conduction, and particularly complete AVB are, in fact, observed in patients without pericardiomyocardial lesions, and when they exist usually develop a long time after the onset of the cardiac lesion. All patients had been treated with antimalarials, however, and the onset of the DC was associated with a chloroquine myopathy in some of them. Three of the five complete AVB were observed during the course of CDL in patients without cardiac lesions, this being a supplementary argument for implicating synthetic antimalarials.

Adult↗