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Christine Bellanné Chantelot - One of the best experts on this subject based on the ideXlab platform.
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REVIEW Open Access Congenital Neutropenia: diagnosis, molecular bases and patient management
2013Co-Authors: Jean Donadieu, Odile Fenneteau, Ine Beaupain, Nizar Mahlaoui, Christine Bellanné ChantelotAbstract:The term congenital Neutropenia encompasses a family of neutropenic disorders, both permanent and intermittent, severe (<0.5 G/l) or mild (between 0.5-1.5 G/l), which may also affect other organ systems such as the pancreas, central nervous system, heart, muscle and skin. Neutropenia can lead to life-threatening pyogenic infections, acute gingivostomatitis and chronic parodontal disease, and each successive infection may leave permanent sequelae. The risk of infection is roughly inversely proportional to the circulating polymorphonuclear neutrophil count and is particularly high at counts below 0.2 G/l. When Neutropenia is detected, an attempt should be made to establish the etiology, distinguishing between acquired forms (the most frequent, including post viral Neutropenia and auto immune Neutropenia) and congenital forms that may either be isolated or part of a complex genetic disease. Except for ethnic Neutropenia, which is a frequent but mild congenital form, probably with polygenic inheritance, all other forms of congenital Neutropenia are extremely rare and have monogenic inheritance, which may be X-linked or autosomal, recessive or dominant. About half the forms of congenital Neutropenia with no extra-hematopoetic manifestations and normal adaptive immunity are due to neutrophil elastase (ELANE) mutations. Some patients have severe permanent neutropeni
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Congenital Neutropenia: diagnosis, molecular bases and patient management
Orphanet Journal of Rare Diseases, 2011Co-Authors: Jean Donadieu, Odile Fenneteau, Nizar Mahlaoui, Blandine Beaupain, Christine Bellanné ChantelotAbstract:The term congenital Neutropenia encompasses a family of neutropenic disorders, both permanent and intermittent, severe (
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congenital Neutropenia diagnosis molecular bases and patient management
Orphanet Journal of Rare Diseases, 2011Co-Authors: Jean Donadieu, Odile Fenneteau, Nizar Mahlaoui, Blandine Beaupain, Christine Bellanné ChantelotAbstract:The term congenital Neutropenia encompasses a family of neutropenic disorders, both permanent and intermittent, severe (<0.5 G/l) or mild (between 0.5-1.5 G/l), which may also affect other organ systems such as the pancreas, central nervous system, heart, muscle and skin. Neutropenia can lead to life-threatening pyogenic infections, acute gingivostomatitis and chronic parodontal disease, and each successive infection may leave permanent sequelae. The risk of infection is roughly inversely proportional to the circulating polymorphonuclear neutrophil count and is particularly high at counts below 0.2 G/l. When Neutropenia is detected, an attempt should be made to establish the etiology, distinguishing between acquired forms (the most frequent, including post viral Neutropenia and auto immune Neutropenia) and congenital forms that may either be isolated or part of a complex genetic disease. Except for ethnic Neutropenia, which is a frequent but mild congenital form, probably with polygenic inheritance, all other forms of congenital Neutropenia are extremely rare and have monogenic inheritance, which may be Xlinked or autosomal, recessive or dominant. About half the forms of congenital Neutropenia with no extra-hematopoetic manifestations and normal adaptive immunity are due to neutrophil elastase (ELANE) mutations. Some patients have severe permanent Neutropenia and frequent infections early in life, while others have mild intermittent Neutropenia. Congenital Neutropenia may also be associated with a wide range of organ dysfunctions, as for example in Shwachman-Diamond syndrome (associated with pancreatic insufficiency) and glycogen storage disease type Ib (associated with a glycogen storage syndrome). So far, the molecular bases of 12 neutropenic disorders have been identified. Treatment of severe chronic Neutropenia should focus on prevention of infections. It includes antimicrobial prophylaxis, generally with trimethoprim-sulfamethoxazole, and also granulocyte-colony-stimulating factor (G-CSF). G-CSF has considerably improved these patients’ outlook. It is usually well tolerated, but potential adverse effects include thrombocytopenia, glomerulonephritis, vasculitis and osteoporosis. Long-term treatment with G-CSF, especially at high doses, augments the spontaneous risk of leukemia in patients with congenital Neutropenia.
Mary L. Disis - One of the best experts on this subject based on the ideXlab platform.
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American Journal of Hematology 72:204–208 (2003) Fatal Bacillus cereus Sepsis Following Resolving Neutropenic Enterocolitis During the Treatment of Acute Leukemia
2013Co-Authors: Amy Sarah Ginsburg, Lupe G. Salazar, Lawrence D. True, Mary L. DisisAbstract:Bacillus cereus is increasingly being acknowledged as a serious bacterial pathogen in immunosuppressed hosts. We report a case of fatal B. cereus sepsis in a patient with newly diagnosed acute leukemia following resolving neutropenic enterocolitis. Gastrointestinal complaints are common during induction chemotherapy, yet some antimicrobial coverage suitable for generalized Neutropenia is not optimal for the eradication of B. cereus. This case demonstrates that, in the neutropenic patient with gastrointestinal complaints or in the setting of resolving neutropenic enterocolitis, it is important to anticipate possible B. cereus infection and sepsis. Am. J. Hematol. 72:204–208, 2003. © 2003 Wiley-Liss, Inc. Key words: Bacillus cereus; gastrointestinal; leukemia; neutropeni
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Fatal Bacillus cereus sepsis following resolving neutropenic enterocolitis during the treatment of acute leukemia.
American journal of hematology, 2003Co-Authors: Amy Sarah Ginsburg, Lupe G. Salazar, Lawrence D. True, Mary L. DisisAbstract:Bacillus cereus is increasingly being acknowledged as a serious bacterial pathogen in immunosuppressed hosts. We report a case of fatal B. cereus sepsis in a patient with newly diagnosed acute leukemia following resolving neutropenic enterocolitis. Gastrointestinal complaints are common during induction chemotherapy, yet some antimicrobial coverage suitable for generalized Neutropenia is not optimal for the eradication of B. cereus. This case demonstrates that, in the neutropenic patient with gastrointestinal complaints or in the setting of resolving neutropenic enterocolitis, it is important to anticipate possible B. cereus infection and sepsis.
Francesca Fioredda - One of the best experts on this subject based on the ideXlab platform.
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infectious complications in children with severe congenital autoimmune or idiopathic Neutropenia a retrospective study from the italian Neutropenia registry
Pediatric Infectious Disease Journal, 2013Co-Authors: Francesca Fioredda, Michaela Calvillo, Sonia Bonanomi, Fabio Tucci, Roberta Ghilardi, Oriana Burlando, Francesca Riccardi, Ilaria Caviglia, Baldassare Martire, Piero FarruggiaAbstract:We describe the incidence and characteristics of infections in children with severe congenital Neutropenia (SCN), autoimmune Neutropenia (AN) and idiopathic Neutropenia (IN). Data extracted from the Italian Neutropenia Registry on 73 patients with 108 episodes of infections were collected from 2000 to 2009. All SCN patients with SCN and one third of AN and IN experienced at least 1 infectious episode, equating to 5.7 infections/patient in SCN and approximately 0.6 in AN and IN. The rate of infections before diagnosis of Neutropenia was 6.35/1000 patient-days at risk in SCN, 0.48 in AN and 0.71 in IN (P < 0.001) and significantly decreased after diagnosis. Skin and subcutaneous abscesses and cellulitis were the most frequent types of infection encountered, followed by pneumonia. Infections are an important clinical issue in the management of neutropenic patients, even in those considered at lower risk.
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congenital and acquired Neutropenias consensus guidelines on therapy and follow up in childhood from the Neutropenia committee of the marrow failure syndrome group of the aieop associazione italiana emato oncologia pediatrica
American Journal of Hematology, 2012Co-Authors: Francesca Fioredda, Michaela Calvillo, Sonia Bonanomi, Tiziana Coliva, Fabio Tucci, Piero Farruggia, Marta Pillon, Baldassarre Martire, Roberta Ghilardi, Ugo RamenghiAbstract:The management of congenital and acquired Neutropenias presents some differences according to the type of the disease. Treatment with recombinant human granulocyte-colony stimulating factor (G-CSF) is not standardized and scanty data are available on the best schedule to apply. The frequency and the type of longitudinal controls in patients affected with Neutropenias are not usually discussed in the literature. The Neutropenia Committee of the Marrow Failure Syndrome Group (MFSG) of the Associazione Italiana di Emato-Oncologia Pediatrica (AIEOP) elaborated this document following design and methodology formerly approved by the AIEOP board. The panel of experts reviewed the literature on the topic and participated in a conference producing a document that includes recommendations on Neutropenia treatment and timing of follow-up.
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a prospective study on the epidemiology of febrile episodes during chemotherapy induced Neutropenia in children with cancer or after hemopoietic stem cell transplantation
Clinical Infectious Diseases, 2007Co-Authors: Elio Castagnola, Francesca Fioredda, Ilaria Caviglia, Vincenzo Fontana, Silvia Caruso, Maura Faraci, Maria Luisa Garre, Cristina Moroni, Massimo Conte, Giuseppe LosurdoAbstract:Background. The purpose of our study was to evaluate the incidence and clinical characteristics of febrile episodes during Neutropenia following chemotherapy in children with cancer. Patients and methods. A prospective, 3-year single-center observational study of periods of Neutropenia was performed. Epidemiology and clinical diagnoses of febrile episodes occurring during the neutropenic periods were evaluated, taking into consideration different categories of anticancer treatment based on the type of tumor and phase of therapy. Results. A total of 703 febrile episodes were observed during 614 (34%) of 1792 neutropenic periods (34%), for a total of 28,001 days at risk, accounting for a rate of 0.76 episodes per 30 days at risk. The highest proportions of neutropenic periods with primary febrile episodes were observed after autologous hemopoietic stem cell transplantation (58%), aggressive treatment for acute leukemia or non-Hodgkin lymphoma (48%), and allogeneic hemopoietic stem cell transplantation (44%); the lowest proportion (9%) was observed during maintenance chemotherapy for acute leukemia (P<.001). The most frequent clinical diagnosis was fever of unknown origin (in 79% of cases), followed by bacteremia (10%); invasive mycosis was diagnosed in only 2% of cases. Conclusions. The overall incidence of febrile Neutropenia and severe infectious complications in children with cancer is low, with differences according to the aggressiveness of chemotherapy. This fact must be considered when designing clinical trials on the management of infectious complications in children with cancer.
Jean Donadieu - One of the best experts on this subject based on the ideXlab platform.
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REVIEW Open Access Congenital Neutropenia: diagnosis, molecular bases and patient management
2013Co-Authors: Jean Donadieu, Odile Fenneteau, Ine Beaupain, Nizar Mahlaoui, Christine Bellanné ChantelotAbstract:The term congenital Neutropenia encompasses a family of neutropenic disorders, both permanent and intermittent, severe (<0.5 G/l) or mild (between 0.5-1.5 G/l), which may also affect other organ systems such as the pancreas, central nervous system, heart, muscle and skin. Neutropenia can lead to life-threatening pyogenic infections, acute gingivostomatitis and chronic parodontal disease, and each successive infection may leave permanent sequelae. The risk of infection is roughly inversely proportional to the circulating polymorphonuclear neutrophil count and is particularly high at counts below 0.2 G/l. When Neutropenia is detected, an attempt should be made to establish the etiology, distinguishing between acquired forms (the most frequent, including post viral Neutropenia and auto immune Neutropenia) and congenital forms that may either be isolated or part of a complex genetic disease. Except for ethnic Neutropenia, which is a frequent but mild congenital form, probably with polygenic inheritance, all other forms of congenital Neutropenia are extremely rare and have monogenic inheritance, which may be X-linked or autosomal, recessive or dominant. About half the forms of congenital Neutropenia with no extra-hematopoetic manifestations and normal adaptive immunity are due to neutrophil elastase (ELANE) mutations. Some patients have severe permanent neutropeni
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Congenital Neutropenia: diagnosis, molecular bases and patient management
Orphanet Journal of Rare Diseases, 2011Co-Authors: Jean Donadieu, Odile Fenneteau, Nizar Mahlaoui, Blandine Beaupain, Christine Bellanné ChantelotAbstract:The term congenital Neutropenia encompasses a family of neutropenic disorders, both permanent and intermittent, severe (
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congenital Neutropenia diagnosis molecular bases and patient management
Orphanet Journal of Rare Diseases, 2011Co-Authors: Jean Donadieu, Odile Fenneteau, Nizar Mahlaoui, Blandine Beaupain, Christine Bellanné ChantelotAbstract:The term congenital Neutropenia encompasses a family of neutropenic disorders, both permanent and intermittent, severe (<0.5 G/l) or mild (between 0.5-1.5 G/l), which may also affect other organ systems such as the pancreas, central nervous system, heart, muscle and skin. Neutropenia can lead to life-threatening pyogenic infections, acute gingivostomatitis and chronic parodontal disease, and each successive infection may leave permanent sequelae. The risk of infection is roughly inversely proportional to the circulating polymorphonuclear neutrophil count and is particularly high at counts below 0.2 G/l. When Neutropenia is detected, an attempt should be made to establish the etiology, distinguishing between acquired forms (the most frequent, including post viral Neutropenia and auto immune Neutropenia) and congenital forms that may either be isolated or part of a complex genetic disease. Except for ethnic Neutropenia, which is a frequent but mild congenital form, probably with polygenic inheritance, all other forms of congenital Neutropenia are extremely rare and have monogenic inheritance, which may be Xlinked or autosomal, recessive or dominant. About half the forms of congenital Neutropenia with no extra-hematopoetic manifestations and normal adaptive immunity are due to neutrophil elastase (ELANE) mutations. Some patients have severe permanent Neutropenia and frequent infections early in life, while others have mild intermittent Neutropenia. Congenital Neutropenia may also be associated with a wide range of organ dysfunctions, as for example in Shwachman-Diamond syndrome (associated with pancreatic insufficiency) and glycogen storage disease type Ib (associated with a glycogen storage syndrome). So far, the molecular bases of 12 neutropenic disorders have been identified. Treatment of severe chronic Neutropenia should focus on prevention of infections. It includes antimicrobial prophylaxis, generally with trimethoprim-sulfamethoxazole, and also granulocyte-colony-stimulating factor (G-CSF). G-CSF has considerably improved these patients’ outlook. It is usually well tolerated, but potential adverse effects include thrombocytopenia, glomerulonephritis, vasculitis and osteoporosis. Long-term treatment with G-CSF, especially at high doses, augments the spontaneous risk of leukemia in patients with congenital Neutropenia.
Melissa M Hudson - One of the best experts on this subject based on the ideXlab platform.
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oral cefixime is similar to continued intravenous antibiotics in the empirical treatment of febrile neutropenic children with cancer
Clinical Infectious Diseases, 2001Co-Authors: Jerry L Shenep, Donald K Baker, Patricia M Flynn, Seth Hetherington, Melissa M HudsonAbstract:Empiric oral antibiotic therapy for febrile neutropenic cancer patients has been suggested as a means to decrease hospitalization, but the safety of this approach has not been adequately studied in children. We compared continued iv antibiotic therapy with switching treatment to orally administered cefixime in a group of selected febrile neutropenic children for whom blood cultures were sterile after 48 h of incubation. Two hundred episodes of febrile Neutropenia were studied (156 patients), and 100 episodes were randomized to receive each treatment. Failure to respond to therapy was defined by documented or suspected bacterial infection, recurrent fever, or discontinuation of assigned therapy for any reason before Neutropenia resolved. Rates of treatment failure were similar in the oral cefixime group (28%) and in the iv antibiotic group (27%; P=1.0). Results support the safety of oral cefixime therapy for low-risk febrile neutropenic children, a therapeutic approach that would facilitate earlier outpatient management and decrease the costs of treatment.
