Search PubMed⌕ Search

Biomedical subjects

E Hachulla

Publications and source records attributed to E Hachulla.

At least 73 records · Page 4Linked to original sources
PubMed2001

[DRESS (Drug Rash with Eosinophilia and Systemic Symptoms) syndrome after sulfasalazine and carmazepine: report of two cases].

INTRODUCTION: To better individualize drug hypersensitivity reaction, Bocquet et al. have recently called this adverse drug reaction DRESS (Drug Rash with Eosinophilia and Systemic Symptoms). EXEGESIS: We report two cases of DRESS and highlight the main characteristics of this syndrome. Two patients presented severe febrile skin eruption following drug intake (carbamazepine or sulfazalazine), with hypereosinophilia and hepatitis. All symptoms resolved after drug withdrawal and corticosteroid therapy. DRESS syndrome is an idiosyncratic reaction characterised by febrile eruption, occurring 2 to 6 weeks after the beginning of the treatment, accompanied by systemic symptoms and biological abnormalities (hypereosinophilia, hepatitis). Some complications can occur. This syndrome can be fatal. Numerous drugs can be responsible for this reaction to medication. The physiopathology has not yet been elucidated, and the treatment is not codified, but the triggering agent must immediately be stopped. Corticotherapy is sometimes used. CONCLUSION: It is important to recognize this entity recently named DRESS syndrome because it can mimic other pathologies, is potentially serious, and because withdrawal of the incriminating drug is imperative.

Adolescent↗
PubMed2001

[Pulmonary hypertension screening in systemic scleroderma: a cohort study of 67 patients].

PURPOSE: Pulmonary hypertension is a severe complication of systemic sclerosis and has emerged as a major cause of morbidity and mortality in this condition. Treatment is all the more efficient as pulmonary hypertension is early diagnosed. A good knowledge of the clinical, biological and functional features of pulmonary hypertension in systemic sclerosis is therefore necessary to suspect and to diagnose pulmonary hypertension as early as possible. METHODS: Sixty seven patients with systemic sclerosis were retrospectively studied. We compared clinical, immunological, functional (spirometry) and morphological (pulmonary fibrosis) features according to the presence (n = 25) and the characteristic of pulmonary hypertension (isolated or secondary) or the absence (n = 42) of pulmonary hypertension, assessed by Doppler echocardiography. RESULTS: CREST syndrome (calcinosis, Raynaud's phenomenon, oesophageal involvement, sclerodactyly and telangiectasia) was more frequent in patients with isolated pulmonary hypertension than in patients without PH (72.7% vs 28.5%, P < 0.05; odds-ratio [OR] = 6.6) and dyspnea was more severe (P < 0.001; OR = 11.4). The age at time of pulmonary hypertension diagnosis was higher in patients with secondary pulmonary hypertension than in patients with isolated from (median: 62.5 years (range: 32-35) vs 53 years (range: 37-85), P < 0.05). Patients with isolated pulmonary hypertension had anticardiolipin antibodies more frequently than patients without pulmonary hypertension (72.7% vs 35.7%, P < 0.05). Isolated reduction of diffusing capacity was preferentially observed among patients with isolated pulmonary hypertension than among those without pulmonary hypertension. A linear relation between systolic pulmonary artery pressure values and diffusing capacity values (r = 0.72, P < 0.01) was found. Isolated reduction of diffusing capacity was more frequent in patients with isolated pulmonary hypertension than in patients without pulmonary hypertension (63.6% vs 14.3%, P < 0.001; OR = 10.5). CONCLUSION: The severity of pulmonary hypertension in systemic sclerosis justifies a systematic screening by Doppler echocardiography and diffusing capacity measurement. Our results allow us to better define the characteristics of sclerodermic patients with isolated or secondary pulmonary hypertension. The search for pulmonary hypertension should be repeated with time and clinicians should be particularly vigilant in the case of a patient presenting these characteristics.

Adult↗
PubMed2001

[Interstitial lung diseases in polymyositis and dermatomyositis].

PURPOSE: Interstitial lung disease is one of the most common respiratory manifestations in polymyositis and dermatomyositis. It still remains a severe complication of the disease, leading to death related to ventilatory insufficiency in 30-66% of patients. CURRENT KNOWLEDGE AND KEY POINTS: Time onset of interstitial lung disease is variable, although interstitial lung disease onset precedes initial manifestations of polymyositis/dermatomyositis in roughly half of the patients. Moreover, clinical presentation of interstitial lung disease can be dichotomized, according to patients' pulmonary manifestations, into: 1) both acute and aggressive lung disease similar to Hamman-Rich syndrome; 2) slowly progressive lung disease; and 3) an asymptomatic pattern. The methods of choice adopted for early diagnosis of interstitial lung disease are high-resolution computed tomography scan and pulmonary function tests, which should be performed during both initial evaluation of polymyositis/dermatomyositis and follow-up. Because anti-JO1 antibody is considered to be a marker of interstitial lung disease in polymyositis/dermatomyositis, close pulmonary follow-up of anti-JO1-positive patients with polymyositis is therefore required for early detection of subclinical impairment. Furthermore, histological lung findings provide prognostic data; patients with bronchiolitis obliterans organizing pneumonia (BOOP) indeed appear to have a more favorable outcome than those with usual interstitial pneumonia or diffuse alveolar damage. Finally, as a guide to both the severity and progress of interstitial lung disease, the significance of other investigations, notably bronchoalveolar lavage, remains controversial. FUTURE PROSPECTS AND PROJECTS: Specific therapy of interstitial lung disease has not yet been clearly established in polymyositis/dermatomyositis patients. Corticosteroid therapy is considered the first line of therapy for polymyositis/dermatomyositis patients with interstitial lung disease. The association of cyclophosphamide and corticosteroids may be the most effective in patients with steroid-resistant interstitial lung disease. Early diagnosis and management of this disease is therefore of the utmost importance.

Adrenal Cortex Hormones↗
PubMed2001

[Detection of anti-DNA antibodies for the diagnosis of disseminated lupus erythematosus. Comparative study of immunoenzyme methods and a Farr test].

Among the biological assays used for the diagnosis of systemic lupus erythematosus, the detection of anti-double strand DNA (dsDNA) antibodies is highly specific, especially at a high level. Different methods are using for the detection of this specificity, particularly enzyme-based immunoassay (EIA) kits that become widespread in autoimmunity because of several advantages like standardization, simple use and automation. We selected 80 sera in order to evaluate six commercial anti-dsDNA EIA kits and a Farr assay. The results were compared with clinical data, to appreciate both the sensitivity and the specificity of each method. EIA assays are highly sensitive, but are less specific than the Farr assay. These EIA methods should remain organized in an integrative biological step, following an evocative clinical examination. Moreover, the EIA results require confirmations with a more specific assay.

Antibodies, Antinuclear↗
PubMed2001

Diagnosing thoracic outlet syndrome: contribution of provocative tests, ultrasonography, electrophysiology, and helical computed tomography in 48 patients.

OBJECTIVE: To evaluate the diagnostic usefulness of provocative tests, Doppler ultrasonography, electrophysiological investigations, and helical computed tomography (CT) angiography in thoracic outlet syndrome (TOS). PATIENTS AND METHODS: We prospectively evaluated 48 patients with a clinical suspicion of thoracic outlet syndrome. Standardized provocative tests, an electromyogram and somatosensory evoked responses, a Doppler ultrasonogram, and a helical CT arterial and/or venous angiogram with dynamic maneuvers were done on each patient. The final diagnosis was established by excluding all other causes based on all available data. The agreement between the results of each investigation and the final diagnosis was evaluated. RESULTS: Provocative tests had mean sensitivity and specificity values of 72% and 53%, respectively, with better values for the Adson test (positive predictive value [PPV], 85%), the hyperabduction test (PPV, 92%), and the Wright test. Using several tests in combination improved specificity. Doppler ultrasonography visualized vascular parietal abnormalities and confirmed the diagnosis in patients with at least five positive provocative tests. Electrophysiological studies were useful mainly for the differential diagnosis or for detecting concomitant abnormalities. Although helical CT angiography provided accurate information on the location and mechanism of vascular compression, the usefulness of this investigation for establishing the diagnosis of TOS and for obtaining pretherapeutic information remains unclear.

Adult↗
PubMed2001

Scleromyxedema (lichen myxedematosus) associated with dermatomyositis.

A 41-year-old white man is described with papules of the lower and upper back, the neck and the upper chest, a marked deposition of mucin in the upper reticular dermis, and an IgG lambda monoclonal gammopathy strongly evocative of scleromyxedema (lichen myxedematosus). Additionally, he developed intense myalgia, muscle weakness and rhabdomyolysis, which were associated with heliotrope erythema, photosensitivity and an erythematous rash of the dorsum of the hands with Gottron's papules. Muscle biopsy revealed an inflammatory myositis, and dermatomyositis was diagnosed. The association of dermatomyositis and secondary mucinosis, or muscle involvement in primary papular mucinosis are not rare. However, the association between scleromyxedema and dermatomyositis has only exceptionally been reported.

Adult↗
PubMed2001

The chondroitin sulfate chain of bikunin-containing proteins in the inter-alpha-inhibitor family increases in size in inflammatory diseases.

Inter-alpha-inhibitor (IalphaI) and pre-alpha-inhibitor (PalphaI) are the main members of a set of multichain serine proteinase inhibitors. Present in human plasma, they may be involved in control of the inflammatory process. They are composed of homologous heavy chains (H1 and H2 for IalphaI; H3 for PalphaI) covalently linked by a protein-glycosaminoglycan-protein cross-link to bikunin, which is a chondroitin 4-sulfate proteoglycan. During the acute-phase response, biosynthesis of IalphaI and PalphaI is downregulated and upregulated, respectively. In this work, we provide evidence that, in inflammatory diseases, the chondroitin sulfate chain of bikunin increases in size proportionally to the severity of the inflammatory response. As a consequence, all IalphaI-related components that contain bikunin are structurally modified. Therefore, the changes in glycosylation of the acute-phase proteins are not restricted to N-linked glycans but also affect glycosaminoglycans. The implications of these findings are discussed with regard to biosynthesis and biological role, especially the anti-inflammatory effects of IalphaI-related proteinase inhibitors.

Acute-Phase Proteins↗
PubMed2001

Acute myelopathies: Clinical, laboratory and outcome profiles in 79 cases.

The main aetiologies of acute myelopathy (AM) are: multiple sclerosis, systemic disease (SD), spinal cord infarct (SCI), parainfectious myelopathy (PIM) and delayed radiation myelopathy (DRM). Although a large amount of data have been published for each individual aetiology, comparison studies are scarce. The aim of this study was to assess the various aetiological and outcome profiles of AM. We studied 79 cases: 34 (43%) in multiple sclerosis; 13 (16.5%) in SD; 11 (14%) in SCI; five (6%) in PIM; and three (4%) in DRM. Myelopathies were of unknown origin in 13 (16.5%) patients. We evaluated clinical, spinal cord and brain MRI, CSF and evoked potentials data at admission, MRI outcome at 6 months and clinical outcome at 12 months. A statistical comparison of clinical, laboratory and outcome data was only performed between multiple sclerosis, SD and SCI patients due to the small number of cases in the other groups. A motor deficit was more frequent in SD and SCI than in multiple sclerosis where initial symptoms were predominantly sensory (P < 0.001). Spinal cord MRI showed lateral or posterior lesions of less than two vertebral levels in multiple sclerosis, in contrast to SD and SCI, where lesions involved more vertebral levels and were centromedullar (P < 0.001). Brain MRI was most frequently abnormal in multiple sclerosis (68%), but was also abnormal in 31% of SD patients (P < 0.05). Oligoclonal bands in CSF were more frequent in multiple sclerosis than in SD (P < 0.001) and were never found in SCI. Clinical outcome at 12 months was good in 88% of multiple sclerosis cases, and poor or fair in 91% of SCI and 77% of SD. Aetiologies of AM may be differentiated on the basis of clinical, spinal cord and brain MRI, CSF and outcome data, and allow a probable diagnosis to be made in previously undetermined cases. These findings may have therapeutic implications for cases with a questionable diagnosis.

Acute Disease↗
PubMed2001

Cannabis arteritis revisited--ten new case reports.

The purpose of this paper was to revisit the old concept of cannabis arteritis first described in the 1960s and report 10 new cases. Ten male patients, with a median age of 23.7 years developed subacute distal ischemia of lower or upper limbs, leading to necrosis in the toes and/or fingers and sometimes to distal limb gangrene. Two of the patients also presented with venous thrombosis and three patients were suffering from a recent Raynaud's phenomenon. Biological test results did not show evidence of the classical vascular risk factors for thrombosis. Arteriographic evaluation in all cases revealed distal abnormalities in the arteries of feet, legs, forearms, and hands resembling those of Buerger's disease. A collateral circulation sometimes with opacification of the vasa nervorum was noted. In some cases, arterial proximal atherosclerotic lesions and venous thrombosis were observed. All patients were moderate tobacco smokers and regular cannabis users. Despite treatment with ilomedine and heparin in all cases, five amputations were necessary in four patients. The vasoconstrictor effect of cannabis on the vascular system has been known for a long time. It has been shown that delta-8- and delta-9-tetrahydrocanabinols may induce peripheral vasoconstrictor activity. Cannabis arteritis resembles Buerger's disease, but patients were moderate tobacco smokers and regular cannabis users. These cases show that prolonged use of cannabis could be an additive risk factor for juvenile and young adult arteritis. Cannabis arteritis is a forgotten and severe occlusive vascular disease occurring in young adults. Search for cannabis use may be an important tool for a better knowledge of arteritis in young smokers.

Adolescent↗
PubMed2001

Oxidation of beta2-glycoprotein I (beta2GPI) by the hydroxyl radical alters phospholipid binding and modulates recognition by anti-beta2GPI autoantibodies.

We investigated whether beta2-glycoprotein I (beta2GPI), the key antigen in the antiphospholipid syndrome, is susceptible to oxidative modifications by the hydroxyl radical (*OH) that may influence its lipid-binding and antigenic properties. The effects on human and bovine beta2GPI of *OH free radicals generated by gamma-radiolysis of water with 137Cs were studied. Radiolytic *OH caused a dose-dependent loss of tryptophan, production of dityrosine and carbonyl groups. dimerization and/or extensive aggregation of beta2GPI. It ensued a reduction in affinity binding to cardiolipin liposomes and loss of beta2GPI-dependent autoantibody binding to immobilized cardiolipin. Patient anti-beta2GPI antibodies (n = 20) segregated into two groups based on the effect in the beta2GPI-ELISA of beta2GPI pretreatment with *OH: enhancement (group A, n = 10) or suppression (group B, n = 10) of IgG binding. The avidities of group A antibodies for fluid-phase beta2GPI were low but increased in a dose-dependent manner upon beta2GPI irradiation, in relation to protein crosslinking. Distinguishing features of group B antibodies included higher avidities for fluid-phase beta2GPI that was no longer recognized after *OH treatment, and negative anticardiolipin tests suggesting epitope location near the phospholipid binding site. The *OH scavengers thiourea and mannitol efficiently protected against all above changes. Therefore, oxidative modifications of beta2GPI via *OH attack of susceptible amino acids alter phospholipid binding, and modulate recognition by autoantibodies depending on their epitope specificities. These findings may be of clinical relevance for the generation and/or reactivity of anti-beta2GPI antibodies.

Animals↗
PubMed2001

Relationship between parity and clinical and biological features in patients with systemic sclerosis.

OBJECTIVE: To assess the influence of parity on the clinical and biological features of systemic sclerosis (SSc). METHODS: We recorded the following clinical and biological data of 100 consecutive women with SSc: age, disease duration before diagnosis, cutaneous extension of sclerosis according to LeRoy's classification, pulmonary involvement, and antinuclear antibodies. We compared these features to the number and sex of children who were born before SSc onset. Date of birth of the first children was systematically recorded. RESULTS: Patients with limited SSc had more children before SSc onset than patients with diffuse SSc (2.4 +/- 1.8 vs 1.7 +/- 1.5; p < 0.05). The interval between first birth and SSc onset was shorter for patients with limited SSc than for patients with diffuse SSc (11.0 +/- 9.9 vs 23.5 +/- 14.5 yrs; p < 0.01). Patients with pulmonary fibrosis had more children than patients without pulmonary fibrosis (2.5 +/- 1.9 vs 2.0 +/- 1.6; p < 0.05). Age at first birth was significantly higher when the child was a girl than a boy (26.8 +/- 7.5 vs 22.9 +/- 5.3 yrs; p < 0.05). The interval between the first birth and SSc onset was shorter when the child was a girl than a boy (16.2 +/- 9.6 vs 25.4 +/- 13.4 yrs; p < 0.05). CONCLUSION: Pregnancy related microchimerism could be preferentially associated with limited SSc and pulmonary fibrosis. Microchimerism may be facilitated in cases in which the fetus is female.

Adult↗
PubMed2001

[Myelopathy - Sjogren's syndrome association: analysis of clinical and radiological findings and clinical course].

Myelopathies associated with Sjögren's syndrome has been rarely described especially concerning magnetic resonance imaging (MRI) and treatment aspects. The aim of this study was to determine the clinical, laboratory and radiological features of myelopathies occurring in Sjögren's syndrome. Eleven patients were studied, 7 with an acute myelopathy and 4 with a chronic form. Acute myelopathy were clinically severe with a feature of transverse myelitis necessitating immunosuppressive drugs. On the other hand, chronic forms were closely similar to progressive multiple sclerosis (MS), for clinical and laboratory data. In 7 cases optic neuritis was found associated with myelopathy and fulfilled the diagnostic criteria of Devic's syndrome in 4 cases. The diagnosis of myelopathy associated with Sjögren's syndrome may be difficult especially compared with MS, HTLV1 or HIV myelopathy and sarcoidosis, in the chronic form but also with other vasculitis, MS or viral infection in the acute forms. However, in this last form, magnetic resonance imaging and cerebrospinal fluid data should bring to the diagnosis of Sjögren syndrome and confirmed by appropriate tests. This diagnosis will have direct consequences for an early treatment by immunosuppressive drugs.

Adult↗