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Biomedical subjects

B Devulder

Publications and source records attributed to B Devulder.

At least 91 records · Page 5Linked to original sources
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

[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

[Detection of the Arg 3500-->Gln mutation of B apolipoprotein. Value in clinical practice].

OBJECTIVE: Familial defective apolipoprotein B-100, caused by a mutation in position 3500 of apolipoprotein B, induces hypercholesterolaemia, a major risk factor of coronary artery disease. The objective is to evaluate the interest of the detecting subjects bearing the Arg3500-->Gln mutation and to observe the response of these subjects to different physiological and therapeutic situations, in clinical practice. METHODS: We performed a systematic screening among hypercholesterolaemic outpatients attending a lipid clinic. The heterozygote subjects were followed up during more than one year and, in some of them, we compared the efficiency of cholesterol-lowering drugs belonging to the different classes: fibrate and statine. RESULTS: Two probands and 3 related subjects were detected. Three patients were treated. For two patients, reduction of LDL-cholesterol plasma levels was observed with both drugs but was significantly higher with statine than with fibrate. For the third one, carrying the E2E4 phenotype, the statine did not seemed to have a major effect. CONCLUSION: The number of probands is in agreement with the frequency reported in literature. A possible interaction of the effect of statine with the apo E2 isoform is discussed.

Adult↗
PubMed1995

[Testicular localization of systemic diseases].

Acute orchitis or a mass in testis usually evokes a neoplasm, a torsion or infectious disease for the clinician. Rarely, a systemic disease is involved. Nevertheless, testicular involvement occurs during vasculitis, Behçet or granulomatous diseases, but is uncommonly the first manifestation. Histologic changes after biopsy or orchiectomy usually give the diagnosis. Testicular localization of systemic disease does not change the general treatment.

Behcet Syndrome↗
PubMed1995

[Neurologic manifestations associated with antiphospholipid antibodies. Or what remains of neurolupus?].

Antiphospholipid antibody is associated with a clinical syndrome of vascular thrombosis, thrombocytopenia, recurrent fetal loss and livedo reticularis, whether or not a clinical diagnosis of systemic lupus erythematosus (SLE) coexists. Central nervous system involvement in SLE is multifactorial, thrombotic events, antineuronal antibodies, hypertension, infection, side effects of drugs etc. Antiphospholipid antibodies may play a role in focal neurological manifestations in SLE. In the absence of SLE, different neurological symptoms are well associated with antiphospholipid antibodies including stroke, seizures, dementia, migraine, ocular ischemia, chorea, transverse myelopathy, cerebral phlebitis. Other association are more controversal like Guillain Barré syndrome, motor neuron disease, communicating hydrocephalus. In all patients with antiphospholipid antibodies with neurological involvement, cerebral MRI may be performed with an echocardiographic study because a possible association with Libman and Sacks endocarditis, valve dysfunction or cardiac thrombus source of cerebral ischemia.

Antibodies, Antiphospholipid↗
PubMed1995

[Familial sarcoidosis: 3 cases in the same family].

Familial sarcoidosis is uncommon, representing less than 5% of the cases. We reported three cases of sarcoidosis in the same family (two sisters and a brother) with different clinical presentation in spite of the presence in each patient of HLA B8 DR3. These HLA phenotype may be related with favourable sarcoidosis. Until the two sisters had articular and benign lymph nodes diseases, the brother had multivisceral sarcoidosis involving the liver, the lung and the mediastinum. Corticosteroid were used during 6 month with favourable evolution. There is 16 years between the three sarcoidosis history but the disease appeared till the third decade. The two sisters and the brother did not live in the same city. These suggest than genetic predisposition is more effective than environmental basis.

Adult↗
PubMed1995

[Digital arteritis, thrombosis and hypereosinophilic syndrome: an uncommon complication].

Digital necrosis has been exceptionally described in association with the hypereosinophilic syndrome. We report a case of a smoking patient with idiopathic hypereosinophilic syndrome who developed digital arteritis with necrosis. A radial artery biopsy confirmed the vasculitis with inflammatory infiltrates which were predominantly eosinophilic granulocytes. The eosinophils may have an active role in the development of the vasculitis.

Adult↗
PubMed1995

Tuberculosis associated haemophagocytic syndrome: two cases with a favourable outcome.

Haemophagocytic syndrome is a heterogenous disease characterized by disordered macrophage activation associated with viral, bacterial or parasitic infection. The few reports of haemophagocytosis occurring in the presence of mycobacterial infection show a high mortality rate and we present two further cases notable for their favourable issue. Rapidity of diagnosis and immediate treatment could explain the avoidance of a fatal outcome.

Aged↗
PubMed1995

Participation of cryoglobulinaemia in the severe peripheral neuropathies of primary Sjögren's syndrome.

A peripheral neuropathy is encountered in 10 to 50% of patients with primary Sjögren's syndrome (pSS). The pathogenesis of this complication remains unknown, but severe cases of peripheral neuropathies seem to result from a vasculitis. Since about 20% of pSS are associated with a cryoglobulinaemia, we initiated a retrospective study to assess the participation of cryoglobulinaemia in the severe peripheral neuropathies (SPN) of pSS. We found 6 cases of SPN (presence of pain and/or muscle weakness) in a group of 115 pSS (5%): 3 polyneuropathies and 3 mononeuritis multiplex. In all cases, the involvement was sensoromotor and axonal. Four patients had a biopsy-documented cutaneous vasculitis, and 2 of them had also a muscular lymphocytic vasculitis. A cryoglobulinaemia was detected in sera of 5 patients. Although the research was negative in the remaining patient, the presence of an IgM monoclonal gammapathy and of an hypo-complementaemia suggested also the presence of a cryoglobulinaemia. Complement was activated in the 6 cases. Treatment was aggressive: oral corticosteroid (6 cases), methylprednisolone pulses (4 cases), chloraminophen (1 case), plasma exchanges (2 cases), intravenous immunoglobulins (1 case). A stabilization or a partial regression of the neuropathies were observed in 1 case and 5 cases, respectively. This was associated with a reduction of cryoglobulin level and complement activation in 5 cases and 6 cases, respectively. This study suggests that SPN of pSS result from a cryoglobulin-mediated vasculitis.

Adult↗
PubMed1995

Association of myelodysplastic syndrome and relapsing polychondritis: further evidence.

We report five patients with both a myelodysplastic syndrome (MDS) and relapsing polychondritis (RP), that represented 0.6% of all MDS and 28% of all RP diagnosed over a period of 14 years. Ten other cases had previously been reported (four in detail), supporting a non-fortuitous association between the two disorders, already suggested for MDS and some other immunological disorders.

Adult↗
PubMed1995

[Course of ischemic risk in treated atheromatous hypertensive patients. The PRIHAM study].

Hypertension is one of the major cardiovascular risk factors. However 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. Amongst diabetic and dyslipidemic patients, an absence of deleterious metabolic effects was noticed, parallel with the effectiveness. 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↗
PubMed1994

[Behçet disease. Comparative aspect of diagnostic criteria].

OBJECTIVES: Different diagnostic criteria for Behçet's disease are proposed in the literature. We compared the criteria proposed by Mason and Barnes with those proposed by the International Study Group and with those proposed by the Japanese committee. METHODS: This retrospective study included 41 patients with Behçet's disease according to the criteria set by Mason and Barnes. There were 16 patients of French origin and 25 patients of North African origin. RESULTS: Genital ulcerations were more frequent in the North African population (p < 0.007) and typical Behçet skin lesions were more frequent in the French population (p < 0.021). Comparing the clinical data among the 41 patients with Behçet's disease according to the Mason-Barnes criteria revealed that only 83% met the criteria set by the International Study Group and only 54% the criteria of the Japanese committee. For the International Study Group criteria, the requirement for recurrent buccal ulcerations with at least 3 episodes over a 12 month period (a major criteria) was the most limiting element. Likewise, genital ulcerations were not always recurrent. CONCLUSION: These findings confirm the variability of clinical manifestations of Behçet's disease with ethnic origin. Proposed diagnostic criteria vary depending on the study committee and should be used as diagnostic guidelines rather than as criteria required for positive diagnosis.

Adult↗
PubMed1994

Percutaneous vertebroplasty with acrylic cement in the treatment of a Langerhans cell vertebral histiocytosis.

A 25-year-old man developed multiple eosinophilic granuloma of bone including vertebral and sacral localization. Radiotherapy was initially administered. One year later, a relapse occurred in another vertebrae which was previously irradiated. Percutaneous vertebroplasty was, for the first time to our knowledge performed, in this indication, with a good clinical result with follow-up now for one year. We emphasize that such treatment is permissible only in symptomatic, progressive lesions, with threatened decompensation of spinal stability. This technique should be used only on an adult.

Adult↗