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1. Systematic review of response criteria and endpoints in autoimmune hepatitis by the International Autoimmune Hepatitis Group

Author

Pape, S, Snijders, R, Gevers, T, Chazouilleres, O, Dalekos, G, Hirschfield, G, Lenzi, M, Trauner, M, Manns, M, Vierling, J, Montano-Loza, A, Lohse, A, Schramm, C, Drenth, J, Heneghan, M, Almasio, P, Alvarez, F, Andrade, R, Arikan, C, Assis, D, Bardou-Jacquet, E, Biewenga, M, Cancado, E, Cazzagon, N, Colloredo, G, Cuarterolo, M, Debray, D, Robles-Diaz, M, Dyson, J, Efe, C, Engel, B, Ferri, S, Fontana, R, Gatselis, N, Gerussi, A, Halilbasic, E, Halliday, N, van Hoek, B, Horby Jorgensen, M, Indolfini, G, Iorio, R, Jeong, S, Jones, D, Kelly, D, Kerkar, N, Lacaille, F, Lammert, C, Leggett, B, Levy, C, Liberal, R, Lleo, A, Ines Lopez, S, de Martin, E, Mclin, V, Mieli-Vergani, G, Milkiewicz, P, Mohan, N, Muratori, L, Nebbia, G, van Nieuwkerk, C, Oo, Y, Ortega, A, Pares, A, Pop, T, Pratt, D, Purnak, T, Ranucci, G, Rushbrook, S, Stattermayer, A, Swain, M, Tanaka, A, Taubert, R, Terrabuio, D, Terziroli, B, Valentino, P, van den Brand, F, Villamil, A, Wahlin, S, Ytting, H, Zachou, K, Zeniya, M, Pape S., Snijders R. J. A. L. M., Gevers T. J. G., Chazouilleres O., Dalekos G. N., Hirschfield G. M., Lenzi M., Trauner M., Manns M. P., Vierling J. M., Montano-Loza A. J., Lohse A. W., Schramm C., Drenth J. P. H., Heneghan M. A., Almasio P., Alvarez F., Andrade R., Arikan C., Assis D., Bardou-Jacquet E., Biewenga M., Cancado E., Cazzagon N., Colloredo G., Cuarterolo M., Dalekos G., Debray D., Robles-Diaz M., Drenth J., Dyson J., Efe C., Engel B., Ferri S., Fontana R., Gatselis N., Gerussi A., Halilbasic E., Halliday N., Heneghan M., Hirschfield G., van Hoek B., Horby Jorgensen M., Indolfini G., Iorio R., Jeong S., Jones D., Kelly D., Kerkar N., Lacaille F., Lammert C., Leggett B., Levy C., Liberal R., Lleo A., Lohse A., Ines Lopez S., de Martin E., McLin V., Mieli-Vergani G., Milkiewicz P., Mohan N., Muratori L., Nebbia G., van Nieuwkerk C., Oo Y., Ortega A., Pares A., Pop T., Pratt D., Purnak T., Ranucci G., Rushbrook S., Stattermayer A., Swain M., Tanaka A., Taubert R., Terrabuio D., Terziroli B., Valentino P., van den Brand F., Villamil A., Wahlin S., Ytting H., Zachou K., Zeniya M., Pape, S, Snijders, R, Gevers, T, Chazouilleres, O, Dalekos, G, Hirschfield, G, Lenzi, M, Trauner, M, Manns, M, Vierling, J, Montano-Loza, A, Lohse, A, Schramm, C, Drenth, J, Heneghan, M, Almasio, P, Alvarez, F, Andrade, R, Arikan, C, Assis, D, Bardou-Jacquet, E, Biewenga, M, Cancado, E, Cazzagon, N, Colloredo, G, Cuarterolo, M, Debray, D, Robles-Diaz, M, Dyson, J, Efe, C, Engel, B, Ferri, S, Fontana, R, Gatselis, N, Gerussi, A, Halilbasic, E, Halliday, N, van Hoek, B, Horby Jorgensen, M, Indolfini, G, Iorio, R, Jeong, S, Jones, D, Kelly, D, Kerkar, N, Lacaille, F, Lammert, C, Leggett, B, Levy, C, Liberal, R, Lleo, A, Ines Lopez, S, de Martin, E, Mclin, V, Mieli-Vergani, G, Milkiewicz, P, Mohan, N, Muratori, L, Nebbia, G, van Nieuwkerk, C, Oo, Y, Ortega, A, Pares, A, Pop, T, Pratt, D, Purnak, T, Ranucci, G, Rushbrook, S, Stattermayer, A, Swain, M, Tanaka, A, Taubert, R, Terrabuio, D, Terziroli, B, Valentino, P, van den Brand, F, Villamil, A, Wahlin, S, Ytting, H, Zachou, K, Zeniya, M, Pape S., Snijders R. J. A. L. M., Gevers T. J. G., Chazouilleres O., Dalekos G. N., Hirschfield G. M., Lenzi M., Trauner M., Manns M. P., Vierling J. M., Montano-Loza A. J., Lohse A. W., Schramm C., Drenth J. P. H., Heneghan M. A., Almasio P., Alvarez F., Andrade R., Arikan C., Assis D., Bardou-Jacquet E., Biewenga M., Cancado E., Cazzagon N., Colloredo G., Cuarterolo M., Dalekos G., Debray D., Robles-Diaz M., Drenth J., Dyson J., Efe C., Engel B., Ferri S., Fontana R., Gatselis N., Gerussi A., Halilbasic E., Halliday N., Heneghan M., Hirschfield G., van Hoek B., Horby Jorgensen M., Indolfini G., Iorio R., Jeong S., Jones D., Kelly D., Kerkar N., Lacaille F., Lammert C., Leggett B., Levy C., Liberal R., Lleo A., Lohse A., Ines Lopez S., de Martin E., McLin V., Mieli-Vergani G., Milkiewicz P., Mohan N., Muratori L., Nebbia G., van Nieuwkerk C., Oo Y., Ortega A., Pares A., Pop T., Pratt D., Purnak T., Ranucci G., Rushbrook S., Stattermayer A., Swain M., Tanaka A., Taubert R., Terrabuio D., Terziroli B., Valentino P., van den Brand F., Villamil A., Wahlin S., Ytting H., Zachou K., and Zeniya M.

Abstract

Background & Aims: Autoimmune hepatitis (AIH) has been well characterised and codified through the development of diagnostic criteria. These criteria have been adapted and simplified and are widely used in clinical practice. However, there is a need to update and precisely define the criteria for both treatment response and treatment. Methods: A systematic review was performed and a modified Delphi consensus process was used to identify and redefine the response criteria in autoimmune hepatitis. Results: The consensus process initiated by the International Autoimmune Hepatitis Group proposes that the term ‘complete biochemical response’ defined as ‘normalization of serum transaminases and IgG below the upper limit of normal’ be adopted to include a time point at 6 months after initiation of treatment. An insufficient response by 6 months was a failure to meet the above definition. Non-response was defined as ‘<50% decrease of serum transaminases within 4 weeks after initiation of treatment’. Remission is defined as liver histology with a Hepatitis Activity Index <4/18. Intolerance to treatment was agreed to stand for ‘any adverse event possibly related to treatment leading to potential drug discontinuation’. Conclusions: These definitions provide a simple and reproducible framework to define treatment response and non-response, irrespective of the therapeutic intervention. A consensus on endpoints is urgently required to set a global standard for the reporting of study results and to enable inter-study comparisons. Future prospective database studies are needed to validate these endpoints. Lay summary: Consensus among international experts on response criteria and endpoints in autoimmune hepatitis is lacking. A consensus on endpoints is urgently required to set a global standard for the reporting of study results and to enable the comparison of results between clinical trials. Therefore, the International Autoimmune Hepatitis Group (IAIHG) herein presents

Published
2022

2. The challenges of primary biliary cholangitis: What is new and what needs to be done

Author

Terziroli Beretta-Piccoli, B, Mieli-Vergani, G, Vergani, D, Vierling, J, Adams, D, Alpini, G, Banales, J, Beuers, U, Bjornsson, E, Bowlus, C, Carbone, M, Chazouilleres, O, Dalekos, G, De Gottardi, A, Harada, K, Hirschfield, G, Invernizzi, P, Jones, D, Krawitt, E, Lanzavecchia, A, Lian, Z, Ma, X, Manns, M, Mavilio, D, Quigley, E, Sallusto, F, Shimoda, S, Strazzabosco, M, Swain, M, Tanaka, A, Trauner, M, Tsuneyama, K, Zigmond, E, Gershwin, M, Terziroli Beretta-Piccoli B., Mieli-Vergani G., Vergani D., Vierling J. M., Adams D., Alpini G., Banales J. M., Beuers U., Bjornsson E., Bowlus C., Carbone M., Chazouilleres O., Dalekos G., De Gottardi A., Harada K., Hirschfield G., Invernizzi P., Jones D., Krawitt E., Lanzavecchia A., Lian Z. -X., Ma X., Manns M., Mavilio D., Quigley E. M., Sallusto F., Shimoda S., Strazzabosco M., Swain M., Tanaka A., Trauner M., Tsuneyama K., Zigmond E., Gershwin M. E., Terziroli Beretta-Piccoli, B, Mieli-Vergani, G, Vergani, D, Vierling, J, Adams, D, Alpini, G, Banales, J, Beuers, U, Bjornsson, E, Bowlus, C, Carbone, M, Chazouilleres, O, Dalekos, G, De Gottardi, A, Harada, K, Hirschfield, G, Invernizzi, P, Jones, D, Krawitt, E, Lanzavecchia, A, Lian, Z, Ma, X, Manns, M, Mavilio, D, Quigley, E, Sallusto, F, Shimoda, S, Strazzabosco, M, Swain, M, Tanaka, A, Trauner, M, Tsuneyama, K, Zigmond, E, Gershwin, M, Terziroli Beretta-Piccoli B., Mieli-Vergani G., Vergani D., Vierling J. M., Adams D., Alpini G., Banales J. M., Beuers U., Bjornsson E., Bowlus C., Carbone M., Chazouilleres O., Dalekos G., De Gottardi A., Harada K., Hirschfield G., Invernizzi P., Jones D., Krawitt E., Lanzavecchia A., Lian Z. -X., Ma X., Manns M., Mavilio D., Quigley E. M., Sallusto F., Shimoda S., Strazzabosco M., Swain M., Tanaka A., Trauner M., Tsuneyama K., Zigmond E., and Gershwin M. E.

Abstract

Primary Biliary Cholangitis (PBC) is an uncommon, chronic, cholangiopathy of autoimmune origin and unknown etiology characterized by positive anti-mitochondrial autoantibodies (AMA), female preponderance and progression to cirrhosis if left untreated. The diagnosis is based on AMA- or PBC-specific anti-nuclear antibody (ANA)-positivity in the presence of a cholestatic biochemical profile, histologic confirmation being mandatory only in seronegative cases. First-line treatment is ursodeoxycholic acid (UDCA), which is effective in preventing disease progression in about two thirds of the patients. The only approved second-line treatment is obeticholic acid. This article summarizes the most relevant conclusions of a meeting held in Lugano, Switzerland, from September 23rd-25th 2018, gathering basic and clinical scientists with various background from around the world to discuss the latest advances in PBC research. The meeting was dedicated to Ian Mackay, pioneer in the field of autoimmune liver diseases. The role of liver histology needs to be reconsidered: liver pathology consistent with PBC in AMA-positive individuals without biochemical cholestasis is increasingly reported, raising the question as to whether biochemical cholestasis is a reliable disease marker for both clinical practice and trials. The urgent need for new biomarkers, including more accurate markers of cholestasis, was also widely discussed during the meeting. Moreover, new insights in interactions of bile acids with biliary epithelia in PBC provide solid evidence of a role for impaired epithelial protection against potentially toxic hydrophobic bile acids, raising the fundamental question as to whether this bile acid-induced epithelial damage is the cause or the consequence of the autoimmune attack to the biliary epithelium. Strategies are needed to identify difficult-to-treat patients at an early disease stage, when new therapeutic approaches targeting immunologic pathways, in addition to bile acid

Published
2019

3. Systemic and Local Cytokine Profile in Biliary Atresia

Author

Saito, T., Sakamoto, A., Hatano, M., Iwai, J., Higashimoto, Y., Yoshida, H., Riepenhoff-Talty, M., Gouvea, V., Evans, M.J., Tyler, K.L., Sokol, R.J., Oberhaus, S.M., Desmet, V.J., Suskind, D.L., Rosenthal, P., Heyman, M.B., Petersen, C., Davenport, M., Shivakumar, P., Campbell, K.M., Sabla, G.E., Mack, C.L., Tucker, R.M., Lu, B.R., Bezerra, J.A., Tiao, G., Ryckman, F.C., Li, J, Bessho, K., Shinkai, M., Shinkai, T., Puri, P., Stringer, M.D., Nobili, V., Marcellini, M., Giovannelli, L., Wu, J.F., Kao, P.C., Chen, H.L., Urushihara, N., Iwagaki, H., Yagi, T., Narayanaswamy, B., Gonde, C., Tredger, J.M., Hussain, M., Vergani, D., Sakaguchi, S., Yamaguchi, T., Nomura, T., Ono, M., Lan, R.Y., Cheng, C., Lian, Z.X., Sakaki, M., Hiroishi, K., Baba, T., Yang, Y, Liu, Y.J., Tang, S.T., Brindley, S.M., Lanham, A.M., Karrer, F.M., Fontenot, A.P., Uchida, K., Inoue, M., Otake, K., Vejchapipat, P., Poomsawat, S., Chongsrisawat, V., Honsawek, S., Poovorawan, Y., Dong, R., Dong, K., Wang, X., Chen, G., Shen, C., Zheng, S., Mourya, R., Mieli-Vergani, G., Minnick, K.E., Kreisberg, R., Dillon, P.W., Ghoneim, E.M., Sira, M.M., Elaziz, A.M. Abd, Khalil, F.O., Sultan, M.M., Mahmoud, A.B., Fenoglio, D., Bernuzzi, F., Battaglia, F., Beriou, G., Costantino, C.M., Ashley, C.W., Koenen, H.J.P.M., Smeets, R.L., Vink, P.M., Rijssen, E. van, Boots, A.M., Saito, T., Sakamoto, A., Hatano, M., Iwai, J., Higashimoto, Y., Yoshida, H., Riepenhoff-Talty, M., Gouvea, V., Evans, M.J., Tyler, K.L., Sokol, R.J., Oberhaus, S.M., Desmet, V.J., Suskind, D.L., Rosenthal, P., Heyman, M.B., Petersen, C., Davenport, M., Shivakumar, P., Campbell, K.M., Sabla, G.E., Mack, C.L., Tucker, R.M., Lu, B.R., Bezerra, J.A., Tiao, G., Ryckman, F.C., Li, J, Bessho, K., Shinkai, M., Shinkai, T., Puri, P., Stringer, M.D., Nobili, V., Marcellini, M., Giovannelli, L., Wu, J.F., Kao, P.C., Chen, H.L., Urushihara, N., Iwagaki, H., Yagi, T., Narayanaswamy, B., Gonde, C., Tredger, J.M., Hussain, M., Vergani, D., Sakaguchi, S., Yamaguchi, T., Nomura, T., Ono, M., Lan, R.Y., Cheng, C., Lian, Z.X., Sakaki, M., Hiroishi, K., Baba, T., Yang, Y, Liu, Y.J., Tang, S.T., Brindley, S.M., Lanham, A.M., Karrer, F.M., Fontenot, A.P., Uchida, K., Inoue, M., Otake, K., Vejchapipat, P., Poomsawat, S., Chongsrisawat, V., Honsawek, S., Poovorawan, Y., Dong, R., Dong, K., Wang, X., Chen, G., Shen, C., Zheng, S., Mourya, R., Mieli-Vergani, G., Minnick, K.E., Kreisberg, R., Dillon, P.W., Ghoneim, E.M., Sira, M.M., Elaziz, A.M. Abd, Khalil, F.O., Sultan, M.M., Mahmoud, A.B., Fenoglio, D., Bernuzzi, F., Battaglia, F., Beriou, G., Costantino, C.M., Ashley, C.W., Koenen, H.J.P.M., Smeets, R.L., Vink, P.M., Rijssen, E. van, and Boots, A.M.

Abstract

Item does not contain fulltext

Published
2017

4. Systemic and Local Cytokine Profile in Biliary Atresia

Author

Saito, T., Sakamoto, A., Hatano, M., Iwai, J., Higashimoto, Y., Yoshida, H., Riepenhoff-Talty, M., Gouvea, V., Evans, M.J., Tyler, K.L., Sokol, R.J., Oberhaus, S.M., Desmet, V.J., Suskind, D.L., Rosenthal, P., Heyman, M.B., Petersen, C., Davenport, M., Shivakumar, P., Campbell, K.M., Sabla, G.E., Mack, C.L., Tucker, R.M., Lu, B.R., Bezerra, J.A., Tiao, G., Ryckman, F.C., Li, J, Bessho, K., Shinkai, M., Shinkai, T., Puri, P., Stringer, M.D., Nobili, V., Marcellini, M., Giovannelli, L., Wu, J.F., Kao, P.C., Chen, H.L., Urushihara, N., Iwagaki, H., Yagi, T., Narayanaswamy, B., Gonde, C., Tredger, J.M., Hussain, M., Vergani, D., Sakaguchi, S., Yamaguchi, T., Nomura, T., Ono, M., Lan, R.Y., Cheng, C., Lian, Z.X., Sakaki, M., Hiroishi, K., Baba, T., Yang, Y, Liu, Y.J., Tang, S.T., Brindley, S.M., Lanham, A.M., Karrer, F.M., Fontenot, A.P., Uchida, K., Inoue, M., Otake, K., Vejchapipat, P., Poomsawat, S., Chongsrisawat, V., Honsawek, S., Poovorawan, Y., Dong, R., Dong, K., Wang, X., Chen, G., Shen, C., Zheng, S., Mourya, R., Mieli-Vergani, G., Minnick, K.E., Kreisberg, R., Dillon, P.W., Ghoneim, E.M., Sira, M.M., Elaziz, A.M. Abd, Khalil, F.O., Sultan, M.M., Mahmoud, A.B., Fenoglio, D., Bernuzzi, F., Battaglia, F., Beriou, G., Costantino, C.M., Ashley, C.W., Koenen, H.J.P.M., Smeets, R.L., Vink, P.M., Rijssen, E. van, Boots, A.M., Saito, T., Sakamoto, A., Hatano, M., Iwai, J., Higashimoto, Y., Yoshida, H., Riepenhoff-Talty, M., Gouvea, V., Evans, M.J., Tyler, K.L., Sokol, R.J., Oberhaus, S.M., Desmet, V.J., Suskind, D.L., Rosenthal, P., Heyman, M.B., Petersen, C., Davenport, M., Shivakumar, P., Campbell, K.M., Sabla, G.E., Mack, C.L., Tucker, R.M., Lu, B.R., Bezerra, J.A., Tiao, G., Ryckman, F.C., Li, J, Bessho, K., Shinkai, M., Shinkai, T., Puri, P., Stringer, M.D., Nobili, V., Marcellini, M., Giovannelli, L., Wu, J.F., Kao, P.C., Chen, H.L., Urushihara, N., Iwagaki, H., Yagi, T., Narayanaswamy, B., Gonde, C., Tredger, J.M., Hussain, M., Vergani, D., Sakaguchi, S., Yamaguchi, T., Nomura, T., Ono, M., Lan, R.Y., Cheng, C., Lian, Z.X., Sakaki, M., Hiroishi, K., Baba, T., Yang, Y, Liu, Y.J., Tang, S.T., Brindley, S.M., Lanham, A.M., Karrer, F.M., Fontenot, A.P., Uchida, K., Inoue, M., Otake, K., Vejchapipat, P., Poomsawat, S., Chongsrisawat, V., Honsawek, S., Poovorawan, Y., Dong, R., Dong, K., Wang, X., Chen, G., Shen, C., Zheng, S., Mourya, R., Mieli-Vergani, G., Minnick, K.E., Kreisberg, R., Dillon, P.W., Ghoneim, E.M., Sira, M.M., Elaziz, A.M. Abd, Khalil, F.O., Sultan, M.M., Mahmoud, A.B., Fenoglio, D., Bernuzzi, F., Battaglia, F., Beriou, G., Costantino, C.M., Ashley, C.W., Koenen, H.J.P.M., Smeets, R.L., Vink, P.M., Rijssen, E. van, and Boots, A.M.

Abstract

Item does not contain fulltext

Published
2017

5. Systemic and Local Cytokine Profile in Biliary Atresia

Author

Saito, T., Sakamoto, A., Hatano, M., Iwai, J., Higashimoto, Y., Yoshida, H., Riepenhoff-Talty, M., Gouvea, V., Evans, M.J., Tyler, K.L., Sokol, R.J., Oberhaus, S.M., Desmet, V.J., Suskind, D.L., Rosenthal, P., Heyman, M.B., Petersen, C., Davenport, M., Shivakumar, P., Campbell, K.M., Sabla, G.E., Mack, C.L., Tucker, R.M., Lu, B.R., Bezerra, J.A., Tiao, G., Ryckman, F.C., Li, J, Bessho, K., Shinkai, M., Shinkai, T., Puri, P., Stringer, M.D., Nobili, V., Marcellini, M., Giovannelli, L., Wu, J.F., Kao, P.C., Chen, H.L., Urushihara, N., Iwagaki, H., Yagi, T., Narayanaswamy, B., Gonde, C., Tredger, J.M., Hussain, M., Vergani, D., Sakaguchi, S., Yamaguchi, T., Nomura, T., Ono, M., Lan, R.Y., Cheng, C., Lian, Z.X., Sakaki, M., Hiroishi, K., Baba, T., Yang, Y, Liu, Y.J., Tang, S.T., Brindley, S.M., Lanham, A.M., Karrer, F.M., Fontenot, A.P., Uchida, K., Inoue, M., Otake, K., Vejchapipat, P., Poomsawat, S., Chongsrisawat, V., Honsawek, S., Poovorawan, Y., Dong, R., Dong, K., Wang, X., Chen, G., Shen, C., Zheng, S., Mourya, R., Mieli-Vergani, G., Minnick, K.E., Kreisberg, R., Dillon, P.W., Ghoneim, E.M., Sira, M.M., Elaziz, A.M. Abd, Khalil, F.O., Sultan, M.M., Mahmoud, A.B., Fenoglio, D., Bernuzzi, F., Battaglia, F., Beriou, G., Costantino, C.M., Ashley, C.W., Koenen, H.J.P.M., Smeets, R.L., Vink, P.M., Rijssen, E. van, Boots, A.M., Saito, T., Sakamoto, A., Hatano, M., Iwai, J., Higashimoto, Y., Yoshida, H., Riepenhoff-Talty, M., Gouvea, V., Evans, M.J., Tyler, K.L., Sokol, R.J., Oberhaus, S.M., Desmet, V.J., Suskind, D.L., Rosenthal, P., Heyman, M.B., Petersen, C., Davenport, M., Shivakumar, P., Campbell, K.M., Sabla, G.E., Mack, C.L., Tucker, R.M., Lu, B.R., Bezerra, J.A., Tiao, G., Ryckman, F.C., Li, J, Bessho, K., Shinkai, M., Shinkai, T., Puri, P., Stringer, M.D., Nobili, V., Marcellini, M., Giovannelli, L., Wu, J.F., Kao, P.C., Chen, H.L., Urushihara, N., Iwagaki, H., Yagi, T., Narayanaswamy, B., Gonde, C., Tredger, J.M., Hussain, M., Vergani, D., Sakaguchi, S., Yamaguchi, T., Nomura, T., Ono, M., Lan, R.Y., Cheng, C., Lian, Z.X., Sakaki, M., Hiroishi, K., Baba, T., Yang, Y, Liu, Y.J., Tang, S.T., Brindley, S.M., Lanham, A.M., Karrer, F.M., Fontenot, A.P., Uchida, K., Inoue, M., Otake, K., Vejchapipat, P., Poomsawat, S., Chongsrisawat, V., Honsawek, S., Poovorawan, Y., Dong, R., Dong, K., Wang, X., Chen, G., Shen, C., Zheng, S., Mourya, R., Mieli-Vergani, G., Minnick, K.E., Kreisberg, R., Dillon, P.W., Ghoneim, E.M., Sira, M.M., Elaziz, A.M. Abd, Khalil, F.O., Sultan, M.M., Mahmoud, A.B., Fenoglio, D., Bernuzzi, F., Battaglia, F., Beriou, G., Costantino, C.M., Ashley, C.W., Koenen, H.J.P.M., Smeets, R.L., Vink, P.M., Rijssen, E. van, and Boots, A.M.

Abstract

Item does not contain fulltext

Published
2017

6. The outcome of combined antiviral therapy in tolerant children with chronic hepatitis B is associated with emergence of mutations within HBV core gene immunodominant epitopes

Author

Carey, I, Cavers, M, D'Antiga, L, Ma, Y, Bansal, S, Byrne, J, Mieli-Vergani, G, Vergani, D, Carey I, Cavers M, D'Antiga L, Ma Y, Bansal S, Byrne J, Mieli-Vergani G, Vergani D, Carey, I, Cavers, M, D'Antiga, L, Ma, Y, Bansal, S, Byrne, J, Mieli-Vergani, G, Vergani, D, Carey I, Cavers M, D'Antiga L, Ma Y, Bansal S, Byrne J, Mieli-Vergani G, and Vergani D

Published
2006

7. Mutations in HBV core18-27 immunodominant epitope evade viral control of cytotoxic T lymphocytes during combined antiviral treatment in tolerant children with chronic hepatitis B

Author

Carey, I, Cavers, M, D'Antiga, L, Longhi, M, Ma, Y, Bansal, S, Byrne, J, Mieli-Vergani, G, Vergani, D, Carey I, Cavers M, D'Antiga L, Longhi MS, Ma Y, Bansal S, Byrne JA, Mieli-Vergani G, Vergani D, Carey, I, Cavers, M, D'Antiga, L, Longhi, M, Ma, Y, Bansal, S, Byrne, J, Mieli-Vergani, G, Vergani, D, Carey I, Cavers M, D'Antiga L, Longhi MS, Ma Y, Bansal S, Byrne JA, Mieli-Vergani G, and Vergani D

Published
2006

8. Longitudinal analysis of mutations within the HBV core gene in children with chronic hepatitis B treated with lamivudine/interferon-alpha combination therapy

Author

Kraslova, I, D'Antiga, L, Cavers, M, Ma, Y, Bansal, S, Byrne, J, Mieli-Vergani, G, Vergani, D, Kraslova I, D'Antiga L, Cavers M, Ma Y, Bansal S, Byrne J, Mieli-Vergani G, Vergani D, Kraslova, I, D'Antiga, L, Cavers, M, Ma, Y, Bansal, S, Byrne, J, Mieli-Vergani, G, Vergani, D, Kraslova I, D'Antiga L, Cavers M, Ma Y, Bansal S, Byrne J, Mieli-Vergani G, and Vergani D

Published
2005

9. HBV core gene mutations evade immune control

Author

Kraslova-Carey, I, Longhi, M, Cavers, M, D'Antiga, L, Ma, Y, Bansal, S, Byrne, J, Mieli-Vergani, G, Vergani, D, Kraslova-Carey I, Longhi MS, Cavers M, D'Antiga L, Ma Y, Bansal S, Byrne JA, Mieli-Vergani G, Vergani D, Kraslova-Carey, I, Longhi, M, Cavers, M, D'Antiga, L, Ma, Y, Bansal, S, Byrne, J, Mieli-Vergani, G, Vergani, D, Kraslova-Carey I, Longhi MS, Cavers M, D'Antiga L, Ma Y, Bansal S, Byrne JA, Mieli-Vergani G, and Vergani D

Published
2005

10. Emergence of HBV core mutations is associated to a weaker HBV core specific T-cell response and failure to clear hepatitis B virus during combined lamivudine and interferon alpha therapy in infancy-acquired infection

Author

Kraslova-Carey, I, Cavers, M, D'Antiga, L, Ma, Y, Bansal, S, Mieli-Vergani, G, Vergani, D, Kraslova-Carey I, Cavers M, D'Antiga L, Ma Y, Bansal S, Mieli-Vergani G, Vergani D, Kraslova-Carey, I, Cavers, M, D'Antiga, L, Ma, Y, Bansal, S, Mieli-Vergani, G, Vergani, D, Kraslova-Carey I, Cavers M, D'Antiga L, Ma Y, Bansal S, Mieli-Vergani G, and Vergani D

Published
2005

11. A broad and strong HBV core-specific T cell reactivity characterizes sustained viral control in children with chronic hepatitis B treated with combined lamivudine and interferon-alpha

Author

Kraslova, I, Cavers, M, D'Antiga, L, Ma, Y, Barisal, S, Mieli-Vergani, G, Vergani, D, Kraslova I, Cavers M, D'Antiga L, Ma Y, Barisal S, Mieli-Vergani G, Vergani D, Kraslova, I, Cavers, M, D'Antiga, L, Ma, Y, Barisal, S, Mieli-Vergani, G, Vergani, D, Kraslova I, Cavers M, D'Antiga L, Ma Y, Barisal S, Mieli-Vergani G, and Vergani D

Published
2005

12. HBV DNA levels at week 9 and number of HBV core gene mutations predict outcome of lamivudine/interferon a treatment in infancy-acquired chronic hepatitis B

Author

Kraslova-Carey, I, D'Antiga, L, Cavers, M, Ma, Y, Bansal, S, Byrne, J, Mieli-Vergani, G, Vergani, D, Kraslova-Carey I, D'Antiga L, Cavers M, Ma Y, Bansal S, Byrne JA, Mieli-Vergani G, Vergani D, Kraslova-Carey, I, D'Antiga, L, Cavers, M, Ma, Y, Bansal, S, Byrne, J, Mieli-Vergani, G, Vergani, D, Kraslova-Carey I, D'Antiga L, Cavers M, Ma Y, Bansal S, Byrne JA, Mieli-Vergani G, and Vergani D

Published
2005

13. Effect of combined lamivudine and interferon alpha treatment on the T-cell response to HBV core peptides in patients with chronic hepatitis B virus infection

Author

Cavers, M, Aw, M, Sharma, U, D'Antiga, L, William, R, Moorat, A, Greensmith, M, Atkins, M, Mieli-Vergani, G, Vergani, D, Cavers M, Aw M, Sharma U, D'Antiga L, William R, Moorat A, Greensmith M, Atkins M, Mieli-Vergani G, Vergani D, Cavers, M, Aw, M, Sharma, U, D'Antiga, L, William, R, Moorat, A, Greensmith, M, Atkins, M, Mieli-Vergani, G, Vergani, D, Cavers M, Aw M, Sharma U, D'Antiga L, William R, Moorat A, Greensmith M, Atkins M, Mieli-Vergani G, and Vergani D

Published
2002

14. Lamivudine and alpha interferon treatment for low responders HBV infected children: A preliminary report

Author

D'Antiga, L, Aw, M, Atkins, M, Moorat, A, Greensmith, M, Smith, H, Vergani, D, Mieli-Vergani, G, D'Antiga L, Aw M, Atkins M, Moorat A, Greensmith M, Smith H, Vergani D, Mieli-Vergani G, D'Antiga, L, Aw, M, Atkins, M, Moorat, A, Greensmith, M, Smith, H, Vergani, D, Mieli-Vergani, G, D'Antiga L, Aw M, Atkins M, Moorat A, Greensmith M, Smith H, Vergani D, and Mieli-Vergani G

Published
2001

16. Effect of combined lamivudine and interferon alpha treatment on the T-cell response to HBV core peptides in paediatric patients infected with chronic hepatitis B virus

Author

Cavers, M, Aw, M, D'Antiga, L, Malacarne, F, Williams, R, Moorat, A, Greensmith, M, Atkins, M, Mieli-Vergani, G, Vergani, D, Cavers M, Aw M, D'Antiga L, Malacarne F, Williams R, Moorat A, Greensmith M, Atkins M, Mieli-Vergani G, Vergani D, Cavers, M, Aw, M, D'Antiga, L, Malacarne, F, Williams, R, Moorat, A, Greensmith, M, Atkins, M, Mieli-Vergani, G, Vergani, D, Cavers M, Aw M, D'Antiga L, Malacarne F, Williams R, Moorat A, Greensmith M, Atkins M, Mieli-Vergani G, and Vergani D

Published
2001

17. The diagnostic spectrum of surgical jaundice in infancy

Author

Davenport, M, Betalli, P, D'Antiga, L, Mieli-Vergani, G, Howard, E, Davenport M, Betalli P, D'Antiga L, Mieli-Vergani G, Howard E, Davenport, M, Betalli, P, D'Antiga, L, Mieli-Vergani, G, Howard, E, Davenport M, Betalli P, D'Antiga L, Mieli-Vergani G, and Howard E

Published
2001

18. Bone changes in children following liver transplantation

Author

Moniz, C, D'Antiga, L, Abraha, W, Buxton-Thomas, M, Heaton, N, Rela, M, Mieli-Vergani, G, Dhawan, A, Moniz C, D'Antiga L, Abraha W, Buxton-Thomas M, Heaton N, Rela M, Mieli-Vergani G, Dhawan A, Moniz, C, D'Antiga, L, Abraha, W, Buxton-Thomas, M, Heaton, N, Rela, M, Mieli-Vergani, G, Dhawan, A, Moniz C, D'Antiga L, Abraha W, Buxton-Thomas M, Heaton N, Rela M, Mieli-Vergani G, and Dhawan A

Published
2001

19. Bone changes in children with chronic liver disease following transplantation

Author

Moniz, C, D'Antiga, L, Abraha, H, Sangupta, C, Heaton, N, Rela, M, Buxton-Thomas, M, Mieli-Vergani, G, Dhawan, A, Moniz C, D'Antiga L, Abraha H, Sangupta C, Heaton N, Rela M, Buxton-Thomas M, Mieli-Vergani G, Dhawan A, Moniz, C, D'Antiga, L, Abraha, H, Sangupta, C, Heaton, N, Rela, M, Buxton-Thomas, M, Mieli-Vergani, G, Dhawan, A, Moniz C, D'Antiga L, Abraha H, Sangupta C, Heaton N, Rela M, Buxton-Thomas M, Mieli-Vergani G, and Dhawan A

Published
2001

20. HBsAg plasma level kinetics: a new role for an old marker as a therapy response predictor in vertically infected children on combination therapy

Author

Carey, I, Bruce, M, Horner, M, Zen, Y, D'Antiga, L, Bansal, S, Vergani, D, Mieli-Vergani, G, Carey, I, Bruce, M, Horner, M, Zen, Y, D'Antiga, L, Bansal, S, Vergani, D, and Mieli-Vergani, G

Abstract

We aimed to investigate the ability of HBsAg plasma level kinetics to predict therapy response by studying 23 children with infancy-acquired chronic hepatitis B (CHB) during combination sequential therapy with lead-in lamivudine (LAM) and add-on interferon-alpha (IFN-alpha) [5 responders (R = anti-HBs seroconversion) and 18 non-responders (NR)] and to assess their relationship with pretreatment intrahepatic HBV-DNA and cccDNA and HBsAg and HBcAg liver expression. Plasma HBsAg levels were measured in samples before (treatment week 0 = TW0), during (TW9, TW28, TW52) and after (follow-up week = FUW24) therapy by Abbott ARCHITECT (R) assay [log(10)IU/mL]. Baseline liver HBV-DNA and cccDNA were quantified by real-time TaqMan PCR [log(10)copies/ng genomic DNA]. HBsAg and HBcAg liver expression was evaluated by immunostaining of formalin-fixed, paraffin-embedded specimens [number of positive cells/1000 hepatocytes]. All results are presented as medians. Plasma: at baseline, on-treatment and during follow-up, HBsAg levels were lower in R than NR (TW0: 4.36 vs 4.75; TW28: 2.44 vs 4.35; TW52: 0 vs 4.08 and FUW24: 0.17 vs 4.35, all P < 0.05). Liver: baseline HBV-DNA (3.82 vs 4.71, P = 0.16) and cccDNA (1.98 vs 2.26, P = 0.18) tended to be lower in R than NR, HBsAg expression was lower in R than NR (0.5 vs 4.7, P = 0.03), and HBcAg expression was similar between R and NR. There were positive correlations between plasma HBsAg levels and liver HBV-DNA (r = 0.44, P = 0.04), cccDNA (r = 0.41, P = 0.04) and HBsAg liver expression (r = 0.38, P = 0.05). Lower baseline HBsAg plasma levels, lower HBsAg expression in liver and on-treatment decline of plasma HBsAg levels heralds HBsAg clearance and response to treatment in tolerant children with CHB.

Published
2015

21. Histological features during graft dysfunction in paediatric liver transplantation

Author

D'Antiga, L, Dhawan, A, Khan, S, Cheeseman, P, Rela, M, Heaton, N, Mieli-Vergani, G, Portmann, B, D'Antiga L, Dhawan A, Khan S, Cheeseman P, Rela M, Heaton ND, Mieli-Vergani G, Portmann B, D'Antiga, L, Dhawan, A, Khan, S, Cheeseman, P, Rela, M, Heaton, N, Mieli-Vergani, G, Portmann, B, D'Antiga L, Dhawan A, Khan S, Cheeseman P, Rela M, Heaton ND, Mieli-Vergani G, and Portmann B

Published
2000

22. The effect of liver transplantation (LT) on hepatic osteodystrophy in children

Author

D'Antiga, L, Dhawan, A, Moniz, C, Thomas, M, Abraha, H, Baker, A, Heaton, N, Rela, M, Mieli-Vergani, G, D'Antiga L, Dhawan A, Moniz C, Thomas MB, Abraha H, Baker AJ, Heaton ND, Rela M, Mieli-Vergani G, D'Antiga, L, Dhawan, A, Moniz, C, Thomas, M, Abraha, H, Baker, A, Heaton, N, Rela, M, Mieli-Vergani, G, D'Antiga L, Dhawan A, Moniz C, Thomas MB, Abraha H, Baker AJ, Heaton ND, Rela M, and Mieli-Vergani G

Published
2000

24. Neonatal haemochromatosis in eleven families: Pattern of presentation and outcome

Author

Rodrigues, F, Kallas, M, Nash, R, Sandell, J, D'Antiga, L, Mieli-Vergani, G, Rodrigues FM, Kallas M, Nash R, Sandell J, D'Antiga L, Mieli-Vergani G, Rodrigues, F, Kallas, M, Nash, R, Sandell, J, D'Antiga, L, Mieli-Vergani, G, Rodrigues FM, Kallas M, Nash R, Sandell J, D'Antiga L, and Mieli-Vergani G

Published
1999

26. High circulating levels of interleukin-6 and tumor necrosis factor-alpha are associated with hepatitis B virus clearance during alpha interferon treatment

Author

Ma, Y, Hussain, M, D'Antiga, L, Cheeseman, P, Williams, R, Mieli-Vergani, G, Vergani, D, Ma Y, Hussain MJ, D'Antiga L, Cheeseman P, Williams R, Mieli-Vergani G, Vergani D, Ma, Y, Hussain, M, D'Antiga, L, Cheeseman, P, Williams, R, Mieli-Vergani, G, Vergani, D, Ma Y, Hussain MJ, D'Antiga L, Cheeseman P, Williams R, Mieli-Vergani G, and Vergani D

Published
1999

27. Biochemical markers of bone metabolism and bone mineral density (BMD) in children before and after liver transplantation

Author

D'Antiga, L, Dhawan, A, Moniz, C, Thomas, M, Abraha, H, Baker, A, Heaton, N, Rela, M, Mieli-Vergani, G, D'Antiga L, Dhawan A, Moniz C, Thomas MB, Abraha H, Baker AJ, Heaton ND, Rela M, Mieli-Vergani G, D'Antiga, L, Dhawan, A, Moniz, C, Thomas, M, Abraha, H, Baker, A, Heaton, N, Rela, M, Mieli-Vergani, G, D'Antiga L, Dhawan A, Moniz C, Thomas MB, Abraha H, Baker AJ, Heaton ND, Rela M, and Mieli-Vergani G

Published
1999

28. Intestinal absorption and permeability in paediatric short-bowel syndrome: A pilot study

Author

D'Antiga, L, Dhawan, A, Davenport, M, Mieli-Vergani, G, Bjarnason, I, D'Antiga L, Dhawan A, Davenport M, Mieli-Vergani G, Bjarnason I, D'Antiga, L, Dhawan, A, Davenport, M, Mieli-Vergani, G, Bjarnason, I, D'Antiga L, Dhawan A, Davenport M, Mieli-Vergani G, and Bjarnason I

Abstract

Background: Sugar absorption tests are an effective, noninvasive way to assess intestinal permeability. The role of intestinal barrier integrity in complications and outcome of short-bowel syndrome is not known. The purpose of the study was to evaluate whether such tests provide information on the status of intestinal mucosa of these patients. Methods: Six children with short-bowel syndrome - median age, 12 months, and median small bowel length at birth, 30 cm - had a sugar test with 3-o-methyl-D-glucose, D-xylose, D- rhamnose, and melibiose approximately 2 months after operation. The melibiose/L-rhamnose ratio was used as an index of permeability, and percentages of 3-o-methyl-D-glucose and D-xylose absorbed were used as indices of absorption. Parenteral nutrition requirement, bowel length, liver disease, recent sepsis, and bacterial overgrowth were recorded. Results: Three patients had increased permeability, and all of them had had a recent episode of sepsis and severe liver disease. All subjects had malabsorption of 3-o-methyl-D-glucose, and five of six had malabsorption of D-xylose and L- rhamnose. The absorption of 3-o-methyl-D-glucose correlated with bowel length (r2 = 0.78; P = 0.04), whereas the absorption of D-xylose correlated with parenteral requirement (r2 = 0.66; P = 0.04) at that time. Conclusions: Increased permeability was observed in three of six patients with short-bowel syndrome associated with a recent episode of sepsis and severe liver disease. Other indices of malabsorption correlated significantly with different clinical features of the disease. A prospective larger scale study in a homogeneous population is indicated to assess at multiple points during the disease course whether the test can be helpful in the management of these patients.

Published
1999

29. Late acute rejection in paediatric liver transplantation: Aetiology and outcome

Author

D'Antiga, L, Dhawan, A, Francavilla, R, Rela, M, Heaton, N, Mieli-Vergani, G, D'Antiga L, Dhawan A, Francavilla R, Rela M, Heaton N, Mieli-Vergani G, D'Antiga, L, Dhawan, A, Francavilla, R, Rela, M, Heaton, N, Mieli-Vergani, G, D'Antiga L, Dhawan A, Francavilla R, Rela M, Heaton N, and Mieli-Vergani G

Published
1998

30. Management of chronic hepatitis B in childhood:ESPGHAN clinical practice guidelines.

Author

UCL - (SLuc) Service de gastro-entérologie et hépatologie pédiatrique, UCL - SSS/IREC/PEDI - Pôle de Pédiatrie, Sokal, Etienne, Paganelli, M, Wirth, S, Socha, P, Vajro, P, Lacaille, F, Kelly, D, Mieli-Vergani, G, UCL - (SLuc) Service de gastro-entérologie et hépatologie pédiatrique, UCL - SSS/IREC/PEDI - Pôle de Pédiatrie, Sokal, Etienne, Paganelli, M, Wirth, S, Socha, P, Vajro, P, Lacaille, F, Kelly, D, and Mieli-Vergani, G

Abstract

More than 360 million persons worldwide (6% of the world population) are chronically infected by the hepatitis B Virus (HBV). Although the incidence of HBV infection has dramatically declined since the implementation of universal immunization programs in several countries and blood-donor screening, a significant number of children are still infected each year, often developing chronic infection and requiring appropriate followup [1]. Despite a rather benign course of chronic hepatitis B (CHB) during childhood and adolescence, 3-5% and 0.01-0.03% of chronic carriers develop cirrhosis or hepatocellular carcinoma (HCC), respectively, before adulthood [2,3]. Such a risk for HCC rises to 9-24% when considering the whole lifetime, with an incidence of cirrhosis of 2-3% per year [4,5]. Worldwide universal vaccination remains the goal for eliminating HBV infection and its complications. Treatment of CHB in childhood has been hampered by the chronic delay in licensing new drugs for pediatric use. Safe and effective antiviral therapies are available in adults, but few are labeled for the use in children, and an accurate selection of whom to treat and the identification of the right timing for treatment are needed to optimize response and reduce the risk of antiviral resistance. Although several guidelines on the management of adult patients with CHB have been published by major international societies, the clinical approach to infected children is still evolving, and is mostly based on consensus of expert opinion [6-9].

Published
2013

31. Severe bile salt export pump deficiency: 82 different ABCB11 mutations in 109 families

Author

Strautnieks, S S, Byrne, J A, Pawlikowska, L, Cebecauerová, D, Rayner, A, Dutton, L, Meier, Y, Antoniou, A, Stieger, B, Arnell, H, Özçay, F, Al-Hussaini, H F, Bassas, A F, Verkade, H J, Fischler, B, Németh, A, Kotalová, R, Shneider, B L, Cielecka-Kuszyk, J, McClean, P, Whitington, P F, Sokal, É, Jirsa, M, Wali, S H, Jankowska, I, Pawłowska, J, Mieli-Vergani, G, Knisely, A S, Bull, L N, Thompson, R J, Strautnieks, S S, Byrne, J A, Pawlikowska, L, Cebecauerová, D, Rayner, A, Dutton, L, Meier, Y, Antoniou, A, Stieger, B, Arnell, H, Özçay, F, Al-Hussaini, H F, Bassas, A F, Verkade, H J, Fischler, B, Németh, A, Kotalová, R, Shneider, B L, Cielecka-Kuszyk, J, McClean, P, Whitington, P F, Sokal, É, Jirsa, M, Wali, S H, Jankowska, I, Pawłowska, J, Mieli-Vergani, G, Knisely, A S, Bull, L N, and Thompson, R J

Abstract

BACKGROUND & AIMS: Patients with severe bile salt export pump (BSEP) deficiency present as infants with progressive cholestatic liver disease. We characterized mutations of ABCB11 (encoding BSEP) in such patients and correlated genotypes with residual protein detection and risk of malignancy. METHODS: Patients with intrahepatic cholestasis suggestive of BSEP deficiency were investigated by single-strand conformation polymorphism analysis and sequencing of ABCB11. Genotypes sorted by likely phenotypic severity were correlated with data on BSEP immunohistochemistry and clinical outcome. RESULTS: Eighty-two different mutations (52 novel) were identified in 109 families (9 nonsense mutations, 10 small insertions and deletions, 15 splice-site changes, 3 whole-gene deletions, 45 missense changes). In 7 families, only a single heterozygous mutation was identified despite complete sequence analysis. Thirty-two percent of mutations occurred in >1 family, with E297G and/or D482G present in 58% of European families (52/89). On immunohistochemical analysis (88 patients), 93% had abnormal or absent BSEP staining. Expression varied most for E297G and D482G, with some BSEP detected in 45% of patients (19/42) with these mutations. Hepatocellular carcinoma or cholangiocarcinoma developed in 15% of patients (19/128). Two protein-truncating mutations conferred particular risk; 38% (8/21) of such patients developed malignancy versus 10% (11/107) with potentially less severe genotypes (relative risk, 3.7 [confidence limits, 1.7-8.1; P = .003]). CONCLUSIONS: With this study, >100 ABCB11 mutations are now identified. Immunohistochemically detectable BSEP is typically absent, or much reduced, in severe disease. BSEP deficiency confers risk of hepatobiliary malignancy. Close surveillance of BSEP-deficient patients retaining their native liver, particularly those carrying 2 null mutations, is essential.

Published
2008

33. Neonatal hemochromatosis - Medical treatment vs. transplantation: The King's experience

Author

Rodrigues, F, Kallas, M, Nash, R, Cheeseman, P, D'Antiga, L, Rela, M, Heaton, N, Mieli-Vergani, G, Heaton, ND, Rodrigues, F, Kallas, M, Nash, R, Cheeseman, P, D'Antiga, L, Rela, M, Heaton, N, Mieli-Vergani, G, and Heaton, ND

Abstract

The aim of our study was to compare the outcome of medical treatment vs. liver transplantation in infants with neonatal hemochromatosis (NH) referred to King's College Hospital from 1990-2002. We conducted a retrospective review of 19 children from 14 families. Fifteen children presented at birth and 4 during the first week of life. One child was diagnosed by cordocentesis at 30 weeks of gestation. NH recurred in 7 of 9 families with further children. In one family, 2 children from different fathers were affected. All patients had elevated ferritin levels, hypoalbuminemia, and coagulopathy. Liver histology showed parenchymal collapse, diffuse fibrosis, and moderate to severe hepatocyte hemosiderin deposition. Extrahepatic siderosis was demonstrated by magnetic resonance in 2 patients, lip biopsy in 3, and autopsy in 10. Ten patients received a chelation-antioxidant cocktail: 1 survived, 4 died, and 5 required liver transplantation, of whom 2 died. One of the 9 infants who did not receive the cocktail survived with medical support, 3 died, and 5 required transplantation, of whom 3 died. Seven children are alive, 5 after transplantation, at a median follow-up of 5.6 years, with excellent quality of life and no recurrence of the disease. In conclusion, chelation-antioxidant treatment does not appear to modify the prognosis of NH, at least in severe cases. Liver transplantation, with 50% long-term survival, remains the treatment of choice and should be promptly offered to those infants who do not improve with supportive medical treatment.

Published
2005

34. The spectrum of surgical jaundice in infancy

Author

Davenport, M, Betalli, P, D'Antiga, L, Cheeseman, P, Mieli-Vergani, G, Howard, E, Howard, ER, Davenport, M, Betalli, P, D'Antiga, L, Cheeseman, P, Mieli-Vergani, G, Howard, E, and Howard, ER

Abstract

Background: Conjugated jaundice arising during infancy may be caused by a number of different surgical conditions. The aim of this study was to compare clinical features, management, and outcome of all types of surgical jaundice presenting in the first year of life. Methods:A retrospective review was conducted of all infants born in the United Kingdom with jaundice caused by a surgical cause referred to the authors' institution from January 1992 to December 1999. Results: There were 171 infants who could be separated into 3 specific groups: biliary atresia (BA, n = 137), inspissated bile syndrome (IBS; n = 14), and choledochal malformation (CM; n = 12) together with a group containing various miscellaneous conditions (n = 8). Infants with BA had higher bilirubin (P < .01) and aspartate aminotransferase levels (P < .001) and came to surgery earlier (P < .01) than infants with either IBS or CM. Infants with IBS and CM were more likely to be premature and have other malformations, respectively. Ultrasound scan was the principal investigation in the differentiation of BA from other causes of jaundice. Accurate prelaparotomy diagnosis was made by percutaneous liver biopsy in 87% of cases later shown to be BA. Currently, 88 (64%) of children with BA are alive with their native liver postportoenterostomy, 4 have died, and 45 have undergone liver transplantation (with 1 death postoperatively). A policy of primary portoenterostomy for BA followed by transplantation, if necessary, resulted in a survival rate of over 95%. All children in the other diagnostic groups are alive and anicteric after appropriate surgical intervention. Conclusions: Approximately 80% of infants presenting with surgical jaundice have biliary atresia, whereas those with inspissated bile syndrome and choledochal malformations make up most of the remainder. Mortality in this age-group is confined to infants with BA, but even on these infants an overall survival rate of greater than 95% is current

Published
2003

35. Mutations in the sterol 27-hydoxylase gene (CYP27A) cause hepatitis of infancy as well as cerebrotendinous xanthomatosis.

Author

Clayton, P.T., Verrips, A., Sistermans, E.A., Mann, A., Mieli-Vergani, G., Wevers, R.A., Clayton, P.T., Verrips, A., Sistermans, E.A., Mann, A., Mieli-Vergani, G., and Wevers, R.A.

Abstract

Item does not contain fulltext, Follow-up investigations were undertaken on a previously reported patient who had severe familial giant cell hepatitis in infancy associated with substantially increased urinary excretion of bile alcohol glucuronides. By the age of 11 years, he had developed a profile of cholanoids in plasma and urine that closely resembled the pattern seen in cerebrotendinous xanthomatosis (CTX). Sequencing of the sterol 27-hydroxylase gene (CYP27A) showed that he was homozygous for a deletion (525/526delG) that causes a frameshift and a premature stop codon. This genotype has previously been described in an adult female withclassical symptoms of CTX (tendon xanthomata, cataracts and deteriorating cognitive function). A review of past medical histories of a group of patients with CTX revealed that prolonged neonatal cholestatic jaundice was common. The family histories also revealed fetal and neonatal deaths among siblings of patients with CTX. We conclude that defective activity of cholesterol 27-hydroxylase can lead to neonatal cholestatic jaundice ('hepatitis of infancy'), which may be self-limiting. After a latent period, however, progressive accumulation of cholesterol and cholestanol can lead to the xanthomata, neurodegeneration, cataracts and atherosclerosis that are typical of CTX.

Published
2002

36. Mutations in the sterol 27-hydoxylase gene (CYP27A) cause hepatitis of infancy as well as cerebrotendinous xanthomatosis.

Author

Clayton, P.T., Verrips, A., Sistermans, E.A., Mann, A., Mieli-Vergani, G., Wevers, R.A., Clayton, P.T., Verrips, A., Sistermans, E.A., Mann, A., Mieli-Vergani, G., and Wevers, R.A.

Abstract

Item does not contain fulltext, Follow-up investigations were undertaken on a previously reported patient who had severe familial giant cell hepatitis in infancy associated with substantially increased urinary excretion of bile alcohol glucuronides. By the age of 11 years, he had developed a profile of cholanoids in plasma and urine that closely resembled the pattern seen in cerebrotendinous xanthomatosis (CTX). Sequencing of the sterol 27-hydroxylase gene (CYP27A) showed that he was homozygous for a deletion (525/526delG) that causes a frameshift and a premature stop codon. This genotype has previously been described in an adult female withclassical symptoms of CTX (tendon xanthomata, cataracts and deteriorating cognitive function). A review of past medical histories of a group of patients with CTX revealed that prolonged neonatal cholestatic jaundice was common. The family histories also revealed fetal and neonatal deaths among siblings of patients with CTX. We conclude that defective activity of cholesterol 27-hydroxylase can lead to neonatal cholestatic jaundice ('hepatitis of infancy'), which may be self-limiting. After a latent period, however, progressive accumulation of cholesterol and cholestanol can lead to the xanthomata, neurodegeneration, cataracts and atherosclerosis that are typical of CTX.

Published
2002

37. Mutations in the sterol 27-hydoxylase gene (CYP27A) cause hepatitis of infancy as well as cerebrotendinous xanthomatosis.

Author

Clayton, P.T., Verrips, A., Sistermans, E.A., Mann, A., Mieli-Vergani, G., Wevers, R.A., Clayton, P.T., Verrips, A., Sistermans, E.A., Mann, A., Mieli-Vergani, G., and Wevers, R.A.

Abstract

Item does not contain fulltext, Follow-up investigations were undertaken on a previously reported patient who had severe familial giant cell hepatitis in infancy associated with substantially increased urinary excretion of bile alcohol glucuronides. By the age of 11 years, he had developed a profile of cholanoids in plasma and urine that closely resembled the pattern seen in cerebrotendinous xanthomatosis (CTX). Sequencing of the sterol 27-hydroxylase gene (CYP27A) showed that he was homozygous for a deletion (525/526delG) that causes a frameshift and a premature stop codon. This genotype has previously been described in an adult female withclassical symptoms of CTX (tendon xanthomata, cataracts and deteriorating cognitive function). A review of past medical histories of a group of patients with CTX revealed that prolonged neonatal cholestatic jaundice was common. The family histories also revealed fetal and neonatal deaths among siblings of patients with CTX. We conclude that defective activity of cholesterol 27-hydroxylase can lead to neonatal cholestatic jaundice ('hepatitis of infancy'), which may be self-limiting. After a latent period, however, progressive accumulation of cholesterol and cholestanol can lead to the xanthomata, neurodegeneration, cataracts and atherosclerosis that are typical of CTX.

Published
2002

38. Late cellular rejection in paediatric liver transplantation: Aetiology and outcome

Author

D'Antiga, L, Dhawan, A, Portmann, B, Francavilla, R, Rela, M, Heaton, N, Mieli-Vergani, G, D'Antiga, L, Dhawan, A, Portmann, B, Francavilla, R, Rela, M, Heaton, N, and Mieli-Vergani, G

Abstract

Background. Acute cellular rejection, though mostly encountered during the first 3 months after liver transplant, may occur later on. Clinical features and outcome of children experiencing late cellular rejection (LCR) have not been described to date. Patients and Methods. A total of 20 children who developed acute rejection 6 months or more after liver transplant were studied for history of early rejection, levels of immunosuppression, causes of low immunosuppression, viral infections, signs of autoimmunity, and HLA mismatch. All liver biopsies were reviewed. Fifteen patients with no history of LCR were randomly selected as controls. Results. Of 20 children 5 were appropriately immunosuppressed, although 15 were not. Causes of low immunosuppression were: unknown (seven), poor compliance (three), posttransplant lymphoproliferative disease, (two), hepatocellular carcinoma recurrence (one), tuberculosis (one), gastroenteritis (one). Of 20 patients early rejection occurred in 13 and cytomegalovirus infection in 5. At last follow-up 10 children have persistent graft dysfunction, of whom 5 have progressed to de novo autoimmune hepatitis, 2 to chronic rejection, one has persistent graft dysfunction with recurrent cytomegalovirus activation, one has hepatic artery thrombosis and one is likely to have persistent poor compliance. None of the 15 controls developed de novo autoimmune hepatitis or any other form of persistent graft dysfunction at the time of last follow-up. Conclusion. Late cellular rejection in children is usually due to low or decreased immunosuppression and is associated with long-term complications. Prompt intervention to correct inadequate immunosuppression and careful follow-up of the other patients to identify other treatable conditions is essential.

Published
2002

39. Bone mineral density and height gain in children with chronic cholestatic liver disease undergoing transplantation

Author

D'Antiga, L, Moniz, C, Buxton-Thomas, M, Cheeseman, P, Gray, B, Abraha, H, Baker, A, Heaton, N, Rela, M, Mieli-Vergani, G, Dhawan, A, Baker, AJ, Heaton, ND, D'Antiga, L, Moniz, C, Buxton-Thomas, M, Cheeseman, P, Gray, B, Abraha, H, Baker, A, Heaton, N, Rela, M, Mieli-Vergani, G, Dhawan, A, Baker, AJ, and Heaton, ND

Abstract

Background. Osteodystrophy is a well-described complication of chronic liver disease. Previous reports in adults and children undergoing liver transplantation (LT) were discordant, with the former showing no improvement of bone disease in the first year after transplantation and the latter demonstrating remarkable benefit from it. Our aim was to perform a pilot study on osteodystrophy in children undergoing LT and evaluate the contribution of growth on bone mineral density (BMD) changes. Methods. We studied six patients (two male), with a median age at transplantation of 8.8 (range 3.8-16.6) years. Indications for transplantation were biliary atresia and progressive familial intrahepatic cholestasis (three patients each). BMD was studied with dual-energy x-ray absorptiometry and biochemical markers of liver and bone function in patients before and at 3, 6, and 12 months after LT. Results. Median L2-L4 spinal BMD was 0.54 g/cm2 (range 0.29-0.87) before LT, and 0.58 g/cm2 (0.27-0.86) at 3 months, 0.66 g/cm2 (0.36-1.00) at 6 months, and 0.76 g/cm2 (0.44-1.02) at 12 months after LT (P=0.005). Median height was 133 (range 93-167) cm before LT, and 134 (93-167) at 3 months, 136 (97-167) at 6 months, and 139 (102-167) at 12 months after LT. There was direct correlation between height gain and total body BMD improvement (r=0.929, P=0.007). Conclusion. BMD in children with chronic cholestatic liver disease improves remarkably by 12 months after LT. Catch-up growth in children can account for the different effect of LT on bone density between adult and pediatric populations in the first year after surgery.

Published
2002

40. A dose ranging study of the pharmacokinetics, safety, and preliminary efficacy of lamivudine in children and adolescents with chronic hepatitis B.

Author

UCL - MD/GYPE - Département de gynécologie, d'obstétrique et de pédiatrie, Sokal, Etienne, Roberts, E A, Mieli-Vergani, G, McPhillips, P, Johnson, M, Barber, J, Dallow, N, Boxall, E, Kelly, D., UCL - MD/GYPE - Département de gynécologie, d'obstétrique et de pédiatrie, Sokal, Etienne, Roberts, E A, Mieli-Vergani, G, McPhillips, P, Johnson, M, Barber, J, Dallow, N, Boxall, E, and Kelly, D.

Abstract

Fifty-three patients with chronic hepatitis B and active viral replication were studied for 4 weeks while on treatment and for 12 weeks after treatment with the oral nucleoside analogue lamivudine. Children aged 2 to 12 years were randomized to receive twice-daily doses of 0.35, 1.5, or 4 mg of lamivudine solution per kg of body weight or once-daily doses of 3 mg of lamivudine solution per kg. Adolescents aged 13 to 17 years received lamivudine at 100 mg (as tablets). Blood samples for pharmacokinetic assay were taken on days 1 and 28. Lamivudine was rapidly absorbed following oral administration, with the maximum concentration in serum being reached 0.5 to 1 h postdosing. Apparent oral clearance (CL/F) was higher in younger children and decreased with age, with CL/F values for adolescents reaching those seen for adults by the age of 12. All doses produced a dramatic fall in serum hepatitis B virus (HBV) DNA levels, with a median reduction of >/=99.5% after 4 weeks of treatment and with the levels returning to the baseline levels posttreatment. The correlation of dose, area under the concentration-time curve (AUC), and changes in HBV DNA levels, as measured by the Chiron Quantiplex assay, showed maximal antiviral effects (99.9% inhibition and a reduction of the amount of HBV DNA of approximately 3 log(10)) at 3 mg/kg/day, with no discernible increase in effect seen whether the drug was given at 4 mg/kg twice daily or whether it was given once daily or twice daily. The limit of detection of the assay (2.5 pg/ml) was reached for some but not all patients across the dose ranges, with the smallest number (n = 2) of those having values negative by the Chiron Quantiplex assay being in the lowest-dose group. The 13- to 17-year-olds showed a similar overall response in terms of the HBV DNA level reduction compared to that for patients younger than age 13. Analysis of the same samples by PCR, which has a lower limit of sensitivity than the Chiron Quantiplex assay, also showe

Published
2000

41. A gene encoding a liver-specific ABC transporter is mutated in progressive familial intrahepatic cholestasis.

Author

Strautnieks, SS, Bull, LN, Knisely, AS, Kocoshis, SA, Dahl, N, Arnell, H, Sokal, E, Dahan, K, Childs, S, Ling, V, Tanner, MS, Kagalwalla, AF, Nemeth, A, Pawlowska, J, Baker, A, Mieli-Vergani, G, Freimer, NB, Gardiner, RM, Thompson, RJ, Strautnieks, SS, Bull, LN, Knisely, AS, Kocoshis, SA, Dahl, N, Arnell, H, Sokal, E, Dahan, K, Childs, S, Ling, V, Tanner, MS, Kagalwalla, AF, Nemeth, A, Pawlowska, J, Baker, A, Mieli-Vergani, G, Freimer, NB, Gardiner, RM, and Thompson, RJ

Published
1998

42. A gene encoding a liver-specific ABC transporter is mutated in progressive familial intrahepatic cholestasis.

Author

UCL - MD/GYPE - Département de gynécologie, d'obstétrique et de pédiatrie, Strautnieks, S S, Bull, L N, Knisely, A S, Kocoshis, S A, Dahl, N, Arnell, H, Sokal, Etienne, Dahan, Karin, Childs, S, Ling, V, Tanner, M S, Kagalwalla, A F, Németh, A, Pawlowska, J, Baker, A., Mieli-Vergani, G, Freimer, N B, Gardiner, R M, Thompson, R J, UCL - MD/GYPE - Département de gynécologie, d'obstétrique et de pédiatrie, Strautnieks, S S, Bull, L N, Knisely, A S, Kocoshis, S A, Dahl, N, Arnell, H, Sokal, Etienne, Dahan, Karin, Childs, S, Ling, V, Tanner, M S, Kagalwalla, A F, Németh, A, Pawlowska, J, Baker, A., Mieli-Vergani, G, Freimer, N B, Gardiner, R M, and Thompson, R J

Abstract

The progressive familial intrahepatic cholestases (PFIC) are a group of inherited disorders with severe cholestatic liver disease from early infancy. A subgroup characterized by normal serum cholesterol and gamma-glutamyltranspeptidase (gammaGT) levels is genetically heterogeneous with loci on chromosomes 2q (PFIC2) and 18q. The phenotype of the PFIC2-linked group is consistent with defective bile acid transport at the hepatocyte canalicular membrane. The PFIC2 gene has now been identified by mutations in a positional candidate, BSEP, which encodes a liver-specific ATP-binding cassette (ABC) transporter, sister of p-glycoprotein (SPGP). The product of the orthologous rat gene has been shown to be an effective bile acid transporter in vitro. These data provide evidence that SPGP is the human bile salt export pump (BSEP).

Published
1998

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