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  • 1
    Language: English
    In: Pediatrics, June 2014, Vol.133(6), pp.e1759-63
    Description: Atypical hemolytic uremic syndrome (aHUS) is a genetic, life-threatening, chronic disease that can affect patients of all ages. aHUS is caused by uncontrolled complement activation due to genetic defects of complement regulation. Plasma exchange or infusion has been used to manage aHUS and may transiently maintain hematologic variables in some patients, but as the underlying complement dysregulation persists, end-stage renal disease or death occurs in 33% to 40% of patients during the first clinical manifestation. Here we present a pediatric case showing that first-line eculizumab treatment successfully blocked the progression of thrombotic microangiopathy in aHUS.
    Keywords: Atypical Hemolytic Uremic Syndrome ; Complement ; Eculizumab ; Infant ; Antibodies, Monoclonal, Humanized -- Therapeutic Use ; Hemolytic-Uremic Syndrome -- Drug Therapy
    ISSN: 00314005
    E-ISSN: 1098-4275
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  • 2
    Language: English
    In: PLoS ONE, 2010, Vol.5(1), p.e8663
    Description: Familial hemophagocytosis (FHL) is a rare disease associated with defects in proteins involved in CD8 + T-cell cytotoxicity. Hyperactivation of immune cells results in a perilous, Th1-driven cytokine storm. We set out to explore the regulation of cytokines in an FHL patient who was clinically stable on low-dose immunosuppressive therapy after bone marrow transplantation over a six-month period. During this period, chimerism analyses showed that the fraction of host cells was between 1 and 10%. Both parents of the patient as well as healthy volunteers were studied for comparison. ; Using ELISA, quantitative real-time PCR, and clinical laboratory methods, we investigated constitutive and inducible cytokines, polymorphisms, and clinical parameters in whole blood and whole blood cultures. Although routine laboratory tests were within the normal range, the chemokines IP-10 and IL-8 as well as the cytokine IL-27p28 were increased up to 10-fold under constitutive and stimulated conditions compared to healthy controls. Moreover, high levels of IFNγ and TNFα were produced upon stimulation. Unexpectedly, IFNγ induction of IL-18 binding protein (IL-18BP) was markedly reduced (1.6-fold vs 5-fold in controls). The patient's mother featured intermediately increased cytokine levels, whereas levels in the father were similar to those in the controls. ; Since IL-18 plays a major role in perpetuating hemophagocytosis, the failure of IFNγ to induce IL-18BP may constitute a fundamental pathogenetic mechanism. Furthermore, increased production of IL-8 and IL-27 appears to be associated with this disease. Such dysregulation of cytokines was also found in the heterozygous parents, providing a novel insight into genotype-phenotype correlation of FHL which may encourage future research of this rare disease.
    Keywords: Research Article ; Immunology ; Immunology -- Autoimmunity ; Hematology -- Pediatric Hematology ; Oncology -- Myelodysplastic Syndrome And Bone Marrow Diseases ; Pediatrics And Child Health -- Pediatric Hematology ; Rheumatology -- Autoimmunity, Autoimmune, And Inflammatory Diseases
    E-ISSN: 1932-6203
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  • 3
    Language: English
    In: European Journal of Paediatric Neurology, May 2013, Vol.17(3), pp.302-307
    Description: This study was performed to confirm the hypothesis that pre-operative apparent diffusion coefficient (ADC) can be used to distinguish between “low grade” and “high grade” tumours in paediatric patients. ADC values were retrospectively evaluated in thirty-six paediatric brain tumours. Twenty-one children with low grade brain tumours (12 WHO I astrocytomas, 1 giant cell tumour, 1 astrocytoma, 4 WHO II astrocytomas, 2 and 1 ganglioglioma) and 15 children with high grade brain tumours (6 medulloblastomas, 3 WHO III ependymomas, 1 PNET, 1 malignant rhabdoid tumour, 1 malignant germ cell tumour, 1 WHO III astrocytoma, 1 WHO IV astrocytoma, 1 rhabdomyosarcoma metastasis) were included in this study. Minimum and mean ADC values were compared between low grade and high grade tumours and cut-off values were evaluated. The cut-off values to differentiate low and high grade paediatric brain tumours were 0.7 × 10  mm /s and 1.0 × 10  mm /s for minimum ADC and average ADC values respectively. All but one high grade infratentorial ependymoma showed significantly lower ADC values than low grade brain tumours in children. Combining the information obtained from conventional MR imaging with the ADC values may increase the accuracy of pre-operative differentiation between low grade and high grade paediatric tumours. Cut-off values can help to discern low from high grade tumours. However, it has to be considered that there is a substantial overlap between tumour types previously described in the literature.
    Keywords: Brain Tumours ; Children ; Dwi ; Adc ; Medicine
    ISSN: 1090-3798
    E-ISSN: 1532-2130
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  • 4
    Language: English
    In: Pediatric Blood & Cancer, 2015, Vol.62(11), p.2018(3)
    Description: To purchase or authenticate to the full-text of this article, please visit this link: http://onlinelibrary.wiley.com/doi/10.1002/pbc.25621/abstract Byline: Michael T. Meister, Sandra Voss, Dirk Schwabe Patients with ataxia telangiectasia (AT) with malignancies face poor prognosis due to increased treatment-related toxicity. Here, we report a 14-year-old male with AT and Hodgkin lymphoma (HL) who received brentuximab vedotin and reduced COPP plus rituximab courses. This treatment resulted in complete remission and showed no severe toxicity. Pediatr Blood Cancer [c] 2015 Wiley Periodicals, Inc. Article Note: Conflict of interest: Nothing to declare
    Keywords: Lymphomas – Care and Treatment ; Ataxia Telangiectasia – Care and Treatment ; Ataxia – Care and Treatment
    ISSN: 1545-5009
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  • 5
    Language: English
    In: PLoS ONE, 2011, Vol.6(11), p.e27351
    Description: In an ongoing clinical phase I/II study, 16 pediatric patients suffering from high risk leukemia/tumors received highly purified donor natural killer (NK) cell immunotherapy (NK-DLI) at day (+3) +40 and +100 post haploidentical stem cell transplantation. However, literature about the influence of NK-DLI on recipient's immune system is scarce. Here we present concomitant results of a noninvasive in vivo monitoring approach of recipient's peripheral blood (PB) cells after transfer of either unstimulated (NK-DLI (unstim) ) or IL-2 (1000 U/ml, 9–14 days) activated NK cells (NK-DLI (IL-2 stim) ) along with their ex vivo secreted cytokine/chemokines. We performed phenotypical and functional characterizations of the NK-DLIs, detailed flow cytometric analyses of various PB cells and comprehensive cytokine/chemokine arrays before and after NK-DLI. Patients of both groups were comparable with regard to remission status, immune reconstitution, donor chimerism, KIR mismatching, stem cell and NK-DLI dose. Only after NK-DLI (IL-2 stim) was a rapid, almost complete loss of CD56 (bright) CD16 (dim/−) immune regulatory and CD56 (dim) CD16 (+) cytotoxic NK cells, monocytes, dendritic cells and eosinophils from PB circulation seen 10 min after infusion, while neutrophils significantly increased. The reduction of NK cells was due to both, a decrease in patients' own CD69 (−) NCR (low) CD62L (+) NK cells as well as to a diminishing of the transferred cells from the NK-DLI (IL-2 stim) with the CD56 (bright) CD16 (+/−) CD69 (+) NCR (high) CD62L (−) phenotype. All cell counts recovered within the next 24 h. Transfer of NK-DLI (IL-2 stim) translated into significantly increased levels of various cytokines/chemokines (i.e. IFN-γ, IL-6, MIP-1β) in patients' PB. Those remained stable for at least 1 h, presumably leading to endothelial activation, leukocyte adhesion and/or extravasation. In contrast, NK-DLI (unstim) did not cause any of the observed effects. In conclusion, we assume that the adoptive transfer of NK-DLI (IL-2 stim) under the influence of ex vivo and in vivo secreted cytokines/chemokines may promote NK cell trafficking and therefore might enhance efficacy of immunotherapy.
    Keywords: Research Article ; Biology ; Medicine ; Immunology ; Hematology ; Oncology ; Pediatrics And Child Health
    E-ISSN: 1932-6203
    Source: PLoS
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  • 6
    Language: English
    In: Blood, 10 August 2017, Vol.130(6), pp.808-816
    Description: Several studies showed that neutralizing anti-factor VIII (anti-fVIII) antibodies (inhibitors) in patients with acquired hemophilia A (AHA) and congenital hemophilia A (HA) are primarily directed to the A2 and C2 domains. In this study, the frequency and epitope specificity of anti-C1 antibodies were analyzed in acquired and congenital hemophilia inhibitor patients (n = 178). The domain specificity of antibodies was studied by homolog-scanning mutagenesis (HSM) with single human domain human/porcine fVIII proteins and antibody binding to human A2, C1, and C2 domains presented as human serum albumin (HSA) fusion proteins. The analysis with HSA-fVIII domain proteins confirmed the results of the HSM approach but resulted in higher detection levels. The higher detection levels with HSA-fVIII domain proteins are a result of antibody cross-reactivity with human and porcine fVIII leading to false-negative HSM results. Overall, A2-, C1-, and C2-specific antibodies were detected in 23%, 78%, and 68% of patients with AHA (n = 115) and in 52%, 57%, and 81% of HA inhibitor patients (n = 63). Competitive binding of the human monoclonal antibody (mAb) LE2E9 revealed overlapping epitopes with murine C1-specific group A mAbs including 2A9. Mutational analyses identified distinct crucial binding residues for LE2E9 (E2066) and 2A9 (F2068) that are also recognized by anti-C1 antibodies present in patients with hemophilia. A strong contribution of LE2E9- and 2A9-like antibodies was particularly observed in patients with AHA. Overall, our study demonstrates that the C1 domain, in addition to the A2 and C2 domains, contributes significantly to the humoral anti-fVIII immune response in acquired and congenital hemophilia inhibitor patients.
    Keywords: Antibodies, Monoclonal -- Immunology ; Antibodies, Neutralizing -- Immunology ; Factor VIII -- Immunology ; Hemophilia A -- Immunology ; Immunoglobulin G -- Immunology
    ISSN: 00064971
    E-ISSN: 1528-0020
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  • 7
    Language: English
    In: European Journal of Paediatric Neurology, January 2014, Vol.18(1), pp.25-29
    Description: It has been described that hyperintensity in diffusion-weighted imaging (DWI) correlates with high-grade tumours, and high signal-intensity in T2-weighted (T2w) images identifies low-grade tumours. We aimed to investigate the potential of routine conventional MRI sequences, such as DWI and T2-w, to pre-operatively distinguish between low-grade and high-grade brain tumours in paediatric patients. Two raters, blinded to the histological diagnosis, rated the aspect and signal intensity of MR images (T2w and DWI) from 37 children with newly diagnosed brain tumours. Histological diagnoses included 18 low-grade and 19 high-grade brain tumours. The inter-rater agreement was 81–95%. High-grade tumours were never hypointense on DWI and low-grade tumours were usually hyperintense on T2w. Specificity was 100% for low-grade tumours and 90% for high-grade tumours. About 95% of the high-grade tumours and about 70% of the low-grade tumours were correctly diagnosed. The combination of general morphological aspect of the tumours and signals on T2-w and DWI yield a high accuracy of pre-operative differentiation between low-grade and high-grade paediatric tumours.
    Keywords: Brain Tumours ; Children ; Mri ; Dwi ; Medicine
    ISSN: 1090-3798
    E-ISSN: 1532-2130
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  • 8
    Language: English
    In: PLoS ONE, 01 January 2011, Vol.6(11), p.e27351
    Description: In an ongoing clinical phase I/II study, 16 pediatric patients suffering from high risk leukemia/tumors received highly purified donor natural killer (NK) cell immunotherapy (NK-DLI) at day (+3) +40 and +100 post haploidentical stem cell transplantation. However, literature about the influence of NK-DLI on recipient's immune system is scarce. Here we present concomitant results of a noninvasive in vivo monitoring approach of recipient's peripheral blood (PB) cells after transfer of either unstimulated (NK-DLI(unstim)) or IL-2 (1000 U/ml, 9-14 days) activated NK cells (NK-DLI(IL-2 stim)) along with their ex vivo secreted cytokine/chemokines. We performed phenotypical and functional characterizations of the NK-DLIs, detailed flow cytometric analyses of various PB cells and comprehensive cytokine/chemokine arrays before and after NK-DLI. Patients of both groups were comparable with regard to remission status, immune reconstitution, donor chimerism, KIR mismatching, stem cell and NK-DLI dose. Only after NK-DLI(IL-2 stim) was a rapid, almost complete loss of CD56(bright)CD16(dim/-) immune regulatory and CD56(dim)CD16(+) cytotoxic NK cells, monocytes, dendritic cells and eosinophils from PB circulation seen 10 min after infusion, while neutrophils significantly increased. The reduction of NK cells was due to both, a decrease in patients' own CD69(-) NCR(low)CD62L(+) NK cells as well as to a diminishing of the transferred cells from the NK-DLI(IL-2 stim) with the CD56(bright)CD16(+/-)CD69(+)NCR(high)CD62L(-) phenotype. All cell counts recovered within the next 24 h. Transfer of NK-DLI(IL-2 stim) translated into significantly increased levels of various cytokines/chemokines (i.e. IFN-γ, IL-6, MIP-1β) in patients' PB. Those remained stable for at least 1 h, presumably leading to endothelial activation, leukocyte adhesion and/or extravasation. In contrast, NK-DLI(unstim) did not cause any of the observed effects. In conclusion, we assume that the adoptive transfer of NK-DLI(IL-2 stim) under the influence of ex vivo and in vivo secreted cytokines/chemokines may promote NK cell trafficking and therefore might enhance efficacy of immunotherapy.
    Keywords: Sciences (General)
    E-ISSN: 1932-6203
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  • 9
    In: Pediatric Blood & Cancer, November 2015, Vol.62(11), pp.2018-2020
    Description: Patients with ataxia telangiectasia (AT) with malignancies face poor prognosis due to increased treatment‐related toxicity. Here, we report a 14‐year‐old male with AT and Hodgkin lymphoma (HL) who received brentuximab vedotin and reduced COPP plus rituximab courses. This treatment resulted in complete remission and showed no severe toxicity. Pediatr Blood Cancer © 2015 Wiley Periodicals, Inc.
    Keywords: Ataxia Telangiectasia ; Brentuximab Vedotin ; Hodgkin Lymphoma
    ISSN: 1545-5009
    E-ISSN: 1545-5017
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  • 10
    In: Pediatric Blood & Cancer, May 2016, Vol.63(5), pp.947-947
    Description: Byline: Michael T. Meister, Sandra Voss, Dirk Schwabe ***** No abstract is available for this article. ***** Article Note: Conflict of interest: Nothing to declare.
    Keywords: Lymphomas – Care and Treatment ; Telangiectasis – Care and Treatment;
    ISSN: 1545-5009
    E-ISSN: 1545-5017
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