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| 1 | Aspirin responsive platelet thrombophilia in essential thrombocythemia and polycythemia vera显示文摘Essential thrombocythemia(ET) and polycythemia vera(PV) frequently present with erythromelalgia and acrocyanotic complications, migraine-like microvascular cerebral and ocular transient ischemic attacks(MIAs) and/or acute coronary disease. The spectrum of MIAs in ET range from poorly localized symptoms of transient unsteadiness, dysarthria and scintillating scotoma to focal symptoms of transient monocular blindness, transient mono- or hemiparesis or both. The attacks all have a sudden onset, occur sequentially rather than simultaneously, last for a few seconds to several minutes and are usually associated with a dull, pulsatile or migraine-like headache. Increased hematocrit and blood viscosity in PV patients aggravate the microvascular ischemic syndrome of thrombocythemia to major arterial and venous thrombotic complications. Phlebotomy to correct hematocrit to normal in PV significantly reduces major arterial and venous thrombotic complications, but fails to prevent the platelet-mediated erythromelalgia and MIAs. Complete long-term relief of the erythromelalgic microvascular disturbances, MIAs and major thrombosis in ET and PV patients can be obtained with low dose aspirin and platelet reduction to normal, but not with anticoagulation. Skin punch biopsies from the erythromelalgic area show fibromuscular intimal proliferation of arterioles complicated by occlusive plateletrich thrombi leading to acrocyanotic ischemia. Symptomatic ET patients with erythromelalgic microvascular disturbances have shortened platelet survival, increased platelet activation markers β-thromboglobulin(β-TG), platelet factor 4(PF4) and thrombomoduline(TM), increased urinary thromboxane B2(TXB2) excretion, and no activation of the coagulation markers thrombin fragments F1+2 and fibrin degradation products. Inhibition of platelet cyclooxygenase(COX1) by aspirin is followed by the disappearance and no recurrence of microvascular disturbances, increase in platelet number, correction of the shortened platelet survival times to normal, and reduction of increased plasma levels of β-TG, PF4, TM and urinary TXB2 excretion to normal. These results indicate that platelet-mediated fibromuscular intimal proliferation and platelet-rich thrombi in the peripheral, cerebral and coronary end-arterial microvasculature are responsible for the erythromelalgic ischemic complica-tions, MIAs and splanchnic vein thrombosis. Baseline platelet P-selectin levels and arachidonic acid induced COX1 mediated platelet activation showed a highly significant increase of platelet P-selectin expression(not seen in ADP and collagen stimulated platelets), which was significantly higher in JAK2V617 F mutated compared to JAK2 wild type ET. | Jan Jacques Michiels Fibo WJ Ten Kate Peter J Koudstaal Perry JJ Van Genderen | 2013 | World Journal of Hematology2013，2，2： | 4 |
| 2 | Differentiation of malignant and benign proximal bile duct strictures: The diagnostic dilemma显示文摘AIM: To identify the criteria for the differentiation of hilar cholangiocarcinoma (HCCA) from benign strictures. METHODS: A total of 68 patients underwent resection of lesions suspicious for HCCA between 1998 and 2006. The results of laboratory investigations, imaging studies and brush cytology were collected. These fi ndings were analyzed to obtain the fi nal diagnosis. RESULTS: Histological examination of the resected specimens confirmed HCCA in 58 patients (85%, group Ⅰ) whereas 10 patients (15%, groupⅡ) were diagnosed to have benign strictures. The most common presenting symptom was obstructive jaundice in 77% patients (79% group Ⅰvs 60% group Ⅱ P = 0.23). Laboratory findings showed greater elevation of transaminase levels in group Ⅰcompared to group Ⅱ The various imaging modalities showed vascular involvement exclusively in the malignant group (36%,P < 0.05). Brush cytology was positive for malignant cells in only 50% patients in group Ⅰ whereas none in group Ⅱ showed malignant cells. CONCLUSION: Despite improvements in imaging techniques, 10 patients (15%) with a presumptive diagnosis of HCCA were ultimately found to have benign strictures. Except for vascular involvement which was associated significantly with malignancy, there were no conclusive features of malignancy on regular imaging modalities. This uncertainty should be taken into account when patients with a suspicious lesion at the liver hilum are considered for resection. | Jaap Jacob Kloek Otto Marinus van Delden Deha Erdogan Fibo Jan ten Kate Erik Anthoni Rauws Olivier Robert Busch Dirk Joan Gouma Thomas Mathijs van Gulik | 2008 | World Journal of Gastroenterology2008，14，32： | 2 |
| 3 | Evaluation of 28 years of surgical treatment of children and young adults with familial adenomatous polyposis显示文摘 | Klaske A.C. Booij Elisabeht M.H. Mathus-Vliegen Jan A.J.M. Taminiau Fibo J.W. Ten Kate J. Frederick M. Slors Merit M. Tabbers Daniel C. Aronson | 2010 | Journal of Pediatric Surgery2010，，3： | 1 |
| 4 | Ex-vivo evaluation of gene therapy vectors in human pancreatic (cancer) tissue slices显示文摘AIM:To culture human pancreatic tissue obtained from small resection specimens as a pre-clinical model for examining virus-host interactions. METHODS:Human pancreatic tissue samples (malignant and normal)were obtained from surgical specimens and processed immediately to tissue slices. Tissue slices were cultured ex vivo for 1-6 d in an incubator using 95%O2.Slices were subsequently analyzed for viability and morphology.In addition the slices were incubated with different viral vectors expressing the reporter genes GFP or DsRed. Expression of these reporter genes was measured at 72 h after infection.RESULTS:With the Krumdieck tissue slicer,uniform slices could be generated from pancreatic tissue but only upon embedding the tissue in 3%low melting agarose.Immunohistological examination showed the presence of all pancreatic cell types.Pancreatic normal and cancer tissue slices could be cultured for up to 6 d,while retaining viability and a moderate to good morphology.Reporter gene expression indicated that the slices could be infected and transduced efficiently by adenoviral vectors and by adeno associated viral vectors,whereas transduction with lentiviral vectors was limited.For the adenoviral vector,the transduction seemed limited to the peripheral layers of the explants. CONCLUSION:The presented system allows reproducible processing of minimal amounts of pancreatic tissue into slices uniform in size,suitable for pre-clinical evaluation of gene therapy vectors. | Michael A van Geer Koert FD Kuhlmann Conny T Bakker Fibo JW ten Kate Ronald PJ Oude Elferink Piter J Bosma | 2009 | World Journal of Gastroenterology2009，15，11： | 1 |
| 5 | The European clinical,molecular,and pathological (ECMP) criteria and the 2007/2008 revisions of the World Health Organization for the diagnosis, classification, and staging of prefihrotic myeloproliferative neoplasms carrying the JAK2V617F mutation显示文摘 | Jan Jacques Michiels Fibo Ten Kate King H Lam | 2014 | Turk J Haematol2014，31，3： | 1 |
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