| Autor(en) |
Titel |
Quelle |
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| Naik RP, Streiff MB, Haywood C Jr, Nelson JA, Lanzkron S |
Venous thromboembolism in adults with sickle cell disease: a serious and under-recognized complication. [+] |
The American journal of medicine 2013, 126: 443 |
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| Sickle cell disease is recognized as a hypercoagulable state; however, the frequency and characteristics of venous thromboembolism in sickle cell patients have not been well defined. The purpose of this study was to establish the prevalence and risk factors for venous thromboembolism in a large cohort of patients with sickle cell disease and determine the relationship between venous thromboembolism and mortality. |
| Namouni F, Doz F, Tanguy ML, Quintana E, Michon J, Pacquement H, Bouffet E, Gentet JC, Plantaz D, Lutz P, Vannier JP, Validire P, Neuenschwander S, Desjardins L, Zucker JM |
High-dose chemotherapy with carboplatin, etoposide and cyclophosphamide followed by a haematopoietic stem cell rescue in patients with high-risk retinoblastoma: a SFOP and SFGM study. [+] |
European journal of cancer (Oxford, England : 1990) 1997, 33: 2368 |
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| This study investigates the role of high-dose chemotherapy with haematopoietic stem cell rescue as consolidation treatment in high-risk retinoblastoma (extraocular disease at diagnosis or relapse or invasion of cut end of optic nerve). 25 patients received high-dose chemotherapy including carboplatin (250 mg/m2/day from day 1 to day 5 for the 6 first patients and 350 mg/m2/day from day 1 to day 5 for the other patients), etoposide (350 mg/m2/day from day 1 to day 5) and cyclophosphamide (1.6 g/m2/day from day 2 to day 5) (CARBOPEC) followed by autologous haematopoietic stem cell rescue. 19 patients received this drug combination for chemosensitive extraocular relapse. The other 6 patients with histological high-risk factors were given this treatment as consolidation after enucleation and conventional chemotherapy. The three year disease-free survival was 67.1%. In 7 of the 9 relapsing patients, the first site of relapse was the central nervous system. All patients with central nervous system disease died except one. The main toxicity was haematological and digestive (mucositis and diarrhoea). 2 of the 13 evaluable patients had grade III and IV ototoxicity. One patient experienced an acute grade I reversible cardiotoxicity. The CARBOPEC regimen seems to be a promising therapeutic strategy in patients with high-risk retinoblastoma, especially those with bone and/or bone marrow involvement. This treatment did not improve the outcome of patients with central nervous system disease. |
| The National Academies - National Research Council |
Report in Brief. Beir VII: Health Risks from Exposure to Low Levels of Ionizing Radiation. |
National Academy Press 2006 |
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| Nazar GB, Hoffman HJ, Becker LE, Jenkin D, Humphreys RP, Hendrick EB |
Infratentorial ependymomas in childhood: prognostic factors and treatment. |
J Neurosurg 1990, 72: 408 |
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| Needle MN, Goldwein JW, Grass J, Cnaan A, Bergman I, Molloy P, Sutton L, Zhao H, Garvin JH Jr, Phillips PC |
Adjuvant chemotherapy for the treatment of intracranial ependymoma of childhood. [+] |
Cancer 1997, 80: 341 |
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| BACKGROUND: Current treatment for childhood intracranial ependymomas with surgery and radiation therapy (RT) yields 5-year survival rates ranging from 50-70% after complete resection to 0-30% after incomplete surgical resection. The role of chemotherapy in the treatment of ependymoma has not been established. In this pilot study, children with newly diagnosed intracranial ependymoma were treated with RT and chemotherapy using agents comparable to those found to be active in the treatment of intracranial ependymoma in infants. METHODS: Nineteen children age 3-14 years (median, 7.5 years) were treated with postoperative RT and chemotherapy. Chemotherapy was comprised of carboplatin, 560 mg/m2, with vincristine, 1.5 mg/m2, weekly for 3 weeks, alternating at 4-week intervals with ifosfamide, 1.8 g/m2, and etoposide, 100 mg/m2, for 5 consecutive days for a total of 4 cycles. RESULTS: The 5-year progression free survival (PFS) estimate was 74%. The extent of surgical resection was not a significant prognostic factor in this study. By contrast, ependymomas located in the posterior fossa were associated with a higher rate of progression (P = 0.036). Toxicity, limited predominantly to myelosuppression, was manageable. CONCLUSIONS: The PFS for children with postoperative residual ependymoma treated with RT and chemotherapy in this study was higher than published survival results for RT alone. These results suggest a role for multialkylator chemotherapy in incompletely resected intracranial ependymoma and provide the rationale for a randomized trial comparing this strategy with conventional postoperative RT. |
| Nenning UC, Eckert C, Wellmann S, Barth A, Henze G, Seeger K |
Re: Prognostic significance of a short sequence insertion in the MCL-1 promoter in chronic lymphocytic leukemia. |
Journal of the National Cancer Institute 2005, 97: 1091-2; author reply 1093 |
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| Neth O, Seidemann K, Jansen P, Mann G, Tiemann M, Ludwig W, Riehm H, Reiter A |
Precursor B-cell lymphoblastic lymphoma in childhood and adolescence. |
Med Pediatr Oncol 2000, 35: 20 |
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| Neuendank A, Hartmann R, Fengler R, Erttmann R, Zintl F, Koscielniak E, Henze G |
Interim results of a phase II study with idarubicin in relapsed childhood acute lymphoblastic leukemia. |
Haematol Blood Transfus 1995, 254 |
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| von Neuhoff C, Reinhardt D, Sander A, Zimmermann M, Bradtke J, Betts DR, Zemanova Z, Stary J, Bourquin JP, Haas OA, Dworzak MN, Creutzig U |
Prognostic Impact of Specific Chromosomal Aberrations in a Large Group of Pediatric Patients With Acute Myeloid Leukemia Treated Uniformly According to Trial AML-BFM 98. [+] |
Journal of clinical oncology 2010, 28: 2682 |
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| PURPOSE Because cytogenetic data are essential for risk stratification of childhood acute myeloid leukemia (AML), the impact of chromosomal aberrations is crucial. PATIENTS AND METHODS Data of a large group of patients younger than 18 years treated according to study AML-Berlin-Frankfurt-Münster (BFM) 98 (n = 454), including their cytogenetics, were analyzed. Results The favorable outcome in the subgroups of patients with t(8;21), inv(16), and t(15;17), with an overall survival of 91% (SE, 4%), 92% (SE, 6%), and 87% (SE, 5%), respectively, was confirmed. Within this group, the 5-year probability of event-free survival (pEFS) of all 17 children with t(8;21) and additional aberrations apart from del(9q) or -X/-Y was 100%. As expected, the cytogenetic finding of a complex karyotype (n = 35; pEFS, 33%; SE, 8%) or a monosomy 7 (n = 12; pEFS, 17%; SE, 11%) was associated with a poor outcome. Compared with remaining patients with cytogenetic data (pEFS, 48%; SE, 2%), prognosis in patients with an MLL rearrangement (n = 91) was inferior (pEFS, 34%; SE, 5%; P = .0005). Particularly, children with t(9;11) and additional aberrations (n = 13; pEFS, 31%; SE, 14%) and MLL rearrangements other than t(9;11) and t(11;19) (n = 41; pEFS, 24%; SE, 7%) had an unfavorable outcome. Nine patients with aberrations in 12p showed an adverse prognosis (pEFS, 11%; SE, 10%). The outcome of patients with aberrations of chromosome 5 (n = 13) was better than expected (pEFS, 50%; SE, 13%). CONCLUSION Because the prognostic value of rare recurrent chromosomal aberrations still has to be elucidated, these data will contribute to future risk stratification for the treatment of pediatric AML. |
| Neunert C, Lim W, Crowther M, Cohen A, Solberg L Jr, Crowther MA, American Society of Hematology |
The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia. [+] |
Blood 2011; 117: 4190 |
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| Immune thrombocytopenia (ITP) is commonly encountered in clinical practice. In 1996 the American Society of Hematology published a landmark guidance paper designed to assist clinicians in the management of this disorder. Since 1996 there have been numerous advances in the management of both adult and pediatric ITP. These changes mandated an update in the guidelines. This guideline uses a rigorous, evidence-based approach to the location, interpretation, and presentation of the available evidence. We have endeavored to identify, abstract, and present all available methodologically rigorous data informing the treatment of ITP. We provide evidence-based treatment recommendations using the GRADE system in those areas in which such evidence exists. We do not provide evidence in those areas in which evidence is lacking, or is of lower quality--interested readers are referred to a number of recent, consensus-based recommendations for expert opinion in these clinical areas. Our review identified the need for additional studies in many key areas of the therapy of ITP such as comparative studies of |
| Neunert CE |
Individualized treatment for immune thrombocytopenia: predicting bleeding risk. [+] |
Seminars in hematology 2013, 50 Suppl 1:S55 |
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| Treatment of patients with immune thrombocytopenia (ITP) is often directed at increasing the platelet count and preventing significant hemorrhage even when there is minimal bleeding present. This approach, however, requires that a large number of patients receive prophylactic treatment to prevent major bleeding events. Identification of initial risk factors for development of severe bleeding would allow for more directed and personalized therapy. This review provides a summary of the current literature with the intent to explore various clinical and laboratory risk factors for severe bleeding including mucosal bleeding, platelet count, and aspects of platelet function. |
| Newton HB, Henson J, Walker RW |
Extraneural metastases in ependymoma. |
J Neurooncol 1992, 14: 135 |
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| Nicholson JC, Punt J, Hale J, Saran F, Calaminus G, Germ Cell Tumour Working Groups of the United Kingdom Children's Cancer Study Group (UKCCSG) and International Society of Paediatric Oncology (SIOP) |
Neurosurgical management of paediatric germ cell tumours of the central nervous system--a multi-disciplinary team approach for the new millennium. |
British journal of neurosurgery 2002, 16: 93 |
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| Niemeyer C, Stollmann-Gibbels B, Ebell W, Gaedicke G, Creutzig U |
Myelodysplastic diseases in childhood. |
Klin Pädiatr 1992, 204: 190 |
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| Niemeyer C, Stollmann-Gibbels B, Ebell W, Gaedicke G, Creutzig U |
Myelodysplastic diseases in childhood. |
Klin Pädiatr 1992, 204: 190 |
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| Niethammer D, Dopfer R, Henze G, Bender-Götze C, Burdach S, Ebell W, Ehninger G, Friedrich W, Gadner H, Klingebiel T, Peters C, Riehm H, Suttorp M |
Allogeneic bone marrow transplantation for childhood acute lymphoblastic leukemia in second remission as part of the treatment strategies within the German BFM-and CoALL-protocols. |
Haematol Blood Transfus 1992, 34 |
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| Niemeyer C, Arico M, Basso G, Biondi A, Cantu Rajnoldi, Creutzig U, Haas O, Harbott J, Hasle H, Kerndrup G, Locatelli F, Mann G, Stollmann-Gibbels B, Veer-Korthof E, van Wering E, Zimmermann M |
Chronic myelomonocytic leukemia in childhood. |
Blood 1997, 89: 3534 |
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| Niemeyer C, Fenu S, Hasle H, Mann G, Stary J, van Wering E |
Differentiating juvenile myelomonocytic leukemia from infectious disease. |
Blood 1998, 91: 365 |
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| Niehues T, Kapaun P, Harms D, Burdach S, Kramm C, Körholz D, Janka-Schaub G, Göbel U |
A classification based on T cell selection-related phenotypes identifies a subgroup of childhood T-ALL with favorable outcome in the COALL studies. |
Leukemia 1999, 13: 614 |
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| Niemeyer C |
Myelodysplastic syndrome and aplastic anemia in children. |
Europ School of Oncology 2000, 31 |
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| Niehuis T, Schellong G, Dörffel W, Bucsky P, Gadner H, Körholz D, Göbel U |
Immunodeficiency and Hodgkin's disease. |
Klin Pädiatr 2003,im Druck |
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| Niemeyer C, Kratz C |
Juvenile myelomonocytic leukemia. |
Curr Treat Options Oncol 2003, 4: 203 |
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| Niemeyer C, Kontny U |
Tumoren der Ewing-Sarkom-Familie, in: Gutjahr P (Hrsg.): Krebs bei Kindern und Jugendlichen. |
Deutscher Ärzte-Verlag Köln 5. Aufl. 2004, 491 |
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| Nielsen P, Gaedicke, G |
Physiologie und Pathophysiologie des Eisenstoffwechsels. |
In: Helmut Gadner, Gerhard Gaedicke, Charlotte Niemeyer (Hrsg.): Pädiatrische Hämatologie und Onkologie 2006 |
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| Niewerth D, Creutzig U, Bierings MB, Kaspers GJ |
A review on allogeneic stem cell transplantation for newly diagnosed pediatric acute myeloid leukemia. [+] |
Blood 2010, |
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| Survival of pediatric acute myeloid leukemia (AML) has improved considerably over the past decades. Since 1985, allo-SCT is widely recommended for patients that have a matched sibling donor. However, it remains controversial whether allo-SCT is superior to chemotherapy for newly diagnosed children with AML. This review summarizes phase III clinical trials which compared allo-SCT with chemotherapy (including auto-SCT) in pediatric AML, excluding studies that did not use the intention-to-treat analysis or correct for time-to-transplant. While allo-SCT might prevent more relapses than chemotherapy, the number needed to transplant (with allo-SCT) in order to prevent one relapse is in the order of 10 patients. Moreover, overall survival is similar with both modalities in most recent studies, apparently because of increased salvagability of a relapse when initial therapy concerned chemotherapy only, and because of a higher treatment-related mortality (TRM) with allo-SCT. Because allo-SCT also gives more severe side-effects and results more often in secondary malignancies than chemotherapy, we do not recommend allo-SCT in first remission for pediatric AML in general. Further research should focus on the possibility that subgroups might benefit from allo-SCT, aiming at further improvements in the prognosis of pediatric AML. |
| Niemeyer CM, Kang MW, Shin DH, Furlan I, Erlacher M, Bunin NJ, Bunda S, Finklestein JZ, Sakamoto KM, Gorr TA, Mehta P, Schmid I, Kropshofer G, Corbacioglu S, Lang PJ, Klein C, Schlegel PG, Heinzmann A, Schneider M, Starý J, van den Heuvel-Eibrink MM, Hasle H, Locatelli F, Sakai D, Archambeault S, Chen L, Russell RC, Sybingco SS, Ohh M, Braun BS, Flotho C, Loh ML |
Germline CBL mutations cause developmental abnormalities and predispose to juvenile myelomonocytic leukemia. [+] |
Nat Genet 2010, 42: 794 |
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| CBL encodes a member of the Cbl family of proteins, which functions as an E3 ubiquitin ligase. We describe a dominant developmental disorder resulting from germline missense CBL mutations, which is characterized by impaired growth, developmental delay, cryptorchidism and a predisposition to juvenile myelomonocytic leukemia (JMML). Some individuals experienced spontaneous regression of their JMML but developed vasculitis later in life. Importantly, JMML specimens from affected children show loss of the normal CBL allele through acquired isodisomy. Consistent with these genetic data, the common p.371Y>H altered Cbl protein induces cytokine-independent growth and constitutive phosphorylation of ERK, AKT and S6 only in hematopoietic cells in which normal Cbl expression is reduced by RNA interference. We conclude that germline CBL mutations have developmental, tumorigenic and functional consequences that resemble disorders that are caused by hyperactive Ras/Raf/MEK/ERK signaling and include neurofibromatosis type 1, Noonan syndrome, Costello syndrome, cardiofaciocutaneous syndrome and Legius syndrome. |
| Niemeyer CM, Baumann I |
Classification of childhood aplastic anemia and myelodysplastic syndrome. [+] |
Hematology / the Education Program of the American Society of Hematology. American Society of Hematology. Education Program 2011, 2011, 84 |
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| Hypoplastic BM disorders in children and adolescents comprise a broad spectrum of disorders. Acquired severe aplastic anemia (SAA), refractory cytopenia of childhood (RCC), a subtype of myelodysplastic syndrome (MDS), and inherited BM failure (IBMF) disorders are the main and most difficult hematological differential diagnoses. Whereas IBMF disorders can often be diagnosed by their clinical features and/or underlying genetic aberrations, the morphological distinction between SAA and hypocellular RCC has been controversial. The histopathological pattern of RCC consists of islands of immature erythroid precursors accompanied by sparsely distributed granulocytic cells. Megakaryocytes are significantly decreased or absent and, rarely, micromegakaryocytes are detected on immunohistochemistry. Because fatty tissue between areas of hematopoiesis can mimic SAA, 2 biopsies are recommended to facilitate the detection of representative BM spaces. Recent data indicate that the response to immunosuppressive therapy is inferior in RCC compared with SAA. Furthermore, approaches to allogeneic hematopoietic transplantation differ. Controlled prospective clinical studies in patients with hypoplastic BM failure disorders will require comprehensive guidelines for diagnosing SAA, RCC, and the different IBMF disorders. |
| Nitz A, Kontopantelis E, Bielack S, Koscielniak E, Klingebiel T, Langer T, Paulides M |
Prospective evaluation of cisplatin- and carboplatin-mediated ototoxicity in paediatric and adult soft tissue and osteosarcoma patients. [+] |
Oncology letters 2013, 5: 311 |
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| Platinum-compound chemotherapy is known to have ototoxic side-effects. However, there is a paucity of literature examining hearing function prospectively and longitudinally in cohorts containing paediatric and adult patients treated within the same cisplatin- or carboplatin-containing treatment trial protocols. In Germany, Austria and Switzerland, late effects of treatment for osteosarcoma and soft tissue sarcoma have been prospectively and longitudinally registered by the Late Effects Surveillance System since 1998. The aim of this study was to analyse cisplatin- and carboplatin-induced ototoxity in a group of 129 osteosarcoma and soft tissue sarcoma patients treated within the COSS-96, CWS-96 and CWS-2002P treatment trials. The cohort consisted of 112 children and 17 adults. The median age at diagnosis was 13.56 (IQR, 10.26-16.27) years. Follow-up was 6.97 (IQR, 0.87-15.63) months. Hearing function was examined by audiometry before and after platinum treatment. A total of 108 patients were treated with cisplatin with a median cumulative dose of 360 mg/m(2). Thirteen patients received carboplatin with a median cumulative dose of 1500 mg/m(2) and 8 patients were treated with both platinum compounds (median cisplatin dose, 240 mg/m(2); IQR, 240-360 mg/m(2) and median carboplatin dose: 1200 mg/m(2); IQR, 600-3000 mg/m(2)). Following cessation of therapy, 47.3% of the patients demonstrated a hearing impairment, namely 55 children (49.1%) and 6 adults (42.1%). Out of thirteen children treated with carboplatin with a cumulative dose of 1500 mg/m(2), six revealed a significant hearing impairment. Although ototoxicity caused by platinum compounds is considered irreversible, we identified hearing improvements over time in 11 children (9.8%) and 3 adults (17.6%). None of these patients received irradiation to the head. We conclude that hearing loss is frequent in children treated with protocols containing platinum compounds and recommend prospective testing via audiometry. |
| Nowak-Göttl U, Schaudin E, Hoffmann C, Eckhoff-Donovan S, Mertes N, Winkelmann W, Jürgens H |
Intraoperative clotting factor dilution and activated hemostasis in children with Ewing's sarcoma or osteosarcoma. |
haematologica 1995, 80: 311 |
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| Nowak-Göttl U, Münchow N, Klippel U, Paulussen M, Bielack S, Ullrich K, Ehrenforth S |
The course of fibrinolytic proteins in children with malignant bone tumours. |
Eur J Pediatr 1999, 158 Suppl 3:S151-S153 |
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| Nowacki S, Skowron M, Oberthuer A, Fagin A, Voth H, Brors B, Westermann F, Eggert A, Hero B, Berthold F, Fischer M |
Expression of the tumour suppressor gene CADM1 is associated with favourable outcome and inhibits cell survival in neuroblastoma. [+] |
Oncogene 2008, 27: 3329 |
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| Cell adhesion molecule 1 (CADM1) is a putative tumour suppressor gene, which is downregulated in many solid tumours. In neuroblastoma, loss of CADM1 expression has recently been found in disseminated tumours with adverse outcome, prompting us to investigate its role in neuroblastoma tumour progression. Oligonucleotide-microarray analysis of 251 neuroblastoma specimens demonstrated that CADM1 downregulation is associated with unfavourable prognostic markers like disseminated stage 4, age >18 months, MYCN amplification and chromosome 11q alterations (P<0.001 each). Furthermore, low CADM1 expression was significantly correlated with unfavourable gene expression-based classification (P<0.001) and adverse patient outcome (P<0.001). Bisulphite sequencing and genetic analysis of 18 primary neuroblastomas suggested that neither haploinsufficiency nor hypermethylation is regularly involved in CADM1 gene silencing in neuroblastoma, which is in contrast to results obtained in other malignancies. In addition, no mutations disrupting the CADM1 reading frame were found in 25 primary neuroblastomas. Over-expression of CADM1 in neuroblastoma cells resulted in significant reduction of proliferation, viability and colony formation in soft agar. Collectively, our results suggest that downregulation of CADM1 tumour suppressor gene expression is a critical event in neuroblastoma pathogenesis resulting in tumour progression and unfavourable patient outcome. |
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