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E Hachulla

Publications and source records attributed to E Hachulla.

232 records · Page 13Linked to original sources
PubMed1988

Alpha 1-antichymotrypsin microheterogeneity in crossed immunoaffinoelectrophoresis with free concanavalin A: a useful diagnostic tool in inflammatory syndrome.

Using crossed immunoaffinoelectrophoresis with free concanavalin A (Con A) in the first dimension and alpha-methyl-D-glucoside incorporated in the second-dimension gel, we examined the microheterogeneity of alpha 1-antichymotrypsin (alpha 1Achy) in sera from healthy donors (N) and in sera from patients with various inflammatory syndromes. We studied three groups: patients with acute inflammation (myocardial infarction: MI, septic inflammation: SI), patients with chronic inflammation (metastatic breast cancer: MBC), and patients with chronic inflammation accompanied by acute attacks (connective-tissue disease: CTD). All pathological sera had a high alpha 1Achy concentration. Compared with N, MI and SI showed an increased proportion of Con A-reactive fraction and a decreased proportion of Con A-nonreactive fraction, which was more pronounced in SI. Unlike the patients with acute inflammation, patients with CTD showed an increased proportion of Con A-nonreactive fraction. Thus alpha 1Achy microheterogeneity in crossed immunoaffinoelectrophoresis may afford a means of differentiating between various inflammatory syndromes. In particular, it can provide a simple test: if the Con A-nonreactive fraction is in a proportion less than 17%, septic origin of an acute-phase reaction may be suspected.

Adult↗
PubMedDate not supplied

[Clinical, flowmetric and radiologic study of the thoracic outlet in 95 healthy subjects: physiologic limitations and practical impact].

Ninety-five healthy subjects have been examined regarding the presence of symptomatic compression of the brachial plexus and subclavian vessels (Thoracic Outlet Syndrome: TOS). Each subject was examined clinically and by Doppler flowmetry during performance of Adson, hyperabduction and abduction-external-rotation manoeuvres. In all subjects Roos test and X-ray examinations of the cervical spine and thoracic aperture were performed. The response was considered positive when the radial pulse disappeared for the clinical test, and when the flow was totally arrested for the Doppler flowmetry. Adson's manoeuvre showed a 1% clinical positive response and a 0% doppler positive response. Hyperabduction at 45 degrees showed a 0% positive response both clinically and by Doppler flowmetry; at 90 degrees, it showed positive response of 6% and 1% respectively, and at 180 degrees it showed positive responses of 40% and 11%. Abduction-external-rotation manoeuvres showed 14% clinical and 7% Doppler positive responses. The Roos test was positive for 8% of the subjects and X-ray was abnormal for 13% of the subjects. We conclude that: Doppler flowmetry is useful for the TOS diagnosis only when the clinical evaluation is abnormal. Total arrest of flow is sometimes temporary; arterial flow must be examined at least 20 seconds. Total arrest of flow is never seen during Adson manoeuvre or hyperabduction at 45 degrees or 90 degrees in healthy subjects. Clinical or Doppler perturbation is not significantly higher for healthy subjects presenting an X-ray abnormality.

Adult↗
PubMedDate not supplied

[Value of biopsy of accessory salivary glands for the diagnosis of amyloidosis].

There are few reports of amyloidosis diagnosed by deliberate biopsy of accessory salivary glands. Usually, a biopsy performed for dry mouth syndrome reveals an unsuspected amyloidosis. We report the case of 2 patients with lambda-type light chain monoclonal gammapathy complicated by generalized amyloidosis and in whom biopsy of the accessory salivary glands showed signs of amyloidosis. In the first patient accessory salivary gland biopsy was performed because these glands were enlarged, and the monoclonal dysglobulinaemia was subsequently diagnosed by serum immunoelectrophoresis. In the second patient with nephrotic syndrome, renal biopsy could not be carried out owing to the presence of a renal malformation; amyloidosis was confirmed by periumbilical fat aspiration, and a systematic biopsy of accessory salivary glands also showed evidence of amyloidosis. Biopsy of accessory salivary glands seems to be a particularly simple and safe method to detect generalized amyloidosis in patients with chronic inflammatory disease or monoclonal dysglobulinaemia.

Aged↗
PubMedDate not supplied

[Double heart valve replacement disclosing antiphospholipid syndrome].

In patients with systemic lupus erythematosus (SLE) heart valve lesions are usually discovered at echocardiography; their haemodynamic repercussions are uncommon, and valve replacement is exceptional. We report the case of a woman who had undergone aortic and mitral valve replacement before antiphospholipid antibodies were found associated with 4 ARA criteria of SLE. Histopathological examination confirmed the diagnosis of Libman-Sachs specific endocarditis. The presence of antiphospholipid antibodies leads to a discussion of their role in the physiopathology of the heart valve lesions and vascular accidents that occurred in this patient. The overlap observed between the diagnostic criteria of SLE and those of primary antiphospholipid syndrome is discussed. Heart valve lesions may be one of the modes of access to the antiphospholipid syndrome.

Adult↗
PubMedDate not supplied

[The mysteries of lipoprotein (a): a bridge between thrombosis and atheroma].

Nearly 30 years have elapsed since Berg discovered a genetic variant of low density lipoproteins (LDL) which he called lipoprotein (a), abbreviated Lp (a). Lp (a) rapidly appeared as an independent factor of atherosclerosis. Its physico-chemical characteristics are well-known, but this cannot be said of its function, its metabolism and the exact mechanism of its contribution to atherogenesis. The plasma Lp (a) concentration is determined genetically and its seems that few factors can lower it. Owing to its structural analogy with plasminogen, Lp (a) has been suspected to bind fibrin by a competitive mechanism, thereby facilitating thrombosis, a necessary step in the progression of atherosclerosis. However, these results remain controverted, and indeed there is some evidence of a possible interaction between Lp (a) and alpha-2-antiplasmin, a physiological inhibitor of fibrinolysis, an effect that would accentuate fibrinolysis. This paradoxical action of Lp (a) and the various mysteries which still shroud this lipoprotein are perhaps due to the diversity of its isotypes.

Arteriosclerosis↗
PubMedDate not supplied

[Adult Still's disease. A too often unrecognized illness. A study of a series of 11 cases].

The authors report eleven new cases of adult Still's disease diagnosed during the last 5 years. This rare, though not exceptional, disease of unknown pathogenesis is difficult to diagnose in the absence of specific sign; it is in fact diagnosed by elimination. The clinical, laboratory and anatomical findings, as well as the treatment and outcome of these 11 cases are described, and this is followed by a discussion of the nosological, therapeutic and above all prognostic problems raised by the disease.

Adolescent↗
PubMedDate not supplied

Prognostic factors and long-term evolution in a cohort of 133 patients with giant cell arteritis.

OBJECTIVE: Survival in patients with giant cell arteritis (GCA) has generally been found to be similar to that of the general population. The aim of our study was to assess outcome and survival of different subgroups of patients with GCA in relation to clinical, biological data or treatment modalities. METHODS: From 1977 and 1995, 176 patients were treated in the Department of Internal Medicine for GCA. The patient, family or local practitioner were contacted prior to the study (July-October 1995). Treatment modalities and follow-up were obtained for 133 patients. All patients (except 11) had 3 or more 1990 ACR classification criteria for GCA. The 11 patients with 2 criteria had a positive temporal biopsy and were included in the study. RESULTS: Relapse during corticosteroid tapering treatment was observed in 83 patients (62.4%) with a mean 1.57 relapses per patient. No correlation was found in age, sex, initial dose or type of steroid used (i.e. prednisone or prednisolone). Only a slight correlation in the initial erythrocyte sedimentation rate (ESR) was observed (p < 0.01, r = 0.23). In 56 patients free of treatment (mean treatment duration: 40 months), 27 (48%) developed a relapse of the disease 1 to 25 months later. No correlation was found in age, sex, initial dose of steroid, number of relapses during treatment, or initial ESR. Survival analysis was performed using the Kaplan-Meier and Mantel-Menszel methods for comparison of groups. At the time of the study, 41 patients had died (30.7%). A significant reduction of survival was found with the presence of permanent visual loss vs absence (p = 0.04), in patients who required more than 10 mg/d of glucocorticoid (p < 0.001) at 6 months treatment and in patients treated with prednisone (vs prednisolone) (p < 0.01). However, these factors were not independently associated with survival in the multivariate analysis. CONCLUSION: Relapse was observed in 62.4% of the patients during corticosteroid tapering (correlated with initial ESR). A relapse of the disease was also observed in 48% of patients 1 to 25 months after the end of the treatment and was associated with prednisolone use. Long term survival was better in patients with no initial ocular manifestations, in patients who took less than 10 mg/day of corticosteroids at 6 months of the treatment and in patients treated with prednisolone.

Aged↗
PubMedDate not supplied

An uncommon association: celiac disease and dermatomyositis in adults.

We describe the case of a patient with a two-year history of adult dermatomyositis (DM) who developed malabsorption revealing celiac disease. Our observation raises the question of an association between DM and celiac disease as part of a continuum, suggesting that celiac disease may be included within the spectrum of the gastrointestinal manifestations of DM and polymyositis (PM). From a practical point of view, our data indicate that the diagnosis of celiac disease should be suspected in PM/DM patients exhibiting malabsorption syndrome. Based on our findings, we further emphasize that an evaluation for celiac disease, including anti-gliadin antibodies, anti-endomysium antibody and tissue trans-glutaminase antibodies should be considered in PM/DM patients presenting with unusual and unexplained gastrointestinal features. This could lead to the early management of such patients, resulting in decreased morbidity (i.e., malnutrition and malignancy) related to misdiagnosed celiac disease.

Celiac Disease↗
PubMedDate not supplied

Serum amyloid A concentrations in giant-cell arteritis and polymyalgia rheumatica: a useful test in the management of the disease.

A prospective clinical study of 23 patients with giant-cell arteritis (GCA) and/or polymyalgia rheumatica (PMR) was undertaken in order to assess the behaviour of the non-specific markers of the disease activity, the erythrocyte sedimentation rate (ESR) and other acute phase markers, particularly the C-reactive protein (CPR) and serum amyloid A apolipoprotein (apo SAA) levels during induction of disease remission by prednisone therapy, and possible further recurrence of GCA and/or PMR. The apo SAA measurement is more sensitive than the CRP measurement in determining disease activity (97% and 61%, respectively). The specificity of apo SAA is greater than ESR in the determination of inactive disease (86% and 77%, respectively). In some cases with clinically active disease the ESR and CRP were normal, whereas the apo SAA was always elevated. We conclude that the apo SAA measurement in combination with clinical data and other laboratory parameters may be useful in the management of GCA and/or PMR.

Acute-Phase Proteins↗
PubMedDate not supplied

[Calcium antagonists and Raynaud's phenomenon].

Epidemiologic studies indicate that episodic vasospasm of arterioles (Raynaud's phenomenon) is a common finding in the general population. The prevalence has been estimated at approximately 10%. Raynaud's phenomenon follows a benign course in most cases and may improve or even disappear in time. Occasionally, Raynaud's phenomenon may be induced by some drugs (beta-blockers, ergot, vinblastine...) or some occupations (pneumatic hammer operators, hand-arm vibration syndrome...) or may be a sign of connective tissue disease (systemic sclerosis, systemic lupus erythematosus, Sjögren's syndrome...). Several different physiologic mechanisms may be responsible for vasospasm: hyperactivity of the sympathetic nervous system and abnormal adrenergic receptor function appear to be most important. Calcium channel blockers have been studied extensively for the treatment of primary and secondary Raynaud's phenomenon. There have been shown to be particularly effective for inhibiting vascular responses evoked by alpha 2-adrenoceptor activity. Calcium channel blockers have been mostly effective in Raynaud's phenomenon.

Calcium Channel Blockers↗
PubMedDate not supplied

Increased risk of Pneumocystis carinii pneumonia in patients with Wegener's granulomatosis.

Combining cyclophosphamide (Cy) and corticosteroids has dramatically improved the prognosis of Wegener's granulomatosis (WG). But this treatment carries the risks of severe infectious complications and drug toxicity. During a 10-month period, we observed 6 cases of Pneumocystis carinii pneumonia (PCP) in 23 patients with biopsy-proven WG and renal involvement. These 23 patients were enrolled in a multicenter controlled clinical trial designed to evaluate the efficacy and safety of either intermittent high-dose pulse Cy or daily oral low-dose Cy in combination with oral prednisone. Mean delay of onset of PCP was 2.5 months after the beginning of the immunosuppressive therapy. In all cases, the diagnosis of PCP was established by cytological examination of bronchoalveolar lavage fluid. None of the patients experienced severe leukopenia at the time of diagnosis, but the mean lymphocyte count decreased to 495/mm3 (range 100 to 830/mm3) and 2 patients had inverted CD4/CD8 T-cell ratios. Renal function was significantly impaired (creatininemia = 493.5 vs 195.4 micromol/l; p = 0.03) in the 6 patients presenting PCP vs those without. High-dose co-trimoxazole therapy was successful in 3 patients, but 3 others who required mechanical ventilation died. Treatment of WG with daily prednisone and either pulse or oral Cy may have contributed to higher rates of PCP in the past than previously thought and, therefore, patients currently receiving such a regimen may be at greater risk for PCP. For these patients, this opportunistic infection must remain highly suspect in order to reach a diagnosis earlier and rapidly initiate treatment. In addition, recommendations for prophylactic therapy are needed.

Adrenal Cortex Hormones↗