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Biomedical subjects

Jean-François Cordier

Publications and source records attributed to Jean-François Cordier.

At least 19 recordsLinked to original sources
PubMed2007

Pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia: a series of 126 patients.

Hereditary hemorrhagic telangiectasia (HHT) is a genetic disorder characterized by epistaxis, telangiectasia, and visceral vascular manifestations. Infectious and ischemic central nervous system (CNS) manifestations due to embolism through pulmonary arteriovenous malformations (PAVMs) represent the main causes of morbidity. To improve the phenotypic characterization of HHT with PAVM, we conducted a retrospective multicenter study of patients with HHT and at least 1 PAVM detected by chest computed tomography (CT) and/or pulmonary angiography, with particular attention to CNS and infectious manifestations. The study included 126 patients (47 men, 79 women), with a mean age of 43.1 +/- 17.4 years; 45 patients had a mutation of the ENG gene and 16 had a mutation of ACVRL1. PAVMs were diagnosed as a result of systematic screening procedures (29%), incidental imaging findings (15%), dyspnea (22%), or CNS symptoms (13%). The PAVMs were diagnosed at a mean age of 43 +/- 17 years, with a linear distribution of diagnosis between 20 and 75 years. Dyspnea on exertion was present in 56% of patients. Four patients had a hemothorax, including 1 during pregnancy. Fifty-three CNS events directly related to HHT (excluding migraine) were observed in 35% of patients: cerebral abscess (19.0%), ischemic cerebral stroke (9.5%), transient cerebral ischemic attack (6.3%), and cerebral hemorrhage (2.4%). The median age of onset was 33 years for cerebral abscesses (range, 11-66 yr), and 53.5 years for ischemic cerebral events (range, 2-72 yr). Migraine was reported in 16% of patients. The diagnoses of PAVM and HHT were made at the time of the cerebral abscess in 13 cases (54%). Forty-three percent of patients were hypoxemic at rest. Contrast echocardiography showed intrapulmonary right-to-left shunting in 87% of tested patients. PAVMs were seen on chest radiograph in 54% of patients, and on the CT scan in all patients. One hundred five patients (83%) underwent treatment of the PAVM, by percutaneous embolization (71%) and/or by surgical resection (23%). A high frequency of CNS and infectious complications was observed in this large series of patients with HHT-related PAVM. Physicians may not be sufficiently aware of the clinical manifestations of this orphan disorder. Patients diagnosed with HHT should be informed by physicians and patient associations of the risk of PAVM-related complications, and systematic screening for PAVM should be proposed, regardless of a patient's symptoms, familial history, or genetic considerations.

Abscess↗
PubMed2006

[Idiopathic pulmonary fibrosis].

Idiopathic pulmonary fibrosis is a fibroproliferative disorder characterized histopathologically by heterogenous interstitial fibrosis with fibroblastic foci and honeycomb lung. The diagnosis is based on a pluridisciplinary approach, integrating the clinical evaluation, the chest high-resolution computerized tomography features, and the pathological pattern of usual interstitial pneumonia on video-thoracoscopic lung biopsy when performed. When the tomodensitometric pattern is typical (50% of the cases), the diagnosis may be made without a lung biopsy. No treatment has proven benefit in survival. Treatment with N-acetylcysteine in association with corticosteroids and azathioprine has recently shown a modest functional improvement. Clinical trials are strongly needed in this still uncurable disease with median survival at 3 years of only about 50%.

Biopsy↗
PubMed2006

Chemotherapy is the cornerstone of the combined surgical treatment of lung cancer with synchronous brain metastases.

BACKGROUND: Lung cancer accounts for about 50% of brain metastases, of which nearly 25% are eligible for neurosurgery, providing a neurological control rate of up to 70% when followed by whole brain radiation therapy. How to manage the primary lung carcinoma remains elusive. METHODS: We undertook a retrospective study of consecutive patients who underwent surgical resection for synchronous brain metastases from non-small cell lung cancer in a single institution, to determine overall survival and prognostic factors, with particular attention to the treatment of the primary lung tumor. RESULTS: Fifty-one patients underwent surgical resection of synchronous brain metastases from non-small cell lung cancer. Median survival was 13.2 months. Prognosis mainly depended of the treatment of the lung tumor, with a marked survival advantage in the 29 patients receiving a focal treatment (thoracic surgery or radiotherapy), compared to the 22 other patients: median, 1-year, and 2-year survival were 22.5 months, 69%, and 42%, versus 7.1 months, 33%, and 5%, respectively (p<0.001); response to pre-operative chemotherapy before focal treatment was the main favorable prognostic factor (p=0.023), and further identified patients who had benefit from resection of the lung tumor, with a significantly better outcome. CONCLUSIONS: Chemotherapy, by its therapeutic and prognostic value, may be considered as the cornerstone of the combined medical and surgical therapeutic sequence whereby brain metastasectomy is followed by chemotherapy and further focal treatment of the primary lung tumor in responders to chemotherapy.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2006

Idiopathic chronic eosinophilic pneumonia.

Idiopathic chronic eosinophilic pneumonia (ICEP) is characterized by subacute or chronic respiratory and general symptoms, alveolar and/or blood eosinophilia, and peripheral pulmonary infiltrates on chest imaging. Eosinophilia is present in most cases, usually in excess of 1000/mm3. In absence of significant blood eosinophilia, a diagnosis of ICEP is supported by the demonstration of bronchoalveolar lavage eosinophilia. ICEP is typically associated with eosinophil counts higher than lymphocyte counts in the bronchoalveolar lavage. ICEP is a rare disorder of unknown cause. Its exact prevalence remains unknown. ICEP may affect every age group but is rare in childhood. It is twice as frequent in women as in men. One third to one half of the ICEP patients have a history of asthma. The mainstay of treatment of ICEP is systemic corticosteroids. Response to oral corticosteroid therapy is dramatic and has led to the consideration of corticosteroid challenge as a diagnostic test for ICEP. Nevertheless, relapses or development of severe asthma are frequent when tapering or withdrawing treatment. Long-term oral corticosteroid therapy is necessary in up to half of the patients.

Adult↗
PubMed2006

[Hypereosinophilic asthma].

Hypereosinophilic asthma (defined by blood eosinophils > 1 G/L, especially > 1.5 G/L) may be solitary (and often difficult to treat), or a manifestation of a hypereosinophilic disorder of determined cause (parasitic, iatrogenic, allergic bronchopulmonary aspergillosis) or idiopathic (idiopathic chronic eosinophilic pneumonia, Churg-Strauss syndrome, idiopathic hypereosinophilic syndrome). In addition to etiologic treatment when available long term corticosteroids are necessary in a majority of patients.

Asthma↗
PubMed2006

Pulmonary arterial hypertension in France: results from a national registry.

RATIONALE: Pulmonary arterial hypertension (PAH) is an orphan disease for which the trend is for management in designated centers with multidisciplinary teams working in a shared-care approach. OBJECTIVE: To describe clinical and hemodynamic parameters and to provide estimates for the prevalence of patients diagnosed for PAH according to a standardized definition. METHODS: The registry was initiated in 17 university hospitals following at least five newly diagnosed patients per year. All consecutive adult (> or = 18 yr) patients seen between October 2002 and October 2003 were to be included. MAIN RESULTS: A total of 674 patients (mean +/- SD age, 50 +/- 15 yr; range, 18-85 yr) were entered in the registry. Idiopathic, familial, anorexigen, connective tissue diseases, congenital heart diseases, portal hypertension, and HIV-associated PAH accounted for 39.2, 3.9, 9.5, 15.3, 11.3, 10.4, and 6.2% of the population, respectively. At diagnosis, 75% of patients were in New York Heart Association functional class III or IV. Six-minute walk test was 329 +/- 109 m. Mean pulmonary artery pressure, cardiac index, and pulmonary vascular resistance index were 55 +/- 15 mm Hg, 2.5 +/- 0.8 L/min/m(2), and 20.5 +/- 10.2 mm Hg/L/min/m(2), respectively. The low estimates of prevalence and incidence of PAH in France were 15.0 cases/million of adult inhabitants and 2.4 cases/million of adult inhabitants/yr. One-year survival was 88% in the incident cohort. CONCLUSIONS: This contemporary registry highlights current practice and shows that PAH is detected late in the course of the disease, with a majority of patients displaying severe functional and hemodynamic compromise.

Adolescent↗
PubMed2006

Idiopathic chronic eosinophilic pneumonia.

Idiopathic chronic eosinophilic pneumonia is a rare disorder of unknown cause with nonspecific respiratory and systemic symptoms but rather characteristic peripheral alveolar infiltrates on imaging. The disorder is highly responsive to oral corticosteroid therapy. However, relapses are frequent when tapering or after stopping treatment. Moreover, some patients develop severe asthma at some time in the follow-up. The high incidence of relapses and prevalence of severe asthma is responsible for the great proportion of patients with idiopathic chronic eosinophilic pneumonia who require prolonged oral corticosteroid therapy. There are tight links between asthma and idiopathic chronic eosinophilic pneumonia. These links might help in the comprehension of the pathogenesis of both diseases. Interestingly, there might exist a continuum between hypereosinophilic asthma, idiopathic chronic eosinophilic pneumonia, and the Churg-Strauss syndrome.

Adrenal Cortex Hormones↗
PubMed2005

Early variations of circulating interleukin-6 and interleukin-10 levels during thoracic radiotherapy are predictive for radiation pneumonitis.

PURPOSE: To investigate variations of circulating serum levels of interleukin-6 (IL-6), tumor necrosis factor alpha (TNFalpha), and interleukin-10 (IL-10) during three-dimensional conformal radiation therapy (3D-CRT) in patients with non-small-cell lung cancer and correlate these variations with the occurrence of radiation pneumonitis. PATIENTS AND METHODS: Ninety-six patients receiving 3D-CRT for stage I to III disease were evaluated prospectively. Circulating cytokine levels were determined before, every 2 weeks during, and at the end of treatment. Radiation pneumonitis was evaluated prospectively between 6 and 8 weeks after 3D-CRT. The predictive value of clinical, dosimetric, and biologic (cytokine levels) factors was evaluated both in univariate and multivariate analyses. RESULTS: Forty patients (44%) experienced score 1 or more radiation pneumonitis. No association was found between baseline cytokine levels and the risk of radiation pneumonitis. In the whole population, mean levels of TNFalpha, IL-6, and IL-10 remained stable during radiotherapy. IL-6 levels were significantly higher (P = .047) during 3D-CRT in patients with radiation pneumonitis. In the multivariate analysis, covariations of IL-6 and IL-10 levels during the first 2 weeks of 3D-CRT were evidenced as independently predictive of radiation pneumonitis in this series (P = .011). CONCLUSION: Early variations of circulating IL-6 and IL-10 levels during 3D-CRT are significantly associated with the risk of radiation pneumonitis. Variations of circulating IL-6 and IL-10 levels during 3D-CRT may serve as independent predictive factors for this complication.

Adult↗
PubMed2005

Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome.

BACKGROUND: Since testing for antineutrophil cytoplasmic antibodies (ANCA) became available for routine evaluation, no large homogeneous cohort of patients with the Churg-Strauss syndrome has been studied. OBJECTIVE: To define the clinical and biological characteristics of newly diagnosed Churg-Strauss syndrome, according to the presence or absence of ANCA. DESIGN: Cross-sectional analysis of manifestations of participants who were enrolled in treatment trials between December 1995 and December 2002. SETTING: Multicenter study in 63 clinical centers in France, Belgium, Latvia, and the United Kingdom, coordinated by the French Vasculitis Study Group. PARTICIPANTS: 112 patients with Churg-Strauss syndrome that was recently diagnosed on the basis of current classifications. MEASUREMENTS: The authors compared principal demographic, clinical, and laboratory features according to ANCA status at diagnosis. RESULTS: The authors detected ANCA in 43 (38%) patients. Positive ANCA status at diagnosis was associated with renal involvement, peripheral neuropathy, and biopsy-proven vasculitis, whereas negative ANCA status was associated with heart disease and fever. LIMITATIONS: The authors assessed ANCA by immunofluorescence, but they did not assess ANCA centrally or systematically retest if ANCA was undetected at diagnosis. CONCLUSIONS: Phenotypically, ANCA-positive and ANCA-negative Churg-Strauss syndrome might differ. The association of ANCA positivity with clinical symptoms that indicate inflammation and necrosis of small vessels might characterize a predominantly vasculitic pattern of the Churg-Strauss syndrome.

Adolescent↗
PubMed2005

Pulmonary varix mimicking pulmonary arteriovenous malformation in a patient with Turner syndrome.

A 36-year-old asymptomatic female with Turner syndrome was referred for a 3-cm opacity of the left lung detected by routine chest X-ray. A computed tomography scan of the chest suggested a vascular lesion such as pulmonary arteriovenous malformation, and transcatheter embolotherapy was considered. The lack of a right-to-left shunt on contrast echocardiography led to suspect an alternate diagnosis. Magnetic resonance imaging and pulmonary angiography eventually demonstrated a pulmonary varix associated with a partial anomalous pulmonary venous return. Contrast echocardiography may help to distinguish between pulmonary varix and arteriovenous malformation.

Adult↗