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Biomedical subjects

O Blétry

Publications and source records attributed to O Blétry.

At least 19 recordsLinked to original sources
PubMed1997

[Can amyloidosis regress?].

Amyloidosis always worsens in the absence of treatment. Suitable treatments may improve the prognosis, but results depend on the type of amyloidosis. AA amyloidosis can improve according to clinical and biological criteria after the treatment of underlying disease, or after colchicine therapy in familial mediterranean fever. Histological regression is very unusual. A small clinical improvement or at least a stabilisation can be observed in familial amyloidosis with mutation in plasma transthyretin, after liver transplantation. However, the follow-up is short and the mortality is high. In AL amyloidosis, the survival is usually less than 15 months. Some patients have a better survival when they receive chemotherapy similar to that given in multiple myeloma. This could indicate an amyloidosis improvement, or at least a stabilisation.

Amyloidosis↗
PubMed1997

Systemic capillary leak syndrome: report on 13 patients with special focus on course and treatment.

BACKGROUND: Systemic capillary leak syndrome (SCLS) is a rare condition characterized by unexplained episodic capillary hyperpermeability due to a shift of fluid and protein from the intravascular to the interstitial space. This results in diffuse swelling, weight gain, and renal shut-down. From the first publication in 1960, only 34 cases have been reported. OBJECTIVE: To collate enough patients to observe the natural history of the disease and evaluate the efficacy of empiric treatments. DESIGN: Multicentric retrospective study. RESULTS: Thirteen patients (6 women and 7 men) were collated with a mean follow-up of 6.4 years. Eight patients are still alive after a mean of 5.6 years (range 1 to 15). Three patients out of the 11 who were not lost to follow-up died; 1 during an attack and 2 because of a progression towards multiple myeloma. CONCLUSIONS: Our series shows an improvement in the prognosis of SCLS due most likely to improved management during attacks. Some patients' disease could evolve into a multiple myeloma. Treatment is still empiric and no prophylactic therapy, including terbutaline associated with aminophylline, has clearly proven its efficacy.

Acute Disease↗
PubMed1997

Outcome of planned pregnancies in systemic lupus erythematosus: a prospective study on 62 pregnancies.

We conducted a prospective study in order to determine planned pregnancy outcome in systemic lupus erythematosus followed in a tertiary referral centre. Pregnancy was authorized if disease was inactive on 20 mg/day prednisone or less for at least 1 yr. Upon the diagnosis of pregnancy, systematic corticosteroids consisting of 10 mg/day prednisone or more were started. In the case of antiphospholipid antibodies, 100 mg/day aspirin was added, replaced by heparin in the pre-partum period. In the case of antiphospholipid syndrome complicated by previous thrombotic events or fetal losses despite aspirin, heparin was prescribed. One woman with a history of atrioventricular block was treated with dexamethasone. Patients were monitored by medical and obstetrical examination, and laboratory tests carried out at least monthly and a quarterly echography. Among 62 pregnancies in 38 women, lupus flare was observed in 27% of the cases, 6% of which occurred in the post-partum period. Flares were moderate except in one renal involvement in a woman with prior diffuse proliferative glomerulonephritis. Therapy was not modified in half of the cases. Pregnancy ended in early spontaneous abortion not related to lupus flare (n = 10), stillbirth (n = 2). induced abortion (n = 2), preterm birth (n = 29) and full-term birth (n = 19). Caesarean section was performed in nine cases. A severe infection occurred in two premature neonates. Another premature neonate was growth retarded. Two children had cutaneous neonatal lupus. No child died, neither had atrioventricular block. Stillbirth and severe prematurity were more common in mothers with antiphospholipid syndrome. After exclusion of early spontaneous and induced abortions, the live birth rate was 96%, that is close to the French general population. The main problem remains a high rate of prematurity, but without maternal or neonatal death.

Adrenal Cortex Hormones↗
PubMed1996

[Planned pregnancy program in systemic lupus erythematosus].

OBJECTIVES: Maternal and fetal risk is high during pregnancy for young women with systemic lupus erythematosus. We analyzed outcome after a planned pregnancy program for these patients. METHODS: Between 1982 and 1994, 58 pregnancy were planned in 34 women with systemic lupus. Nine of them had renal and 5 central nervous system involvement; antiphospholipid syndrome was present in 8; steroid therapy was given in 24, immunosuppressors in 2 and plasma exchange in 1. At diagnosis of pregnancy, prednisone was prescribed (at least 10 mg/d), associated with aspirin in all non-symptomatic patients with antiphospholipid antibodies followed by heparin at pre partum. Heparin was used in case of antiphospholipid syndrome. Women with anti-SSA or B antibodies and no past history of congenital atrioventricular block were not given any specific treatment. RESULTS: An acute lupus flare-up occurred in 27% of the cases including 6% in post partum. The flare-up was mild in all cases and treatment had to be changed in half of the cases. There were 9 early abortions, 1 induced abortion for congenital malformation, 2 fetal deaths, 28 premature deliveries and 18 term deliveries. Cesarean section was indicated in 8 cases. Severe neonatal infection occurred in 2 premature infants and 1 other was growth retarded. Cutaneous neonatal lupus was observed in 2 infants. No atrioventricular blocks occurred. CONCLUSION: Fetal death or very premature birth were more frequent in patients with antiphospholipid syndrome. When pregnancy is planned in women with systemic lupus erythematosus, live birth rate reaches 96% after exclusion of early and therapeutic abortions. This rate is close to the rate in the general population. The high rate of premature birth is the main risk, but there were no maternal nor neonatal deaths.

Abortion, Spontaneous↗
PubMed1996

[Fatal infectious complications in 2 patients with adult onset Still disease].

If adult Still's disease (ASD) can sometimes lead to severe destructive joint lesions and to various systemic manifestations, life-threatening complications are very rare. However, a long-term and high-dose corticosteroid therapy is often required to control the disease, with frequent corticodependence. Some authors have proposed methotrexate as a second line drug for ASD, that could permit a corticosteroid sparing effect. We report two cases of acute fatal infectious complications--legionella pneumonitis and multiple brain abscess caused by Nocardia asteroides--in two patients treated for ASD with both corticosteroids and methotrexate. These two cases raise the problem of the immunodepression induced by this combination therapy and point out the difficulties in aggressive forms of ASD.

Adult↗
PubMed1996

[Pleuropulmonary manifestations of systemic vasculitis].

Pleuropulmonary manifestations of systemic vasculitis are common, polymorphic and of ambiguous significance: the same pulmonary lesion may reveal a specific manifestation of vasculitis as well as a therapy-induced complication, especially infection which may favor per se a flare-up. Two questions will be successively studied: what are the pleuropulmonary characteristics of Wegener's granulomatosis, Churg-Strauss syndrome, periarteritis nodosa, Behçet's disease, Takayasu's disease and temporal arteritis? What are the major adverse effects that may occur in the course of a treated systemic vasculitis?

Humans↗
PubMed1996

[The heart and antiphospholipid antibodies. Personal experience and review of the literature].

Since the recognition of the antiphospholipid syndrome, a great number of cardiac manifestations have been reported in association with these antibodies: valvular disease, coronary artery disease, cardiomyopathy and intracardiac thrombosis. However this association raises numerous questions related to the pathogenic role of antiphospholipids, their prognostic significance and their frequency in a non-selected population with a definite cardiac manifestation. In view of the literature and our personal experience, it seems necessary to distinguish two kinds of situations. During systemic lupus and primary antiphospholipid syndrome (which must be systematically looked for in patients with history of thrombo-embolic disease), antiphospholipids antibodies certainly play a role in the occurrence of cardiac manifestations, but the precise place of thrombosis has to be best defined along with immunologic/inflammatory mechanisms. On the other hand, in a non-selected population, antiphospholipids antibodies may just be the consequence of the cardiac lesion and do not seem to have prognostic implications. This distinction, actually hypothetical, should be supported on the basis of distinct specificities of antiphospholipids antibodies and especially their dependence on beta 2-glycoprotein I, which would help to distinguish the harmful antibodies from those which probably just appear as an epiphenomenon.

Antibodies, Anticardiolipin↗
PubMed1996

Prospective and serial study of primary amyloidosis with serum amyloid P component scintigraphy: from diagnosis to prognosis.

OBJECTIVE: The purpose of this study was to assess the value of the serum amyloid P (SAP) component scintigraphy in patients with primary amyloidosis (AL). MATERIAL AND METHODS: Pure human SAP labeled with iodine-123 (123I-SAP) was given intravenously to 24 patients with biopsy-proven systemic amyloidosis (15 without multiple myeloma = group 1, and 9 with multiple myeloma = group 2) and to 6 patients with multiple myeloma without any clinical or biological signs of amyloidosis (group 3). Whole-body images as well as regional views and tissue retention levels were obtained after 24 hours. Our study was approved by the institutional review committee and all individuals gave informed consent and were prospectively studied (median 13 months, range 1 to 47 from the date of the scintigraphy to May 1995). RESULTS: Organ localization of 123I-SAP, indicating the presence of substantial visceral amyloid deposits, was observed in all patients in group 1 and 2. The organ uptake of 123I-SAP included the spleen (1 patient was splenectomized) in 20 of 23 cases (87%), the liver in 15 of 24 (60%), and the kidneys in 6 of 24 (25%). Myocardial 123I-SAP was never seen although 13 out of the 24 patients had clinical or echographic data for amyloidosis. Twenty-four hour tissue retention was significantly elevated in all patients (group 1 and group 2): 55.66% +/- 19.16% in group 1 and 34.37% +/- 24.92% in group 2, as compared with normal levels < 24%. The sensitivity of the technique was 79% when only organ uptake was considered but reached 100% when tissue retention was also considered. The 24-hour tissue retention might be correlated with the severity of the amyloidosis: mean survival in patients with tissue retention greater than 50% was 11.3 months versus 24.5 months in patients with levels less or equal to 50%. Five of the 6 patients with multiple myeloma without evidence of amyloidosis had abnormal 123I-SAP imaging and 24-hour tissue retention levels. In 2 of them, amyloidosis was secondly detected. In the 9 patients who had two scintigraphies, variations in 24-hour tissue retention values were in accordance with the clinical evaluation. CONCLUSIONS: Spleen and liver distribution of amyloidosis is mostly revealed by 123I-SAP scintigraphy in patients with AL amyloidosis. The uptake of 123I-SAP appeared in proportion to the quantity of amyloidosis present in different tissues, and the relative quantity of amyloid deposits in the myocardium, carpal tunnel, digestive tract, and kidneys was often small and seldom visualized by 123I-SAP scintigraphy. In contrast 24-hour tissue retention levels were abnormal in all cases of known AL amyloidosis. This may be a positive argument for the diagnosis of amyloidosis when histopathological tests are normal. Tissue retention levels appear important as they may be correlated with survival.

Aged↗
PubMed1996

Immunological disorders in C virus chronic hepatitis.

Hepatitis C virus infection can be accompanied by a number of systemic, non-specific or autoimmune disorders and by extra-hepatic biological abnormalities. Their exact prevalence remains to be determined, together with their association with certain pathologies such as non-Hodgkin lymphoma. The most frequent biological peculiarity is cryoglobulinaemia, found in more than 50% of patients. It is only symptomatic in less than one-third of cases (as purpura, Raynaud's syndrome, neuropathy or renal failure), and could be the origin of benign lymphoproliferative haematological pathology, then of a malignant one (non-Hodgkin lymphoma). Rheumatoid arthritis may develop in 20-25% of cases. Periarteritis nodosa is a rare but possible occurrence. Autoimmune disorders proper include hepatitis with anti-LKM1 antibodies, thyroiditis, skin manifestations, complex connectivitis and Gougerot-Sjögren's syndrome. This syndrome is histologically detected in 15-50% of patients. It affects women preferentially and is usually not accompanied by anti-SSA or anti-SSB antibodies. Thyroidites are different from those occurring under Interferon-alpha therapy, and should be systematically investigated before initiating that therapy. Lichen planus can be associated to HCV infection. The proven induction of autoimmune disorders by Interferon therapy requires that immune disorders be fully assessed before treatment initiation, and close monitoring for their occurrence is necessary throughout the treatment course. TSH and thyroid hormone in particular should be closely monitored.

Animals↗
PubMed1996

[Course of ischemic risk in treated atheromatous hypertensive patients. The PRIHAM study (Prognosis of Ischemic Risk in Atheromatous Patients under Mediatensyl)].

Hypertension is one of the major cardiovascular risk factors. However it seldom occurs alone in a patient and the antihypertensive treatment should be taken into account when assessing global cardiovascular status. The PRIHAM study was an open study which enrolled 3,216 hypertensive atheromatous patients who already has had a clinical ischaemic accident. Patients were followed-up for 3 years. The lowering effect on cardiovascular risk, starting from the fifteenth month was confirmed to the end of 36-month study. The effect on blood pressure was observed from the third month. Stabilized blood pressure observed within the first year persisted throughout the three years without tachyphylaxis. It was effective on SBP (-14.4%) and on DBP (-15%) while maintaining a stable heart rate both in the supine position or immediately upon standing or after 3 minutes in the standing position. Tolerance was globally judged as good or very good by the investigating practitioners and the patients expressed an improvement in their feeling of well-being. In conclusion, from the level of effectiveness obtained and the low incidence of adverse effects, urapidil appears well adapted and easy to manage in a population with a particularly high cardiovascular risk.

Adult↗
PubMed1995

M mode and Doppler echocardiographic assessment of left ventricular diastolic function in primary antiphospholipid syndrome.

BACKGROUND: High titres of serum antiphospholipid antibodies are a possible pathogenic factor for cardiac lesions in patients with systemic lupus erythematosus. OBJECTIVE: To test the hypothesis of a causal link between high titres of antiphospholipid antibodies in the serum and myocardial involvement in patients without systemic lupus erythematosus. PATIENTS AND DESIGN: 18 patients with primary antiphospholipid syndrome (recurrent fetal loss, arterial and/or venous thrombosis, high titres of antiphospholipid antibodies, and no criteria for systemic lupus erythematosus) were prospectively studied by cross sectional, M mode, and pulsed Doppler echocardiography, and compared with 18 healthy controls. The pulsed Doppler indices of left ventricular diastolic function included isovolumic relaxation time and four mitral outflow indices: peak velocity of early flow, peak velocity of late flow, early to late peak flow velocity ratio, and rate of deceleration of early flow. Four computerised M mode indices were also measured: peak rate of left ventricular enlargement in diastole, peak rate of posterior wall thinning, peak velocity of lengthening of the posterior wall, and velocity of circumferential chamber lengthening. RESULTS: Compared with controls, patients with primary antiphospholipid syndrome had higher values for isovolumic relaxation time and peak velocity of late mitral outflow and lower values for early to late mitral peak outflow velocity ratio, rate of deceleration of early mitral outflow, peak rate of left ventricular enlargement in diastole, peak rate of posterior wall thinning, peak velocity of lengthening of the posterior wall and velocity of circumferential chamber lengthening. CONCLUSION: This abnormal pattern reflects an impairment of myocardial relaxation and filling dynamics of the left ventricle in patients with primary antiphospholipid syndrome who were free of any clinically detectable heart disease. These data suggest that high serum titres of antiphospholipid antibodies may be associated with subclinical myocardial damage.

Adult↗