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Combined liver-kidney transplantation for children with autosomal recessive polycystic kidney disease (ARPKD): indication and outcome.

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Combined liver-kidney transplantation for children with autosomal recessive polycystic kidney disease (ARPKD): indication and outcome. / Brinkert, Florian; Lehnhardt, Anja; Montoya, Carmen; Helmke, Knut; Schäfer, Hansjoerg; Fischer, Lutz; Nashan, Bjoern; Bergmann, Carsten; Ganschow, Rainer; Kemper, Markus J.

in: TRANSPL INT, Jahrgang 26, Nr. 6, 6, 2013, S. 640-650.

Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung

Harvard

Brinkert, F, Lehnhardt, A, Montoya, C, Helmke, K, Schäfer, H, Fischer, L, Nashan, B, Bergmann, C, Ganschow, R & Kemper, MJ 2013, 'Combined liver-kidney transplantation for children with autosomal recessive polycystic kidney disease (ARPKD): indication and outcome.', TRANSPL INT, Jg. 26, Nr. 6, 6, S. 640-650. https://doi.org/10.1111/tri.12098

APA

Brinkert, F., Lehnhardt, A., Montoya, C., Helmke, K., Schäfer, H., Fischer, L., Nashan, B., Bergmann, C., Ganschow, R., & Kemper, M. J. (2013). Combined liver-kidney transplantation for children with autosomal recessive polycystic kidney disease (ARPKD): indication and outcome. TRANSPL INT, 26(6), 640-650. [6]. https://doi.org/10.1111/tri.12098

Vancouver

Brinkert F, Lehnhardt A, Montoya C, Helmke K, Schäfer H, Fischer L et al. Combined liver-kidney transplantation for children with autosomal recessive polycystic kidney disease (ARPKD): indication and outcome. TRANSPL INT. 2013;26(6):640-650. 6. https://doi.org/10.1111/tri.12098

Bibtex

@article{2b87f960e69b41c88ff55facb04d3a1a,
title = "Combined liver-kidney transplantation for children with autosomal recessive polycystic kidney disease (ARPKD): indication and outcome.",
abstract = "In ARPKD, mutations in the PKHD1 gene lead to remodeling of the kidneys and liver. These may result in progressive liver fibrosis with portal hypertension requiring combined liver and kidney transplantation (CLKT). There is currently no consensus on the indication for CLKT and data on long-term outcomes are scarce. We analyzed in detail the pretransplant liver symptomatology, laboratory and ultrasound data, histological studies, and genotypes in eight patients undergoing CLKT. The median age was 10.1 years (range 1.7-16) and median follow-up was 4.6 years (range 1.1-8.9). All patients had clinical signs of portal hypertension and abnormal ultrasound findings. Congenital hepatic fibrosis was present in all pretransplant biopsies (6 out of 8 patients) and in all explanted livers. All patients survived; liver and kidney graft survival was 72% and 88%, respectively. Liver and kidney function were stable in all patients with a median eGFR of 70 ml/min/1.73 m² (range 45-108 ml/min/1.73 m²). Height-SDS improved significantly after 12, 24, and 36 months (P = 0.016, 0.022 and 0.018 respectively). The indication for CLKT remains challenging and controversial. A favorable outcome for patients with ARPKD can be achieved by using the degree of portal hypertension, longitudinal ultrasound examinations, and preoperative liver histology as parameters for CLKT.",
keywords = "Adolescent, Child, Child, Preschool, Female, Graft Survival, Humans, Hypertension, Portal, Infant, Kidney Failure, Chronic, Kidney Transplantation, Liver, Liver Cirrhosis, Liver Transplantation, Male, Polycystic Kidney, Autosomal Recessive, Receptors, Cell Surface, Retrospective Studies",
author = "Florian Brinkert and Anja Lehnhardt and Carmen Montoya and Knut Helmke and Hansjoerg Sch{\"a}fer and Lutz Fischer and Bjoern Nashan and Carsten Bergmann and Rainer Ganschow and Kemper, {Markus J}",
note = "{\textcopyright} 2013 The Authors Transplant International {\textcopyright} 2013 European Society for Organ Transplantation. Published by Blackwell Publishing Ltd.",
year = "2013",
doi = "10.1111/tri.12098",
language = "English",
volume = "26",
pages = "640--650",
journal = "TRANSPL INT",
issn = "0934-0874",
publisher = "Wiley-Blackwell",
number = "6",

}

RIS

TY - JOUR

T1 - Combined liver-kidney transplantation for children with autosomal recessive polycystic kidney disease (ARPKD): indication and outcome.

AU - Brinkert, Florian

AU - Lehnhardt, Anja

AU - Montoya, Carmen

AU - Helmke, Knut

AU - Schäfer, Hansjoerg

AU - Fischer, Lutz

AU - Nashan, Bjoern

AU - Bergmann, Carsten

AU - Ganschow, Rainer

AU - Kemper, Markus J

N1 - © 2013 The Authors Transplant International © 2013 European Society for Organ Transplantation. Published by Blackwell Publishing Ltd.

PY - 2013

Y1 - 2013

N2 - In ARPKD, mutations in the PKHD1 gene lead to remodeling of the kidneys and liver. These may result in progressive liver fibrosis with portal hypertension requiring combined liver and kidney transplantation (CLKT). There is currently no consensus on the indication for CLKT and data on long-term outcomes are scarce. We analyzed in detail the pretransplant liver symptomatology, laboratory and ultrasound data, histological studies, and genotypes in eight patients undergoing CLKT. The median age was 10.1 years (range 1.7-16) and median follow-up was 4.6 years (range 1.1-8.9). All patients had clinical signs of portal hypertension and abnormal ultrasound findings. Congenital hepatic fibrosis was present in all pretransplant biopsies (6 out of 8 patients) and in all explanted livers. All patients survived; liver and kidney graft survival was 72% and 88%, respectively. Liver and kidney function were stable in all patients with a median eGFR of 70 ml/min/1.73 m² (range 45-108 ml/min/1.73 m²). Height-SDS improved significantly after 12, 24, and 36 months (P = 0.016, 0.022 and 0.018 respectively). The indication for CLKT remains challenging and controversial. A favorable outcome for patients with ARPKD can be achieved by using the degree of portal hypertension, longitudinal ultrasound examinations, and preoperative liver histology as parameters for CLKT.

AB - In ARPKD, mutations in the PKHD1 gene lead to remodeling of the kidneys and liver. These may result in progressive liver fibrosis with portal hypertension requiring combined liver and kidney transplantation (CLKT). There is currently no consensus on the indication for CLKT and data on long-term outcomes are scarce. We analyzed in detail the pretransplant liver symptomatology, laboratory and ultrasound data, histological studies, and genotypes in eight patients undergoing CLKT. The median age was 10.1 years (range 1.7-16) and median follow-up was 4.6 years (range 1.1-8.9). All patients had clinical signs of portal hypertension and abnormal ultrasound findings. Congenital hepatic fibrosis was present in all pretransplant biopsies (6 out of 8 patients) and in all explanted livers. All patients survived; liver and kidney graft survival was 72% and 88%, respectively. Liver and kidney function were stable in all patients with a median eGFR of 70 ml/min/1.73 m² (range 45-108 ml/min/1.73 m²). Height-SDS improved significantly after 12, 24, and 36 months (P = 0.016, 0.022 and 0.018 respectively). The indication for CLKT remains challenging and controversial. A favorable outcome for patients with ARPKD can be achieved by using the degree of portal hypertension, longitudinal ultrasound examinations, and preoperative liver histology as parameters for CLKT.

KW - Adolescent

KW - Child

KW - Child, Preschool

KW - Female

KW - Graft Survival

KW - Humans

KW - Hypertension, Portal

KW - Infant

KW - Kidney Failure, Chronic

KW - Kidney Transplantation

KW - Liver

KW - Liver Cirrhosis

KW - Liver Transplantation

KW - Male

KW - Polycystic Kidney, Autosomal Recessive

KW - Receptors, Cell Surface

KW - Retrospective Studies

U2 - 10.1111/tri.12098

DO - 10.1111/tri.12098

M3 - SCORING: Journal article

C2 - 23582048

VL - 26

SP - 640

EP - 650

JO - TRANSPL INT

JF - TRANSPL INT

SN - 0934-0874

IS - 6

M1 - 6

ER -