| Autor(en) |
Titel |
Quelle |
Links |
| Jacobi G, Korinthenberg R, Rutkowski S |
Diagnostik der Hirntumoren. |
Interdisziplinäre Leitlinie der Deutschen Krebsgesellschaft (in Zusammenarbeit der Gesellschaft für Neuropädiatrie und der Gesellschaft für Pädiatrische Onkologie und Hämatologie 2006 |
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| Jaffe ES et al. |
World Health Organization classification of tumours. Pathology and genetics of tumours of hematopoietic and lymphoid tissues. |
IARC Press, Lyon 2001 |
|
| Jaffe N, Bruland O S, Bielack S |
Pediatric and Adolescent Osteosarcoma. |
Cancer Treatment and Research Vol. 152 2010 |
|
| James RM, Kinsey SE |
The investigation and management of chronic neutropenia in children. [+] |
Archives of disease in childhood 2006, 91: 852 |
|
| Unravelling the cause of a neutropenia poses a complex diagnostic challenge. The differential diagnosis ranges from life threatening disease to transient benign causes of little clinical significance. This review offers a practical guide to investigating the neutropenic child, and highlights features that merit specialist referral. Therapeutic options, the role of long term follow up, and the complications of severe chronic neutropenia are considered. |
| Janka-Schaub G, Stuehrk H, Kortuem B, Graubner U, Haas R, Göbel U, Jürgens H, Spaar H, Winkler K |
Bone marrow blast count at day 28 as the single most important prognostic factor in childhood acute lymphoblastic leukemia. |
Haematol Blood Transfus 1992, 34A92211, 233 |
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| Janka-Schaub G |
Immunological factors and childhood leukaemias-pathophysiological and clinical perspectives. |
Anonymous Medizinische Forschung 1993, 183 |
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| Janka-Schaub G, Gobrecht O, Gross S |
Dose intensity and prognosis in acute lymphocytic leukemia in childhood. |
Hämatol Blood Transf 1994, 36 |
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| Janka-Schaub G, Harms D, Goebel U, Graubner U, Gutjahr P, Haas R, Jürgens H, Spaar H, Winkler K |
Randomized comparison of rotational chemotherapy in high-risk acute lymphoblastic leukaemia of childhood--follow up after 9 years. Coall Study Group. |
Eur J Pediatr 1996, 155: 640 |
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| Janka-Schaub G, Harms D, Den Boer M, Veerman A, Pieters R |
In vitro drug resistance as independent prognostic factor in the study COALL-O5-92 Treatment of childhood acute lymphoblastic leukemia; two-tiered classification of treatments based on accepted risk criteria and drug sensitivity profiles in study COALL-06-97. |
Klin Pädiatr 1999, 211: 233 |
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| Janoueix-Lerosey I, Penther D, Thioux M, de Cremoux P, Derre J, Ambros P, Vielh P, Benard J, Aurias A, Delattre O |
Molecular analysis of chromosome arm 17q gain in neuroblastoma. |
Genes Chromosomes Cancer 2000, 28: 276 |
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| Jankovic M, Reciputo A, Haupt R, Micalizzi C, Manganini C, Frey E, Lackner H, Maurus R, Beck J, Langer T, Marx M, Krappmann P, Magyarosy E, Feldges A, Weel-Sipman M |
ELTEC. |
Med Pediatr Oncol 2002, 38 |
|
| Janßen K |
Chemische Umwelttoxikologie, in: Gutjahr P (Hrsg.): Krebs bei Kindern und Jugendlichen. |
Deutscher Ärzte-Verlag Köln 5. Aufl. 2004, 64 |
|
| Janka GE |
Familial and acquired hemophagocytic lymphohistiocytosis. [+] |
European journal of pediatrics 2007, 166: 95 |
|
| Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition of severe hyperinflammation caused by the uncontrolled proliferation of activated lymphocytes and histiocytes secreting high amounts of inflammatory cytokines. Cardinal signs and symptoms are prolonged fever, hepatosplenomegaly and pancytopenia. Characteristic biochemical markers include elevated triglycerides, ferritin and low fibrinogen. HLH occurs on the basis of various inherited or acquired immune deficiencies. Impaired function of natural killer (NK) cells and cytotoxic T-cells (CTL) is shared by all forms of HLH. Genetic HLH occurs in familial forms (FHLH) in which HLH is the primary and only manifestation, and in association with the immune deficiencies Chédiak-Higashi syndrome 1 (CHS 1), Griscelli syndrome 2 (GS 2) and x-linked lymphoproliferative syndrome (XLP), in which HLH is a sporadic event. Most patients with acquired HLH have no known underlying immune deficiency. Both acquired and genetic forms are triggered by infections, mostly viral, or other stimuli. HLH also occurs as a complication of rheumatic diseases (macrophage activation syndrome) and of malignancies. Several genetic defects causing FHLH have recently been discovered and have elucidated the pathophysiology of HLH. The immediate aim of therapy in genetic and acquired HLH is suppression of the severe hyperinflammation, which can be achieved with immunosuppressive/immunomodulatary agents and cytostatic drugs. Patients with genetic forms have to undergo stem cell transplantation to exchange the defective immune system with normally functioning immune effector cells.In conclusion, awareness of the clinical symptoms and of the diagnostic criteria of HLH is crucial in order not to overlook HLH and to start life-saving therapy in time. |
| Janka GE |
Hemophagocytic syndromes. [+] |
Blood reviews 2007, 21: 245 |
|
| Hemophagocytic syndromes (hemophagocytic lymphohistiocytosis, HLH) represent a severe hyperinflammatory condition with the cardinal symptoms prolonged fever, cytopenias, hepatosplenomegaly, and hemophagocytosis by activated, morphologically benign macrophages. Biochemical markers include elevated ferritin and triglycerides, and low fibrinogen. Whereas in children several inherited immune deficiencies may lead to this syndrome, most adults with HLH have no known underlying immune defect. Nevertheless, impaired function of natural killer (NK) cells and cytotoxic T-cells (CTL) is characteristic for both genetic and acquired forms of HLH. Frequent triggers are infectious agents, mostly viruses of the herpes group. Malignant lymphomas, especially in adults, may be associated with HLH. A special form of HLH in rheumatic diseases is called macrophage-activation syndrome. Initially HLH may masquerade as a normal infection since all symptoms, even though less pronounced, may also be found in immune competent patients. Patients with HLH, however, cannot control the hyperinflammatory response which, if untreated, is fatal in genetic cases and in a high percentage of acquired cases. Awareness of the clinical symptoms and of the diagnostic criteria of HLH is important to start life-saving therapy with immunosuppressive/immunomodulatory agents in time. |
| Janka GE |
Familial and acquired hemophagocytic lymphohistiocytosis. [+] |
Annual review of medicine 2012, 63: 233 |
|
| Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome in which an uncontrolled and ineffective immune response, triggered in most cases by infectious agents, leads to severe hyperinflammation. Familial forms of HLH (FHL), which are increasingly found also in adolescents and adults, are due to genetic defects leading to impaired function of natural killer cells and cytotoxic T cells. These mutations occur either in the perforin gene or in genes important for the exocytosis of cytotoxic granules. Cytotoxic granules contain perforin and granzymes, which induce apoptosis upon entering (infected) target cells. Additionally, perforin is important for the downregulation of the immune response. Acquired forms of HLH are encountered in association with (usually) viral infections, autoinflammatory/autoimmune diseases, malignant diseases, and acquired immune deficiency states (e.g., after organ transplantation). Treatment of HLH includes immune-suppressive and immune-modulatory agents, cytostatic drugs, and biological response modifiers. For patients with FHL, stem cell transplantation is indicated and can be curative. |
| Jenkin D, Goddard K, Armstrong D, Becker L, Berry M, Chan H, Doherty M, Greenberg M, Hendrick B, Hoffman H, et al. |
Posterior fossa medulloblastoma in childhood: treatment results and a proposal for a new staging system. [+] |
Int J Radiat Oncol Biol Phys 1990, 19: 265 |
|
| Seventy-two children with posterior fossa medulloblastoma were diagnosed at the Hospital for Sick Children, Toronto, from 1977 to 1987 and treated by standard methods. The 5- and 10-year survival and disease-free survival rates were 71% and 63%, and 64% and 63%, respectively. Total tumor resection, as determined by the surgeon was the most significant favorable prognostic factor. Post-operative meningitis, a residual enhancing mass lesion on the post-operative, pre irradiation CT scan and dissemination to the brain or cord at diagnosis were unfavorable factors. These four easily definable factors were used to define a staging system with prognostic significance. Five-year disease-free survival rates were for Stage I (total resection, no adverse factor) 100%, Stage II (total resection with one or more adverse factor or less than total resection with no other adverse factor) 78%, and Stage III (less than total resection with one or more adverse factor) 18%. Evaluation of treatment results in medulloblastoma requires that these prognostic factors be known. |
| Jensen M, Ernestus K, Kemshead J, Klehr M, Von Bergwelt-Baildon MS, Schinkothe T, Schultze JL, Berthold F |
The bi-specific CD3 x NCAM antibody: a model to preactivate T cells prior to tumour cell lysis. [+] |
Clinical and experimental immunology 2003, 134: 253 |
|
| To target the neural cell adhesion molecule (NCAM, CD56) on neuroblastoma by T cell-based immunotherapy we have generated a bi-specific CD3 x NCAM antibody (OE-1). This antibody can be used to redirect T cells to NCAM+ cells. Expectedly, the antibody binds specifically to NCAM+ neuroblastoma cells and CD3+ T cells. OE-1 induces T cell activation, expansion and effector function in peripheral blood mononuclear cell (PBMC)-derived CD4+ and CD8+ T cells. T cell activation was shown to depend on the presence of normal natural killer (NK) cells in the culture. Interestingly, while PBMC- derived T cells were activated by OE-1, NK cells were almost completely depleted, suggesting that T cells activated by OE-1 deleted the NK cells. Activated CD4+ and CD8+ T cells differentiate into a larger CCR7+ central memory and a smaller CCR7- effector memory cell population. Most importantly, preactivated T cells were highly cytotoxic for neuroblastoma cells. In eight of 11 experiments tumour-directed cytotoxicity was enhanced when NK cells were present during preactivation with OE-1. These data strongly support a bi-phasic therapeutic concept of primarily stimulating T cells with the bi-specific antibody in the presence of normal NCAM+ cells to induce T cell activation, migratory capacity and finally tumour cell lysis. |
| Jensen M, Tawadros S, Sedlacek HH, Schultze JL, Berthold F |
NK cell depletion diminish tumour-specific B cell responses. [+] |
Immunology letters 2004, 93(2-3): 205 |
|
| Natural killer (NK) cells can exercise immediate cytotoxicity against malignant cells and thus far modulate the development of tumour directed T cell immunity. To investigate the impact of NK cells on the development of tumour directed B cell immunity mice were immunised with IMR5-75 human neuroblastoma cells with or without prior in vivo NK cell depletion. Flow cytometry analyses gave evidence for an impaired IgG response against the cells immunised with. Dissection of Th1 (IgG2a) and Th2 (IgG1) oriented B cell responses revealed Th1 responses as primarily affected, while Th2 oriented B cell responses as measured by flow cytometry and GD2 ganglioside-specific ELISA were enforced. The data reveal an unexpected impact of NK cells on the development of tumour directed B cell responses. Consequently, NK cell function has also to be taken into account when developing B cell-based cancer immunotherapy. |
| Johnson P, Glennie M |
The mechanisms of action of rituximab in the elimination of tumor cells. |
Semin Oncol 2003, 30 (Suppl 2): 3 |
|
| Jordan MB, Allen CE, Weitzman S, Filipovich AH, McClain KL |
How I treat hemophagocytic lymphohistiocytosis. [+] |
Blood 2011, 118: 4041 |
|
| Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of pathologic immune activation, occurring as either a familial disorder or a sporadic condition, in association with a variety of triggers. This immune dysregulatory disorder is prominently associated with cytopenias and a unique combination of clinical signs and symptoms of extreme inflammation. Prompt initiation of immunochemotherapy is essential for survival, but timely diagnosis may be challenging because of the rarity of HLH, its variable presentation, and the time required to perform diagnostic testing. Therapy is complicated by dynamic clinical course, high risk of treatment-related morbidity, and disease recurrence. Here, we review the clinical manifestations and patterns of HLH and describe our approach to the diagnosis and therapy for this elusive and potentially lethal condition. |
| Jozwiak S, Schwartz RA, Janniger CK, Bielicka-Cymerman J |
Usefulness of diagnostic criteria of tuberous sclerosis complex in pediatric patients. |
J Child Neurol 2000, 15: 652 |
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| Juergens C, Weston C, Lewis I, Whelan J, Paulussen M, Oberlin O, Michon J, Zoubek A, Jürgens H, Craft A |
Safety assessment of intensive induction with vincristine, ifosfamide, doxorubicin, and etoposide (VIDE) in the treatment of Ewing tumors in the EURO-E. W.I. N.G. 99 clinical trial. |
Pediatric blood & cancer 2006, 47: 22 |
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| Jumaa H, Bossaller L, Portugal K, Storch B, Lotz M, Flemming A, Schrappe M, Postila V, Riikonen P, Pelkonen J, Niemeyer C, Reth M |
Deficiency of the adaptor SLP-65 in pre-B-cell acute lymphoblastic leukaemia. |
Nature 2003, 423: 452 |
|
| Jung R, Jacobs U, Krumbholz M, Langer T, Keller T, De Lorenzo P, Valsecchi MG, van der Velden VH, Moericke A, Stanulla M, Teigler-Schlegel A, Panzer-Gruemayer ER, van Dongen JJ, Schrappe M, den Boer ML, Pieters R, Rascher W, Metzler M |
Bimodal distribution of genomic MLL breakpoints in infant acute lymphoblastic leukemia treatment. |
Leukemia 2010, 24: 903 |
|
| Jurklies C |
Das Retinoblastom - Diagnose und Therapie. |
WIR Informationsschrift der Aktion für krebskranke Kinder e.V. (Bonn) 2007, 1: 26 |
|
| Jürgens H, Janka-Schaub G, Ibrahim M, Tonert C, Winkler K, Göbel U |
Prognostic significance of exposure to intermediate-dose Methotrexate in children with standard risk ALL:the COALL 82/85 experience. |
Hämatol Blood Transf 1992, 34: 238 |
|
| Jürgens H |
Interdisciplinary therapy of Ewing sarcoma. |
Schweiz Rundsch Med Prax 1995, 84: 1005 |
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| Jürgens H, Ritter J |
Lungenmetastasen bei Malignomen im Kindesalter. |
Z Herz-Thorax-Gefäáchir 1996, 10: 9 |
|
| Jürgens H |
Recent advances in childhood cancer. |
Eur J Cancer 1997, 33: 15 |
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| Juergens C, Weston C, Lewis I, Whelan J, Paulussen M, Oberlin O, Michon J, Zoubek A, Jürgens H, Craft A |
Safety assessment of intensive induction with vincristine, ifosfamide, doxorubicin, and etoposide (VIDE) in the treatment of Ewing tumors in the EURO-E. W.I. N.G. 99 clinical trial. [+] |
Pediatric blood & cancer 2006, 47: 22 |
|
| BACKGROUND: The EUROpean Ewing tumour Working Initiative of National Groups 1999 (EURO-E.W.I.N.G. 99) protocol prescribes six courses of vincristine, ifosfamide, doxorubicin, and etoposide (VIDE) as intensive induction chemotherapy for Ewing tumors (ET). Granulocyte-colony stimulating factor (G-CSF) is recommended. Adverse reactions (AR) were evaluated; quality assurance of data collection reviewed. PROCEDURE: Safety data from 4,746 courses of VIDE in 851 patients less than 50 years with ET were collected using a checklist and evaluated using descriptive statistics with sub-groups including gender, age, and tumor volume, analyzed by Wilcoxon and Kruskal-Wallis tests. RESULTS: Myelosuppression and infections were the major AR but with appropriate supportive therapy targeted dose intensity was maintained. Five VIDE-related deaths with three due to sepsis were reported. Renal and cardiac toxicity were reflected by glomerular filtration rate (GFR) <39 ml/min/1.73 m2 in 0.1%, tubular phosphate reabsorption < or = 0.80 in 1.9%, and left ventricular shortening fracture <28% in 2.5% VIDE courses. Statistically significant gender-associated AR concerning hemoglobin and platelets were observed with females > males as were age-associated AR concerning hemoglobin, WBC, platelets, stomatitis, and vomiting with AR decreasing with age, that is, children > adolescents > adults. No association of AR to tumor volume was found. In VIDE courses with and without G-CSF, neutropenia-related fever in 60.8% and 65.8%, and infection in 54.7% and 61.0% courses, respectively, were recorded. CONCLUSIONS: AR under VIDE remained within the expected range. Some AR, for example, hematotoxicity were significantly influenced by age and gender but not by tumor volume. G-CSF did not significantly influence neutropenia-related fever and infection. Solicited safety collection with checklists adequately reflects the burden per course. |
| Jürgens H, Paulussen M, Zoubek A |
Ewing-Tumoren, in: Gadner H, Gaedicke G, Niemeyer CH, Ritter J (Hrsg.): Pädiatrische Hämatologie und Onkologie. |
Springer Verlag 2006, 894 |
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