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31.
Verena Limperger Ulrich C. Klostermeier Gili Kenet Susanne Holzhauer Martine Alhenc Gelas Ulrich Finckh Ralf Junker Christine Heller Barbara Zieger Karin Kurnik Ralf Knöfler Rolf Mesters Susan Halimeh Ulrike Nowak‐Göttl 《British journal of haematology》2014,167(3):385-393
Venous thromboembolism [TE] is a multifactorial disease and protein C deficiency [PCD] constitutes a major risk factor. In the present study the prevalence of PCD and the clinical presentation at TE onset, including neonatal purpura fulminans, in a cohort of children are reported. In 367 unselected children (0·1–19 years) recruited between July 1996 and December 2013, a comprehensive thrombophilia screening was performed along with recording of anamnestic data. Twenty‐five of 338 children (7·4%) had PCD. Mean age at first TE onset was 10 years (range 0·1–18). Leading thromboembolic manifestations were neonatal purpura fulminans (n = 5), TE of cerebral veins (n = 3), stroke (n = 2) deep veinthrombosis (DVT) of the leg (n = 10), DVT & pulmonary embolism (n = 2) and DVT & pelvic veins (n = 3). Concomitant risk factors for TE were identified in 12 patients, whereas 13 children spontaneously developed TE. A positive family history of DVT was found in 10 children. In this unselected cohort of paediatric patients with symptomatic TE the overall prevalence of PCD was 7·4%; 1·5% presented with neonatal purpura fulminans. Given its clinical implication for patients and family members, thrombophilia testing should be performed and the benefit of medical or educational interventions should be evaluated in this high‐risk population. 相似文献
32.
Elizabeth Kiwanuka Florian Hackl Edward J. Caterson Daniel Nowinski Johan P.E. Junker Bengt Gerdin Elof Eriksson 《The Journal of surgical research》2013
Background
CCN2 (previously known as connective tissue growth factor) is a multifunctional matricellular protein that has numerous effects on cell life and cell interactions with the connective tissue. Although the importance of CCN2 for the fibrotic process in wound healing has been well studied, the involvement of CCN2 in keratinocyte function has not yet been explored. Therefore, the aim of the present study was to investigate the role of CCN2 in the epidermis during wound healing.Materials and methods
Immunohistochemistry was done on sections from full-thickness porcine wounds. The effect of CCN2 on the migration of cultured human keratinocytes exposed to scratch wounds, the effect on phosphorylation of extracellular signal-related kinases (ERK), and the effect of adding inhibitors to the ERK/mitogen-activated protein kinase pathway to human keratinocytes were studied.Results
The CCN2 protein was transiently expressed in vivo at the leading keratinocyte edge during re-epithelialization of full-thickness porcine wounds. In vitro, exogenous addition of CCN2 to human keratinocyte cultures regulated keratinocyte migration and resulted in phosphorylation of ERK. The addition of inhibitors of ERK/mitogen-activated protein kinase counteracted the effect of CCN2 on migration.Conclusions
CCN2 was transiently expressed at the leading keratinocyte edge in vivo. The biologic importance of this was supported in vitro, because CCN2 regulated human keratinocyte migration through activation of the Ras-mitogen-activated protein kinase kinase-ERK signal transduction pathway. 相似文献33.
Jan Rustemeyer Stefan Zwerger Matthies Pörksen Klaus Junker 《Oral and maxillofacial surgery》2013,17(2):141-144
Background
Desmoplastic trichoepithelioma (DT) is a benign appendageal tumour predominately localized on the facial skin. The histological diagnosis can be difficult in some cases. Partial malignant transformation of a DT is a rarity and a complete transformation has never been described in literature.Case report
A DT of the upper lip was diagnosed histologically by a small biopsy 4 years previously. At presentation, the tumour had enlarged and had partly infiltrated the left side of the upper lip and subnasal region. Histological evaluation confirmed a microcystic adnexal carcinoma but without evidence of malignant transformation of the DT. It appeared that a too-small initial biopsy had led to the incorrect histological diagnosis of a benign tumour. Thus, it was necessary to perform a tumour resection and reconstruction using a two-flap technique including a rotation flap and an Abbé flap. Functional and aesthetic outcomes were good after 6 months. There were no recurrences during a 12-month follow-up.Conclusion
A facial DT should be resected completely. Patients should be attended for follow-ups, keeping in mind the difficulty of making a proper histological diagnosis from small biopsies or excisions and the consequences of ablative facial surgery. However, in particular cases, subtotal defects of the upper lip region are amenable to reconstruction without gross functional or aesthetic deficits. 相似文献34.
Rachael I. Scahill Nicola Z. Hobbs Miranda J. Say Natalie Bechtel Susie M.D. Henley Harpreet Hyare Douglas R. Langbehn Rebecca Jones Blair R. Leavitt Raymund A.C. Roos Alexandra Durr Hans Johnson Stéphane Lehéricy David Craufurd Christopher Kennard Stephen L. Hicks Julie C. Stout Ralf Reilmann Sarah J. Tabrizi the TRACK‐HD investigators 《Human brain mapping》2013,34(3):519-529
TRACK‐HD is a multicentre longitudinal observational study investigating the use of clinical assessments and 3‐Tesla magnetic resonance imaging as potential biomarkers for future therapeutic trials in Huntington's disease (HD). The cross‐sectional data from this large well‐characterized dataset provide the opportunity to improve our knowledge of how the underlying neuropathology of HD may contribute to the clinical manifestations of the disease across the spectrum of premanifest (PreHD) and early HD. Two hundred and thirty nine gene‐positive subjects (120 PreHD and 119 early HD) from the TRACK‐HD study were included. Using voxel‐based morphometry (VBM), grey and white matter volumes were correlated with performance in four domains: quantitative motor (tongue force, metronome tapping, and gait); oculomotor [anti‐saccade error rate (ASE)]; cognition (negative emotion recognition, spot the change and the University of Pennsylvania smell identification test) and neuropsychiatric measures (apathy, affect and irritability). After adjusting for estimated disease severity, regionally specific associations between structural loss and task performance were found (familywise error corrected, P < 0.05); impairment in tongue force, metronome tapping and ASE were all associated with striatal loss. Additionally, tongue force deficits and ASE were associated with volume reduction in the occipital lobe. Impaired recognition of negative emotions was associated with volumetric reductions in the precuneus and cuneus. Our study reveals specific associations between atrophy and decline in a range of clinical modalities, demonstrating the utility of VBM correlation analysis for investigating these relationships in HD. Hum Brain Mapp, 2013. © 2011 Wiley Periodicals, Inc. 相似文献
35.
Daniel?Junker Thomas?R.?W.?Herrmann Markus?Bader Jasmin?Bektic Gregor?Henkel Stephan?Kruck Markus?Sandbichler David?Schilling Georg?Sch?fer Udo?Nagele the Training Research in Urological Surgery Therapy group 《World journal of urology》2016,34(2):245-252
Introduction
Experience from interdisciplinary cooperation revealed the need for a prostate mapping scheme to communicate multiparametric MRI (mpMRI) findings between radiologists, urologists, and pathologists, which should be detailed, yet easy to memorize. For this purpose, the ‘Prostate interdisciplinary communication and mapping algorithm for biopsy and pathology’ (PIC-MABP) was developed. This study evaluated the accuracy of the PIC-MABP system.Methods
PIC-MABP was tested and validated in findings of 10 randomly selected patients from routine clinical practise with 18 histologically proven cancer lesions. Patients received an mpMRI of the prostate prior to prostatectomy. After surgery the prostates were prepared as whole-mount step sections. Cancer lesions, which were found suspicious on mpMRI, were assigned to the according PIC-MABP sectors by a radiologist. MpMRI slides were masked and sent to seven urologists from different centres, providing only the PIC-MABP location of each lesion. Urologists marked the accordant regions. Then mpMRI slides were unmasked, and the correctness of each mark was evaluated.Results
One hundred and seventeen of the 126 marks (93 %) were correctly assigned. Detection rates differed for lesions >0.5 cc compared with lesions <0.5 cc (p < 0.005): 3/7 (43 %) marks were correctly assigned in lesions <0.3 cc, 16/21 (76 %) in lesions with 0.3–0.5 cc, and 98/98 (100 %) in lesions >0.5 cc. Interobserver agreement was good for lesions >0.5 cc and poor for lesions <0.3 cc (Fleiss Kappa 1 vs. 0.0175).Conclusion
PIC-MABP seems to be a reliable system to communicate the location of mpMRI findings >0.5 cc between different disciplines and can be a useful guidance for cognitive mpMRI/TRUS fusion biopsy.36.
Florian Hackl Elizabeth KiwanukaJustin Philip Philipp GernerPejman Aflaki J. Rodrigo Diaz-SisoGeoffroy Sisk E.J. CatersonJohan P.E. Junker Elof Eriksson 《Burns : journal of the International Society for Burn Injuries》2014
Transplantation of skin micrografts in a 1:100 ratio regenerate the epidermis of full-thickness wounds in pigs within 14 days in a wet environment. The aim of the current study was to combine micrografts and commercially available moist dressings. We hypothesized that micrografts regenerate the epidermis when covered with a moist dressing. 5 cm × 5 cm and 10 cm × 10 cm full-thickness wounds were created on the backs of pigs. Wounds were transplanted with 0.8 mm × 0.8 mm micrografts created from a split-thickness skin graft in a 1:100 ratio. 5 cm × 5 cm wounds were treated with wound chambers, moist dressings or dry gauze (non-transplanted control group). 10 cm × 10 cm wounds were compared to non-transplanted wounds, both covered with moist dressings. Reepithelialization was assessed in biopsies from day 10, 14 and 18 post-transplantation. 5 cm × 5 cm transplanted wounds covered with moist dressings showed 69.5 ± 20.6% reepithelialization by day 14 and 90.5 ± 10.4% by day 18, similar to wounds covered with a wound chamber (63.9 ± 16.7 and 86.2 ± 11.9%, respectively). 18 days post-transplantation, 10 cm × 10 cm transplanted wounds covered with moist dressings showed 66.1 ± 10.3% reepithelialization, whereas nontransplanted wounds covered with moist dressings were 40.6 ± 6.6% reepithelialized. We conclude that micrografts combined with clinically available moist dressings regenerate the epidermis of full-thickness wounds. 相似文献
37.
Maternal administration of granulocyte colony-stimulating factor improves neonatal rat survival after a lethal group B streptococcal infection 总被引:2,自引:0,他引:2
Novales JS; Salva AM; Modanlou HD; Kaplan DL; del Castillo J; Andersen J; Medlock ES 《Blood》1993,81(4):923-927
Maternally administered recombinant human granulocyte colony- stimulating factor (rhG-CSF) has been shown to cross the placenta and induce a peripheral neutrophilia and increases in the marrow and spleen neutrophil storage pools in fetal and newborn rats. In the present study, we have used this model system to investigate the efficacy of prenatally administered rhG-CSF on neonatal defense to a lethal challenge with Group B-beta hemolytic Streptococcus (GBS). Pregnant rats were injected with rhG-CSF twice daily beginning 6 days before parturition. At birth, all pups were infected with a dose of GBS that is lethal for 90% of infected pups (LD90). Survival was monitored daily for 5 days. Survival of infected pups from saline-treated mothers beyond 60 hours after infection was 10%. No difference in survival was observed among pups from mothers treated 2 and 4 days before parturition. In contrast, we determined that survival was 82.5% among infected pups from mothers treated for 6 days before parturition with rhG-CSF. Our results demonstrate that maternal administration of rhG- CSF augments neonatal defenses against a lethal bacterial challenge. 相似文献
38.
M Storr P Born E Frimberger N Weigert T Rösch A Meining M Classen HD Allescher 《BMC gastroenterology》2002,2(1):19-8
Background
It has been suggested that intrasphincteric injection of botulinum toxin (BTX) may represent an alternative therapy to balloon dilatation in achalasia. The aim of the present study was to test the effectiveness of botulinum toxin injections in achalasia patients, as assessed using lower oesophageal sphincter pressure (LOSP) and symptom scores, and to compare the response in patients with different types of pretreatment (no previous treatment, balloon dilatation, myotomy, BTX injection). 相似文献39.
Patterns of cytokine gene expression in peripheral T-cell lymphoma of angioimmunoblastic lymphadenopathy type 总被引:3,自引:0,他引:3
Foss HD; Anagnostopoulos I; Herbst H; Grebe M; Ziemann K; Hummel M; Stein H 《Blood》1995,85(10):2862-2869
40.
von den Driesch P; Bhardwaj R; Flad HD; Neugebauer DC; Pielken HJ; Urbanitz D; Kolsch E 《Blood》1989,74(1):430-436
An immunoglobulin M (IgM)-positive cell line, Ms 28, apparently spontaneously transformed by Epstein-Barr virus (EBV) was established from peripheral blood cells of a patient with immature myeloblastic leukemia. It has been characterized according to phenotype, cytochemistry, and membrane antigen pattern. The cell line expresses lymphoid markers like CD 19, CD 22, and CD 30 and synthesizes and secretes IgM. Monocyte markers CD 11c, CD 14, and CD 15 are absent. Neither interleukin-1 (IL-1), nor tumor necrosis factor (TNF-alpha) are produced. But Ms 28 cells show strong phagocytic activity and engulf Latex particles and sheep RBCs (SRBCs) that need not to be opsonized. The phagocytic activity can be inhibited by chloroquine. Both phagocytosis and EBV nuclear-antigen (EBNA) expression can be observed in one and the same cell. Ms 28 cells might be useful to study immunologic activities like antigen processing and presentation. 相似文献