9 results on '"De Sainte Marie B"'
Search Results
2. [Health status and quality of life in β-thalassemia adults in Marseille, France].
- Author
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Soubrier C, Jean E, De Sainte Marie B, Agouti I, Seguier J, Lavoipierre V, Clapasson C, Iline N, Gonin J, Giorgi R, Schleinitz N, Thuret I, Badens C, and Bernit E
- Subjects
- Humans, France epidemiology, Male, Female, Adult, Retrospective Studies, Young Adult, Middle Aged, Blood Transfusion statistics & numerical data, Iron Overload epidemiology, Iron Overload etiology, Surveys and Questionnaires, Adolescent, Quality of Life, beta-Thalassemia therapy, beta-Thalassemia epidemiology, beta-Thalassemia complications, beta-Thalassemia psychology, Health Status
- Abstract
Introduction: The life expectancy of β-thalassemia patients has increased over the last 20 years. In this study, we evaluated the current health status and quality of life of these patients managed in a reference center in Marseille., Methods: This is a single-center, descriptive study conducted between June and August 2019 in patients over 18 years of age with β-thalassemia major or intermedia. Clinical and paraclinical data were collected retrospectively and the SF-36 health survey questionnaire was proposed to each patient., Results: 43 of 64 selected patients were included and divided into 2 groups: 35 patients with transfusion-dependent β-thalassemia and 8 patients with non-transfusion-dependent β-thalassemia. Liver iron overload is the most frequent complication, present in 80% of transfusion-dependent and 62.5% of non-transfusion-dependent patients. Cardiac iron overload is present only in the transfusion dependent β-thalassemia group (20%). Hypogonadotropic hypogonadism remains the most common endocrine disorder (41.9%) followed by osteoporosis (37.2%). Among the 31 patients who completed the SF-36 questionnaire, physical and mental quality of life scores were lowered in transfusion dependent (respectively 42.7 and 46.8) as in non-transfusion-dependent patients (respectively 43.8 and 28.9)., Conclusion: Despite an improvement in medical care, our patients with β-thalassemia show an alteration in their quality of life that will need to be characterized in the entire French cohort., (Copyright © 2024 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)
- Published
- 2024
- Full Text
- View/download PDF
3. Pleuropulmonary Manifestations of Vacuoles, E1 Enzyme, X-Linked, Autoinflammatory, Somatic (VEXAS) Syndrome.
- Author
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Borie R, Debray MP, Guedon AF, Mekinian A, Terriou L, Lacombe V, Lazaro E, Meyer A, Mathian A, Ardois S, Vial G, Moulinet T, Terrier B, Jamilloux Y, Heiblig M, Bouaziz JD, Zakine E, Outh R, Groslerons S, Bigot A, Flamarion E, Kostine M, Henneton P, Humbert S, Constantin A, Samson M, Bertrand NM, Biscay P, Dieval C, Lobbes H, Jeannel J, Servettaz A, Adelaide L, Graveleau J, de Sainte-Marie B, Galland J, Guillotin V, Duroyon E, Templé M, Bourguiba R, Georgin Lavialle S, Kosmider O, and Audemard-Verger A
- Subjects
- Male, Humans, Aged, Female, Prednisone, Lung diagnostic imaging, Lung pathology, Syndrome, Mutation, Vacuoles, Pulmonary Fibrosis pathology
- Abstract
Background: The vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is a newly identified autoinflammatory disorder related to somatic UBA1 mutations. Up to 72% of patients may show lung involvement., Research Question: What are the pleuropulmonary manifestations in VEXAS syndrome?, Study Design and Methods: One hundred fourteen patients were included in the French cohort of VEXAS syndrome between November 2020 and May 2021. Each patient included in the study who had an available chest CT scan was discussed in an adjudication multidisciplinary team and classified as showing potentially pleuropulmonary-specific involvement of VEXAS syndrome or others., Results: Fifty-one patients had a CT scan available for review and 45 patients (39%) showed pleuropulmonary abnormalities on chest CT scan that were considered related to VEXAS syndrome after adjudication. Most patients were men (95%) with a median age 67.0 years at the onset of symptoms. Among these 45 patients, 44% reported dyspnea and 40% reported cough. All 45 patients showed lung opacities on chest CT scan (including ground-glass opacities [87%], consolidations [49%], reticulation [38%], and septal lines [51%]) and 53% of patients showed pleural effusion. Most patients showed improvement with prednisone, but usually required > 20 mg/d. The main clinical and biological features as well the median survival did not differ between the 45 patients with pleuropulmonary involvement and the rest of the cohort, suggesting that the prevalence of pleuropulmonary involvement might have been underdiagnosed in the rest of the cohort., Interpretation: Pulmonary manifestations are frequent in VEXAS syndrome, but rarely are at the forefront. The initial outcome is favorable with prednisone and does not seem to lead to pulmonary fibrosis., (Copyright © 2022 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.)
- Published
- 2023
- Full Text
- View/download PDF
4. [Why must we integrate carbon footprint in our prescriptions? The example of inhaler prescribing].
- Author
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de Sainte Marie B, Arnal P, Seguier J, Faucher B, Gaigne L, Briantais A, Bermudez J, Ebbo M, Schleinitz N, and Andre B
- Subjects
- Humans, Prescriptions, Carbon Footprint, Nebulizers and Vaporizers
- Published
- 2022
- Full Text
- View/download PDF
5. [Eye and skin lesions].
- Author
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Gomes de Pinho Q, de Sainte Marie B, Seguier J, Ebbo M, and Schleinitz N
- Subjects
- Humans, Cryopyrin-Associated Periodic Syndromes, Skin Diseases diagnosis, Urticaria
- Published
- 2022
- Full Text
- View/download PDF
6. A descriptive study of IgG4-related disease in children and young adults.
- Author
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de Sainte Marie B, Ebbo M, Grados A, Rebours V, Reumaux H, Briantais A, Urbina D, Cury J, Morel N, Lhote F, Rohmer B, Lazaro E, Agbo-Kpati KP, Deroux A, Domont F, Delacroix I, Lavigne C, Perlat A, Kahn JE, Godeau B, Hamidou M, Launay D, Bader-Meunier B, and Schleinitz N
- Subjects
- Child, Humans, Immunoglobulin G, Young Adult, Immunoglobulin G4-Related Disease diagnosis
- Published
- 2022
- Full Text
- View/download PDF
7. [Bilateral adrenal hemorrhage under apixaban in primary antiphospholipid syndrome].
- Author
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Lavoipierre V, Talbot M, Soubrier C, De Sainte Marie B, Seux V, Solas C, Morange P, Bernit E, Ebbo M, Faucher B, Harlé JR, and Schleinitz N
- Subjects
- Adrenal Gland Diseases diagnosis, Adrenal Glands drug effects, Adrenal Glands pathology, Antiphospholipid Syndrome complications, Hemorrhage diagnosis, Humans, Male, Middle Aged, Necrosis chemically induced, Necrosis diagnosis, Pyrazoles therapeutic use, Pyridones therapeutic use, Venous Thromboembolism complications, Venous Thromboembolism drug therapy, Adrenal Gland Diseases chemically induced, Antiphospholipid Syndrome diagnosis, Hemorrhage chemically induced, Pyrazoles adverse effects, Pyridones adverse effects
- Abstract
Introduction: Adrenal hemorrhage is a classical but rare complication of antiphospholipid syndrome, revealing diagnosis in one third of the cases. Anti-vitamin K therapy is the standard treatment but direct oral anticoagulants are discussed as an alternative. In the latest recommendations, it is advised not to use direct oral anticoagulants in the setting of antiphospholipid syndrome., Case Report: We present a case of bilateral adrenal hemorrhage revealing primary antiphospholipid syndrome with triple positive antibody profile, in a 47-year-old man treated by apixaban for previous venous thromboembolism., Conclusion: To our knowledge, it is the first case of adrenal hemorrhage occurring during apixaban treatment in a patient with antiphospholipid syndrome. This case illustrates the inefficacy of direct oral anticoagulants to prevent thrombotic events in antiphospholipid syndrome, in accordance with the latest recommendations., (Copyright © 2019 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)
- Published
- 2020
- Full Text
- View/download PDF
8. [Sudden epigastric pain].
- Author
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de Sainte Marie B, Coze S, Grégoire E, Jean R, Durand JM, and Chiche L
- Subjects
- Abdominal Pain etiology, Abdominal Pain surgery, Animals, Bone and Bones, Diagnosis, Differential, Duodenitis etiology, Duodenitis surgery, Duodenum pathology, Duodenum surgery, Endoscopy, Digestive System, Fishes, Foreign Bodies diagnosis, Foreign Bodies surgery, Humans, Intestinal Perforation complications, Intestinal Perforation surgery, Male, Middle Aged, Peritonitis etiology, Peritonitis surgery, Tomography, X-Ray Computed, Abdominal Pain diagnosis, Duodenitis diagnosis, Duodenum injuries, Foreign Bodies complications, Intestinal Perforation diagnosis, Peritonitis diagnosis
- Published
- 2015
- Full Text
- View/download PDF
9. Leptospirosis presenting as honeymoon fever.
- Author
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de Sainte Marie B, Delord M, Dubourg G, Gautret P, Parola P, Brouqui P, and Lagier JC
- Subjects
- Adult, Anti-Bacterial Agents therapeutic use, Ceftriaxone therapeutic use, Colombia, Diagnosis, Differential, Doxycycline therapeutic use, Female, Fever drug therapy, Fever microbiology, France, Humans, Leptospirosis complications, Leptospirosis drug therapy, Malaria diagnosis, Male, Rickettsia Infections diagnosis, Travel, Treatment Outcome, Typhoid Fever diagnosis, Fever diagnosis, Leptospirosis diagnosis
- Abstract
An increasing number of travelers from western countries visit tropical regions, questioning western physicians on the prophylaxis, the diagnosis and the therapeutic management of patients with travel-associated infection. In July 2014, a French couple stayed for an adventure-travel in Columbia without malaria prophylaxis. A week after their return the woman presented with fever, myalgia, and retro-orbital pain. Three days later, her husband presented similar symptoms. In both patients, testing for malaria, arboviruses and blood cultures remained negative. An empirical treatment with doxycycline and ceftriaxone was initiated for both patients. Serum collected from the female patient yielded positive IgM for leptospirosis but was negative for her husband. Positive Real-Time PCR were observed in blood and urine from both patients, confirming leptospirosis. Three lessons are noteworthy from this case report. First, after exclusion of malaria, as enteric fever, leptospirosis and rickettsial infection are the most prevalent travel-associated infections, empirical treatment with doxycycline and third generation cephalosporin should be considered. In addition, the diagnosis of leptospirosis requires both serology and PCR performed in both urine and blood samples. Finally, prophylaxis using doxycycline, also effective against leptospirosis, rickettsial infections or travellers' diarrhea should be recommended for adventure travelers in malaria endemic areas., (Copyright © 2015 The Authors. Published by Elsevier Ltd.. All rights reserved.)
- Published
- 2015
- Full Text
- View/download PDF
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