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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
PubMed1997

POEMS syndrome: report on six patients with unusual clinical signs, elevated levels of cytokines, macrophage involvement and chromosomal aberrations of bone marrow plasma cells.

POEMS syndrome is a multisystemic disorder characterized by the association of polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes and various other systemic clinical signs. The pathophysiology of this syndrome remains largely unknown. In order to gain insight into its pathophysiology, we studied the clinical characteristics and performed serum analysis (auto-antibodies, cytokine levels) and phenotypic and cytogenetic studies of bone marrow plasma cells (BMPC) in six patients with unequivocal POEMS syndrome. Two unusual clinical signs were present in these patients: pulmonary hypertension (two patients) and diffuse cutaneous necrosis (one patient). No auto-antibodies against peripheral nerve (PN) antigens (SGPG and SGLPG glycolipids, GM1, GD1a, GD1b and GT1b gangliosides) were found. Sequential evaluations of serum cytokines (IL-1-beta, IL-6 and TNF-alpha) showed a moderate to marked elevations of IL-6 and TNF-alpha in all patients (up to six-fold for TNF-alpha and 16-fold for IL-6). Using in situ hybridization of these cytokines mRNAs on lymph node specimens of two patients who had an angiofollicular lymph node hyperplasia, a strong positivity was found with the IL-1-beta antisense probe in lymph node macrophages. On skin biopsy a high number of cells expressing TNF-alpha mRNA was observed in the dermis. The biological features of BMPC: phenotype (expression of CD19 and CD56 antigens), kinetics (Ki-67 index), karyotype, DNA content and chromosomal in situ hybridization remained those of BMPC found in monoclonal gammopathy of undetermined significance. We conclude that POEMS syndrome is a hypercytokinemic syndrome in which BMPC are not of malignant type. Macrophages are involved in this syndrome and their role has to be further investigated as well as treatments which act through an anti-cytokine mechanism.

Adult↗
PubMed1997

'Localized amyloidosis': 123I-labelled SAP component scintigraphy and labial salivary gland biopsy.

In apparently localized amyloidosis, there is no appropriate test to determine whether systemic deposits exist. We studied the value of serum amyloid P component (SAP) scintigraphy and labial salivary gland (LSG) biopsy on patients with apparently localized amyloidosis in 12 patients who had neither clinical nor biological evidence of systemic amyloidosis. All patients had an LSG biopsy and echocardiography. Iodine-123-labelled serum amyloid P component (123I-SAP) scintigraphy was performed in all patients. Whole-body scintigraphy was done, and tissue retention was evaluated at 24 h and 48 h. Of these 12 patients, three had amyloidosis in their LSG and had abnormal 123I-SAP scintigraphy; these three had a secondary clinical history of systemic amyloidosis. Three other patients had abnormal 123I-SAP scintigraphy without detectable systemic amyloid deposits, but one had a previous history of bilateral carpal tunnel syndrome treated with infiltration. 123I-SAP scintigraphy in association with LSG biopsy may be helpful in determining the localized or systemic character of amyloid disease.

Adult↗
PubMed1997

Assessment of anti-endothelial cell antibodies in systemic sclerosis and Sjögren's syndrome.

OBJECTIVES: Anti-endothelial cell antibodies (AECA) have been detected in 19 to 30% of patients with systemic sclerosis (SSc). The objective of this study was first to assess the role of a secondary Sjögren's syndrome (SS) in the occurrence of AECA in SSc. Secondly, we researched AECA in patients with primary SS, and investigated whether AECA were associated with vascular manifestations (Raynaud's phenomenon and vasculitis). METHODS: IgG-AECA were tested by an ELISA method in serum samples from 50 patients with SSc (16 of them had also a secondary SS), 50 patients with primary SS, and 50 healthy controls. RESULTS: AECA levels were significantly higher in patients with SSc or primary SS than in healthy controls (p < 0.01 and p < 0.01, respectively). In patients with SSc, AECA values were significantly higher in patients with secondary SS (p < 10(-5)). In patients with primary SS, AECA levels were significantly higher in patients with Raynaud's phenomenon (p < 0.01), but not in patients with vasculitis. CONCLUSION: In patients with SSc, AECA are associated with a secondary SS. In patients with primary SS, AECA are associated with Raynaud's phenomenon, but not with vasculitis.

Adolescent↗
PubMed1997

Elastase derived elastin peptides: putative autoimmune targets in giant cell arteritis.

OBJECTIVE: Histological analysis of giant cell arteritis (GCA) reveals a granulomatous reaction around the internal elastic lamina. Elastolysis by multinucleated giant cells has also been reported. We investigated elastin derived peptides as putative recall antigens for peripheral blood mononuclear cells (PBMC) from patients with GCA. METHODS: PBMC were collected from 17 patients with GCA (Group 1), 17 patients with vascular diseases, connective tissue diseases, or polymyalgia rheumatica without GCA (Group 2), and 17 healthy controls (Group 3). Cultures of PBMC with different elastin derived peptides or elastase were analyzed. RESULTS: A proliferative response was obtained only with elastate derived elastin peptides in 12/13 untreated patients with GCA. Steroid treatment was believed to abolish this proliferative response in 4 patients with GCA. PBMC from only 3/34 non-GCA subjects responded to these antigens. No proliferative response was obtained for other elastin derived peptides or elastase in any subject. CONCLUSION: Degradation of native elastin by leukocyte elastase can provide elastin derived peptides that act as autoimmune targets for T cells in GCA.

Adrenal Cortex Hormones↗
PubMed1996

[Glycoforms of alpha-1 antichymotrypsin in infectious process. Diagnostic value and follow-up].

OBJECTIVES: Three alpha 1-antichymotrypsin (alpha 1-ACT) glycans have been identified. We followed their levels during the septic processes in 24 patients in order to determine their diagnostic value in correlation with C-reactive peptide in patients with infection. METHODS: Sera were collected for assay on days 0, 3 and 6 after starting antibiotics. Erythrocyte sedimentation rate, alpha 1-ACT and C-reactive protein were determined. RESULTS: Erythrocyte sedimentation rate changed little while C-reactive protein fell sharply as in other inflammatory processes. Crossed immunoaffinoelectrophoresis showed a decreased formation of the Con-A non reactive fraction which disappeared rapidly after initiating antibiotics. Glycan microheterogeneity returned to normal at 6 days while C-reactive protein and alpha 1-ACT were still elevated. CONCLUSION: Synthesis and glycosylation of alpha 1-ACT are independent. Study of alpha 1-ACT glycan microheterogeneity may provide a useful test in the diagnosis of inflammatory processes of unknown origin and may be helpful in following-up patients with sepsis.

Adult↗
PubMed1996

Early expression of E-selectin, tumor necrosis factor alpha, and mast cell infiltration in the salivary glands of patients with systemic sclerosis.

OBJECTIVE: To assess the predictive value of early endothelial E-selectin and tumor necrosis factor alpha (TNF alpha) expression, as well as mast cell infiltration, in the subsequent progression to systemic sclerosis (SSc) in patients with Raynaud's phenomenon (RP) and abnormal nailfold capillaroscopic findings. METHODS: Clinical criteria were evaluated, and immunostaining was performed on lip biopsy samples from 22 patients with RP and abnormal capillaroscopic results. None of these patients initially fulfilled the American College of Rheumatology criteria for SSc. RESULTS: E-selectin, TNF alpha, and mast cell infiltration were found in 9, 10, and 8 of 11 patients, respectively, whose disease progressed to SSc, and in 0, 2, and 1 of 11 patients, respectively, whose disease did not progress to SSc (P < 0.001, P < 0.01, and P < 0.01, respectively). CONCLUSION: E-selectin, TNF alpha, and mast cell infiltration are detectable in the very early stages of SSc, prior to the onset of skin changes.

Adult↗
PubMed1996

[Initial manifestations and late diagnosis in chronic atrophic polychondritis: apropos of a series of 15 patients].

An auricular or nasal chondritis or a saddle nose deformity are the initial manifestation in half of cases of relapsing polychondritis; the other initial manifestations are various and less evocative; polyarthritis, laryngo-tracheal symptoms, episcleritis which delay the diagnosis. From 15 cases of relapsing polychondritis, the diagnosis time from the first symptom are studied; this one is long, about 3 years and 6 months (from 3 months to 17 years) in 13/15 of the cases even if the first manifestation is typical (external chondritis). In two cases only, the diagnosis was established after the first attack. This late of diagnosis had socioeconomical impact and exposed to severe complications like tracheal chondritis.

Adult↗
PubMed1996

[Sicca syndrome and hepatitis C virus infection: a Gougerot-Sjögren pseudo-syndrome?].

These last years, different virus have been incriminated in the etiopathogeny of the primary-Sjögren's syndrome and more particularly the hepatitis C virus. We have led a prospective study over 23 patients presenting a primary Sjögren's syndrome, searching for a sign of infection by the hepatitis C virus and over 23 patients presenting an active chronic hepatitis C virus searching for a Gougerot-Sjögren syndrome. The overcoming of the hepatitis C virus in the Sjögren group was 4.7% which was not significatively higher than in our sample of population. Parallelly, the search of the Sjögren's syndrome, in the hepatitis C virus group, found 4 patients (feminine, average age 48.5 years old) whose clinical board was compatible with this diagnostic. Antinuclear antibodies have not been found in any of the 23 patients. The lymphocytic typing of the infiltrate in the minor salivary glands biopsies showed a predominance of the CD8 lymphocytes in a proportion of 2/1, contrasting with what is observed in primary Sjögren's syndrome. We concluded that hepatitis C virus may be more associated with a chronic lymphocytic sialadenitis than an authentic primary Sjögren's syndrome.

Adult↗
PubMed1996

[Role of biopsy of the accessory salivary glands in Löfgren's syndrome and other forms of sarcoidosis].

The diagnosis of sarcoidosis requires histopathological analysis. Easy accessible site of biopsy is seldom. A systematic labial salivary gland biopsy was performed in 62 suspected sarcoidosis: 22 patients with Löfgren syndrome (group I), and 40 patients with systemic sarcoidosis (group II). Systematic bronchial biopsy was performed in eight patients of group I and ten patients of group II. If systematic biopsies were negative, direct biopsies were performed (lymph node, skin, kidney, liver). In group I, 8/22 labial salivary gland biopsies and 1/8 bronchial biopsies were positive; in group II, 17/40 labial salivary gland biopsies and 5/10 bronchial biopsies were positive. In the other patients, direct biopsies were positive: 27 lymph nodes, eight skin, eight hepatic, four kidney biopsies. In conclusion, labial salivary gland biopsy (even in the absence of sicca syndrome) is more reliable than systematic bronchial biopsies, particularly in Löfgren syndrome and may avoid in 30 to 50% of the cases more aggressive and dangerous biopsies such as liver, kidney or deep lymph nodes biopsies.

Adult↗
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↗