Plasma Exchange Research Articles

How to make a decision for the use of plasma exchange in patients with acute liver failure?

How to make a decision for the use of plasma exchange in patients with acute liver failure?

Plasmapheresis for extracorporeal membrane oxygenation (ECMO)-induced hemolysis in infants

Background: Intravascular hemolysis is a known complication of extracorporeal membrane oxygenation (ECMO). Characterized by elevated plasma-free hemoglobin (PFH), intravascular hemolysis is associated with cytotoxic effects leading to renal replacement therapy (RRT), longer ECMO runs and mortality. Therapeutic plasma exchange (TPE) in tandem with ECMO was described as a therapy for various pathologic conditions, but there are no ELSO guidelines for the treatment of ECMO-induced hemolysis. We describe the use of TPE in the management of severe ECMO-induced hemolysis. Methods: Two term neonates receiving veno-arterial (VA) ECMO developed severe PFH, with peak values over 500 mg/dL. TPE was performed in tandem with the ECMO circuit. Packed red cells were used to prime the TPE circuit, and citrate anticoagulation was added to establish the interface, which could not be achieved with existing heparin in the ECMO circuit. Therapy was completed with saline solution as a decoy for citrate, to avoid hypocalcemia and intracranial bleeding. Plasma volume was replaced by fresh frozen plasma (FFP). Results: In one patient PFH fell to 120 mg/dL, but rebounded to close to 500 mg/dL, only to stabilize between 210 and 300 mg/dL after the second TPE. He was liberated from ECMO, but could not survive a respiratory decompensation. The other patient’s PFH improved to 360 mg/dL after one TPE and continued to decline to 120 mg/dL over the ensuing days. Despite that improvement, care was withdrawn. Conclusion: TPE is effective in decreasing the burden of PFH, is well tolerated in tandem with ECMO, and a database of infants with ECMO-induced hemolysis needs to be created to assess the current practice, and establish clinical guidelines for its most appropriate therapy.

The Difficulties of Treating Complement-3–Mediated Glomerulopathy

Background: C3 glomerulopathy (C3G) is a rare disease affecting the complement alternative pathway, categorized into dense deposit disease and C3 glomerulonephritis. Dense deposit disease predominantly affects younger individuals, while C3 glomerulonephritis tends to manifest in older populations. The diseases are characterized by dysregulation of the complement alternative pathway, leading to the deposition of complement components in the glomeruli and subsequent renal dysfunction. Notably, the incidence of C3G in the United States is low, with 1–3 cases per 1,000,000 and a prevalence of 5 cases per 1,000,000. Areas of Uncertainty: Numerous uncertainties persist in comprehending the etiology and pathophysiology of C3G. While biomarkers such as C3 nephritic factor, autoantibodies, and relevant genetic mutations have been identified, their pathogenicity and clinical utility remain unclear. Standard workups involve complement assays and autoantibody panels, yet the definitive diagnostic test remains a kidney biopsy. Nuanced challenges lie in deciphering the sensitivity and specificity of these diagnostic tools, especially in the presence of phenotypical variations among individuals. Therapeutic Advancement: Current therapeutic approaches, albeit lacking robust evidence, encompass a spectrum ranging from supportive care to targeted B-cell therapy and immunosuppression with mycophenolate mofetil and glucocorticoids. For severe and refractory cases, the monoclonal antibody eculizumab, targeting C5 in the complement cascade, is recommended. These treatments, while offering some relief, pose challenges related to their cost and obtaining insurance approval. Exploratory avenues delve into the potential of plasma exchange and innovative treatments such as oral complement inhibitors, reflecting the ongoing quest for effective therapeutic modalities. Trials investigating various complement inhibitors underscore the dynamic landscape of therapeutic advancements in C3G management. Conclusion: In conclusion, the article highlights the complexities of C3G management. The need for further understanding, large-scale trials, and ongoing investigations into disease etiology and pathophysiology is emphasized.

Therapeutic Plasma Exchange is Associated with Increased Survival in Heart Transplant Recipients Experiencing Severe Primary Graft Dysfunction

Therapeutic Plasma Exchange is Associated with Increased Survival in Heart Transplant Recipients Experiencing Severe Primary Graft Dysfunction

Numerical Investigation of G–V Measurements of metal – A Nitride GaAs junction

In this study, a Schottky diode consisting of Au/GaN/GaAs was fabricated using a radiofrequency nitrogen plasma source. The voltage-conductance characteristics (G/ω-V) of this diode structure were investigated at room temperature. To interpret the changes in the electrical properties of the nitrided GaAs-based Schottky structure, we developed a simulation program. This program employs a numerical model to calculate the G-V characteristics, allowing us to validate the experimental measurements conducted on the Schottky diodes. The geometric model used in our simulation considers not only the GaN layer formed between the metal and GaAs substrate but also the density and distribution of trapped states within the band gap. The program utilizes the numerical resolution of the Poisson and continuity equations to calculate the electrostatic potential and the concentrations of both n and p mobile carriers. These parameters are then used to determine the electric charge, current, capacitance, and conductance. The simulation results were subsequently compared to the experimental measurements to ensure their accuracy.

Real-World Data on Effectiveness and Safety of First-Line Use of Caplacizumab in Italian Centers for the Treatment of Thrombotic Thrombocytopenic Purpura: The Roscapli Study.

Background/Objectives: Immune thrombotic thrombocytopenic purpura (iTTP) is a thrombotic microangiopathy caused by the formation of anti-ADAMTS13 antibodies. Caplacizumab is approved for the treatment of acute episodes of iTTP in conjunction with plasma exchange (PEX) and immunosuppression. Real-world data for the use of caplacizumab in Italy have been recently published by a limited number of centers located in the northern and middle regions of the country only. Methods: A total of 38 patients with iTTP were enrolled in the study in six Italian centers spread over the entire territory of the country. The patients' data were registered in eCRF. Results: All patients achieved normalization of platelet count (median 2.0 days, IQR: 2-4), within a time significantly shorter than in the absence of caplacizumab, as previously reported in other studies. As to the secondary aims, patients treated with caplacizumab had a few exacerbations (4/38 (10.5%)) and relapses (2/38, 5.3%). No deaths or refractoriness were observed in these patients. The total length of hospitalization was 12 days (IQR: 9-18) and only one patient required 2 days of stay in the intensive care unit. Interestingly, when caplacizumab was initiated within the first 3 days, the plasma exchange (PEX) duration was 9 days (IQR: 8-10), which was significantly lower than those reported in previous studies conducted in the absence of caplacizumab. No severe adverse event was described in the caplacizumab-treated patients. Conclusions: Caplacizumab reduced exacerbations and refractoriness compared with previously reported standard-of-care regimens. When administered in association with PEX and immunosuppressive therapy, caplacizumab provided rapid normalization of platelet count, which was responsible for lower overall hospitalization time, ICU stay, lower exacerbations and relapses compared to previously reported outcomes of studies carried out without caplacizumab.

Plasma exchange with albumin replacement for Alzheimer's disease treatment induced changes in serum and cerebrospinal fluid inflammatory mediator levels.

There is extensive literature indicating that inflammatory pathways are affected in Alzheimer's disease (AD). We examined whether plasma exchange with albumin replacement (PE-Alb) can impact the inflammatory status of AD patients and alter the relationship between inflammatory mediators and cognitive measures. Serum and cerebrospinal fluid (CSF) samples from 142 AD patients participating in the AMBAR trial (14-month schedule of PE-Alb treatment vs. placebo [sham PE-Alb]) were analyzed for changes from baseline for 19 inflammatory mediators (6 inflammatory cytokines, 9 chemokines, and 4 vascular injury indicators) at representative time points across the AMBAR study (lasting effects) as well as in pre- versus post-PE-Alb procedure (acute effects). Association between mediator changes and clinical outcomes reported in the AMBAR study (cognitive, functional, behavioral function, and global change tests) was assessed. PE-Alb significantly reduced IFN-γ, eotaxin, MIP-1α and ICAM-1 levels in serum, and eotaxin-3 and MIP-1β levels in CSF, at various time points during treatment (p < 0.05; false discovery rate-corrected). Vascular injury indicators were the mediators mostly affected by post- versus pre-PE-Alb level reduction. Increased serum MIP-1α levels were associated with worsening in ADAS-Cog, CDR-sb, and ADCS-CGIC scores in the placebo group, but not in the PE-Alb-treated group. Peripheral intervention could affect AD by reducing inflammatory mediators in both peripheral and central compartments. Changes in MIP-1α due to PE-Alb were associated with changes in clinical outcomes.

Clinical Characteristics and Treatment Courses of Trauma-Induced Thrombotic Microangiopathy: A Retrospective Study.

Introduction: Thrombotic microangiopathy (TMA), defined by thrombocytopenia, microangiopathic hemolytic anemia, and organ injury, is not widely recognized as being trauma-related. This study aimed to describe the clinical features and outcomes of trauma-induced TMA (t-TMA) to assist in early diagnosis and management. Methods: A retrospective review was conducted on 30 trauma patients diagnosed with t-TMA between 2014 and 2019. Demographic, clinical, and laboratory data, as well as treatment methods, were analyzed. Results: Thrombocytopenia (<50,000/µL) occurred, on average, 2.9 days post-trauma, with diagnosis following 3.6 days later. Patients had a mean age of 67.6 years, and 63.3% were male. Clinical presentations included acute kidney injury (AKI) requiring renal replacement therapy (86.7%), altered mental status (53.3%), non-infectious fever (50%), and digital necrosis (43.3%). Eighteen patients were treated with therapeutic plasma exchange (TPE) alone, nine with TPE and methylprednisolone, and three with methylprednisolone alone. Remission was achieved in 96.7% of all cases. The mean TPE duration was 6.1 days, prolonged by prior platelet transfusions. The mortality rate was 26.7% (8/30), with sepsis being the most common cause of death (five patients), particularly for those treated with TPE and methylprednisolone. Conclusions: Trauma-induced TMA should be suspected in trauma patients presenting with unexplained thrombocytopenia, AKI, and elevated LDH. Early diagnosis and prompt treatment are crucial, while unnecessary platelet transfusions should be avoided. Careful infection management is critical to improving patient outcomes, particularly if patients are treated with TPE and methylprednisolone.

Securing commitment and control for the supply of plasma derivatives for public health systems. I: A short review of the global landscape.

The social market economies of the Western world considered the provision of plasma derivatives produced from publicly owned blood services as a legitimate state commitment and, until the last decades of the 20th century, many of the relevant jurisdictions maintained state-supported fractionation plants to convert publicly collected plasma into products for the public health system. This situation started to change in the 1990s, because of several converging factors, and currently, publicly owned/subsidized, not-for-profit fractionation activity has shrunk to a handful of players. However, the collection of plasma from publicly owned blood services has continued and recent developments have increased the interest of state authorities globally to increase the volume of plasma collected to increase the level of strategic independence in the supply of crucial plasma-derived medicines from commercial market pressures, particularly the global for-profit fractionation sector with its dominance of source plasma from paid donors in the United States. This paper reviews the development of the plasma industry and the evolution of the pressures on the supply of plasma, which has led to a situation of scarcity of key plasma-derived medicinal products (PDMPs).

Anticathode effect on electron kinetics in electron beam generated E×B plasma

Abstract Electron beam (e-beam) generated plasmas with applied crossed electric and magnetic (E×B) fields are promising for low damage processing of materials with applications to microelectronics and quantum information systems. In cylindrical e-beam E×B plasmas, radial confinement of electrons and ions is achieved by an axial magnetic field and radial electric field, respectively. To control the axial confinement of electrons, such e-beam generated plasma sources may incorporate a conducting boundary known as an anticathode, which is placed on the axially opposite side of the plasma from the cathode. In this work, it is shown that varying the anticathode voltage bias can control the degree to which the anticathode collects or repels incident electrons, allowing control of warm electron (electron energies in 10-30 eV range) and beam electron population confinement. It is suggested that the effect of the anticathode bias on the formation of these distinct electron populations is also associated with the transition between weak turbulence and strong Langmuir turbulence.

Prospective screening and proposed treatment algorithm for immune checkpoint inhibitor induced myositis in neoadjuvant-treated rectal cancer patients: data from the phase II CHINOREC trial

Abstract Background Myositis is an infrequent (&amp;lt;1%) but potentially life-threatening (case fatality rate 40-50%) immune-related adverse event (irAEs) of immune checkpoint inhibitors (ICI), such as ipilimumab (IPI) and nivolumab (NIVO). The true incidence is likely to be underestimated and may not be representative for curative neoadjuvant treatment approaches in gastrointestinal (GI) cancers, especially in combination with chemoradiotherapy (CRT). Currently, the summary of product characteristics (SmPC) does not suggest any pre-emptive screening or surveillance. Methods The CHINOREC study is an ongoing prospective, randomized, open-label, multicenter, phase II investigator-initiated trial (IIT). Patients with intermediate to locally advanced rectal cancer (LARC) receive either neoadjuvant CRT alone or in combination with a single dose of IPI and 3 cycles of NIVO, following surgical resection. Patients are monitored at baseline and throughout the whole study period for myotoxicity biomarkers, including cardiac troponin T (cTnT) and cardiac troponin I (cTnI). Findings From 06/2020 to 11/2023, 80 patients have been enrolled of whom 50 patients were randomized to the CRT+IPI/NIVO arm. Six patients (12%) developed biopsy-verified myositis. Myositis treatment was promptly implemented using a pragmatic step-up approach. Patients received glucocorticoids (GC) with concomitant intravenous immunoglobulin (IVIG). If myotoxicity biomarkers did not improve, patients received infliximab (INFLIXI) and/or plasma exchange (PLEX). Although all patients had strikingly elevated cTnT (median peak 284 ng/L, 95% CI 39-3097), cTnI levels remained largely normal, correlating with a negative cardiac magnetic resonance (CMR). All patients underwent successful tumor surgery without any major surgical complication. As of today, all patients' creatine kinase (CK) and myoglobin (MB) levels have normalized and they are tumor free without any major sequela. Interpretation Longitudinal biomarker screening (cTnT/cTnI) for ICI-induced myotoxicities is pivotal in curative neoadjuvant-treated cancer patients to initiate an early counter treatment in a holistic step-up approach, without compromising oncological principles.

On the in-situ determination of the effective secondary electron emission coefficient in low pressure capacitively coupled radio frequency discharges based on the electrical asymmetry effect

Abstract We present a method for the in-situ determination of the effective secondary electron emission coefficient (SEEC, γ) in a capacitively coupled plasma (CCP) source based on the γ-dependence of the DC self-bias voltage that develops over the plasma due to the Electrical Asymmetry Effect (EAE). The EAE is established via the simultaneous application of two consecutive radio-frequency harmonics (with a varied phase angle) for the excitation of the discharge. Following the measurement of the DC self-bias voltage experimentally, Particle-in-Cell/Monte Carlo Collision (PIC/MCC) simulations coupled with a diffusion-reaction-radiation (DRR) code to compute the argon atomic excited level dynamics are conducted with a sequence of SEEC values. The actual γ for the given discharge operating conditions is found by searching for the best match between the experimental and computed values of the DC self-bias voltage. The γ ≈ 0.07 values obtained this way are in agreement with typical literature data for the working gas of argon and the electrode material of stainless steel in the CCP source. The method can be applied for a wider range of conditions, as well as for different electrode materials and gases to reveal the effective SEEC for various physical settings and discharge operating conditions.

Inactivation Efficiency of Bacillus atrophaeus Spores on Seeds of Barley, Wheat, Lupine and Rapeseed by Direct Cold Atmospheric Pressure Plasma

The objective of this study was to evaluate the antimicrobial effect of direct cold atmospheric plasma (CAPP) treatment for pre-harvest application using four different crop species: Hordeum vulgare L. (barley), Triticum aestivum L. (wheat), Brassica napus L. (rapeseed) and Lupinus angustifolius L. (lupine). The model bacterium Bacillus atrophaeus served as a proxy for spore-forming plant pathogens on the seed surface. After semi-dry inoculation of spores onto the seeds, treatment with two different plasma sources, a volume-dielectric barrier discharge and a corona discharge, and different exposure times was carried out. Subsequently, recovery of viable spores from the seeds’ surfaces was performed. Moreover, seed viability was determined based on maximum germination, as well as water contact angle as a measure for seed surface hydrophilicity. Direct CAPP treatment was efficient in reducing viable spores of B. atrophaeus with no significant differences between the plasma sources, reaching a mean inactivation of 1 log10 CFU/mL across all treatment times and crops species. Maximum germination of seeds was not negatively affected under any treatment condition. Seed hydrophilicity was increased for both plasma sources tested. Overall, this study provides valuable information on the efficiency of direct CAPP treatment of seeds with the purpose of seed hygienization with the premise of unaltered seed vitality and evaluates the potential application in comparison with previous investigated CAPP methods.

Fatal invasive Aspergillus infection in an elderly patient with hepatitis E: A case report and literature review.

Elderly patients with acute liver failure are highly susceptible to severe complications, such as invasive fungal infections, due to weakened immune systems and altered gut microbiota. A thorough understanding of liver failure and opportunistic infections is crucial for effective management. An 84-year-old male with acute liver failure from hepatitis E experienced worsening jaundice despite standard treatments. He also developed respiratory symptoms, including blood-streaked sputum, raising concerns about a potential fungal infection. The patient was diagnosed with acute liver failure secondary to hepatitis E and an invasive fungal infection caused by Aspergillus fumigatus. Initial treatments included artificial liver plasma exchange and antifungal prophylaxis. Further diagnostics, including bronchoscopy and next-generation sequencing of alveolar lavage fluid, confirmed the Aspergillus infection. Elderly liver failure patients are particularly prone to opportunistic infections, underscoring the need for vigilant monitoring and early intervention. Despite aggressive treatments, including antifungal therapy and artificial liver support, prognosis remains poor, highlighting the importance of prompt diagnosis and comprehensive management to enhance patient outcomes.

Coupling of Fluid and Particle-in-Cell Simulations of Ambipolar Plasma Thrusters

Ambipolar plasma thrusters are an appealing technology due to multiple system-related advantages, including propellant flexibility and the absence of electrodes or neutralizer. Understanding the plasma generation and acceleration mechanisms is key to improving the performance and capabilities of these thrusters. However, the source and plume regions inside are often simulated separately, and no self-consistent strategy exists which can couple these different simulations together. This paper introduces the MUlti-regime Plasma Equilibrium Transport Solver (MUPETS), a self-consistent coupled model integrating a fluid solver for the plasma dynamics in the source, which are collision-driven, with a kinetic Particle-In-Cell (PIC) code for the plasma dynamics in the magnetic nozzle, which involve expansion across a diverging magnetic field. The methodology begins by solving the plasma source with the classical Bohm condition at the thruster’s throat. The resulting plasma profiles (density, temperature, speed) are input into the PIC code for the magnetic nozzle. The PIC code calculates the plasma plume expansion and determines the electric field at the thruster’s throat. This electric field is then used as a boundary condition in the fluid code, where it replaces the Bohm assumption, and the fluid simulation is repeated. This iterative process continues until convergence. In comparing the MUPETS results with those for an experimental thruster, the plasma densities at the thruster’s throat differed by less than 2–5% between the fluid and PIC regions. The thrust predictions agreed with the experimental trend, and were kept well within the measurement’s uncertainty band. These results validate the effectiveness of the coupling strategy for enhancing plasma thruster simulation accuracy.

Use of Therapeutic Plasma Exchange and Intravenous Immunoglobulin to Prevent Complications in a K+ Sensitized Pregnancy

Use of Therapeutic Plasma Exchange and Intravenous Immunoglobulin to Prevent Complications in a K+ Sensitized Pregnancy

Comparative analysis of purity of human albumin preparations for clinical use

BackgroundAlbumin is the most prevalent plasma protein and serves numerous physiological roles, both in body fluid management and in various other capacities. In many diseases, a deficiency of albumin has been observed, and in certain conditions, albumin substitution has been demonstrated to improve outcome in comparison to plasma expansion using crystalloid or other colloid solutions. The favourable effects of using albumin in patients with liver cirrhosis are likely associated with the non-oncotic functions of albumin. Albumin for clinical use is obtained through fractionation of pooled donor plasma. The production procedures are optimized to ensure pure, chemically uncompromised and native protein. ResultsWe have extensively analysed commercial preparations of human albumin for clinical use from six different providers. Parameters that must correspond to the requirements of international pharmacopoeias were assessed (aluminium, ethanol, sodium, the presence of dimers and oligomers) and found to conform in all cases. In addition, we used for the first time nuclear magnetic resonance (NMR) as an additional analytical approach for investigating in greater depth the quality of a biological remedy gained from human plasma. We applied both 1H NMR and 13C-HSQC for confirming the identity of the albumin preparations, which also conformed in all cases. Moreover, we utilized T2-filtered 1H NMR and 13C-HSQC measurements to identify the presence of small molecules in the preparations. This demonstrated similar patterns of additional substances present, but also unveiled certain differences in purity in the products of the different providers. SignificanceOur analyses confirmed that albumin preparations in clinical use conform to the requirements. We furthermore demonstrate that NMR measurements can provide further depth in identity and purity measurements of biologicals. Despite largely standardized protocols in pharmaceutical albumin production, our in-depth analyses revealed differences in purity. Some samples exhibited lower levels of components other than albumin. We discuss possible causes of these observations and their potential implications for clinical therapy.

Design, development and test of single beamlet microwave ion source with spot-dense plasma system

In this paper, an efficient ion source with unique characteristics is introduced and proposed. It outlines the generation of a spot-dense plasma just below the plasma electrode aperture of the extractor system through the utilization of a 2.45 GHz microwave plasma source with a power of 1000 W, in the absence of a magnetic field. This source utilizes a 2.45 GHz microwave plasma generator operating at 1000 W, resulting in the generation of a spot-dense plasma just below the plasma electrode aperture of the extractor system, all achieved without the need for a magnetic field.The spot-dense plasma results in the extraction of an ion beam with high current density. Furthermore, the suggested ion source can be utilized in a smaller size using a higher frequency. Therefore, our findings indicate that the proposed method not only substantially enhances the extracted positive ion beam current but also significantly reduces the size of the ion beam source system. Based on the design and simulation results, an experimental model of the ion beam source is constructed. The diagnostic tools provided the beam current density and system efficiency values of 99mA/cm2 and 7×10−6A/W, respectively.

TGIRT-seq of Inflammatory Breast Cancer Tumor and Blood Samples Reveals Widespread Enhanced Transcription Impacting RNA Splicing and Intronic RNAs in Plasma.

Inflammatory breast cancer (IBC) is the most aggressive and lethal breast cancer subtype but lacks unequivocal genomic differences or robust biomarkers that differentiate it from non-IBC. Here, Thermostable Group II intron Reverse Transcriptase RNA-sequencing (TGIRT-seq) revealed myriad differences in tumor samples, Peripheral Blood Mononuclear Cells (PBMCs), and plasma that distinguished IBC from non-IBC patients and healthy donors across all tested receptor-based subtypes. These included numerous differentially expressed protein-coding gene and non-coding RNAs in all three sample types, a granulocytic immune response in IBC PBMCs, and over- expression of antisense RNAs, suggesting wide-spread enhanced transcription in both IBC tumors and PBMCs. By using TGIRT-seq to quantitate Intron-exon Depth Ratios (IDRs) and mapping reads to both genome and transcriptome reference sequences, we developed methods for parallel analysis of transcriptional and post-transcriptional gene regulation. This analysis identified numerous differentially and non-differentially expressed protein-coding genes in IBC tumors and PBMCs with high IDRs, the latter reflecting rate-limiting RNA splicing that negatively impacts mRNA production. Mirroring gene expression differences in tumors and PBMCs, over-represented protein-coding gene RNAs in IBC patient plasma were largely intronic RNAs, while those in non- IBC patients and healthy donor plasma were largely mRNA fragments. Potential IBC biomarkers in plasma included T-cell receptor pre-mRNAs and intronic, LINE-1, and antisense RNAs. Our findings provide new insights into IBC and set the stage for monitoring disease progression and response to treatment by liquid biopsy. The methods developed for parallel transcriptional and post- transcriptional gene regulation analysis have potentially broad RNA-seq and clinical applications.

Childhood onset C3 glomerulopathy: recurrence after kidney transplantation-a case series.

C3 Glomerulopathy (C3G) is a complement-mediated disease, with predominant C3 deposits, where pathogenic genetic variants in complement system components and circulating autoantibodies result in loss of control of the alternative pathway, have been described. A high incidence of disease recurrence including graft failure has been reported after kidney transplantation (KTx). Currently treatment modalities for preventing and treating post KTx C3G recurrence (plasma exchange, rituximab and eculizumab) in adults have yielded inconsistent results. Data on post KTx C3G recurrence in childhood-onset C3G is still unknown. A comprehensive case study of patients diagnosed with C3G as children or adolescents, who underwent KTx between the years 2015-2023. Data collected included complement workup, treatment modalities, and outcomes. 19 patients with C3G were identified during the study period. Five patients developed ESRD and received a kidney transplant. C3G recurrence was diagnosed post KTx in 100% of patients. Graft function improved in 3 of these patients (two with anti-factor H antibodies) after eculizumab treatment, one patient reached graft failure 9 months after transplantation despite eculizumab, recieved a second successful transplantation with pre-emptive eculizumab treatment and one patient showed histologic signs of disease recurrence without clinical signs. C3G recurrence after KTx in patients diagnosed as children or adolescents may be higher than previously described. Treatment with eculizumab is beneficial in some patients. New treatments are needed for improving post-transplant outcome in patients with C3G.