甲亢危象是一种罕见的、危及生命的内分泌急症,患者有严重的甲状腺毒症临床表现。美国和日本的研究显示,甲亢危象的年发病率分别为(0.57~0.76)/10万和0.2/10万。甲亢危象占甲状腺毒症患者的0.22%,占住院甲状腺毒症患者的5.4%。即使在获...甲亢危象是一种罕见的、危及生命的内分泌急症,患者有严重的甲状腺毒症临床表现。美国和日本的研究显示,甲亢危象的年发病率分别为(0.57~0.76)/10万和0.2/10万。甲亢危象占甲状腺毒症患者的0.22%,占住院甲状腺毒症患者的5.4%。即使在获得及时治疗的情况下,甲亢危象患者的死亡率仍高达10%~30%;若未治疗,则患者的死亡率可达90%。甲亢危象的急诊漏诊和误诊率高达43.48%。导致甲亢危象的诱因可能包括突然停用抗甲状腺药物或急性事件(如感染、创伤、甲状腺或非甲状腺手术、急性碘负荷或分娩)以及其他少见的病因。目前尚无公认的标准或临床工具用于诊断甲亢危象,其诊断依据包括存在甲亢的生化证据(游离T4或T3升高、TSH降低),以及危及生命的严重症状(高热、心血管功能障碍及精神状态改变等)。伯奇-沃托斯基点量表(Burch-Wartofsky point scale,BWPS)近30年来一直被广泛应用于甲亢危象的诊断。甲亢危象的主要治疗包括一般对症治疗及针对甲状腺的特异性治疗,包括去除诱因和治疗并发症,如使用抗甲状腺药物、碘剂、糖皮质激素及β受体阻滞剂等抑制甲状腺激素合成,或阻断外周T4向T3转换或抑制甲状腺激素释放,对上述治疗后病情改善不明显者,则可以尝试血液净化(血浆置换)治疗。此外,支持治疗对于甲亢危象患者亦至关重要。甲亢危象患者经过积极治疗,病情多在1~2d内改善。甲亢危象抢救成功后,应采用根治方法治疗甲亢。展开更多
Acute humoral rejection （AHR） is uncommon after ABO- compatible liver transplantation. Herein, we report two cases of AHR treated with plasmapheresis and rituximab in two ABO-compatible liver-transplant patients wit...Acute humoral rejection （AHR） is uncommon after ABO- compatible liver transplantation. Herein, we report two cases of AHR treated with plasmapheresis and rituximab in two ABO-compatible liver-transplant patients with preformed anti-human leukocyte antigen donor-specific antibodies. Patient 1 experienced a biopsy-proven AHR at day 10 post-transplant. She was treated by steroid pulses, and OKT3. Because of persisting signs of biopsy-proven AHR at day 26, she was treated by plasmapheresis and rituximab. Uver enzyme levels did not improve, and she died on day 41. Patient 2 experienced a biopsy-proven AHR on day 10 post-transplant. She was treated by steroid pulses, plasmapheresis, and rituximab. Liver enzymes returned to within normal range 18 d after diagnosis. Uver biopsies, at 3 and 9 mo post-transplant, showed complete resolution of AHR. We conclude that plasmapheresis should be started as soon as AHR is diagnosed, and be associated with a B-cell depleting agent. Rituximab may be considered as a first-line therapy.展开更多
Autoimmune encephalitis is a poorly understood condition that can present with a combination of neurological and psychiatric symptoms, either of which may predominate. There are many autoantibodies associated with a v...Autoimmune encephalitis is a poorly understood condition that can present with a combination of neurological and psychiatric symptoms, either of which may predominate. There are many autoantibodies associated with a variety of clinical syndromes-anti-N-Methyl-D-Aspartate receptor(NMDAR) is the commonest. Currently, the most widely used therapy is prompt plasmapheresis and steroid treatment(and tumour resection if indicated), followed by second line immunosuppression if this fails. Given the growing awareness of autoimmune encephalitis as an entity, it is increasingly important that we consider it as a potential diagnosis in order to provide timely, effective treatment. We discuss several previously published case reports and one new case. These reports examined the effects of electroconvulsive therapy(ECT) on patients with autoimmune encephalitis, particularly those in whom psychiatric symptoms are especially debilitating and refractory to standard treatment. We also discuss factors predicting good outcome and possible mechanisms by which ECT may be effective. Numerous cases, such as those presented by Wingfield, Tsutsui, Florance, Sansing, Braakman and Matsumoto, demonstrate effective use of ECT in anti-NMDAR encephalitis patients with severe psychiatric symptoms such as catatonia, psychosis, narcolepsy and stupor who had failed to respond to standard treatments alone. We also present a new case of a 71-year-old female who presented to a psychiatric unit initially with depression, which escalated to catatonia, delusions, nihilism and auditory hallucinations. After anti-NMDAR antibodies were isolated, she was treated by the neurology team with plasmapheresis and steroids, with a partial response. She received multiple sessions of ECT and her psychiatric symptoms completely resolved and she returned to her premorbid state. For this reason, we suggest that ECT should be considered, particularly in those patients who are non-responders to standard therapies.展开更多
Background: Plasmapheresis is a desensitization method used prior to ABO-incompatible(ABO-I) living donor liver transplantation. However, studies on its usefulness in the rituximab era are lacking.Methods: Fifty-six a...Background: Plasmapheresis is a desensitization method used prior to ABO-incompatible(ABO-I) living donor liver transplantation. However, studies on its usefulness in the rituximab era are lacking.Methods: Fifty-six adult patients underwent ABO-I living donor liver transplantation between January2012 and October 2015. A single dose of rituximab(300 mg/m~2) was administered 2 weeks before surgery with plasmapheresis in all patients until February 2014(RP group, n = 26). Patients were administered rituximab only, without plasmapheresis between March 2014 and October 2015(RO group, n = 30).Results: The 6-, 12-and 18-month overall survival rates were 92.3%, 80.8% and 76.9% in the RP group and 96.6%, 85.4% and 85.4% in the RO group, respectively(P = 0.574). When the initial isoagglutinin titers < 16, neither group showed a rebound rise of isoagglutinin titers. For patients with initial isoagglutinin titers ≥ 16, the rebound rise of isoagglutinin titers was more prominent in the RP group. There was no difference in time-dependent changes in B cell subpopulations and ABO-I-related complications.Conclusions: Sufficient desensitization for ABO-I living donor liver transplantation can be achieved using rituximab alone. This desensitization strategy does not affect the isoagglutinin titers, ABO-I-related complications and patient survival.展开更多
AIM:To investigate the effect of plasmapheresis via the portal vein for"small-for-size"syndrome(SFSS)aided by extracorporeal continuous portal diversion(ECPD).METHODS:Extensive or total hepatectomy in the pi...AIM:To investigate the effect of plasmapheresis via the portal vein for"small-for-size"syndrome(SFSS)aided by extracorporeal continuous portal diversion(ECPD).METHODS:Extensive or total hepatectomy in the pig is usually adopted as a postoperative liver failure(PLF)or SFSS model.In this study,animals which underwent85%-90%hepatectomy were randomized into either the Systemic group(n=7)or the Portal group(n=7).In the Systemic group,all pigs received temporal plasmapheresis(PP)via the extracorporeal catheter circuit(systemic to systemic circulation)from 24 to 30 h posthepatectomy(PH);in the Portal group,all pigs received ECPD to divert partial portal vein flow(PVF)to the systemic circulation after hepatectomy,then converted to temporal PP from 24 to 30 h PH,and subsequently converted to ECPD again until 48 h PH.In the Portal group,the PVF was preserved at 3.0-3.3 times that of the baseline value,similar to that following 70%hepatectomy,which was regarded as the optimal PVF to the hypertrophic liver remnant.At 48 h PH,all pigs were re-opened and the portal vein pressure(PVP),PVF,and HAF(hepatic artery flow)were measured,and then diversion of the portal venous flow was terminated.After1 h the PVP,PVF,and HAF were re-measured.The portal hemodynamic changes,liver injury,liver regeneration and bacterial/lipopolysaccharide(LPS)translocation were evaluated in the two groups.RESULTS:The PVP in the Portal group was significantly lower than that in the Systemic group during the time period from 2 to 49 h PH(P<0.05).Serum alanine aminotransferase(ALT),total bilirubin(TB)and ammonia were significantly reduced in the Portal group compared with the Systemic group from 24 to 48 h PH(P<0.05).The Portal group may have attenuated sinusoidal endothelial injury and decreased the level of HA compared with the Systemic group.In the Systemic group,there was significant sinusoidal dilation,hydropic changes in hepatocytes and hemorrhage into the hepatic parenchyma,and the sinusoidal endothelial lining was partially destroyed and detached into the sinusoidal space.CD31immunostaining revealed significant destruction of the endothelial lining.In the Portal group,there was no intraparenchymal hemorrhage and the sinusoidal endothelial cells and hepatocytes were well preserved.CD31immunostaining was mild which indicated less destruction of the endothelial lining.HA was significantly decreased in the Portal group compared with the Systemic group from 2 to 48 h PH.The rate of liver remnant regeneration was elevated,while apoptosis was attenuated in the Portal group compared with the Systemic group.Thymidine kinase activity was much higher in the Portal group than in the Systemic group at 48 h PH.The PCNA index was significantly increased and the apoptotic index was significantly decreased in the Portal group compared with the Systemic group.Bacterial translocation and endotoxin,as well as the inflammatory response,were significantly attenuated in the Portal group compared with the Systemic group.LPS,tumor necrosis factor-and interleukin-6 levels were all significantly decreased in the Portal group compared with the Systemic group from 24 to48 h PH,while bacterial DNA level was significantly decreased from 2 to 48 h PH.CONCLUSION:PP plus ECPD via the portal vein can attenuate toxic load and hyperperfusion injury,and should be undertaken instead of PP via the systemic circulation in SFSS or PLF.展开更多
BACKGROUND Focal segmental glomerulosclerosis(FSGS)is one of the most common glomerular diseases leading to renal failure.FSGS has a high risk of recurrence after kidney transplantation.Prevention of recurrent FSGS us...BACKGROUND Focal segmental glomerulosclerosis(FSGS)is one of the most common glomerular diseases leading to renal failure.FSGS has a high risk of recurrence after kidney transplantation.Prevention of recurrent FSGS using rituximab and/or plasmapheresis has been evaluated in multiple small studies with conflicting results.AIM To assess the risk of recurrence of FSGS after transplantation using prophylactic rituximab with or without plasmapheresis,and plasmapheresis alone compared to the standard treatment group without preventive therapy.METHODS This meta-analysis and systematic review were performed by first conducting a literature search of the MEDLINE,EMBASE,and Cochrane databases,from inception through March 2021;search terms included‘FSGS,’’steroid-resistant nephrotic syndrome’,‘rituximab,’and‘plasmapheresis,’.We identified studies that assessed the risk of post-transplant FSGS after use of rituximab with or without plasmapheresis,or plasmapheresis alone.Inclusion criteria were:Original,published,randomized controlled trials or cohort studies(either prospective or retrospective),case-control,or cross-sectional studies;inclusion of odds ratio,relative risk,and standardized incidence ratio with 95%confidence intervals(CI),or sufficient raw data to calculate these ratios;and subjects without interventions(controls)being used as comparators in cohort and cross-sectional studies.Effect estimates from individual studies were extracted and combined using a random effects model.RESULTS Eleven studies,with a total of 399 kidney transplant recipients with FSGS,evaluated the use of rituximab with or without plasmapheresis;thirteen studies,with a total of 571 kidney transplant recipients with FSGS,evaluated plasmapheresis alone.Post-transplant FSGS recurred relatively early.There was no significant difference in recurrence between the group that received rituximab(with or without plasmapheresis)and the standard treatment group,with a pooled risk ratio of 0.82(95%CI:0.47-1.45,I2=65%).Similarly,plasmapheresis alone was not associated with any significant difference in FSGS recurrence when compared with no plasmapheresis;the pooled risk ratio was 0.85(95%CI:0.60-1.21,I2=23%).Subgroup analyses in the pediatric and adult groups did not yield a significant difference in recurrence risk.We also reviewed and analyzed posttransplant outcomes including timing of recurrence and graft survival.CONCLUSION Overall,the use of rituximab with or without plasmapheresis,or plasmapheresis alone,is not associated with a lower risk of FSGS recurrence after kidney transplantation.Future studies are required to assess the effectiveness of rituximab with or without plasmapheresis among specific patient subgroups with high-risk for FSGS recurrence.展开更多
Prolonged cholestasis is a very rare complication of endoscopic retrograde cholangiography (ERC). Only few cases with this complication are reported in the English literature. We report persisting cholestatic jaundice...Prolonged cholestasis is a very rare complication of endoscopic retrograde cholangiography (ERC). Only few cases with this complication are reported in the English literature. We report persisting cholestatic jaundice in a 73-year old man after successful therapeutic ERC for choledocholithiasis. Serologic tests for viral and autoimmune hepatitis were all negative. A second-look ERC was normal also. He denied any medication except for prophylaxis given intravenous 1 g ceftriaxon prior to the ERC procedure. After an unsuccessful trial with ursodeoxycholic acid and cholestyramine for 2 wk, this case was efficiently treated with corticosteroids and plasmapheresis. His cholestatic enzymes became normal and intense pruritis quickly resolved after this treatment which lasted during his follow- up period. We discussed the possible mechanisms and treatment alternatives of intrahepatic cholestasis associated with the ERC procedure.展开更多
BACKGROUND Glycogen storage disease type Ia(GSDIa) is an autosomal recessive inborn error of carbohydrate metabolism that is caused by deficiency of the enzyme glucose-6-phosphatase(G6Pase),leading to disturbed glycog...BACKGROUND Glycogen storage disease type Ia(GSDIa) is an autosomal recessive inborn error of carbohydrate metabolism that is caused by deficiency of the enzyme glucose-6-phosphatase(G6Pase),leading to disturbed glycogenolysis and gluconeogenesis.Patients with GSDIa show severe fasting hypoglycemia,hyperlipidemia,hyperlactacidemia,and hyperuricemia,which are associated with fatal outcomes in pregnant women and fetuses.CASE SUMMARY Herein,we report the case of a 24-year-old female who on her first visit to the hospital,presented with pregnancy combined with extremely high hyperlipidemia and hyperlactic acidosis with anemia,and frequent hypoglycemia occurred during the treatment.Genetic tests revealed a mutation in the G6Pase gene(G6PC) at 17q21,the patient was finally diagnosed with glycogen storage disease type Ia for the first time after 22 years of inaccurate treatment.She has been treated with a continuous double filtration plasmapheresis(DFPP) strategy to remove blood lipids,and a cornstarch diet therapy.The patient did not develop pancreatitis during the course of the disease and a healthy baby girl weighing 3 kg was delivered.CONCLUSION Patients with GSDIa may be misdiagnosed as epilepsy.DFPP can be used to control hyperlipidemia in GSDIa patients during pregnancy.展开更多
Bickerstaff brainstem encephalitis (BBE) is a rare post-infectious neurological syndrome for which an effective treatment strategy has not been established. Here, we report a case of a 71-year-old male who suffered fr...Bickerstaff brainstem encephalitis (BBE) is a rare post-infectious neurological syndrome for which an effective treatment strategy has not been established. Here, we report a case of a 71-year-old male who suffered from an upper respiratory tract infection, and 7 days later, developed numbness of the bilateral upper and lower limbs, unsteady gait and dysarthria. Brain magnetic resonance imaging was normal, nerve conduction study and cerebral spinal fluid analysis were nonspecific. Based on the clinical features, we tentatively diagnosed Guillain-Barré syndrome and started immunoadsorption plasmapheresis. However, consciousness progressively declined to coma level within 10 days. Electroencephalogram showed diffuse slowing, and auditory evoked brainstem response (ABR) demonstrated absence of waves II, III and V. Serum anti-GQ1b IgG autoantibody and anti-GM1b IgG autoantibody were negative. Subsequently, we diagnosed BBE, and clinical symptoms resolved after treatment with intravenous immunoglobulin and methyllprednisolone. On day 62, neurological symptoms were remarkably alleviated with an improvement in ABR. Our observations suggest that immunoadsorption plasmapheresis should be used only when anti-ganglioside antibodies are detected. Combination therapy with intravenous immunoglobulin and methylprednisolone or plasma exchange?is recommended as initial therapy.展开更多
The role of plasmapheresis in liver failure and hepatic encephalopathy is undefined and its use as a strategy to salvage patients with severe allograft dysfunction after liver transplantation remains investigational. ...The role of plasmapheresis in liver failure and hepatic encephalopathy is undefined and its use as a strategy to salvage patients with severe allograft dysfunction after liver transplantation remains investigational. We present a case of early allograft dysfunction following deceased donor liver transplantation(DDLT) where plasmapheresis was effective as a bridge to recovery and possibly avoiding a retransplantation. A 16 years old boy, known to have decompensated Wilson's disease underwent DDLT at our Public Sector Hospital. He received a healthy liver from a brain-dead donor, whose liver was considered too large for the boy. The graft was reduced in situ to a left lobe graft. Surgery was uneventful and the recipient was well for the initial 96 h. On Doppler and further computed tomography scan, a partial portal vein thrombus was noted. He was reexplored and a Fogarty endothombecteomy was performed. Following the second surgery, he developed severe allograft dysfunction with a peak bilirubin of 40 mg/d L. He underwent imaging to rule out technical causes for the dysfunction, followed by a liver biopsy, which revealed acute cellular rejection. Multiple cycles of plasmapheresis were initiated. Over the next two weeks, the graft demonstrated a gradual recovery. He was discharged on the 30 th postoperative day, with a serum bilirubin of 5.5 mg/d L. He remains well on follow-up, with the liver function tests improving further. Our report demonstrates the beneficial effect of plasmapheresis, which appears to be an effective treatment option for early allograft dysfunction following liver transplantation and may obviate the need for retransplantation.展开更多
<strong>Background:</strong> Cold agglutinins are auto-antibodies that can be a nuisance in cross matching and in blood grouping. Here we report an unusual case of a high titer and wide amplitude cold aggl...<strong>Background:</strong> Cold agglutinins are auto-antibodies that can be a nuisance in cross matching and in blood grouping. Here we report an unusual case of a high titer and wide amplitude cold agglutinin reduced by plasmapheresis. <strong>Methods and Materials:</strong> A 56-year-old man with severe anemia requested a transfusion of red blood cells. However, there was a problem in blood for blood grouping. The discrepancy of blood typing was subsequently resolved using group O absorbed plasma along with repetition of forward grouping with warm-washed red blood cells. The presence of high-thermal-amplitude and a high-titer anti-I cold agglutinin were detected in further serologic investigation. It revealed reactivity against autologous and adult O red blood cells at 37<span style="white-space:nowrap;">°</span>C by the thermal amplitude screening test, and demonstrated a very high titer of 65,536 against adult O cells by titration studies at 4<span style="white-space:nowrap;">°</span>C. The patient received two plasma exchange sessions of 1.5 plasma volumes each. There was a significant reduction of the titer of cold agglutinins and of the thermal amplitude by plasmapheresis as well (<em>p</em> < 0.01). <strong>Results:</strong> After successful cross-matching with post plasma exchanges, four units of red blood cells were infused to the patient without any hemolysis symptoms or signs. <strong>Conclusions:</strong> We now reported a patient with abnormally ascended titer of cold agglutinins and wide-thermal-amplitude, but we also successfully performed ABO typing and cross matching after 2 plasma exchange sessions of 1.5 plasma volumes each.展开更多
文摘甲亢危象是一种罕见的、危及生命的内分泌急症,患者有严重的甲状腺毒症临床表现。美国和日本的研究显示,甲亢危象的年发病率分别为(0.57~0.76)/10万和0.2/10万。甲亢危象占甲状腺毒症患者的0.22%,占住院甲状腺毒症患者的5.4%。即使在获得及时治疗的情况下,甲亢危象患者的死亡率仍高达10%~30%;若未治疗,则患者的死亡率可达90%。甲亢危象的急诊漏诊和误诊率高达43.48%。导致甲亢危象的诱因可能包括突然停用抗甲状腺药物或急性事件(如感染、创伤、甲状腺或非甲状腺手术、急性碘负荷或分娩)以及其他少见的病因。目前尚无公认的标准或临床工具用于诊断甲亢危象,其诊断依据包括存在甲亢的生化证据(游离T4或T3升高、TSH降低),以及危及生命的严重症状(高热、心血管功能障碍及精神状态改变等)。伯奇-沃托斯基点量表(Burch-Wartofsky point scale,BWPS)近30年来一直被广泛应用于甲亢危象的诊断。甲亢危象的主要治疗包括一般对症治疗及针对甲状腺的特异性治疗,包括去除诱因和治疗并发症,如使用抗甲状腺药物、碘剂、糖皮质激素及β受体阻滞剂等抑制甲状腺激素合成,或阻断外周T4向T3转换或抑制甲状腺激素释放,对上述治疗后病情改善不明显者,则可以尝试血液净化(血浆置换)治疗。此外,支持治疗对于甲亢危象患者亦至关重要。甲亢危象患者经过积极治疗,病情多在1~2d内改善。甲亢危象抢救成功后,应采用根治方法治疗甲亢。
文摘Acute humoral rejection （AHR） is uncommon after ABO- compatible liver transplantation. Herein, we report two cases of AHR treated with plasmapheresis and rituximab in two ABO-compatible liver-transplant patients with preformed anti-human leukocyte antigen donor-specific antibodies. Patient 1 experienced a biopsy-proven AHR at day 10 post-transplant. She was treated by steroid pulses, and OKT3. Because of persisting signs of biopsy-proven AHR at day 26, she was treated by plasmapheresis and rituximab. Uver enzyme levels did not improve, and she died on day 41. Patient 2 experienced a biopsy-proven AHR on day 10 post-transplant. She was treated by steroid pulses, plasmapheresis, and rituximab. Liver enzymes returned to within normal range 18 d after diagnosis. Uver biopsies, at 3 and 9 mo post-transplant, showed complete resolution of AHR. We conclude that plasmapheresis should be started as soon as AHR is diagnosed, and be associated with a B-cell depleting agent. Rituximab may be considered as a first-line therapy.
文摘Autoimmune encephalitis is a poorly understood condition that can present with a combination of neurological and psychiatric symptoms, either of which may predominate. There are many autoantibodies associated with a variety of clinical syndromes-anti-N-Methyl-D-Aspartate receptor(NMDAR) is the commonest. Currently, the most widely used therapy is prompt plasmapheresis and steroid treatment(and tumour resection if indicated), followed by second line immunosuppression if this fails. Given the growing awareness of autoimmune encephalitis as an entity, it is increasingly important that we consider it as a potential diagnosis in order to provide timely, effective treatment. We discuss several previously published case reports and one new case. These reports examined the effects of electroconvulsive therapy(ECT) on patients with autoimmune encephalitis, particularly those in whom psychiatric symptoms are especially debilitating and refractory to standard treatment. We also discuss factors predicting good outcome and possible mechanisms by which ECT may be effective. Numerous cases, such as those presented by Wingfield, Tsutsui, Florance, Sansing, Braakman and Matsumoto, demonstrate effective use of ECT in anti-NMDAR encephalitis patients with severe psychiatric symptoms such as catatonia, psychosis, narcolepsy and stupor who had failed to respond to standard treatments alone. We also present a new case of a 71-year-old female who presented to a psychiatric unit initially with depression, which escalated to catatonia, delusions, nihilism and auditory hallucinations. After anti-NMDAR antibodies were isolated, she was treated by the neurology team with plasmapheresis and steroids, with a partial response. She received multiple sessions of ECT and her psychiatric symptoms completely resolved and she returned to her premorbid state. For this reason, we suggest that ECT should be considered, particularly in those patients who are non-responders to standard therapies.
文摘Background: Plasmapheresis is a desensitization method used prior to ABO-incompatible(ABO-I) living donor liver transplantation. However, studies on its usefulness in the rituximab era are lacking.Methods: Fifty-six adult patients underwent ABO-I living donor liver transplantation between January2012 and October 2015. A single dose of rituximab(300 mg/m~2) was administered 2 weeks before surgery with plasmapheresis in all patients until February 2014(RP group, n = 26). Patients were administered rituximab only, without plasmapheresis between March 2014 and October 2015(RO group, n = 30).Results: The 6-, 12-and 18-month overall survival rates were 92.3%, 80.8% and 76.9% in the RP group and 96.6%, 85.4% and 85.4% in the RO group, respectively(P = 0.574). When the initial isoagglutinin titers < 16, neither group showed a rebound rise of isoagglutinin titers. For patients with initial isoagglutinin titers ≥ 16, the rebound rise of isoagglutinin titers was more prominent in the RP group. There was no difference in time-dependent changes in B cell subpopulations and ABO-I-related complications.Conclusions: Sufficient desensitization for ABO-I living donor liver transplantation can be achieved using rituximab alone. This desensitization strategy does not affect the isoagglutinin titers, ABO-I-related complications and patient survival.
文摘AIM:To investigate the effect of plasmapheresis via the portal vein for"small-for-size"syndrome(SFSS)aided by extracorporeal continuous portal diversion(ECPD).METHODS:Extensive or total hepatectomy in the pig is usually adopted as a postoperative liver failure(PLF)or SFSS model.In this study,animals which underwent85%-90%hepatectomy were randomized into either the Systemic group(n=7)or the Portal group(n=7).In the Systemic group,all pigs received temporal plasmapheresis(PP)via the extracorporeal catheter circuit(systemic to systemic circulation)from 24 to 30 h posthepatectomy(PH);in the Portal group,all pigs received ECPD to divert partial portal vein flow(PVF)to the systemic circulation after hepatectomy,then converted to temporal PP from 24 to 30 h PH,and subsequently converted to ECPD again until 48 h PH.In the Portal group,the PVF was preserved at 3.0-3.3 times that of the baseline value,similar to that following 70%hepatectomy,which was regarded as the optimal PVF to the hypertrophic liver remnant.At 48 h PH,all pigs were re-opened and the portal vein pressure(PVP),PVF,and HAF(hepatic artery flow)were measured,and then diversion of the portal venous flow was terminated.After1 h the PVP,PVF,and HAF were re-measured.The portal hemodynamic changes,liver injury,liver regeneration and bacterial/lipopolysaccharide(LPS)translocation were evaluated in the two groups.RESULTS:The PVP in the Portal group was significantly lower than that in the Systemic group during the time period from 2 to 49 h PH(P<0.05).Serum alanine aminotransferase(ALT),total bilirubin(TB)and ammonia were significantly reduced in the Portal group compared with the Systemic group from 24 to 48 h PH(P<0.05).The Portal group may have attenuated sinusoidal endothelial injury and decreased the level of HA compared with the Systemic group.In the Systemic group,there was significant sinusoidal dilation,hydropic changes in hepatocytes and hemorrhage into the hepatic parenchyma,and the sinusoidal endothelial lining was partially destroyed and detached into the sinusoidal space.CD31immunostaining revealed significant destruction of the endothelial lining.In the Portal group,there was no intraparenchymal hemorrhage and the sinusoidal endothelial cells and hepatocytes were well preserved.CD31immunostaining was mild which indicated less destruction of the endothelial lining.HA was significantly decreased in the Portal group compared with the Systemic group from 2 to 48 h PH.The rate of liver remnant regeneration was elevated,while apoptosis was attenuated in the Portal group compared with the Systemic group.Thymidine kinase activity was much higher in the Portal group than in the Systemic group at 48 h PH.The PCNA index was significantly increased and the apoptotic index was significantly decreased in the Portal group compared with the Systemic group.Bacterial translocation and endotoxin,as well as the inflammatory response,were significantly attenuated in the Portal group compared with the Systemic group.LPS,tumor necrosis factor-and interleukin-6 levels were all significantly decreased in the Portal group compared with the Systemic group from 24 to48 h PH,while bacterial DNA level was significantly decreased from 2 to 48 h PH.CONCLUSION:PP plus ECPD via the portal vein can attenuate toxic load and hyperperfusion injury,and should be undertaken instead of PP via the systemic circulation in SFSS or PLF.
文摘BACKGROUND Focal segmental glomerulosclerosis(FSGS)is one of the most common glomerular diseases leading to renal failure.FSGS has a high risk of recurrence after kidney transplantation.Prevention of recurrent FSGS using rituximab and/or plasmapheresis has been evaluated in multiple small studies with conflicting results.AIM To assess the risk of recurrence of FSGS after transplantation using prophylactic rituximab with or without plasmapheresis,and plasmapheresis alone compared to the standard treatment group without preventive therapy.METHODS This meta-analysis and systematic review were performed by first conducting a literature search of the MEDLINE,EMBASE,and Cochrane databases,from inception through March 2021;search terms included‘FSGS,’’steroid-resistant nephrotic syndrome’,‘rituximab,’and‘plasmapheresis,’.We identified studies that assessed the risk of post-transplant FSGS after use of rituximab with or without plasmapheresis,or plasmapheresis alone.Inclusion criteria were:Original,published,randomized controlled trials or cohort studies(either prospective or retrospective),case-control,or cross-sectional studies;inclusion of odds ratio,relative risk,and standardized incidence ratio with 95%confidence intervals(CI),or sufficient raw data to calculate these ratios;and subjects without interventions(controls)being used as comparators in cohort and cross-sectional studies.Effect estimates from individual studies were extracted and combined using a random effects model.RESULTS Eleven studies,with a total of 399 kidney transplant recipients with FSGS,evaluated the use of rituximab with or without plasmapheresis;thirteen studies,with a total of 571 kidney transplant recipients with FSGS,evaluated plasmapheresis alone.Post-transplant FSGS recurred relatively early.There was no significant difference in recurrence between the group that received rituximab(with or without plasmapheresis)and the standard treatment group,with a pooled risk ratio of 0.82(95%CI:0.47-1.45,I2=65%).Similarly,plasmapheresis alone was not associated with any significant difference in FSGS recurrence when compared with no plasmapheresis;the pooled risk ratio was 0.85(95%CI:0.60-1.21,I2=23%).Subgroup analyses in the pediatric and adult groups did not yield a significant difference in recurrence risk.We also reviewed and analyzed posttransplant outcomes including timing of recurrence and graft survival.CONCLUSION Overall,the use of rituximab with or without plasmapheresis,or plasmapheresis alone,is not associated with a lower risk of FSGS recurrence after kidney transplantation.Future studies are required to assess the effectiveness of rituximab with or without plasmapheresis among specific patient subgroups with high-risk for FSGS recurrence.
文摘Prolonged cholestasis is a very rare complication of endoscopic retrograde cholangiography (ERC). Only few cases with this complication are reported in the English literature. We report persisting cholestatic jaundice in a 73-year old man after successful therapeutic ERC for choledocholithiasis. Serologic tests for viral and autoimmune hepatitis were all negative. A second-look ERC was normal also. He denied any medication except for prophylaxis given intravenous 1 g ceftriaxon prior to the ERC procedure. After an unsuccessful trial with ursodeoxycholic acid and cholestyramine for 2 wk, this case was efficiently treated with corticosteroids and plasmapheresis. His cholestatic enzymes became normal and intense pruritis quickly resolved after this treatment which lasted during his follow- up period. We discussed the possible mechanisms and treatment alternatives of intrahepatic cholestasis associated with the ERC procedure.
文摘BACKGROUND Glycogen storage disease type Ia(GSDIa) is an autosomal recessive inborn error of carbohydrate metabolism that is caused by deficiency of the enzyme glucose-6-phosphatase(G6Pase),leading to disturbed glycogenolysis and gluconeogenesis.Patients with GSDIa show severe fasting hypoglycemia,hyperlipidemia,hyperlactacidemia,and hyperuricemia,which are associated with fatal outcomes in pregnant women and fetuses.CASE SUMMARY Herein,we report the case of a 24-year-old female who on her first visit to the hospital,presented with pregnancy combined with extremely high hyperlipidemia and hyperlactic acidosis with anemia,and frequent hypoglycemia occurred during the treatment.Genetic tests revealed a mutation in the G6Pase gene(G6PC) at 17q21,the patient was finally diagnosed with glycogen storage disease type Ia for the first time after 22 years of inaccurate treatment.She has been treated with a continuous double filtration plasmapheresis(DFPP) strategy to remove blood lipids,and a cornstarch diet therapy.The patient did not develop pancreatitis during the course of the disease and a healthy baby girl weighing 3 kg was delivered.CONCLUSION Patients with GSDIa may be misdiagnosed as epilepsy.DFPP can be used to control hyperlipidemia in GSDIa patients during pregnancy.
文摘Bickerstaff brainstem encephalitis (BBE) is a rare post-infectious neurological syndrome for which an effective treatment strategy has not been established. Here, we report a case of a 71-year-old male who suffered from an upper respiratory tract infection, and 7 days later, developed numbness of the bilateral upper and lower limbs, unsteady gait and dysarthria. Brain magnetic resonance imaging was normal, nerve conduction study and cerebral spinal fluid analysis were nonspecific. Based on the clinical features, we tentatively diagnosed Guillain-Barré syndrome and started immunoadsorption plasmapheresis. However, consciousness progressively declined to coma level within 10 days. Electroencephalogram showed diffuse slowing, and auditory evoked brainstem response (ABR) demonstrated absence of waves II, III and V. Serum anti-GQ1b IgG autoantibody and anti-GM1b IgG autoantibody were negative. Subsequently, we diagnosed BBE, and clinical symptoms resolved after treatment with intravenous immunoglobulin and methyllprednisolone. On day 62, neurological symptoms were remarkably alleviated with an improvement in ABR. Our observations suggest that immunoadsorption plasmapheresis should be used only when anti-ganglioside antibodies are detected. Combination therapy with intravenous immunoglobulin and methylprednisolone or plasma exchange?is recommended as initial therapy.
文摘The role of plasmapheresis in liver failure and hepatic encephalopathy is undefined and its use as a strategy to salvage patients with severe allograft dysfunction after liver transplantation remains investigational. We present a case of early allograft dysfunction following deceased donor liver transplantation(DDLT) where plasmapheresis was effective as a bridge to recovery and possibly avoiding a retransplantation. A 16 years old boy, known to have decompensated Wilson's disease underwent DDLT at our Public Sector Hospital. He received a healthy liver from a brain-dead donor, whose liver was considered too large for the boy. The graft was reduced in situ to a left lobe graft. Surgery was uneventful and the recipient was well for the initial 96 h. On Doppler and further computed tomography scan, a partial portal vein thrombus was noted. He was reexplored and a Fogarty endothombecteomy was performed. Following the second surgery, he developed severe allograft dysfunction with a peak bilirubin of 40 mg/d L. He underwent imaging to rule out technical causes for the dysfunction, followed by a liver biopsy, which revealed acute cellular rejection. Multiple cycles of plasmapheresis were initiated. Over the next two weeks, the graft demonstrated a gradual recovery. He was discharged on the 30 th postoperative day, with a serum bilirubin of 5.5 mg/d L. He remains well on follow-up, with the liver function tests improving further. Our report demonstrates the beneficial effect of plasmapheresis, which appears to be an effective treatment option for early allograft dysfunction following liver transplantation and may obviate the need for retransplantation.
文摘<strong>Background:</strong> Cold agglutinins are auto-antibodies that can be a nuisance in cross matching and in blood grouping. Here we report an unusual case of a high titer and wide amplitude cold agglutinin reduced by plasmapheresis. <strong>Methods and Materials:</strong> A 56-year-old man with severe anemia requested a transfusion of red blood cells. However, there was a problem in blood for blood grouping. The discrepancy of blood typing was subsequently resolved using group O absorbed plasma along with repetition of forward grouping with warm-washed red blood cells. The presence of high-thermal-amplitude and a high-titer anti-I cold agglutinin were detected in further serologic investigation. It revealed reactivity against autologous and adult O red blood cells at 37<span style="white-space:nowrap;">°</span>C by the thermal amplitude screening test, and demonstrated a very high titer of 65,536 against adult O cells by titration studies at 4<span style="white-space:nowrap;">°</span>C. The patient received two plasma exchange sessions of 1.5 plasma volumes each. There was a significant reduction of the titer of cold agglutinins and of the thermal amplitude by plasmapheresis as well (<em>p</em> < 0.01). <strong>Results:</strong> After successful cross-matching with post plasma exchanges, four units of red blood cells were infused to the patient without any hemolysis symptoms or signs. <strong>Conclusions:</strong> We now reported a patient with abnormally ascended titer of cold agglutinins and wide-thermal-amplitude, but we also successfully performed ABO typing and cross matching after 2 plasma exchange sessions of 1.5 plasma volumes each.
