BACKGROUND: The primary limitation to kidney transplantation is organ shortage. Recent progress in gene editing and immunosuppressive regimens has made xenotransplantation with porcine organs a possibility. However, evidence in pig-to-human xenotransplantation remains scarce, and antibody-mediated rejection (AMR) is a major obstacle to clinical applications of xenotransplantation. METHODS: We conducted a kidney xenotransplantation in a brain-dead human recipient using a porcine kidney with five gene edits (5GE) on March 25, 2024 at Xijing Hospital, China. Clinical-grade immunosuppressive regimens were employed, and the observation period lasted 22 days. We collected and analyzed the xenograft function, ultrasound findings, sequential protocol biopsies, and immune surveillance of the recipient during the observation. RESULTS: The combination of 5GE in the porcine kidney and clinical-grade immunosuppressive regimens prevented hyperacute rejection. The xenograft kidney underwent delayed graft function in the first week, but urine output increased later and the single xenograft kidney maintained electrolyte and pH homeostasis from postoperative day (POD) 12 to 19. We observed AMR at 24 h post-transplantation, due to the presence of pre-existing anti-porcine antibodies and cytotoxicity before transplantation; this AMR persisted throughout the observation period. Plasma exchange and intravenous immunoglobulin treatment mitigated the AMR. We observed activation of latent porcine cytomegalovirus toward the end of the study, which might have contributed to coagulation disorder in the recipient. CONCLUSIONS 5GE and clinical-grade immunosuppressive regimens were sufficient to prevent hyperacute rejection during pig-to-human kidney xenotransplantation. Pre-existing anti-porcine antibodies predisposed the xenograft to AMR. Plasma exchange and intravenous immunoglobulin were safe and effective in the treatment of AMR after kidney xenotransplantation.
Transplantation, Heterologous/methods* ; Kidney Transplantation/methods* ; Heterografts/pathology* ; Immunoglobulins, Intravenous/administration & dosage* ; Graft Survival/immunology* ; Humans ; Animals ; Sus scrofa ; Graft Rejection/prevention & control* ; Kidney/pathology* ; Gene Editing ; Species Specificity ; Immunosuppression Therapy/methods* ; Plasma Exchange ; Brain Death ; Biopsy ; Male ; Aged

Pediatric acute liver failure (PALF) is a severe and rare clinical syndrome characterized by rapid progression and high mortality. Current main treatment strategies include medical therapy, artificial liver support, and liver transplantation. Given the limited efficacy of medical treatment and the challenges of liver transplantation, such as donor scarcity and high costs, the non-biological artificial liver (NBAL) support system has become a widely used and effective alternative in clinical practice. It provides critical time for liver function recovery or as a bridging therapy to transplantation. Common NBAL modalities include plasma exchange (PE), plasma adsorption (PA), albumin dialysis (AD), and various combination therapies. Therapeutic PE removes toxins by replacing plasma and is suitable as adjuvant therapy in liver failure; high-volume PE is used in acute liver failure but is costly. PA and double plasma molecular adsorption systems remove specific toxins while reducing plasma consumption. AD systems eliminate macromolecular toxins through different mechanisms. Hybrid blood purification therapies combine multiple modes to enhance solute clearance efficiency. Elucidating the clinical characteristics and applications of various NBAL techniques in pediatric acute liver failure may provide valuable guidance for the use of NBAL support systems in pediatric clinical practice.
Humans ; Liver Failure, Acute/therapy* ; Liver, Artificial ; Plasma Exchange/methods* ; Child

BACKGROUND: This study examined the outcomes of ABO incompatible living donor liver transplantation (LDLT). The changes in the immunologic factors that might help predict the long term outcomes were also studied. METHODS: Twenty-three patients, who underwent ABO incompatible LDLT from 2010 to 2015, were reviewed retrospectively. The protocol was the same as for ABO compatible LDLT except for the administration of rituximab and plasma exchange. The clinical outcomes and immunologic factors, such as isoagglutinin titer and cluster of differentiation 20+ (CD20+) lymphocyte levels were reviewed. RESULTS: The center showed a 3-year survival of 64% with no case of antibody-mediated rejection. When transplantation-unrelated mortalities (for example, traffic accidents and myocardial infarction) were removed from statistical analysis, the 3-year survival was 77.8%. Although isoagglutinin titers continued to remain at low levels, the CD20+ lymphocyte levels recovered to the pre-Rituximab levels at postoperative one year. CONCLUSIONS: As donor shortages continue, ABO incompatible liver transplantation is a feasible method to expand the donor pool. On the other hand, caution is still needed until more long-term outcomes are reported. Because CD20+ lymphocytes are recovered with time, more immunologic studies will be needed in the future.
ABO Blood-Group System ; Accidents, Traffic ; B-Lymphocytes ; Hand ; Hemagglutinins ; Humans ; Immunologic Factors ; Liver Transplantation* ; Liver* ; Living Donors* ; Lymphocytes ; Methods ; Mortality ; Plasma Exchange ; Retrospective Studies ; Rituximab ; Tissue Donors

Thrombotic thrombocytopenic purpura (TTP) is a rare blood disorder with a mortality rate of over 90% without prompt treatment. It is caused by congenital, idiopathic, or secondary diseases; idiopathic TTP is mainly associated with deficiency of ADAMTS13, a von Willebrand factor cleaving protease or ADAMTS13 inhibitors. The long-term survival rate of TTP has improved since the introduction of therapeutic plasma exchange (TPE), and the therapeutic aims have also been established. However, deciding on the end-point and appropriate treatment method requires careful assessment of clinical conditions of patients. The present study reports a case of a 33-year-old male patient with reduced ADAMTS13 activity and ADAMTS13 inhibitor, who developed symptoms after an early termination of TPE with improved symptoms, which finally improved with retreatment and additionally corticosteroid. We report our case with relevant literature review on TPE in TTP with this case.
Adult ; Humans ; Male ; Methods ; Mortality ; Plasma Exchange* ; Plasma* ; Plasmapheresis ; Purpura, Thrombotic Thrombocytopenic* ; Retreatment ; Survival Rate ; von Willebrand Factor

Systemic lupus erythematosus-related acute pancreatitis (SLEAP) has a poor prognosis with a high mortality. We described the clinical features of SLEAP, and discussed the feasibility of plasma exchange (PE) combined with glucocorticosteroids (GC) in short-term prognosis and possible mechanism in reducing serum inflammatory cytokine IL-6 and removing serum lipids. A retrospective study was performed by an independent rheumatologist. Medical records of SLEAP from March 2010 to December 2014 were retrieved from Tongji Hospital information system, and patients were divided into two groups according to whether PE therapy was adopted. Sixteen patients treated with PE in combination with GC were classified as group A, and the other 10 patients who were treated with merely GC were classified as group B. Patients' clinical remission rate and average daily GC dosage after two-week therapy were compared between the two groups. Patients' serum inflammatory cytokines and lipid concentration were compared between baseline and after two-week treatment in both groups. Pearson correlation test was performed to determine association between serum cytokines and Ranson score. SLEDAI score in group A patients at baseline (14.8±3.1) showed no statistical difference from that in group B (14.1±3.3). At baseline serum IL-6 levels had no significant difference between group A [13.14 (11.12, 16.57) mg/L] and group B [14.63 (11.37, 16.37) mg/L]; after two-week therapy IL-6 decreased significantly in group A [9.16 (7.93, 10.75)mg/L] while it did not show decreasing trend in group B [13.62 (9.29,17.63) mg/L]. Serum lipid concentration after two-week therapy in group A [(TC=5.02±0.53, TG=1.46±0.44) mmol/L] decreased significantly compared to baseline [(TC=6.11±0.50, TG=2.14±1.03) mmol/L], while similar tendency was not observed in group B. The remission rate after two-week therapy was higher in group A (70.0%) than in group B (25.0%). Acute pancreatitis (AP) was one of the clinical manifestations of active SLE. PE combined with GC could reduce serum IL-6 level, and remove serum lipid to improve short-term prognosis. Therefore, it might be a safe and effective way in treating SLEAP and was worth continuing to explore its feasibility.
China ; Female ; Glucocorticoids ; administration & dosage ; Humans ; Interleukin-6 ; blood ; Lipids ; blood ; Lupus Erythematosus, Systemic ; complications ; genetics ; pathology ; therapy ; Male ; Middle Aged ; Pancreatitis ; blood ; etiology ; pathology ; therapy ; Plasma Exchange ; methods ; Prognosis

OBJECTIVETo investigate the clinical application, indication, timing and prognosis of blood purification (artificial liver, BP) in treatment of acute liver failure in children.

METHODArtificial liver was used to treat 30 cases of pediatric acute liver failure (PALF), who were hospitalized in pediatric intensive care unit of Bayi Children's Hospital Affiliated to Beijing Military Command General Hospital, during March 2010 to July 2013. Simple plasma exchange (PE) mode was used for PALF without complications, while PE combined with continuous veno-venous hemodiafiltration (CVVHDF) mode was used for PALF with cerebral edema and/or hepatorenal syndrome and/or serious abnormality of electrolyte and acid-base balance.

RESULTSixteen cases survived and restored hepatic function, with a survival rate of 53.3%. Single PE therapy could significantly decrease total bilirubin (TBIL) from (293.96 ± 214.52) µmol/L to (155.64 ± 140.97) µmol/L (P = 0.033), increase prothrombin time activity (PTA) from (34.50 ± 18.34) % to (60.50 ± 33.97) % (P = 0.013), while it did not significantly influence ammonia from (156.43 ± 67.23) µmol/L to (124.03 ± 62.58) µmol/L (P = 0.156) and alanine transarninase (ALT) from (752.53 ± 1 291.84) U/L to (132.00 ± 98.57) U/L (P = 0.066). PE + CVVHDF therapy could significantly ameliorate TBIL from (326.90 ± 233.85) µmol/L to (157.53 ± 125.31) µmol/L (P = 0.033), ALT from (1 476.64 ± 1 728.18) U/L to (169.38 ± 207.18) U/L (P = 0.019), ammonia from (215.83 ± 83.92) µmol/L to (141.25 ± 63.09) µmol/L (P = 0.022) and PTA from (36.68 ± 23.13)% to (71.75 ± 50.50) % (P = 0.044). Prothrombin time (PT) from (29.71 ± 17.75)s to (16.27 ± 6.38)s (P = 0.008) , ALT from (1 574.11 ± 1 775.96) U/L to (145.81 ± 113.89 ) U/L (P = 0.003) , TBIL from (233.16 ± 219.70) µmol/L to (75.19 ± 86.07) µmol/L (P = 0.012) , ammonia from (182.75 ± 90.07) µmol/L to (101.81 ± 37.14) µmol/L (P = 0.002) and PTA from (38.38 ± 20.39)% to (83.13 ± 41.68)% (P = 0.001) in survived cases significantly ameliorated after BP therapy. TBIL from (394.04 ± 192.80) µmol/L to (249.34 ± 113.97) µmol/L (P = 0.023) in died cases declined significantly after BP therapy, while alteration of PT, ALT, ammonia , and PTA had no statistical significance (P > 0.10) after BP therapy.

CONCLUSIONPE + CVVHDF therapy could significantly ameliorate not only TBIL and PTA but also ammonia and ALT compared with single PE therapy. The decline of only an index like TBIL or ALT after BP therapy could not improve the prognosis. The inconsistency between serum bilirubin and ALT levels was an important factor that suggested poor prognosis of ALF, and it might increase survival rate to use BP therapy before that inconsistency emerged.

Adolescent ; Bilirubin ; blood ; Biomarkers ; blood ; Brain Edema ; etiology ; therapy ; Child ; Child, Preschool ; Female ; Hemodiafiltration ; methods ; Heparin ; pharmacology ; Hepatorenal Syndrome ; etiology ; therapy ; Humans ; Infant ; Liver Failure, Acute ; blood ; complications ; mortality ; therapy ; Liver Function Tests ; Male ; Plasma Exchange ; Prothrombin Time ; Survival Rate ; Treatment Outcome

OBJECTIVE: To explore the effective treatment and prognostic factors for fulminant Wilson's disease (FWD). METHODS: We retrospectively analyzed the clinical characteristics, therapeutic Methods and outcomes of 13 FWD patients. We investigated the treatment effect of the joint use of hormones, decoppering, and plasma exchange therapy in patients with FWD, compared the difference in the clinical features, biochemical data and treatment between the survival group and the death group. RESULTS: Thirteen patients with FWD presented with acute hepatic failure and severe jaundice: 7 accompanied with severe hemolytic anemia, 5 with primary peritonitis, 8 with hepatic encephalopathy. Prothrombin activity (PTA) of 5 was below 30% in the 13 patients. Plasma exchange (PE), dimercaptopropansulfonate sodium (DMPS) and short-term methylprednisolone /dexamethasone administration were performed in 7 patients, in which 6 survived and the other 1 who had primary peritonitis with PTA below 30% died. The other 6 patients without above-mentioned treatments all died, in which 4 accompanied with primary peritonitis with PTA all below 30%. CONCLUSION The level of hepatic failure and the occurrence of infection are the decisive factors for prognosis of patients with FWD. PE with decoppering treatment and corticosteroid administration are effective.
Chelating Agents ; therapeutic use ; Combined Modality Therapy ; Copper ; blood ; Female ; Hepatolenticular Degeneration ; therapy ; Humans ; Male ; Methylprednisolone ; therapeutic use ; Plasma Exchange ; methods ; Prognosis ; Retrospective Studies ; Unithiol ; therapeutic use

OBJECTIVETo evaluate the effects of continuous blood purification on the hemodynamics and oxygenation in patients with acute respiratory distress syndrome (ARDS).

METHODSTwenty-one patients with ARDS were treated with continuous veno-venous hemofiltration (CVVH) combined with plasma exchange. Hemodynamics and oxygenation were measured or calculated at scheduled intervals using Swan-Ganz catheters.

RESULTSThe mean arterial pressure, partial pressure of arterial oxygen, oxygen delivery, oxygen consumption increased, heart rate, mean pulmonary arterial pressure, pulmonary capillary wedge pressure, blood lactate concentration all decreased significantly after the treatment, and the oxygen extraction ratio underwent no obvious changes.

CONCLUSIONSContinuous blood purification can increase blood and oxygen supply but has no significant effects on oxygen extraction ratio in ARDS patients.

Adult ; Female ; Hemodynamics ; Hemofiltration ; methods ; Humans ; Male ; Middle Aged ; Oxygen ; blood ; Oxygen Consumption ; physiology ; Partial Pressure ; Plasma Exchange ; methods ; Pulmonary Gas Exchange ; Respiratory Distress Syndrome, Adult ; therapy

Adsorption ; Bilirubin ; metabolism ; Liver, Artificial ; Plasma Exchange ; methods

Humans ; Liver Failure ; therapy ; Liver, Artificial ; Membranes, Artificial ; Plasma ; Plasma Exchange ; methods ; Plasma Substitutes ; therapeutic use ; Renal Dialysis ; methods ; Serum Albumin ; therapeutic use