Research Topics
Species | Jean DonadieuSummaryCountry: France Publications
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Publications
[Granulopoeisis and leukemogenesis: lessons from congenital neutropenia]Jean Donadieu
Service d hémato oncologie pédiatrique, Registre français des neutropénies congénitales, Hopital Trousseau, 26 avenue du Docteur Arnold Netter, 75012 Paris, France
Med Sci (Paris) 24:284-9. 2008..Leukemia risk does not appear to be related to an oncogenic effect of ELA2 mutations, but much likely to the deepness of the neutropenia, and the intensity of G-CSF therapy...
Congenital neutropenia: diagnosis, molecular bases and patient managementJean Donadieu
Service d Hémato Oncologie Pédiatrique Registre des neutropénies congénitales, AP HP Hopital Trousseau, 26 avenue du Dr Netter, Paris, France
Orphanet J Rare Dis 6:26. 2011..Long-term treatment with G-CSF, especially at high doses, augments the spontaneous risk of leukemia in patients with congenital neutropenia...
Estimation of the radiation dose from thoracic CT scans in a cystic fibrosis populationJean Donadieu
Institut de Veille Sanitaire, Santé et Environnement, 12 Rue du Val d Osne, Saint Maurice 94415, France
Chest 132:1233-8. 2007..To determine the radiation exposure associated with CT scans in a population of patients with cystic fibrosis (CF)...
[Clinical presentation and epidemiology of childhood Langerhans cell Histiocytosis]J Donadieu
, AP-HP, , , 26 av. du Dr Netter, 75012 Paris
Arch Pediatr 15:520-2. 2008
Mutations in the ELA2 gene correlate with more severe expression of neutropenia: a study of 81 patients from the French Neutropenia RegisterChristine Bellanne-Chantelot
Hopital Saint Antoine, Laboratoire de d Embryologie Pathologique et de Cytogénétique, 184 rue du Fbg Saint Antoine, 75012 Paris, France
Blood 103:4119-25. 2004....
Analysis of risk factors for myelodysplasias, leukemias and death from infection among patients with congenital neutropenia. Experience of the French Severe Chronic Neutropenia Study GroupJean Donadieu
Service d hémato oncologie pédiatrique, Hopital Trousseau, Paris, France
Haematologica 90:45-53. 2005..Granulocyte colony-stimulating factor (G-CSF) therapy has significantly reduced the frequency and severity of infections, but its possible influence on the risk of malignancy is not known...
Is pegfilgrastim safe and effective in congenital neutropenia? An analysis of the French Severe Chronic Neutropenia registryBlandine Beaupain
Service d hémato oncologie pédiatrique, Registre Français des Neutropénies Chroniques Sévères, Centre de Référence des Déficits Immunitaires Héréditaires, Hopital Trousseau, Paris, France
Pediatr Blood Cancer 53:1068-73. 2009..To examine the efficacy and safety of pegfilgrastim in patients with congenital neutropenia (CN)...
A single-centre study can improve our knowledge of a rare disease! As exemplified by studies in Langerhans' cell histiocytosisJean Donadieu
Service d hémato oncologie pédiatrique, Hopital Trousseau, Paris, France
Acta Paediatr 94:1010-1. 2005..This study also offers evidence that some sequelae are due to radiotherapy, which should therefore be excluded from the treatment of LCH. The analysis of causes of death shows the important role of haematological dysfunction...
B-cell and T-cell phenotypes in CVID patients correlate with the clinical phenotype of the diseaseGael Mouillot
Immunology Laboratory, INSERM UMR S945, CIB Pitié Salpêtrière, Assistance Publique Hopitaux de Paris, Paris, France
J Clin Immunol 30:746-55. 2010..Common variable immunodeficiency (CVID) is a heterogeneous disorder characterized by recurrent infections and defective immunoglobulin production...
Prevalence of vernal keratoconjunctivitis: a rare disease?D Bremond-Gignac
Service d Ophtalmologie, Hopital Robert Debre, APHP, 48 Boulevard Serurier, 75019 Paris, France
Br J Ophthalmol 92:1097-102. 2008..To determine the prevalence of vernal keratoconjuntivitis (VKC) in Europe...
Late-onset combined immune deficiency: a subset of common variable immunodeficiency with severe T cell defectMarion Malphettes
Departement d Immunologie, Hopital Saint Louis, Assistance Publique Hopitaux de Paris, Paris, France
Clin Infect Dis 49:1329-38. 2009..Common variable immunodeficiency (CVID) is a primary immune deficiency defined by defective antibody production. In most series, a small proportion of patients present with opportunistic infections (OIs)...
Cholesteatoma secondary to temporal bone involvement by Langerhans cell histiocytosis: a complication amenable to curative surgeryGilles Roger
ENT Department, Armand Trousseau Children s Hospital, University Paris VI, AP HP, Paris, France
Otol Neurotol 30:190-3. 2009..To describe secondary acquired cholesteatoma in patients previously treated for Langerhans cell histiocytosis (LCH). To focus on misleading symptoms suggesting LCH relapse...
Cumulative effective doses delivered by radiographs to preterm infants in a neonatal intensive care unitJean Donadieu
Département Santé et Environnement, Institut de Veille Sanitaire, St Maurice, France
Pediatrics 117:882-8. 2006..We sought to determine the number and distribution of radiographs and the cumulative effective radiograph doses (cED) received by a population of preterm infants (PIs) hospitalized in an NICU...
Endocrine involvement in pediatric-onset Langerhans' cell histiocytosis: a population-based studyJean Donadieu
Service d hémato oncologie pédiatrique, Hôpital Trousseau and Orphanet, Hopital Broussais, Paris, France
J Pediatr 144:344-50. 2004..To document the frequency and outcome of endocrine involvement in pediatric-onset Langerhans' cell histiocytosis (LCH), and the association with other types of organ involvement...
A new clinical score for disease activity in Langerhans cell histiocytosisJean Donadieu
Hopital Trousseau, 26 avenue du Dr Netter, Paris, France
Pediatr Blood Cancer 43:770-6. 2004..To develop an objective tool for assessing disease activity in patients with Langerhans cell histiocytosis (LCH)...
Efficacy of vinblastine in central nervous system Langerhans cell histiocytosis: a nationwide retrospective studySophie Ng Wing Tin
APHP UPMC, Service de Neurologie 2 Mazarin, Groupe Hospitalier Pitie Salpetriere, Paris, France
Orphanet J Rare Dis 6:83. 2011..Vinblastine (VBL) is the standard treatment for systemic Langerhans cell histiocytosis (LCH), but little is known about its efficacy in central nervous system (CNS) mass lesions...
Retrospective French nationwide survey of childhood aggressive vascular anomalies of bone, 1988-2009Sébastien Héritier
Service d hématologie oncologie pédiatrique, Centre de référence des histiocytoses, AP HP Hôpital Armand Trousseau, Paris, France
Orphanet J Rare Dis 5:3. 2010..To document the epidemiological, clinical, histological and radiological characteristics of aggressive vascular abnormalities of bone in children...
Incidence of growth hormone deficiency in pediatric-onset Langerhans cell histiocytosis: efficacy and safety of growth hormone treatmentJean Donadieu
, Hopital Trousseau, 75012 Paris, France
J Clin Endocrinol Metab 89:604-9. 2004..Among patients with pituitary involvement, the number of LCH disease episodes appeared not significantly influenced by GHD or GH administration, suggesting an absence of deleterious effect of GH therapy on LCH disease activity...
Complications and treatment of patients with ?-thalassemia in France: results of the National RegistryIsabelle Thuret
Service d Hématologie Pédiatrique, Hopital d Enfants de la Timone, Paris, France
Haematologica 95:724-9. 2010..Epidemiological and clinical data have been collected on living patients with beta-thalassemia major or intermedia, including those who underwent hematopoietic stem cell transplantation...
Herpes-virus infection in patients with Langerhans cell histiocytosis: a case-controlled sero-epidemiological study, and in situ analysisEric Jeziorski
Laboratory of Biology of the Mononuclear Phagocyte System, INSERM U838, University Paris Descartes, Paris, France
PLoS ONE 3:e3262. 2008..Epstein-barr virus (EBV, HHV-4), & Cytomegalovirus (CMV, HHV-5) can infect Langerhans cells, and EBV, CMV and HHV-6 have been proposed to be associated with LCH based on the detection of these viruses in clinical samples...
Expansion of regulatory T cells in patients with Langerhans cell histiocytosisBrigitte Senechal
INSERM, U838, Laboratory of Biology of the Mononuclear Phagocyte System, Necker Enfants Malades Institute, Paris, France
PLoS Med 4:e253. 2007..The present study was designed to further investigate the nature of proliferating cells and the immune mechanisms involved in the LCH granulomas...
Hematopoietic stem cell transplantation for Shwachman-Diamond syndrome: experience of the French neutropenia registryJ Donadieu
Service d hématologie et oncologie pédiatrique, Hopital Trousseau, Paris, France
Bone Marrow Transplant 36:787-92. 2005..The overall 5-year event-free survival rate is 60+/-15%. We conclude that HSCT is feasible for patients with SDS who develop bone marrow failure or malignant transformation...
Multi-centre pilot study of 2-chlorodeoxyadenosine and cytosine arabinoside combined chemotherapy in refractory Langerhans cell histiocytosis with haematological dysfunctionF Bernard
Service d hémato oncologie pédiatrique, Hopital Arnaud de Villeneuve, CHU de Montpellier, 34000 Montpellier, France
Eur J Cancer 41:2682-9. 2005..This series is small, but we conclude that 2-CdA and Ara-C combined chemotherapy probably has major activity in childhood refractory Langerhans cell histiocytosis...
Descriptive epidemiology of childhood Langerhans cell histiocytosis in France, 2000-2004Aurélie Guyot-Goubin
French National Registry of Childhood Hematological malignancies NRCH, Villejuif, France
Pediatr Blood Cancer 51:71-5. 2008..The French National Registry of Childhood Hematopoietic Malignancies (NRCH) has recorded LCH cases of all subtypes since 2000. The present study describes the data on LCH collected on a national scale over a 5-year period...
Epigenetic regulation of protein-coding and microRNA genes by the Gfi1-interacting tumor suppressor PRDM5Zhijun Duan
Division of Medical Genetics, Department of Medicine, University of Washington School of Medicine, Seattle, WA 98195, USA
Mol Cell Biol 27:6889-902. 2007..In neutropenic patients, we identified PRDM5 protein sequence variants perturbing transcriptional function, suggesting a potentially important role in hematopoiesis...
Risk and benefit of treatment of severe chronic neutropenia with granulocyte colony-stimulating factorTammy E Cottle
Severe Chronic Neutropenia International Registry, Department of Medicine, School of Medicine, University of Washington, Seattle, WA 98101, USA
Semin Hematol 39:134-40. 2002..Analysis of data from patients who received filgrastim for up to 11 years did not identify any adverse events associated with increased duration of treatment...
No association between Langerhans cell histiocytosis and human herpes virus 8Michel Slacmeulder
Department of Pediatric Oncology, Institut Gustave Roussy, Villejuif, France
Med Pediatr Oncol 39:187-9. 2002
Langerhans cell histiocytosis: portrait of a disease as a rare tumourJean Donadieu
Eur J Cancer 40:1465-6. 2004
Is G-CSF-mobilized peripheral stem cell harvest harmful?Jean Donadieu
Pediatr Blood Cancer 48:594-5; author reply 595-7. 2007
[Incidence of radiodermatitis following interventional radiology. Results of a feasibility study among members of the French Dermatology Society]Candice Roudier
Institut de Veille Sanitaire, , Saint-Maurice (94
Presse Med 35:955-9. 2006..We recommend the development of a reporting system for this adverse effect as part of the optimization of interventional radiology practices, including dose reduction...
Distinct patterns of mutations occurring in de novo AML versus AML arising in the setting of severe congenital neutropeniaDaniel C Link
Division of Oncology, Department of Medicine, Washington University, St Louis, MO 63110, USA
Blood 110:1648-55. 2007..These data support the hypothesis that mutations of CSF3R may provide the "activated tyrosine kinase signal" that is thought to be important for leukemogenesis...
Reactivations in multisystem Langerhans cell histiocytosis: data of the international LCH registryMilen Minkov
St Anna Children s Hospital, Vienna, Austria
J Pediatr 153:700-5, 705.e1-2. 2008..To assess multisystem Langerhans cell histiocytosis reactivation and its impact on morbidity and mortality...
Improved outcome in multisystem Langerhans cell histiocytosis is associated with therapy intensificationHelmut Gadner
Children s Cancer Research Institute, St Anna Children s Hospital, Vienna, Austria
Blood 111:2556-62. 2008..042). We conclude that intensified treatment significantly increases rapid response and reduces mortality in risk MS-LCH. This trial was registered at http://www.controlled-trials.com as no. ISRCTN57679341...
18F-FDG PET in neurodegenerative Langerhans cell histiocytosis : results and potential interest for an early diagnosis of the diseaseMaria Joao Ribeiro
Service Hospitalier Frederic Joliot, DRM DSV, CEA 4, place du General Leclerc, 91406 Orsay, France
J Neurol 255:575-80. 2008..MRI is the gold standard to investigate CNS lesions of ND-LCH but little is known about functional changes observed in this disease...
