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Isabelle Marie

Publications and source records attributed to Isabelle Marie.

16 recordsLinked to original sources
PubMed2006

High-risk pregnancies in Diamond-Blackfan anemia: a survey of 64 pregnancies from the French and German registries.

We reviewed 64 pregnancies in 26 women with Diamond-Blackfan anemia (DBA) included in the French and German DBA registries. Complications were seen in 42 pregnancies (66%) and included abortion, pre-eclampsia, in utero fetal death, intrauterine growth retardation, retroplacental hematoma, pre-term delivery and fetal malformations. Of the 34 children (53%) born alive, 13 had DBA. No correlations were found between pregnancy outcome and features of either maternal or child DBA. Pregnancies in DBA-affected women are at high risk, especially for complications likely to be of vascular-placental origin. Careful monitoring with prevention of severe anemia and early introduction of aspirin is suggested.

Anemia↗
PubMed2006

Helicobacter heilmanii gastritis in polymyositis.

We report the original case of a patient with polymyositis (PM) who developed severe gastritis related to Helicobacter heilmanii. H. heilmanii eradication, using triple therapy with omeprazole, metronidazole and amoxicillin, resulted in the complete disappearance of digestive manifestations. As an association has been found between gastric adenocarcinoma/MALT lymphoma and both polymyositis/dermatomyositis (PM/DM) and H. heilmanii infection, respectively, we suspect that PM/DM patients who develop H. heilmanii infection may have a higher risk of gastric malignancy occurrence. Further studies are warranted to confirm whether Helicobacter species infections do, in fact, increase the risk of gastric adenocarcinoma/MALT lymphoma in patients with PM/DM.

Journal Article↗
PubMed2006

[Pulmonary damage during polymyositis and dermatomyositis: interstitial lung disease].

Interstitial lung disease is a serious complication of polymyositis/dermatomyositis and leads to death from chronic respiratory insufficiency in 30 to 66% of cases. It is a criterion of poor prognosis in these disorders. Its onset occurs at variable points in the course of polymyositis/dermatomyositis, and precedes them in half of all cases. Presentation may also vary: acute (25%), insidious (60%), or infraclinical, discovered fortuitously (15%). The examinations of choice for early screening are high-resolution computed tomography (CT) and pulmonary function tests, which should be performed during the initial work-up and during ongoing surveillance. Moreover, high-resolution CT also makes it possible to determine the type of histologic lesions in the interstitial lung disease. Today, diagnosis of this disease does not generally require histological confirmation; nonspecific interstitial lung disease seems to be the most common histologic form of lung damage in polymyositis/dermatomyositis (40 to 80%). Anti-Jo1 antibodies are a sensitive marker of interstitial lung disease during polymyositis/dermatomyositis, and close surveillance of lung function is recommended in patients with these autoantibodies. Systematic testing for them is also justified in patients with apparently idiopathic interstitial lung disease, to rule out underlying polymyositis/dermatomyositis. No clear treatment protocols have been established for interstitial lung disease during polymyositis/dermatomyositis. Corticosteroid treatment is the first choice. Its combination with cyclophosphamide may be most effective in corticosteroid-resistant forms of polymyositis/dermatomyositis, especially when begun early; it may also be appropriate to begin corticosteroids as soon as factors predicting poor prognosis are detected.

Acetylcysteine↗
PubMed2006

[Gastrointestinal involvement in systemic sclerosis].

Gastrointestinal involvement is frequent in systemic sclerosis (SSc), occurring in 75 to 90% of patients with diffuse or limited cutaneous SSc. Although all regions of the gut may be affected, the esophagus is the most common gastrointestinal localization of this disease. If not diagnosed at an early stage, resulting complications may include esophagitis (leading to stenosis, strictures, and Barrett's esophagus) and increased risk of interstitial lung disease. Esophageal manometry is the most sensitive test for accurate diagnosis of motor dysfunction. Antisecretory agents (mainly proton-pump inhibitors) are effective for treating esophageal manifestations. Gastrointestinal involvement is reported in 50-88% of patients with SSc. It remains associated with a poor prognosis and leads to death in 6 to 12% of cases. Gastrointestinal impairment may lead to life-threatening complications, including severe dyspepsia, hemorrhage related to watermelon stomach (gastric antral vascular ectasia), malabsorption syndrome (related to bacterial overgrowth), and intestinal pseudo-obstruction. Treatment for the latter two remains difficult, although octreotide has proven to be effective in SSc patients. Small bowel manometry is useful for careful selection of SSc patients who will benefit from this treatment. Anorectal involvement is frequent in SSc patients (50-70%) and causes fecal incontinence and rectal prolapse. Other digestive manifestations, including liver impairment, are less common in SSc.

Barrett Esophagus↗
PubMed2006

Ascites as the first manifestation of polyarteritis nodosa.

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that affects numerous organs, particularly the digestive tract (33-52% of cases). However, isolated ascites has rarely been described in patients with PAN. We observed a patient who had developed ascites revealing PAN, and because of suspected malignancy laparotomy and abdominal surgery were performed, including systematic total abdominal hysterectomy, bilateral salpingo-oophrectomy, submesocolic omontectomy and appendicectomy. Macroscopic examination revealed a nodule involving the left ovary, while histological examination of biopsy specimens demonstrated characteristic damage of necrotizing vasculitis involving the appendix, omentum, uterus, fallopian tubes and right ovary, consistent with PAN. The patient had a favourable outcome of clinical manifestations related to PAN after initiation of combined therapy of prednisone and cyclophosphamide. Our case report therefore reinforces the possibility of an unusual presentation of PAN. As ascites may precede other signs of PAN, we suggest that when unexplained ascites is noted an evaluation for misdiagnosed vasculitis should be carried out, including a search of the constitutional symptoms (e.g. fever, weight loss). Patients undergoing laparoscopy for peritoneal involvement of unknown origin should also have tissue specimens taken for pathologic studies, notably to exclude an underlying vasculitis.

Aged↗
PubMed2005

Opportunistic infections in polymyositis and dermatomyositis.

OBJECTIVE: To assess prevalence and characteristics of opportunistic infections in patients with polymyositis/dermatomyositis (PM/DM). To determine the predictive values for opportunistic infections on clinical presentation, biochemical findings, and paraclinical features of PM/DM to detect patients at risk of opportunistic infections. METHODS: The medical records of 156 consecutive PM/DM patients in 3 medical centers were reviewed. RESULTS: Eighteen PM/DM patients (11.5%) developed opportunistic infections. The majority of patients exhibited an opportunistic infection after the onset of PM/DM (89% of cases). Opportunistic infections occurred most frequently during the first year following PM/DM diagnosis (62.5%). The pathogen microorganisms responsible for opportunistic infections were various, i.e., Candida albicans, Pneumocystis carinii, Aspergillus fumigatus, Geotrichum capitatum, Mycobacterium avium-intracellulare complex, M. xenopi, M. marinum, M. tuberculosis, Helicobacter heilmanii, cytomegalovirus, and herpes simplex virus. Mortality rates were as high as 27.7% in these PM/DM patients. Higher mean daily doses of steroids, lymphopenia, and lower serum total protein levels were significantly more frequent in the group of PM/DM patients with opportunistic infections. CONCLUSION: Our study underscores the high frequency of opportunistic infections in PM/DM, resulting in an increased mortality rate. It also indicates that a great variety of microorganisms are responsible for opportunistic infections, although they were more often due to fungi (>50% of cases). Our series highlights a predominance of both lung and digestive opportunistic infections (89% of cases). In addition, our results suggest that PM/DM patients presenting with factors predictive of opportunistic infection may require closer monitoring.

Adult↗
PubMed2005

Clonal T cells in the blood of patients with systemic sclerosis.

BACKGROUND: It has been suggested that clonal T cells may play a critical role in the pathogenesis of systemic sclerosis. OBSERVATIONS: A monoclonal population of T cells was found in blood samples from 13 (34%) of 38 consecutive patients with a definite diagnosis of systemic sclerosis who were prospectively examined by T-cell receptor gamma gene rearrangement using polymerase chain reaction analysis and denaturating gradient gel electrophoresis. In the healthy control group, the same type of examination revealed a monoclonal population of T cells in the blood samples from only 3 healthy subjects (4%)(odds ratio, 12.28; 95% confidence interval, 2.76-54.64; P = .001). Patients who had a circulating clonal population of T cells were older than those who did not (67 years vs 48 years; P = .04). There was a marked relationship between systemic sclerosis subtypes and the presence of a circulating clonal population of T cells. Twelve (43%) of 28 patients with limited cutaneous sclerosis exhibited a circulating clonal population of T cells, whereas only 1 (10%) of the 10 patients with diffuse cutanous sclerosis had evidence of T-cell clonality (P<.01). CONCLUSIONS: Clonally expanded T cells were more commonly detected in patients with limited cutaneous sclerosis than in those with diffuse cutaneous sclerosis, which is also in accordance with a possible role of clonal T cells in patients with limited cutaneous sclerosis.

Adult↗
PubMed2005

Inflammatory pseudotumor of the liver revealing gynecological Corynebacterium infection.

Inflammatory pseudotumor (IPL) of the liver is a rare benign clinicopathologic entity, which has been associated with infectious conditions, including alimentary, digestive tract and mouth infections. We report a new case, where a patient developed hepatic IPL that revealed endometritis related to Corynebacterium infection; the patient had a favorable outcome of hepatic IPL after appropriate antibiotic therapy initiation. Our findings therefore confirm that antibiotic therapy may be effective in patients with hepatic IPL associated with underlying infections, avoiding unnecessary surgery. Our data also indicate that when hepatic IPL is observed, a complete clinical evaluation, including gynecological evaluation, should be systematically performed.

Adult↗
PubMed2004

Pleural effusion revealing giant cell arteritis.

Pulmonary involvement is considered to be rare in giant cell arteritis (GCA), usually occurring in the course of the disease. We describe the case of a patient who developed left pleural effusion revealing GCA. Thoracic CT scan demonstrated an abundant left pleural effusion and a thickening of the aortic wall. The patient's condition improved rapidly, with regression of pulmonary clinical features and complete clearance of pleural effusion, after institution of steroid therapy. Our case report reinforces the possibility of unusual presentation of GCA; such a diagnosis should, therefore, be considered in elderly patients presenting with pulmonary manifestations, even in the absence of typical clinical features of temporal arteritis (e.g. headache, jaw claudication, blurred vision, scalp tenderness) or polymyalgia rheumatica.

Journal Article↗
PubMed2003

Intracardiac thrombi in primary antiphospholipid syndrome: two case reports.

We describe two patients who developed stroke and ischemia of the left lower limb, related to intracardiac thrombi, as the first manifestation of primary antiphospholipid syndrome (PAPS). Transesophageal echocardiography (TEE) revealed intracardiac thrombi as abnormal hyperechogenic, nonmobile masses, firmly attached to the left atrial appendage and the anterior part of the mitral annulus, respectively. Our patients received high-intensity oral anticoagulant therapy (INR 3.0-4.0), which resulted in the rapid disappearance of clinical symptoms, without subsequent recurrence of thromboembolic manifestations, and the disappearance of intracardiac thrombi on TEE. Our findings underscore that PAPS should be suspected in patients presenting with intracardiac thrombi, even if they have no previous history of thromboembolic disorders. Moreover, because intracardiac thrombi may precede other manifestations of PAPS, this finding in young patients without underlying heart disease should invoke a search for both antiphospholipid and anticardiolipin antibodies.

Journal Article↗
PubMed2002

[Pathogenesis of scleroderma].

Systemic sclerosis is a systemic inflammatory disorder, affecting the skin and other organs, especially the lungs and the digestive tract. The pathogenic mechanisms of systemic sclerosis still remain unknown, although there is evidence that many parameters may play a role in the pathogenesis of the disease, including: vascular involvement, immunity impairment, and both environmental and genetic factors. To date, many authors support the hypothesis that, in patients with genetic predisposition, environmental stimuli (occupational factors and/or virus) may lead to vascular dysfunction, particularly, endothelial damage and immune activation, resulting in tissue fibrosis at late stages of the disorder.

Cardiovascular System↗
PubMedDate not supplied

Calcium imaging of murine thoracic aorta endothelium by confocal microscopy reveals inhomogeneous distribution of endothelial cells responding to vasodilator agents.

The aim of the study was to assess, in intact murine thoracic aorta in vitro, the distribution of endothelial cells responsive to endothelium-dependent vasodilators ACh, ATP, bradykinin and substance P, using laser line confocal microscopy in combination with two Ca2+ fluorescent dyes, Fluo-4 and Fura-red. We observed that 82 +/- 3% of endothelial cells responded to ATP, 33 +/- 5% to Ach, whereas less than 3% of them responded to bradykinin or substance P. In order to determine whether the findings of pharmacological tests agree with confocal microscopy data, endothelium-dependent vasodilators induced relaxation was evaluated using isometric tension measurement. We show a marked correlation between a higher number of activated endothelial cells, using confocal microscopy, and a greater degree of endothelium-dependent relaxation using isometric tension measurement (p = 0.00286). Our results suggest that endothelial cells responding to endothelium-dependent vasodilators are not homogeneously distributed in intact murine thoracic aorta. This could be due to nonhomogeneous distribution of surface receptors or to differences in post-receptor coupling mechanisms.

Acetylcholine↗