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5. Cold Ischemia Time and Graft Fibrosis Are Associated with Autoantibodies after Pediatric Liver Transplantation: A Retrospective Cohort Study of the European Reference Network TransplantChild

Author

Junge, N, Di Giorgio, A, Girard, M, Demir, Z, Kaminska, D, Janowska, M, Urbonas, V, Varnas, D, Maggiore, G, Alterio, T, Leiskau, C, Vondran, F, Richter, N, D'Antiga, L, Mikolajczyk, R, Pfister, E, Baumann, U, Junge N., Di Giorgio A., Girard M., Demir Z., Kaminska D., Janowska M., Urbonas V., Varnas D., Maggiore G., Alterio T., Leiskau C., Vondran F. W. R., Richter N., D'antiga L., Mikolajczyk R., Pfister E. -D., Baumann U., Junge, N, Di Giorgio, A, Girard, M, Demir, Z, Kaminska, D, Janowska, M, Urbonas, V, Varnas, D, Maggiore, G, Alterio, T, Leiskau, C, Vondran, F, Richter, N, D'Antiga, L, Mikolajczyk, R, Pfister, E, Baumann, U, Junge N., Di Giorgio A., Girard M., Demir Z., Kaminska D., Janowska M., Urbonas V., Varnas D., Maggiore G., Alterio T., Leiskau C., Vondran F. W. R., Richter N., D'antiga L., Mikolajczyk R., Pfister E. -D., and Baumann U.

Abstract

The reported prevalence of autoantibodies (AAB) (ANA, SMA, LKM, SLA) after pediatric liver transplantation (pLTX) varies considerably from 26–75%, but their clinical impact on outcome is uncertain. We aimed to study the prevalence of AAB after pLTX, their association with donor-, transplant-, and recipient-characteristics, and their relation to outcome. In our multicenter retrospective study, we aimed to clarify conflicting results from earlier studies. Six ERN TransplantChild centers reported data on 242 patients, of whom 61% were AAB positive. Prevalence varied across these centers. Independent of the interval between pLTX and AAB analysis, a one-hour increase in CIT resulted in an odds ratio (OR) of 1.37 (95% CI 1.11–1.69) for SMA positivity and an OR of 1.42 (95% CI 1.18–1.72) for ANA positivity. Steroid-free immunosuppression (IS) versus steroid-including IS (OR 5.28; 95% CI 1.45–19.28) was a risk factor for SMA positivity. Liver enzymes were not associated with ANA or SMA positivity. We did not observe an association of rejection activity index with ANA or SMA. However, the liver fibrosis score in follow-up biopsies was associated with ANA titer and donor age. In conclusion, this first multicenter study on AAB after pLTX showed high AAB prevalence and varied widely between centers. Longer CIT and prednisolone-free-IS were associated with AAB positivity, whereas AAB were not indicative of rejection, but instead were associated with graft fibrosis. The detection of AAB may be a marker of liver fibrosis and may be taken into consideration when indications for liver biopsy and immunosuppressive regimes, or reduction of im-munosuppression in long-term follow-up, are being discussed. Prospective immunological profiling of pLTX patients, including AAB, is important to further improve our understanding of transplant immunology and silent graft fibrosis.

Published
2022

7. Immune monitoring after pediatric liver transplantation - the prospective ChilSFree cohort study

Author

Goldschmidt, I, Karch, A, Mikolajczyk, R, Mutschler, F, Junge, N, Pfister, E, Mohring, T, D'Antiga, L, Mckiernan, P, Kelly, D, Debray, D, Mclin, V, Pawlowska, J, Hierro, L, Daemen, K, Keil, J, Falk, C, Baumann, U, Goldschmidt I., Karch A., Mikolajczyk R., Mutschler F., Junge N., Pfister E. D., Mohring T., d'Antiga L., McKiernan P., Kelly D., Debray D., McLin V., Pawlowska J., Hierro L., Daemen K., Keil J., Falk C., Baumann U., Goldschmidt, I, Karch, A, Mikolajczyk, R, Mutschler, F, Junge, N, Pfister, E, Mohring, T, D'Antiga, L, Mckiernan, P, Kelly, D, Debray, D, Mclin, V, Pawlowska, J, Hierro, L, Daemen, K, Keil, J, Falk, C, Baumann, U, Goldschmidt I., Karch A., Mikolajczyk R., Mutschler F., Junge N., Pfister E. D., Mohring T., d'Antiga L., McKiernan P., Kelly D., Debray D., McLin V., Pawlowska J., Hierro L., Daemen K., Keil J., Falk C., and Baumann U.

Abstract

Background: Although trough levels of immunosuppressive drugs are largely used to monitor immunosuppressive therapy after solid organ transplantation, there is still no established tool that allows for a validated assessment of functional degree of immunosuppression or the identification of clinically relevant over- or under-immunosuppression, depending on graft homeostasis. Reliable non-invasive markers to predict biopsy proven acute rejection (BPAR) do not exist. Literature data suggest that longitudinal measurements of immune markers might be predictive of BPAR, but data in children are scarce. We therefore propose an observational prospective cohort study focusing on immune monitoring in children after liver transplantation. We aim to describe immune function in a cohort of children before and during the first year after liver transplantation and plan to investigate how the immune function profile is associated with clinical and laboratory findings. Methods: In an international multicenter prospective approach, children with end-stage liver disease who undergo liver transplantation are enrolled to the study and receive extensive immune monitoring before and at 1, 2, 3, 4 weeks and 3, 6, 12 months after transplantation, and whenever a clinically indicated liver biopsy is scheduled. Blood samples are analyzed for immune cell numbers and circulating levels of cytokines, chemokines and factors of angiogenesis reflecting immune cell activation. Statistical analysis will focus on the identification of trajectorial patterns of immune reactivity predictive for systemic non-inflammatory states, infectious complications or BPAR using joint modelling approaches. Discussion: The ChilSFree study will help to understand the immune response after pLTx in different states of infection or rejection. It may provide insight into response mechanisms eventually facilitating immune tolerance towards the graft. Our analysis may yield an applicable immune panel for non-invasive early d

Published
2018

14. Autorenverzeichnis

Author

Graul-Neumann, L., Horn, D., Hübner, C., Huppke, P., König, R., Majewski, F., Meinecke, P., Pankau, R., Rosenbaum, T., Schnabel, D., Schuelke, M., Spranger, J., Theile, U., Tinschert, S., Wilichowski, E., Wollmann, H.A., Zenker, M., Bartmann, P., Bassler, D., Bührer, C., Flemmer, A.W., Forster, J., Franz, A., Gonser, M., Gortner, L., Groneck, P., Hentschel, R., Herting, E., Hoyme, U.B., Hummler, H., Jandeck, C., Jorch, G., Korinthenberg, R., Liese, J., Maier, R.F., Martius, J., Merkenschlager, A., Poets, C.F., Pohlandt, F., Roll, C., Roos, R., Roth, B., Schneider, K.T.M., Speer, Ch., Stopfkuchen, H., Teichmann, A., Thomas, W., Vetter, K., von der Wense, A., Zielen, S., Assmann, B., Hoffmann, G.F., Kölker, S., Lindner, M., Mönch, E., Santer, R., Spiekerkötter, U., Zschocke, J., Bauer, K., Böhles, H.-J., Sinclair, Jack, Jauch, K.W., Jochum, F., Kauth, Thomas, Koletzko, B., Krawinkel, M., Krohn, K., Mihatsch, Walter, Moß, A., Mühlebach, S., Verwied-Jorky, S., Wabitsch, M., Zimmer, K.-P., Albers, N., L'Allemand, D., Binder, G., Brämswig, J.H., Dörr, H.G., Grüters-Kieslich, A., Hauffa, B.P., Heger, S., Hiort, O., Holl, R., Holterhus, P.M., Köhler, B., Korsch, Eckhard, Kratzsch, J., Krude, H., Mohnike, K., Neu, A., Pfäffle, R., Richter-Unruh, A., Riepe, F.G., Simic-Schleicher, G., Schönau, E., Sinnecker, G., Sippell, W., Willgerodt, H., Wölfle, J., Wudy, S.A., Aygören-Pürsün, E., Bas, M., Baumann, U., Biedermann, T., Blume, J., Buchholz, B., Dückers, G., Dunsch, D., Edelhäuser, M., Ehl, S., Feiterna-Sperling, C., Funk, M., Hartmann, K., Königs, C., Kreuz, W., Krudewig, J., Laws, H.-J., Linde, R., Martinez-Saguer, I., Maurer, M., Nadal, David, Niehues, T., Notheis, G., Ott, H., Schulze, I., Wedi, B., Wintergerst, U., Bürk, G., Foeldvari, I., Frosch, M., Girschick, H., Gerhold, K., Guellac, N., Haas, J.P., Häfner, R., Häuser, W., Heiligenhaus, A., Hospach, T., Horneff, G., Huppertz, H.-I., Illhardt, A., Jansson, A.F., Kallinich, T., Michels, H., Mönkemöller, K., Neudorf, U., Richter, M., Schnöbel-Müller, E., Thon, A., Zernikow, B., Behnisch, W., Cario, H., Dickerhoff, R., Eber, S., Führer, M., Kohne, E., Kulozik, A.E., Kunz, J., Muckenthaler, M., Eberl, W., Gaedicke, G., Muntean, W., Streif, W., Beck, J.D., Berthold, F., Bielack, S., Calaminus, G., Claviez, A., Creutzig, U., Dirksen, U., Dworzak, M., Göbel, U., Graf, N., Grießmeier, B., Henze, G., Hero, B., Jürgens, H., Kaiser, U., Klingebiel, T., Koscielniak, E., Kramm, C., Langer, T., Lawrenz, B., Lehrnbecher, T., Leiss, U., Mentzel, H.-J., Minkov, M., Peitz, J., Placzek, R., Reinhardt, D., Reiter, A., Rutkowski, S., Schmittenbecher, P., Schneider, D.T., Schreiber-Gollwitzer, B.M., Schrappe, M., Schroten, H., Schröder, H.M., Schuster, V., von Schweinitz, D., Sörensen, N., Tallen, G., Timmermann, B., Warmuth-Metz, M., Weckesser, M., Wessel, L., Wirth, T., Wolff, J.E.A., Wößmann, W., Zehnhoff-Dinnesen, A. am, Apitz, C., Arnold, R., Baumgartner, H., Bennink, G., Bertram, H., Blankenburg, M., Bönner, G., von der Breek, J., Breuer, J., Buchhorn, R., Bürsch, J., Cesnjevar, R., Dähnert, I., Deisenhofer, I., Diller, G.-P., Doenst, T., Dubowy, K.-O., Eicken, A., Ewert, P., Fink, C., Franke, J., Gebauer, R., Gorenflo, M., Grabitz, Haas, N.A., Häusler, H.-J., Hager, A., Hebebrand, J., Henschel, W., Hirt, M., Hoeper, M.M., Hörer, J., Hofbeck, M., Horke, A., Hraska, V., Hulpke-Wette, M., šek, J. Janou, Jux, C., Kändler, L., Kandolf, R., Kaulitz, R., Kienast, W., Klaassen, S., Knirsch, W., Kramer, H.H., Kreuder, J.G., Kriebel, T., Läer, S., Laser, K.T., Lê, T.-P., Lewin, M.A.G., Lindinger, A., Mackenzie, C.R., Mebus, S., van der Mei, S.H., Miera, O., Ovroutski, S., Paul, T., Photiadis, J., Pozza, R. Dalla, Rickers, C., Rosendahl, W., Ruschewski, W., Sachweh, J.S., Schäfers, H.-J., Scheewe, J., Schirmer, K.-R., Schlensak, C., Schlez, M., Schmaltz, A.A., Schmitt, K., Schneider, H., Schneider, M.B., Schranz, D., Schreiber, C., Schulze-Neick, I., Sieverding, L.F.J., Singer, H., Stieh, J., Sreeram, N., Thies, W.-R., Thul, J., Trauzeddel, R., Tschöpe, C., Uebing, A., Ulmer, H.E., Vogel, M., Vogt, M., Weil, J., Wessel, A., Will, J.C., Wühl, E., Ballmann, M., Barben, J., Bauer, C.P., Bend, J., Berdel, D., Blankenstein, O., Bremer, W., Brunsmann, F., Buchholz, T., Bufe, A., Derichs, N., Eber, E., Friedrichs, F., Frischer, T., Gembruch, U., Gieler, U., Götz, M., Haas, W.H., Hamelmann, E., Hammer, J., Hellermann, M., Jacobeit, J., Jung, A., Keim, V., Kitz, R., Kleinheinz, A., Koletzko, S., Kopp, I., Kopp, M., Lau, S., Lauener, R., Loff, Magdorf, K., Muche-Borowski, C., Müller, F.-M., Müsken, H., Naehrlich, L., Nicolai, T., Nüßlein, Th., Paditz, E., Palm, Frau B., Paul, K., Pfeiffer-Auler, S., Pfeiffer-Kascha, Frau D., Posselt, H.-G., Przybilla, B., Räwer, H.-C., Ratjen, F., Reese, I., Riedler, J., Rietschel, E., Rose, M., Rossi, R., Ruëff, F., Schäfer, T., Schmidt, S., Schmitt-Grohé, S., Schulze, J., Schuster, A., Seidenberg, J., Sitter, H., Smaczny, C., Spindler, T., Staab, D., Stern, M., Strassburg, C.P., Strömer, K., Stuhrmann-Spangenberg, M., Szczepanski, R., Tacke, A., Tiedgen, M., Urschitz, M.S., Vagts, J., Vogelberg, C., Wahn, U., Walker, A., Werfel, T., Wildhaber, J.H., Zach, M., Zimmermann, Th., Ballauff, A., Bannert, N., Böhn, I., Buderus, S., Bufler, P., Burdelski, M., Gerner, P., Grosse, K.-P., Henker, J., Henneke, P., Huber, W., Lang, T., Lentze, M.J., Melter, M., Müller, T., Pfister, E.-D., Rodeck, B., Schmidt-Choudhury, A., Skopnik, H., Wirth, S., Witt, H., Bachmann, H., Dötsch, J., Ehrich, J.H., Fuchshuber, Arno, Hoppe, B., Hoyer, P.F., Kemper, M.J., Michalk, D., Müller, D., Müller-Wiefel, D.E., Pohl, M., Tönshoff, B., Zerres, K., Bast, T., Baumeister, F.A.M., Berner, R., Bode, H., Christen, H.J., Collmann, H., Ebinger, F., Eiffert, H., Evers, S., Gold, R., Groß, S., Hanefeld, F., Heinen, F., Holthausen, H., Hübner, A., Jacobi, G., Karch, D., Kauschke, C., Kerkhoff, G., Kiese-Himmel, C., Klepper, J., Kohlschütter, A., Korn-Merker, E., Krägeloh-Mann, I., Kropp, P., Kurlemann, G., de Langen-Müller, U., Lenard, H.G., Michael, Th., von Moers, A., Felderhoff-Müser, U., Nau, R., Neubauer, B.A., Neuhäuser, G., Neumann, K., Noterdaeme, M., Pothmann, R., Rating, D., Reitter, B., Rickels, E., Ritz, A.M., Rosenkötter, H., Schmitt, B., Stephani, U., Stöver, B., Tibussek, D., Trollmann, R., Trommer, G., Tuxhorn, I., Wohlrab, G., Boergen, K.P., Brosch, S., Delb, W., Frank, R., Herrmann, B., von Hofacker, N., de Camargo, O. Kraus, Kries, R.v., Michaelis, R., Papousek, M., Schlack, H.G., Schriever, J., Skrodzki, K., Straßburg, H.-M., Thyen, U., Becker, K., Fels, T., Fitze, G., Grasshoff-Derr, S., Göbel, P., Illing, P., Lieber, J., Schmidt, A., Wessel, L.M., Berthold, L.D., Hahn, G., Hirsch, W., Moritz, J.D., Schröder, C., Schumacher, R., Stegmann, J., Steinborn, M., Tietze, R., Wunsch, R., Deppe, W., Hermann, T., Kiosz, D., Leidig, E., Mayer, H., Oepen, J., Stachow, R., Ahrens, F., Frey, G., Huttegger, I., Preil, M.-L., Schmittenbecher, P.P., Traupe, H., Eberhardt, O., Hasler, C., Krauspe, R., Meenen, N.M., Meurer, A., Rödl, R., Stücker, R., and Zilkens, C.

Published
2015
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16. European Biliary Atresia Registries: Summary of a Symposium

Author

Petersen, C., primary, Harder, D., additional, Abola, Z., additional, Alberti, D., additional, Becker, T., additional, Chardot, C., additional, Davenport, M., additional, Deutschmann, A., additional, Khelif, K., additional, Kobayashi, H., additional, Kvist, N., additional, Leonhardt, J., additional, Melter, M., additional, Pakarinen, M., additional, Pawlowska, J., additional, Petersons, A., additional, Pfister, E.-D., additional, Rygl, M., additional, Schreiber, R., additional, Sokol, R., additional, Ure, B., additional, Veiga, C., additional, Verkade, H., additional, Wildhaber, B., additional, Yerushalmi, B., additional, and Kelly, D., additional

Published
2008
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20. Biliary Atresia: Lessons Learned from the Voluntary German Registry.

Author

Leonhardt, J., Kuebler, J. F., Leute, P. J., Turowski, C., Becker, T., Pfister, E.-D., Ure, B., and Petersen, C.

Subjects
BILIARY atresia, LIVER transplantation, SURGERY, MEDICAL care, MANAGEMENT
Abstract

Introduction: Aim of the study was to carry out a 5-year survey of German patients with biliary atresia (BA) and to launch a discussion regarding the feasibility of voluntary registries in unregulated healthcare systems. Methods: A retrospective analysis of German BA patients born between 2001 and 2005, based on data collected from the voluntary European Biliary Atresia Registry (EBAR), was carried out and supplemented by data from all BA patients who underwent liver transplantation at the only 4 pediatric transplantation centers (pLTx) in Germany which are so far not registered at EBAR. Survival rates were calculated using Kaplan- Meier analysis and compared by Cox regression to determine the predictive value of age at surgery and the influence of the center size (fewer or more than 5 patients / study period) on overall survival and survival with native liver. Results: A critical review of the 148 German EBAR charts revealed that 11 patients (7.4 % ) had no biliary atresia. The remaining 137 patients from EBAR together with 46 BA patients who underwent LTx without prior registration at EBAR were evaluated with a median follow-up of 39 months (range: 25 - 85 months). 29 hospitals performed a total of 159 Kasai procedures, but only 7 centers treated 5 or more patients (116 patients, range: 5 - 68), and 22 hospitals performed less than 5 KP (43 patients, range: 1 - 4). Primary LTx was performed in 21 patients (11.5 % ) and 3 patients died without surgical intervention. 16 patients were lost to follow-up (8.7 % ). Overall survival after 2 years was 83.3 % (139 patients), including 105 patients (63 % ) who had undergone LTx and 34 patients (20.3 % ) with native liver. 28 patients died (16.7 % ), 8 after LTx (5.8 % ). The experience of the center was the only factor with a significant predictive value for jaundice- free survival with native liver (p = 0.001). Conclusion: 25 % of all German BA patients were not registered at EBAR, and 29 clinics were involved in the surgical management of BA patients. Therefore a new approach consisting of an internet-based decentralized registry for rare neonatal liver diseases is outlined which could improve the future management of patients with BA. The centralization of such patients at experienced centers with higher caseloads is necessary in Germany and would improve the outcome of patients with biliary atresia. [ABSTRACT FROM AUTHOR]

Published
2011
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21. [Wilson's disease : What has been confirmed in diagnostic and therapy?]

Author

Pfister ED

Subjects
Chelating Agents therapeutic use, Chromosome Aberrations, Copper administration & dosage, Copper blood, Diet Therapy, Genes, Recessive genetics, Hepatolenticular Degeneration blood, Hepatolenticular Degeneration genetics, Humans, Liver Transplantation, Molybdenum therapeutic use, Penicillamine therapeutic use, Phenotype, Zinc therapeutic use, Hepatolenticular Degeneration diagnosis, Hepatolenticular Degeneration therapy
Abstract

Wilson's disease (WD) is a rare autosomal recessive disorder characterized by abnormal copper accumulation. Presenting a broad variety of phenotypes and, thus, being a chameleon within the group of metabolic diseases, the manifold clinical symptoms of WD can include hepatologic, neurologic, and psychiatric manifestations. Early onset presentations in infancy and late-onset manifestations in adults older than 70 years of age have been described. If the typical laboratory blood test values are missing, the diagnosis of WD may be difficult and often involves a combination of different parameters. Novel test methods like the identification of the relative exchangeable copper have not been validated within a sufficient cohort of WD patients as of yet and therefore do not currently play a crucial role within the clinical setting. Consequently any patient with reasonable suspected diagnosis of WD needs to be presented to a (pediatric) gastroenterologist and/or (pediatric) neurologist. Different medical treatments including drugs such as copper chelating agents are commonly used in the clinical setting. Liver transplantation may be the ultima ratio in selected patients. Dietary changes involving a low copper diet play only a minor role. Due to the fact the use of tetrathiomolybdate is still not approved, the treatment of advanced and progressive neurologic symptoms remains a major challenge. In any case, life-long medical supervision and treatment governed by a specialist is absolutely essential. Early diagnosis and early and life-long treatment lead to better prognoses and do not negatively influence the overall life expectancy.

Published
2017
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22. "Current state and prospects in managing liver transplanted children".

Author

Pfister ED, McLin VA, Hierro L, Tizzard SA, and Baumann U

Subjects
Child, Humans, Immunosuppression Therapy, Treatment Outcome, Liver Transplantation
Abstract

Pediatric liver transplantation (LTx) has revolutionized life chances and perspectives of children with liver disease. Following rapid establishment of the therapeutic concept in the early years of pediatric transplant medicine, more aspects beyond plain survival become increasingly important. In addition to improving the short to medium-term survival rates, researchers are focusing on themes such as rehabilitation, adherence and quality of life, long-term graft fibrosis and dysfunction, as well as the consequences of long-term immunosuppression. Also, more protocol biopsy data are available to evaluate increasing graft fibrosis. To manage their conditions, patients will need access to highly experienced pediatric liver transplant centers where clinical research will examine modulators of renal disease, endocrine and cardiovascular comorbidity and the development of graft fibrosis and malignancies. Assessment and evaluation of health-related quality of life and factors which influence clinical tolerance, adherence and transition from child to adult care will also be investigated. The analysis of multi-national registry data and more than 40years of experience with large patient cohorts will provide important clues to treatment and will thus get increasing attention. In the future, longitudinal assessment of the outcome for pediatric LTx patients should include more functional aspects than plain survival rates or laboratory parameters., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Published
2015
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23. [Predictive parameters in children with biliary atresia].

Author

Leonhardt J, Kuebler JF, Turowski C, von Wasielewski R, Pfister ED, Becker T, Ure BM, and Petersen C

Subjects
Adolescent, Biliary Atresia mortality, Biliary Atresia pathology, Biomarkers blood, Child, Child, Preschool, Disease Progression, Female, Follow-Up Studies, Humans, Infant, Infant, Newborn, Liver pathology, Liver Cirrhosis, Biliary diagnosis, Liver Cirrhosis, Biliary mortality, Liver Cirrhosis, Biliary pathology, Liver Cirrhosis, Biliary surgery, Liver Function Tests, Male, Portoenterostomy, Hepatic, Predictive Value of Tests, Prognosis, Retrospective Studies, Survival Analysis, Young Adult, gamma-Glutamyltransferase blood, Alanine Transaminase blood, Biliary Atresia diagnosis, Biliary Atresia surgery, Bilirubin blood
Abstract

Most children with biliary atresia require liver transplantation, and only about 20% survive in the long term with their native livers. Prognostic factors that determine disease progression are still lacking. This retrospective survey of 85 BA patients from 1993 to 2003 was aimed to evaluate prognostic factors using the log rank test. After 5 years 40% of the patients are alive with their native livers (35/85), 26 of them with normal bilirubin (31%). Age at Kasai operation (P=0.46), degree of liver fibrosis (P=0.95), and all laboratory test results before Kasai failed to correlate with outcome. Normal levels of bilirubin 3, 6, and 12 months after Kasai and of aspartate aminotransferase with gammaGT after 6 months are associated with survival with native liver. In conclusion our data demonstrate that a lack of predictive factors must prevent primary liver transplantation in BA patients.

Published
2009
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