Le Plessis-Robinson, France
Le Plessis-Robinson, France

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Sellier-Leclerc A.-L.,Hopital Femme Mere Enfants | Belli E.,Center Chirurgical Marie Lannelongue | Guerin V.,Robert Debre Hospital | Dorfmuller P.,Center Chirurgical Marie Lannelongue | And 2 more authors.
Pediatric Nephrology | Year: 2013

Background: We report a 7-year-old boy with high-degree steroid-dependent idiopathic nephrotic syndrome (SDNS) who went into remission with rituximab (RTX) maintenance therapy. Case-Diagnosis/Treatment: Four months after this patient received his first RTX infusion, there was a progressive and sustained decrease of immunoglobulin (Ig)G and IgM levels. Thirteen months after the initiation of RTX therapy he was in sustained remission without any steroid or oral immunosuppressive therapy; however, B cell depletion was still present. At this time he developed a fulminant myocarditis due to enterovirus. Despite aggressive treatment and the administration of intravenous polyvalent immunoglobulins there was no clinical improvement. He successfully underwent heart transplant surgery. Conclusions: We conclude that B cell depletion with RTX is efficacious in the treatment of paediatric SDNS but that it may be associated with severe infectious complications. Therefore, we recommend a close monitoring of Ig levels in children who have received RTX therapy and a supplementation with intravenous Ig as soon as the Ig levels fall below the lower limit of the normal range © 2013 IPNA.


Lador F.,Hopitaux Universitaires Of Geneva | Herve P.,Center Chirurgical Marie Lannelongue | Herve P.,University Paris - Sud
Seminars in Respiratory and Critical Care Medicine | Year: 2013

Recent reports from pulmonary arterial hypertension (PAH) registries suggest that the mean age at diagnosis is increasing, outlining a growing proportion of elderly male patients. As a consequence, the classical description of the disease is shifting and may no more be described as a rare disease typically affecting young women. Potential explanations of this changing picture may include an aging of populations in western countries, the increase in life expectancy and the growing awareness of PAH and emergence of potential efficient treatments. Diagnostic workup of severe pulmonary hypertension (PH) in the elderly should be performed in such a way as to discriminate between the expected consequences of aging, pulmonary vascular disease, and other frequent causes of secondary PH (left heart failure or lung disease). Careful exploration by right heart catheterization is mandatory, but special attention should be paid to several pitfalls specific to this procedure in this age group. This is a matter of concern as clinical trials that aim to study new specific drug therapy for PAH might be biased by the inclusion of misdiagnosed patients. The aim of this review is to highlight the main difficulties in diagnosing PAH in the elderly and to propose a practical approach to distinguish PAH from the other frequent causes of PH in this population. © 2013 by Thieme Medical Publishers, Inc.


Guignabert C.,French Institute of Health and Medical Research | Guignabert C.,University Paris - Sud | Dorfmuller P.,French Institute of Health and Medical Research | Dorfmuller P.,Center Chirurgical Marie Lannelongue | Dorfmuller P.,University Paris - Sud
Seminars in Respiratory and Critical Care Medicine | Year: 2013

Pulmonary hypertension is a devastating, life-threatening disorder with no curative options, characterized by elevated pulmonary vascular resistance and secondary right ventricular failure. Although the etiologies of pulmonary arterial hypertension are multiple and its pathogenesis is complex, there is growing evidence that inflammation, endothelial dysfunction, aberrant vascular wall cell proliferation, as well as mutations in the bone morphogenetic protein receptor type 2 gene play a crucial role in triggering pathological vascular remodeling. The present article outlines the current understanding of this disease from the point of view of pathology and pathobiology. © 2013 by Thieme Medical Publishers, Inc.


Besse B.,Institute Gustave Roussy | Le chevalier T.,Institute Gustave Roussy | Le chevalier T.,Center Chirurgical Marie Lannelongue
Annals of Oncology | Year: 2012

approximately 30 of lung carcinomas are resected and these cases are candidates for adjuvant treatments. The PORT meta-analysis reported in 1999 that postoperative radiotherapy had a detrimental effect for pathological N0 and N1 patients, and a debatable effect for N2 patients. Following the results of the 1995 meta-analysis on the role of chemotherapy (CT) in non-small-cell lung cancer (NSCLC), many randomized, controlled trials were launched to evaluate the effect of adjuvant cisplatin-based CT after the complete resection of NSCLC. The Lung adjuvant Ciplatin Evaluation pooled analysis included a total of 4584 patients recruited in five recent cisplatin-based adjuvant trials. It confirmed that adjuvant CT was associated with an absolute 5-year survival benefit of 5.3 (P = 0.0043). In addition, it showed that adjuvant cisplatin-based CT is detrimental in cases of stage Ia resected NSCLC; it also suggested that the combination of vinorelbine and cisplatin was of more benefit than older two and three drug combinations. The individual data-based meta-analysis was also updated with a total of over 10 000 patients. It confirmed the substantial effect of postoperative CT, with or without postoperative radiotherapy, with a substantial overall benefit of 4 at 5 years. Recent results of biological programs suggest that evaluating the expression of various tumor markers, including excision repair cross-complementation group 1, may allow the identification of patients most likely to benefit from CT. If these results are confirmed, tailored therapy might be the next step forward for resected NSCLC. © The author 2012. Published by Oxford University Press on behalf of the European Society for Medical Oncology.


Paul J.F.,Center Chirurgical Marie Lannelongue
The international journal of cardiovascular imaging | Year: 2011

To evaluate the feasibility of an individually adapted coronary CT protocol based on precontrast attenuation values using different kVp and tube current settings. All images were acquired on a 64-slice CT scanner (Sensation 64, Siemens) in 270 consecutive patients. X-ray tube current and kVp settings were defined in 6 groups depending on the noise measurements at the heart level on pre-control unenhanced CT. The contrast medium was 400 mg/ml of iodine given at 1 ml/kg. The duration of injection was at 17 s in all cases, so the flow rate was adapted accordingly. Contrast enhancement and noise were measured from enhanced scans of the aortic root. The contrast to noise ratio (CNR) was used as an indicator of image quality. The mean contrast enhancement was 391 ± 83 HU. The mean radiation dose was 9.4 mSv (1.4-30.4 mSv). The mean noise was 39.8 ± 7.7 HU; noise was not significantly different between the groups, except for the 80 kVp group (mean noise = 54.5 HU, P < 0.05). The mean CNR was 10.1 ± 2.5, and CNR was similar in groups 1, 3, 4, and 5 (scanned at 80 kVp or 120 kVp), but significantly higher (CNR = 12) in group 2 (100 kVp) and significantly lower (CNR = 7.9) in group 6 (140 kVp). Individual adaptation of radiation dose is feasible in routine clinical coronary multislice CT, at various kVp and mAs settings. Image quality may be preserved with substantial radiation dose savings in patients with low attenuation values on precontrast images.


Paul J.F.,Center Chirurgical Marie Lannelongue | Virag R.,Center Dexploration Et Traitement Of Limpuissance
Journal of Sexual Medicine | Year: 2013

Aim. There are men who suffer from unsustainable erections without any identified cause of erectile dysfunction, raising the question if anatomical alterations could be involved. Since early anatomical studies, it has been proposed that to achieve full penile rigidity, the blood must be blocked inside the penis by compression of the deep dorsal vein (DDV), the main venous collector under pubic symphysis. Using a recently developed caverno computed tomography (CT) scan technique, allowing the evaluation of the venous drainage of the corpora cavernosa (CC) during erection, we have studied some anatomical conditions of this important part of the erectile phenomenon. Methods. Puboischial rami angles were measured in axial CT images and calculated strictly on the upper insertion point of the CC, using axial submillimeter slices in 37 patients divided into 3 groups depending on the results of the caverno CT scan: (i) no leak; (ii) superficial veins leaking; and (iii) drainage through the DDV and/or preprostatic plexus. In addition same angles were measured in two randomly unselected populations of men (N=30), and women (N=23) who underwent pelvic CT scan for various reasons, unrelated to their sexual or genital condition. Main Outcome Measures. The angles made by both puboischial rami were measured in patients with and without veno-occlusive dysfunction and in unselected samples of men and women. Results. There is a significantly wider angle made by both puboischial rami in men without complete erection and without evidence of anomalous venous drainage (group 3) (72.2°±4.7°standard deviation [SD]), compared with both men with normal erection (group 1) (57.5±5°SD) P<0.00001, and men with incomplete erection and evidence of anomalous drainage (group 2) (57.7±6°SD) P<0.00001. Conclusions. If confirmed in larger samples, these results raise new questions on the mechanism and the role of these significant anatomical variations, yet unknown, in maintaining or not full rigid erections. © 2012 International Society for Sexual Medicine.


Trehel-Tursis V.,Cardiothoracic Intensive Care Unit | Louvain-Quintard V.,Center Chirurgical Marie Lannelongue | Zarrouki Y.,Cardiothoracic Intensive Care Unit | Imbert A.,Cardiothoracic Intensive Care Unit | And 2 more authors.
Chest | Year: 2012

Background: The diagnosis of heparin-induced thrombocytopenia (HIT) is problematic in the surgical ICU, as there are multiple potential explanations for thrombocytopenia. We conducted a study to assess the incidence, clinical presentation, and outcome of HIT in a cardiothoracic surgical ICU. Methods: From January 2005 to December 2010, all patients with suspicion of HIT were prospectively identified, and data were collected retrospectively. Detection of anti-PF4/heparin antibodies and functional assays were systematically performed. Results: During the study period, 5,949 patients were admitted to the ICU (2,751 after cardiac surgery and 3,198 after thoracic surgery), of whom 101 were suspected to have HIT (1.7% [95% CI, 1.4%-2.0%]). Suspicion of HIT occurred at a median of 5 (4-9) days after ICU admission. Diagnosis was confirmed in 28 of 5,949 patients (0.47% [95% CI, 0.33%-0.68%]). Thrombosis was detected in 14 patients with HIT (50%) and in 12 patients without HIT (16%) (P = .0006). After receiver operating characteristic analysis (area under curve = 0.78 ± 0.06), a 4Ts score ≥ 5 had a sensitiv ity of 86% and a specifi city of 70%. Course of platelet count was similar between the two groups. Six patients (21%) with HIT and 20 (27%) without died ( P = .77). Conclusions: Even with a prospective platelet monitoring protocol, suspicion for HIT arose in < 2% of patients in a cardiothoracic ICU. Most were found to have other causes of thrombocytopenia, with HIT confirmed in 28 of 101 suspected cases (0.47% of all patients in the ICU). The 4Ts score may have value by identifying patients who should have laboratory testing performed. The mortality of patients with HIT was not different from other very ill thrombocytopenic patients in the ICU. © 2012 American College of Chest Physicians.


Durand S.,Center Chirurgical Marie Lannelongue | Paul J.-F.,Institute Mutualiste Montsouris
European Radiology | Year: 2014

Purpose: To evaluate noise level and contrast-to-noise ratio (CNR) with various kVp-mAs pairs producing the same computed tomography dose index (CTDI) value. The 80 kVp and new 70-kVp settings were compared.Materials and methods: The noise was measured in 10 ovoid water phantoms with different diameters from 10 cm to 28 cm. Contrast was obtained from CTs of iodine-filled tubes. Spiral acquisition protocols at 70 kVp and 80 kVp, with the same CTDI, were applied. In the clinical study, two matched groups, each of 21 paediatric patients, underwent 70-kVp or 80-kVp ECG-gated iodinated-enhanced sequential CT.Results: Noise was significantly higher with 70 kVp than 80-kVp settings for all phantom sizes. Estimated CNR with phantoms was higher at 70 kVp than 80 kVp, and the difference decreased from 17 % to 3 % as phantom size increased. The mean CNR in paediatric patients was 15.2 at 70 kVp and 14.3 at 80 kVp (ns). The CNR difference was significantly larger in the small-child subgroup.Conclusion: Noise level is slightly higher at the 70-kVp than the 80-kVp setting, but the CNR is higher, particularly for small children. Therefore, 70 kVp may be appropriate for contrast-enhanced CT examinations and 80 kVp for non-enhanced CT in small children.Key Points: • 70-kVp settings provide a slightly higher noise level compared to 80-kVp settings.• The CNR is higher with 70-kVp than with 80-kVp settings.• Without contrast, 80-kVp settings may be preferable over 70-kVp settings. © 2014, European Society of Radiology.


Durand S.,Center Chirurgical Marie Lannelongue | Paul J.-F.,Institute Mutualiste Montsouris
European Radiology | Year: 2014

Purpose To evaluate noise level and contrast-to-noise ratio (CNR) with various kVp-mAs pairs producing the same computed tomography dose index (CTDI) value. The 80 kVp and new 70-kVp settings were compared. Materials and methods The noise was measured in 10 ovoid water phantoms with different diameters from 10 cm to 28 cm. Contrast was obtained from CTs of iodine-filled tubes. Spiral acquisition protocols at 70 kVp and 80 kVp, with the same CTDI, were applied. In the clinical study, two matched groups, each of 21 paediatric patients, underwent 70-kVp or 80-kVp ECG-gated iodinated-enhanced sequential CT. Results Noise was significantly higher with 70 kVp than 80-kVp settings for all phantom sizes. Estimated CNR with phantoms was higher at 70 kVp than 80 kVp, and the difference decreased from 17 % to 3 % as phantom size increased. The mean CNR in paediatric patients was 15.2 at 70 kVp and 14.3 at 80 kVp (ns). The CNR difference was significantly larger in the small-child subgroup. Conclusion Noise level is slightly higher at the 70-kVp than the 80-kVp setting, but the CNR is higher, particularly for small children. Therefore, 70 kVp may be appropriate for contrast-enhanced CT examinations and 80 kVp for non-enhanced CT in small children. Key Points • 70-kVp settings provide a slightly higher noise level compared to 80-kVp settings.• The CNR is higher with 70-kVp than with 80-kVp settings.• Without contrast, 80-kVp settings may be preferable over 70-kVp settings. © 2014 European Society of Radiology.


Paul J.-F.,Center Chirurgical Marie Lannelongue | Amato A.,Center Chirurgical Marie Lannelongue | Rohnean A.,Center Chirurgical Marie Lannelongue
International Journal of Cardiovascular Imaging | Year: 2013

To compare image quality of coronary CT angiography in step-and-shoot mode at the diastolic phase at low heart rates (<70 bpm) and systolic phase at high heart rates (≥70 bpm). We prospectively included 96 consecutive patients then excluded 5 patients with arrhythmia. Coronary CT-angiography was performed using a dual-source 128-slice CT machine, at the diastolic phase in the 55 patients with heart rates <70 bpm (group D) and at the systolic phase in the 36 patients with heart rates ≥70 (group S). Image quality was scored on a 5 point-scale (1, not interpretable; 2, insufficient for diagnosis; 3, fair, sufficient for diagnosis; 4, good; 5, excellent). In addition, we compared the number of stair-step artifacts in the two groups. Mean image quality score was 4 (0.78) in group D and 4.1 (0.34) in group S (NS), with an unequal distribution (p = 0.01). Step artifacts were seen in 44 % of group D and 18 % of group S patients (p = 0.02). In 3 group D patients and no group S patients, the image score was <3 due to artifacts, requiring repeat CT-angiography. When performing dual-source 128-slice CT-angiography, step-and-shoot acquisition provides comparable mean image quality in systole, with less variability and fewer stair-step artifacts, compared to diastole. This method may be feasible at any heart rate in most patients in sinus rhythm, allowing low-dose prospective acquisition without beta-blocker premedication. © 2012 Springer Science+Business Media, B.V.

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