Clinical profile and management of pediatric hereditary angioedema in resource-constrained settings: our experience from a single centre in North India.

Hereditary angioedema (HAE) is a rare genetic disorder. The pattern of HAE is different in children as compared to adults. There is limited literature from developing countries where all first-line treatments are either unavailable or not easily accessible. Data of children with HAE were retrieved from medical records of patients registered in the Pediatric Immunodeficiency Clinic at our institute. Of the 206 patients with HAE, 61 were diagnosed before the age of 18 years. Male: female ratio was 1.1:1. Median age at onset of symptoms and diagnosis were 6.2 years (range 1-17 years) and 10.7 years (range 1.5-18 years) respectively. Median delay in diagnosis was 4.9 years (range 0-16 years). The commonest presentation was facial swelling (51/61) followed by swelling of extremities (47/61). Laryngeal edema and abdominal symptoms were reported in 28/61 and 31/61 patients respectively. Abdominal attacks were found to be less common in children as compared to adults. Most patients in our cohort received fresh-frozen plasma (n = 5/61) as on-demand therapy. Long-term prophylaxis included attenuated androgens (n = 25/61) and tranexamic acid (n = 23/61). Median duration of follow-up was 2242 patient months. One patient died on follow-up in this cohort. This is the largest single-centre cohort of pediatric HAE from resource-constrained settings. Facial attacks were more common, and there were significant delays in diagnosis when the age of onset of symptoms was younger. Gastrointestinal symptoms were less common in children than adults. HIGHLIGHTS: One of the largest single-centre cohorts of pediatric HAE and the only one from resource-constrained settings. There were significant delays in diagnosis when the age of onset of symptoms was younger. Abdominal attacks were found to be less common in children as compared to adults.

Proteomic Characterization of Transfusable Blood Components: Fresh Frozen Plasma, Cryoprecipitate, and Derived Extracellular Vesicles via Data-Independent Mass Spectrometry.

Extracellular vesicles (EVs) are a heterogeneous collection of particles that play a crucial role in cell-to-cell communication, primarily due to their ability to transport molecules, such as proteins. Thus, profiling EV-associated proteins offers insight into their biological effects. EVs can be isolated from various biological fluids, including donor blood components such as cryoprecipitate and fresh frozen plasma (FFP). In this study, we conducted a proteomic analysis of five single donor units of cryoprecipitate, FFP, and EVs derived from these blood components using a quantitative mass spectrometry approach. EVs were successfully isolated from both cryoprecipitate and FFP based on community guidelines. We identified and quantified approximately 360 proteins across all sample groups. Principal component analysis and heatmaps revealed that both cryoprecipitate and FFP are similar. Similarly, EVs derived from cryoprecipitate and FFP are comparable. However, they differ between the originating fluids and their derived EVs. Using the R-package MS-DAP, differentially expressed proteins (DEPs) were identified. The DEPs for all comparisons, when submitted for gene enrichment analysis, are involved in the complement and coagulation pathways. The protein profile generated from this study will have important clinical implications in increasing our knowledge of the proteins that are associated with EVs derived from blood components.

A Simple, Fast, and Reliable Method for the Identification of Candida albicans.

Background: Candida is one of the common pathogens in nosocomial infections. Culture is the gold standard for diagnosing candidemia. Candida albicans is identified via the germ tube test, which uses serum as the culture medium, which is costly and time-consuming. This study was conducted to evaluate and compare a relatively simple, fast, and reliable method for the detection of Candida albicans. Methods: We conducted this randomized case study at Taipei City Hospital (TCH) from January 2023 to August 2023, with a total of 30 specimen culture reports collected and confirmed to be cases of Candida albicans infection. A germ tube test was performed in a 37°C water bath using serum, plasma, and safe plasma products (Fresh Frozen Plasma, FFP). Further, the same procedures were repeated with the addition of 22% bovine serum albumin (BSA) to the identification/culture. Results: By adding BSA, more than 50% of the budding phenomenon was observed in 40 minutes, which shortened the diagnosis time compared with the traditional method (2-3 hours). Using BSA can shorten the identification time for early clinical medication and improve the quality of medical care. Conclusion: Using safer plasma products for germ tube test of candidiasis not only reduced the risk of infection for medical technicians but could also replace the serum used in traditional methods to increase convenience and save time. This study proposed BSA as a germ tube induction medium enhancer, which reduced the culture time, thereby enabling quicker diagnosis of C. albicans infections.

Australian fresh frozen plasma audit: A National Blood Transfusion Committee and Blood Matters collaboration.

BACKGROUND: There is a paucity of high-quality data to guide appropriate fresh frozen plasma transfusion with current recommendations based on consensus opinion. The limitations of the product and testing modalities are poorly understood with the rare but potentially serious side effects underappreciated. Combined this has resulted in the widespread misuse of FFP. STUDY DESIGN AND METHODS: Retrospective data capturing FFP transfusion within the 12-month period of April 1, 2022 and March 31, 2023 was entered by Australian health care providers. Appropriate transfusion was assessed by the adjudicators and defined as one in keeping with current recommendations. Descriptive and comparative analyses were performed using SAS Studio version 9.4. RESULTS: During the study period, 935 FFP transfusion episodes were captured. The most frequent indications for FFP were massive hemorrhage 344 (37%), bleeding 141 (15%), and preoperative use 90 (10%). Males received 534 (60%) transfusions. Critical care specialists were the largest users of FFP, prescribing 568 (63%) of transfusions. FFP was used appropriately in 546 (61%) transfusions. However, when massive hemorrhage was excluded only 202 (37%) transfusions were appropriate. Patients with an INR <1.5 received 37% of transfusions. Transfusion associated adverse events were reported in 2% (15) of transfusions including two non-fatal anaphylactic reactions. DISCUSSION: This audit assesses the appropriate use of FFP across all major clinical indications and provides the largest body of evidence of Australian plasma transfusion practices. It highlights the widespread misuse of FFP, which is predominantly guided by consensus recommendations due to a lack of high-quality data.

Monogenic lupus with neuroregression in an infant due to rare compound heterozygous variants in C1QA gene: Case based review.

BACKGROUND: Monogenic lupus is a rare variant of systemic lupus erythematosus (SLE) that develops in patients with a single gene disorder. Early complement component deficiencies were the first forms of monogenic lupus to be described and C1Q gene mutations are one of the most common forms. C1QA complement deficiency has been reported to occur usually due to biallelic variants in C1QA gene and compound heterozygous variants in C1QA gene have rarely been reported. Majority of the monogenic lupus patients with C1Q deficiency present with mucocutaneous, renal, and musculoskeletal manifestations. Our patient is an unusual case of monogenic lupus with severe neurological manifestations along with cutaneous, haematological, and hepatic manifestations secondary to rare compound heterozygous variants in C1QA gene and anti-ribosomal P autoantibody positivity. She was treated with glucocorticoids, rituximab and fresh frozen plasma with partial neurological recovery. Thus, we present a unique case of monogenic lupus due to a rare compound heterozygous variant in C1QA gene with a brief review of literature.

Evaluation of the effect of fresh-frozen plasma transfusion on circulating hyaluronic acid concentration in critically ill dogs: a pilot study.

OBJECTIVE: To describe changes in circulating hyaluronic acid (HA) concentration, a biomarker of endothelial glycocalyx degradation, after administration of fresh-frozen plasma (FFP) in critically ill dogs. ANIMALS: 12 client-owned dogs receiving an FFP transfusion due to underlying disease. METHODS: Plasma samples were collected for HA concentration measurement pre-FFP transfusion (T0) and 10 minutes (T10) and 90 minutes (T90) following completion of FFP transfusion of a minimum volume of 7 mL/kg. Hyaluronic acid was also measured in the transfused FFP units following in-house validation of a commercial HA assay on citrate phosphate dextrose-anticoagulated plasma. Potential associations of the difference between pre-FFP and post-FFP HA plasma concentrations with the volume of FFP transfused, the cumulative volume of IV fluids administered during the study period, and the HA concentration in the transfused unit were explored. RESULTS: Concentrations of HA were not significantly different between pre- and post-FFP transfusion measurements. The volume of FFP transfused, the cumulative volume of other IV fluids administered during the study time, and the concentration of HA in the FFP units had no significant effect on the change in HA concentration following FFP transfusion in this study. CLINICAL RELEVANCE: This pilot study did not demonstrate an association between FFP administration and changes in plasma HA concentration. The results of this study may serve to help design future research. A commercial assay was validated to measure HA in citrate phosphate dextrose-anticoagulated plasma.

Role of plasma exchange in a post-partum case of severe thrombotic thrombocytopenic purpura with acute kidney injury.

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease present with the classic pentad of microangiopathic hemolytic anemia (MAHA), fever, neurologic changes, thrombocytopenia, and renal dysfunction. In a diagnostic dilemma, therapeutic plasma exchange (TPE) is a choice of life-saving intervention. In this, we assess the efficacy of TPE in a suspected case of post-partum TTP. A 27 years old female was admitted in an emergency on day 8 after a lower segment cesarian section (LSCS) with unresponsive behavior for 3 days and with TTP. She was normal 32 days back with her second, 7-month pregnancy. Ultrasonography (USG) showed an umbilical cord around the neck of the baby. On the fifth post-operative day, she was shifted to emergency with fever, generalized anasarca, gastrointestinal tract (GI) bleeding, low platelet count, and low Hb, with a poor Glasgow coma scale (GCS) of 6. On the bases of serum urea and serum creatinine, she presented acute kidney injury with encephalopathy. At emergency, she was unresponsive to mechanical ventilation and supportive treatment; hence, therapeutic plasma exchange was performed. After eight TPE cycles, the patient presented with an improved hematological and renal profile with good GCS. TPE is helpful and life-saving for suspected TTP patients with AKI.

Effects of Fibrinogen Concentrate Supplementation on Postoperative Bleeding in Infants Undergoing Complex Cardiac Surgery.

The use of allogeneic blood products to restore hemostasis during pediatric cardiac surgery is associated with major risks. Consequently, there has been a growing interest in new patient blood management strategies, such as those based on the use of fibrinogen concentrate (FC). Accumulating evidence has shown FC supplementation to be safe and effective. Nevertheless, no guidelines are available on using FC in the pediatric setting, and few objective evaluations have been provided in clinical practice. The endpoint of this monocenter retrospective study was the hemostatic effect of additional FC in infants undergoing complex cardiac surgery with cardiopulmonary bypass to manage persistent clinically relevant bleeding. After weaning from cardiopulmonary bypass and after protamine administration, patients were transfused with conventional allogeneic products such as packed red blood cells, fresh frozen plasma (FFP), and platelets. In the case of redo surgery, according to the institutional protocol, patients also received tranexamic acid. In case of clinically persistent relevant bleeding, according to the anesthesiologist's judgment and thromboelastography, patients received FC supplementation (group with FC) or further FFP transfusions without receiving FC supplementation (group without FC). The primary endpoint was the hemostatic effects of FC. Secondary endpoints were the functional hypofibrinogenemia threshold value (expressed as maximum amplitude fibrinogen, MA-Fib) and postoperative MA-Fib, fibrinogenemia, intraoperative transfusions, and adverse events (AEs). In total, 139 patients who underwent cardiac surgery with CPB and aged less than 2 years were enrolled: 70 patients received allogeneic blood products and FC supplementation (group FC); 69 patients received allogeneic products without FC supplementation (group without FC). Patients that received FC supplementation were characterized by a significantly longer time of extracorporeal circulation (p < 0.001) and aortic cross-clamping (p < 0.001), a significantly lower minimum temperature (p = 0.011), increased use of concentrated prothrombin complex (p = 0.016) and tranexamic acid (p = 0.010), and a significantly higher amount of packed red blood cells, platelets (p < 0.001) and fresh frozen plasma (p = 0.03). Postoperative bleeding and severe bleeding were not statistically different between patients treated with FC and those not treated with FC supplementation (p = 0.786 and p = 0.695, respectively); after adjustment, a trend toward reduced bleeding can be observed with FC (p = 0.064). Overall, 88% of patients with severe bleeding had MA-Fib < 10 mm; a moderate association between severe bleeding and MA-Fib (odds ratio 1.7, 95% CI 0.5-6.5, p = 0.425) was found. Increased MA-Fib and postoperative fibrinogen were higher in the FC group (p = 0.003 and p < 0.001, respectively) than in FFP. AEs in the FC group were comparable to those observed in less complicated surgeries. Our results suggest a potential role of FC in complex surgery in maintaining postoperative bleeding at a level comparable to less complicated surgical procedures and favoring the increase in postoperative MA-Fib and fibrinogen.

Goal-Directed Use of Prothrombin Complex Concentrates in Liver Transplantation: Is a Plasma-Free Procedure Feasible?

Background: Fresh frozen plasma (FFP) transfusions have been the mainstay of hemostatic intervention for the treatment of bleeding and coagulation abnormalities arising during liver transplantation (LT) for decades. However, numerous clinical studies showed that FFP has many side effects, including the risk of pathogen transmission, transfusion-associated circulatory overload (TACO), transfusion-related immunomodulation (TRIM), and transfusion-related acute lung injury (TRALI). These adverse events are particularly challenging in patients undergoing LT, who often suffer from severe portal hypertension, poor renal function and coexisting cardiac disease.The aims of this review are to summarize the pharmacological properties of currently available PCCs, to represent the theoretical benefits and the possible risks related to the use of these drugs in patients undergoing LT, and, finally, to review the current literature on the topic in order to highlight the evidence that currently supports PCC use in LT patients. Methods: The current literature on the topic was reviewed in order to highlight the evidence that currently supports PCC use in LT patients. Results: Prothrombin complex concentrates (PCCs) may offer several advantages when compared to FFP. Indeed, PCCs have been shown to reduce the risk of TACO, which during liver transplantation may deteriorate portal hypertension, increase intraoperative bleeding, and possibly reduce survival rates. One of the major concerns for PCC use is thrombogenicity. However, currently available PCCs are much safer as they contain inactivated forms of the vitamin K-dependent coagulation factors and protein C, protein S, antithrombin and/or heparin. Nowadays, the use of PCCs to correct coagulation abnormalities that occur during LT is an increasingly widespread practice. However, it is not yet clear what level of evidence supports this practice, and what the risks associated with it are. Conclusions: Administration of PCC in LT patients to correct haemostatic abnormalities seems to be well-tolerated, but the relationship between PCC use and thromboembolic events in the postoperative period remains unclear. Adequately powered, methodologically sound trials are urgently required for more definitive conclusions about the efficacy and safety of PCCs in a broad phenotype of LT recipients.

Evaluation of the effectiveness of fresh frozen plasma transfusion as adjuvant treatment in acute organophosphate-poisoned patients: A randomized clinical trial.

Organophosphorus (OP) poisoning is a significant cause of morbidity and mortality worldwide. Recent research has explored new approaches to improving treatment options, which present several challenges. This study aimed to evaluate the role of fresh frozen plasma (FFP) as an adjunctive therapy for acute OP intoxication. A prospective single-blinded randomized clinical trial was conducted on patients of both sexes admitted to the Intensive Care Unit (ICU) of the Poison Control Center at Ain Shams University Hospital (PCC-ASUH) with acute OP toxicity during the period from the beginning of August 2022 to the end of July 2023. According to the Peradeniya score, Group I consisted of 48 patients (52%) with moderate OP poisoning, and Group II consisted of 44 patients (48%) with severe OP poisoning. Patients in the moderate group were assigned to receive either standard treatment (Group Ia, n = 24) or standard treatment plus FFP (Group Ib, n = 24). In addition, patients in the severe group were assigned to receive either standard treatment (Group IIa, n = 22) or standard treatment plus FFP (Group IIb, n = 22). A total of 46 patients received FFP transfusion. The authors demonstrated that the early use of a total of nine packs of FFP (250 mL each) over three consecutive days significantly reduced the total doses of atropine and oximes, the total hospitalization period, and the requirement for mechanical ventilation in patients with OP poisoning, both in the moderate and severe groups.

Successful Combination of Fresh Frozen Plasma and Albumin 5% in Plasma Exchange for a Patient with Concurrent Thyroid Storm and Guillain-Barré Syndrome: A Rare Case Report.

Thyroid storm is an endocrine emergency, and treatment must ensure primary goals, including reducing the production and release of thyroid hormones, mitigating the effects of thyroid hormones, increasing the elimination of thyroid hormones, treating systemic disturbances, and managing triggering factors. However, in a few cases where thyroid storm does not respond to initial treatment, therapeutic plasma exchange (TPE) should be considered. A 50-year-old male patient was admitted to the University Medical Center Ho Chi Minh City due to hypotonia and sensory disturbances gradually spreading from the lower extremities to the entire body. The patient was diagnosed with Guillain-Barré syndrome (GBS) and newly discovered hyperthyroidism. During the treatment course, the patient developed hospital-acquired pneumonia, acting as a trigger factor for a thyroid storm. Despite aggressive treatment for thyroid storm, the patient's condition worsened, leading to the decision to perform TPE. The replacement fluid was a combination of fresh frozen plasma (FFP) and albumin 5%. Subsequently, the patient returned to a euthyroid state and was discharged. Combining FFP and albumin 5% in TPE advantages FFP's high thyroid hormones-binding capacity and albumin's cost-effectiveness, safety, and efficiency. This reduces the drawbacks associated with high volumes of FFP and offers a balanced and effective approach to managing thyroid storms. Moreover, the concurrent presence of GBS and thyroid storm is extremely rare. Through this case, we aim to discuss the role of TPE in the treatment of thyroid storms and the effectiveness of the combination of FFP and albumin 5% as the replacement fluid.

A resonance sonorheometry guided dose reduction of plasma transfusion in repetitive hip surgery in a patient with a severe factor XI deficiency: a case report.

Factor XI deficiency is a rare disorder with an unpredictable bleeding tendency. Here, we report the successful use of the sonic estimation of elasticity via resonance sonorheometry for guiding the management of haemostasis in a patient with a severe factor XI deficiency in repeated revision hip surgeries. Regardless of an administration of fresh frozen plasma, a significant haemorrhage occurred at the first of three hip surgeries. The repeat application of fresh frozen plasma normalised the prolonged activated partial thromboplastin time and the resonance sonorheometry clot time values; the factor XI activity increased to a sufficient level. No significant bleeding occurred in the second and third hip surgery. Using a resonance sonorheometry guided approach in haemostasis management has the potential to improve safety for patients with factor XI deficiency undergoing surgery by ensuring sufficient clotting and preventing side effects.

Complications of therapeutic plasma exchange: A retrospective, single-center study of 2505 procedures performed in 338 patients over 15 years.

INTRODUCTION: Therapeutic plasma exchange (TPE) may involve complications. We aimed to review the demographic data, indications, technical information, and complications. METHODS: Data for TPE procedures (TPEPs) performed between 2004 and 2018 were retrospectively. RESULTS: This study covered 2505 TPEPs performed on 338 patients; 55% of them were female (n = 186), and the median age was 36 years (range, 11-93 years). Most TPEPs were administered for hematological (40.6%) indications. The incidence of complications on the first procedure was 3.2% (n = 80); only 16 procedures (0.6%) were failed. The complication incidence was 19.8% (n = 497), with 789 total complications. Most of the complications were patient-related (90.4%), and the most of them were urticaria (29.1%), occlusion (3.2%), and faulty systems (1.01%), respectively. The use of only fresh frozen plasma as replacement fluid caused a higher complication rate (22.1%, p < 0.01). CONCLUSION: The number of TPEPs is increasing every day. Hematologic indications for TPE and the use of fresh frozen plasma may increase the risk of complications.

Association of perioperative transfusion of fresh frozen plasma and outcomes after cardiac surgery.

BACKGROUND: Fresh frozen plasma (FFP) transfusion is used to manage coagulopathy and bleeding in cardiac surgery patients despite uncertainty about its safety and effectiveness. METHODS: We performed a propensity score matched analysis of the Australian and New Zealand Society of Cardiac and Thoracic Surgeons National Cardiac Surgery Database including patients from 39 centres from 2005 to 2018. We investigated the association of perioperative FFP transfusion with mortality and other clinical outcomes. RESULTS: Of 119,138 eligible patients, we successfully matched 13,131 FFP recipients with 13,131 controls. FFP transfusion was associated with 30-day mortality (odds ratio (OR), 1.41; 99% CI, 1.17-1.71; p < .0001), but not with long-term mortality (hazard ratio (HR), 0.92; 99% CI, 0.85-1.00; p = .007, Holm-Bonferroni α = 0.0004). FFP was also associated with return to theatre for bleeding (OR, 1.97; 99% CI, 1.66-2.34; p < .0001), prolonged intubation (OR, 1.15; 99% CI, 1.05-1.26; p < .0001) and increased chest tube drainage (Mean difference (MD) in mL, 131; 99% CI, 120-141; p < .0001). It was also associated with reduced postoperative creatinine levels (MD in g/L, -6.33; 99% CI, -10.28 to -2.38; p < .0001). CONCLUSION: In a multicentre, propensity score matched analysis, perioperative FFP transfusion was associated with increased 30-day mortality and had variable associations with secondary clinical outcomes.

Long-term clinical outcomes and management of hypertriglyceridemia in children with Apo-CII deficiency.

BACKGROUND AND AIM: APO CII, one of several cofactors which regulate lipoprotein lipase enzyme activity, plays an essential role in lipid metabolism. Deficiency of APO CII is an ultra-rare autosomal recessive cause of familial chylomicronemia syndrome. We present the long-term clinical outcomes of 12 children with APO CII deficiency. METHODS AND RESULTS: The data of children with genetically confirmed APO CII deficiency were evaluated retrospectively. Twelve children (8 females) with a mean follow-up of 10.1 years (±3.9) were included. At diagnosis, the median age was 60 days (13 days-10 years). Initial clinical findings included lipemic serum (41.6%), abdominal pain (41.6%), and vomiting (16.6%). At presentation, the median triglyceride (TG) value was 4341 mg/dL (range 1277-14,110). All patients were treated with a restricted fat diet, medium-chain triglyceride (MCT), and omega-3-fatty acids. In addition, seven patients (58.3%) received fibrate. Fibrate was discontinued in two patients due to rhabdomyolysis and in one patient because of cholelithiasis. Seven (58.3%) patients experienced pancreatitis during the follow-up period. One female experienced recurrent pancreatitis and was treated with fresh frozen plasma (FFP). CONCLUSIONS: Apo CII deficiency is an ultra-rare autosomal recessive condition of hypertriglyceridemia associated with significant morbidity and mortality. Low-fat diet and MCT supplementation are the mainstays of therapy, while the benefit of TG-lowering agents are less well-defined.

Fresh Frozen Plasma Versus Solvent Detergent Plasma for Cardiopulmonary Bypass Priming in Neonates and Infants Undergoing Cardiac Surgery: A Retrospective Cohort Study.

OBJECTIVE: Compared with fresh frozen plasma (FFP), Omniplasma has been attributed to an increased coagulation potential and an increased fibrinolytic potential. This study aimed to compare Omniplasma and FFP used for cardiopulmonary bypass (CPB) priming regarding the incidence of postoperative thrombotic or hemorrhagic complications and outcomes in pediatric patients undergoing cardiac surgery. DESIGN: A retrospective observational cohort study SETTING: This single-center study was performed at the University Medical Center Groningen. PARTICIPANT: All pediatric patients up to 10 kg undergoing cardiac surgery with CPB. INTERVENTIONS: Procedures in which FFP was used for CPB priming were compared with those in which Omniplasma was used. MEASUREMENTS AND MAIN RESULTS: The primary outcome parameter was a composite endpoint consisting of the following: (1) pediatric intensive care unit (PICU) mortality, (2) thromboembolic complications, and (3) hemorrhagic complications during PICU stay. The authors included 143 procedures in the analyses, 90 (63%) in the FFP group and 53 (37%) in the Omniplasma group. The occurrence of the combined primary endpoint (FFP 20% v Omniplasma 11%, p = 0.18) and its components did not differ between the used CPB priming agent). Omniplasma for CPB priming was associated with decreased unfractionated heparin administration per kg bodyweight (585 IU v 510 IU, p = 0.03), higher preoperative and postoperative activated clotting times (ACT) discrepancy (90% v 94%, p = 0.03), a lower postoperative ACT value (125 v 118 seconds, p = 0.01), and less red blood cell transfusion per kilogram bodyweight (78 v 55 mL, p = 0.02). However, none of the variables differed statistically significantly in the multivariate logistic regression analyses. CONCLUSIONS: The authors did not find an association between the plasma used for CPB priming and thromboembolic and hemorrhagic complications and death in neonates and infants undergoing cardiac surgery. Omniplasma seems to be safe to use in this population.

Efficacy of fresh frozen plasma transfusion in decompensated cirrhosis patients with coagulopathy admitted to ICU: a retrospective cohort study from MIMIC-IV database.

We aimed to explore the association between FFP transfusion and outcomes of DC patients with significant coagulopathy. A total of 693 DC patients with significant coagulopathy were analyzed with 233 patients per group after propensity score matching (PSM). Patients who received FFP transfusion were matched with those receiving conventional therapy via PSM. Regression analysis showed FFP transfusion had no benefit in 30-day (HR: 1.08, 95% CI 0.83-1.4), 90-day (HR: 1.03, 95% CI 0.80-1.31) and in-hospital(HR: 1.30, 95% CI 0.90-1.89) mortality, associated with increased risk of liver failure (OR: 3.00, 95% CI 1.78-5.07), kidney failure (OR: 1.90, 95% CI 1.13-3.18), coagulation failure (OR: 2.55, 95% CI 1.52-4.27), respiratory failure (OR: 1.76, 95% CI 1.15-2.69), and circulatory failure (OR: 2.15, 95% CI 1.27-3.64), and even associated with prolonged the LOS ICU (ß: 2.61, 95% CI 1.59-3.62) and LOS hospital (ß: 6.59, 95% CI 2.62-10.57). In sensitivity analysis, multivariate analysis (HR: 1.09, 95%CI 0.86, 1.38), IPTW (HR: 1.11, 95%CI 0.95-1.29) and CAPS (HR: 1.09, 95% CI 0.86-1.38) showed FFP transfusion had no beneficial effect on the 30-day mortality. Smooth curve fitting demonstrated the risk of liver failure, kidney failure and circulatory failure increased by 3%, 2% and 2% respectively, for each 1 ml/kg increase in FFP transfusion. We found there was no significant difference of CLIF-SOFA and MELD score between the two group on day 0, 3, 7, 14. Compared with the conventional group, INR, APTT, and TBIL in the FFP transfusion group significantly increased, while PaO2/FiO2 significantly decreased within 14 days. In conclusion, FFP transfusion had no beneficial effect on the 30-day, 90-day, in-hospital mortality, was associated with prolonged the LOS ICU and LOS hospital, and the increased risk of liver failure, kidney failure, coagulation failure, respiratory failure and circulatory failure events. However, large, multi-center, randomized controlled trials, prospective cohort studies and external validation are still needed to verify the efficacy of FFP transfusion in the future.

Comparative Analysis of AB vs. ABO-specific Plasma for Desensitization in Blood Group O Recipients: An In Vitro Study.

Neutralizing capacity measurement (NCM) of soluble ABH substances (SAS) in plasma was assessed to guide the selection of the appropriate ABO group of fresh-frozen plasma (FFP) for plasma exchange (PE) in blood group O recipients with ABO-incompatible transplantations. Neutralizing capacity was assessed by measuring anti-A and/or anti-B titers in samples comprising one unit of O FFP and 10 O EDTA plasma samples and subtracting the binary logarithm of the titer in each group with a saline dilution. Ten EDTA plasma samples with Lewis b (Leb) antigen positivity and 10 sets of pooled FFP from each blood group were used as diluents. In O FFP, the NCM values (mean±SD) were 3.4±0.52 (2.6±0.52) and 2.6±0.52 (1.5±0.3) in B and AB for IgM (total antibody) anti-B (both P<0.001), and in the 10 O EDTA plasma samples, they were 3.9±0.88 (3.1±0.88) and 3.2±0.79 (2.4±0.97) for IgM (P=0.0013) and total anti-B (P=0.025), respectively. In vitro analysis revealed that B FFP is more effective than AB FFP in reducing IgM and total anti-B antibody titers in O recipients, regardless of Leb antigen positivity.

Deleterious effects of plasma-derived cellular debris in a porcine model of hemorrhagic shock.

BACKGROUND: Recent studies identify large quantities of inflammatory cellular debris within Fresh Frozen Plasma (FFP). As FFP is a mainstay of hemorrhagic shock resuscitation, we used a porcine model of hemorrhagic shock and ischemia/reperfusion to investigate the inflammatory potential of plasma-derived cellular debris administered during resuscitation. METHODS: The porcine model of hemorrhagic shock included laparotomy with 35 % hemorrhage (Hem), 45 min of ischemia from supraceliac aortic occlusion with subsequent clamp release (IR), followed by protocolized resuscitation for 6 h. Cellular debris (Debris) was added to the resuscitation phase in three groups. The four groups consisted of Hem + IR (n = 4), Hem + IR + Debris (n = 3), Hem + Debris (n = 3), and IR + Debris (n = 3). A battery of laboratory, physiologic, cytokine, and outcome data were compared between groups. RESULTS: As expected, the Hem + IR group showed severe time dependent decrements in organ function and physiologic parameters. All animals that included both IR and Debris (Hem + IR + Debris or IR + Debris) died prior to the six-hour end point, while all animals in the Hem + IR and Hem + Debris survived. Cytokines measured at 30-60 min after initiation of resuscitation revealed significant differences in IL-18 and IL-1ß between all groups. CONCLUSIONS: Ischemia and reperfusion appear to prime the immune system to the deleterious effects of plasma-derived cellular debris. In the presence of ischemia and reperfusion, this model showed the equivalency of 100 % lethality when resuscitation included quantities of cellular debris at levels routinely administered to trauma patients during transfusion of FFP. A deeper understanding of the immunobiology of FFP-derived cellular debris is critical to optimize resuscitation for hemorrhagic shock.

Transfusion of non-red blood cell blood products does not reduce survival following cardiac surgery.

OBJECTIVES: The literature supports the assertion that patients undergoing cardiac surgery who receive perioperative packed red blood cell (pRBC) transfusions have increased associated mortality. The aim of the current study is to assess whether there is an association between non-pRBC blood product transfusions and increased mortality. METHODS: Data from our center's Society of Thoracic Surgeons database included patients who underwent cardiac surgery from 2010 to 2018. Patients with pRBC transfusions or circulatory arrest were excluded. Propensity matching was performed (1:1; caliper = 0.2 times the standard deviation of logit of propensity score). Kaplan-Meier estimates and Cox regression were used. Cardiac transplant, ventricular assist devices, transcatheter aortic valves, and patients who had experienced circulatory arrest were excluded from this analysis. RESULTS: A total of 8042 patients met criteria for analysis. Following propensity matching (1:1), 395 patients requiring perioperative non-pRBC blood products (platelets, fresh-frozen plasma, and cryoprecipitate) were matched with 395 nontransfusion patients, yielding equitable patient cohorts. Median follow-up was 4.5 (3.0-6.4) years. Patients received platelets (327 [82.8%]), fresh-frozen plasma (141 [35.7%]), and cryoprecipitate (60 [15.2%]). There was no significant difference in the postoperative mortality (6 [1.5%] vs 4 [1.0%]; P = .52). Reoperation (20 [5.0%] vs 8 [2.0%]; P < .02) and prolonged ventilation (36 [9.1%] vs 19 [4.8%]; P < .02) were greater in the transfusion group. Emergent operation (odds ratio [OR] 2.86 [1.72-4.78]; P < .001), intra-aortic balloon pump (OR 3.24 [1.64-6.39]; P < .001), and multivalve operation (OR 4.34 [2.83-6.67]; P < .001) were significantly associated with blood product use. Blood product transfusion (hazard ratio; 1.15 [0.89-1.48]; P = .3) was not significantly associated with increased mortality risk. There was no significant long-term survival difference between cohorts. CONCLUSIONS: Patients who undergo cardiac surgery requiring blood products alone, without pRBC transfusion, have similar postoperative and long-term survival compared with patients not requiring blood products. These data are based on a limited patient sample, and future studies will aid in improving the generalizability of these results.