クリオ上清中の高分子VWFマルチマー非結合型ADAMTS13の存在:血栓性血小板減少性紫斑病の治療に、より効果的な血漿分画製剤の選択

BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is characterized by deficient ADAMTS13 activity. Treatment involves plasma exchange (PE). Both fresh-frozen plasma (FFP) and cryosupernatant (CSP) are used, but it remains to be determined which is more effective. STUDY DESIGN AND METHODS: To analyze the interaction between von Willebrand factor (VWF) and ADAMTS13, we used large-pore isoelectric focusing (IEF) analysis followed by detection with anti-ADAMTS13 monoclonal antibody. FFP, CSP, cryoprecipitate (CP), and purified ADAMTS13 were analyzed for their effects on high shear stress-induced platelet aggregation (H-SIPA). RESULTS: IEF analysis of normal plasma revealed three groups of ADAMTS13 bands with pI of 4.9 to 5.6, 5.8 to 6.7, and 7.0 or 7.5. Two band groups (pI 4.9-5.6 and 5.8-6.7) were found in plasma of a patient with Type 3 von Willebrand disease, in which VWF is absent, whereas no bands were found in plasma of a patient with congenital ADAMTS13 deficiency. Mixing these plasmas generated the bands at pI 7.0 or 7.5, representing the VWF-ADAMTS13 complex; these bands were absent in CSP. FFP and purified ADAMTS13 down regulated H-SIPA in a dose-dependent manner. However, CP did not inhibit H-SIPA in the initial phase, and the degree of inhibition at the endpoint was almost indistinguishable from those of the other two plasma products. CONCLUSION: Both plasma products (FFP and CSP) are effective for PE in TTP patients. However, CSP may be more favorable, because it has lower levels of VWF and almost normal ADAMTS13 activity, but lower levels of ADAMTS13 in complex with larger VWF multimers.博士（医学）・乙第1322号・平成25年11月27日© 2013 American Association of Blood BanksCopyright © 1999-2018 John Wiley & Sons, Inc. All rights reservedThis is the pre-peer reviewed version of the following article: [https://onlinelibrary.wiley.com/doi/full/10.1111/trf.12182], which has been published in final form at [http://dx.doi.org/10.1111/trf.12182]. This article may be used for non-commercial purposes in accordance with Wiley Terms and Conditions for Use of Self-Archived Versions

アンゼンナ フククウキョウカ タンノウ テキシュツジュツ ノ タメ ノ ジュツゼン ガゾウ シンダン ノ ヤクワリ

Laparoscopic cholecystectomy becomes one of the standard procedures for digestive surgeons. There is consensus that careful dissection and correct elucidation of the anatomy avoids the complications during cholecystectomy. From May 1991 to March 2005, 433 cases of laparoscopic cholecystectomy were retrospectively analyzed. Conversion to open cholecystectomy was required in 19 cases（4.4%）and the rate of vascular injury or bile duct injury was 0.7% each. CT angiography was effective for preoperative evaluation of vascular anatomy. In many cases, middle hepatic vein was located near gallbladder bed. CT cholangiography was also useful for obtaining information of the biliary tract. Careful evaluation of preoperative CT angiography and cholangiography contributes to decrease the risk of complications during laparoscopic cholecystectomy

セイタイ カンイショク ニオケル カショウ グラフト ノ ビョウタイ セイリ ト チリョウ センリャク

Introduction : To save the small-for-size graft in living donor adult liver transplantation （LDALT）, it is necessary to overcome the following problems：１）excessive portal inflow；２） graft congestion；３）small functional liver mass ; and４）inadequate intragraft responses. Treatments for the small-for-size graft. １）To avoid excessive portal inflow（: a）Splenctomy or splenic artery ligation to reduce portal pressure and flow ; and b）Portocaval shunt to reduce portal pressure and flow. ２）To avoid graft congestion : a）Graft venoplasty and graft hepatic vein to the IVC anastomosis in left lobe grafts, and reconstruction of significant venous tributaries from the middle hepatic vein in right lobe grafts ; and（b）Intraportal administration of drugs（PG-E１, etc.）to prevent microcirculatory disturbance. ３）To avoid liver failure due to small functional liver mass : Hyperbaric oxygen therapy is a feasible option for a persistent functional hyperbiliruminemia. ４）To modulate inadequate intragraft responses（: a）Induction of heat shock protein into the graft to suppress up-regulation of inflammatory cytokines, and to improved survival rate after ９５％-hepatectomy（Hx）in rats ; and（b）Slow-down of liver regeneration to reduce liver injury and to improve survival rate after９０％-Hx in rats. Conclusions : Pathophysiology-oriented strategy against small-for-size graft is effective in LDALT

Treatment Strategy for Pancreatic Neuroendocrine Neoplasms (pNEN)

Article信州医学雑誌 68(5): 235-242(2020)journal articl

The changes in treatment strategies in ABOi living donor liver transplantation for acute liver failure

Introduction. Living donor liver transplantation (LDLT) using ABO-incompatible (ABOi) graft for acute liver failure (ALF) is a developing treatment modality. Methods. We reviewed the changes in our treatment strategies in applying ABOi LDLT for FH over our fourteen years of experience. Results. Five patients with ALF received LDLT in adults using ABOi grafts, with different but gradually renewed protocols. The etiologies for acute liver failure included autoimmune hepatitis (n=3) and unknown (n=2). The desensitization protocol for ABOi barrier included Case #1 ; local infusion (portal vein)+plasma exchange (PE), Case #2 ; local infusion (hepatic artery)+rituximab+PE, Case #3 and #4 ; rituximab+PE, and Case #5 ; rituximab+PE under high-flow continuous hemodiafiltration. Local infusion was abandoned since Case #3, because Case #1 had portal vein thrombosis resulting in graft necrosis and Case #2 had hepatic artery dissection. The patients (Case #2 and #3), who received rituximab within 7 days before LDLT, experienced antibody-mediated rejection. Thus, the most recent protocol for ABOi-LDLT is that rituximab is given 2 weeks before LDLT, followed by high-flow continuous hemodiafiltration to obstacle hepatic encephalopathy until LDLT. The four patients except Case #1 are doing well with good graft function over 3.8±3.7 years. Conclusion. Rituximab-based ABOi-LDLT, most-recently under high-flow hemodiafiltration for treating encephalopathy, is a feasible option for applying LDLT for ALF

Sleep status of older adults with sleep apnoea syndrome may vary by body mass index

Obesity and ageing are the most important risk factors for sleep apnoea syndrome (SAS); however, the role of body mass index (BMI) on sleep status in healthy older adults is unclear. To explore sleep parameters according to BMI among active older adults, we cross-sectionally examined the relationship between sleep-related parameters and BMI in 32 Japanese adults aged from 83 to 95 years without long-term care who were unaware of having SAS. Correlation and linear regression analyses were performed. Moderate or severe SAS prevalence was high in both those with low (68.8%) and high (68.8%) BMI. A higher increase in apnoea-hypopnoea index (AHI) was negatively correlated with sleep depth in the high-BMI group. In the low-BMI group, the number of awakenings and age were positively correlated with AHI. Older adults may have SAS regardless of their BMI, and the sleep status of patients with SAS may vary by BMI

Clinical features and significance of leukopenia occurring immediately after endovascular surgery

Purpose Inflammation after stent graft surgery is known as postimplantation syndrome (PIS) and it causes leukocytosis. However, we have experienced leukopenia in the very early postoperative phase of endovascular surgery at our institution. We investigated leukopenia, an under-recognized phenomenon that occurred after transcatheter aortic valve implantation (TAVI), endovascular aortic repair (EVAR), and thoracic endovascular aortic repair (TEVAR). Methods Records of patients who underwent TAVI, EVAR, and TEVAR between March 2018 and February 2019 were retrospectively reviewed. Primary outcomes were the decline rate of white blood cell count (DR-WBC) in the immediate postoperative period and its differences among surgical procedures. The secondary endpoint was the relationship between DR-WBC and infectious complications. Furthermore, the incidence of PIS and its differences among the procedures and associations with DR-WBC were evaluated. Results A total of 108 patients (TAVI 41, EVAR 37, TEVAR 30) were included. DR-WBC immediately after surgery was higher in the TAVI group when compared with other groups (TAVI, 43.1 +/- 22.6%; EVAR, 27.6 +/- 17.3%; TEVAR, 25.4 +/- 27.4%; P < 0.01). DR-WBC was not significantly different regardless of postoperative infection (P = 0.45) or PIS (P = 0.62). The incidence rate of PIS was higher in the EVAR group compared with the TAVI group, and was not associated with DR-WBC. Conclusions Leukopenia was a common phenomenon immediately after endovascular surgery, especially TAVI. It resolved a day after surgery and was not associated with PIS or infectious complications. Therefore, it seems to be a transient abnormal hematological finding and a self-limiting condition