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Transplantation procedures in children with primary hyperoxaluria type 1: outcome and longitudinal growth.

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Transplantation procedures in children with primary hyperoxaluria type 1: outcome and longitudinal growth. / Brinkert, Florian; Ganschow, Rainer; Helmke, Knut; Harps, Egmond; Fischer, Lutz; Nashan, Björn; Hoppe, Bernd; Kulke, Stephanie; Müller-Wiefel, Dirk E.; Kemper, Markus J.

In: TRANSPLANTATION, Vol. 87, No. 9, 9, 2009, p. 1415-1421.

Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review

Harvard

Brinkert, F, Ganschow, R, Helmke, K, Harps, E, Fischer, L, Nashan, B, Hoppe, B, Kulke, S, Müller-Wiefel, DE & Kemper, MJ 2009, 'Transplantation procedures in children with primary hyperoxaluria type 1: outcome and longitudinal growth.', TRANSPLANTATION, vol. 87, no. 9, 9, pp. 1415-1421. <http://www.ncbi.nlm.nih.gov/pubmed/19424045?dopt=Citation>

APA

Brinkert, F., Ganschow, R., Helmke, K., Harps, E., Fischer, L., Nashan, B., Hoppe, B., Kulke, S., Müller-Wiefel, D. E., & Kemper, M. J. (2009). Transplantation procedures in children with primary hyperoxaluria type 1: outcome and longitudinal growth. TRANSPLANTATION, 87(9), 1415-1421. [9]. http://www.ncbi.nlm.nih.gov/pubmed/19424045?dopt=Citation

Vancouver

Brinkert F, Ganschow R, Helmke K, Harps E, Fischer L, Nashan B et al. Transplantation procedures in children with primary hyperoxaluria type 1: outcome and longitudinal growth. TRANSPLANTATION. 2009;87(9):1415-1421. 9.

Bibtex

@article{9f4886831532423f876bde50f80ac2db,
title = "Transplantation procedures in children with primary hyperoxaluria type 1: outcome and longitudinal growth.",
abstract = "BACKGROUND: Cure of the metabolic defect in primary hyperoxaluria type 1 (PH1) is possible with liver transplantation (LTx). Preemptive LTx (PLTx) was promoted to prevent chronic kidney disease due to nephrocalcinosis and urolithiasis. However, timing of this procedure is difficult in view of the heterogeneity of PH1 and effective conservative treatment. Combined liver-kidney transplantation (LKTx) is able to cure metabolic defect and replace renal function at the same time and is effective and indicated for patients with or approaching end-stage renal disease (ESRD). Sometimes a sequential approach for LKTx (first liver, then kidney) has been recommended. METHODS: We report on 13 patients with PH1 since 1995 who underwent transplantation procedures in our center for PH1 at a median age of 4.7 (range 1.4-8.9) years. RESULTS: The first two patients, planned for a sequential strategy, died early after LTx because of infectious complications. Four patients underwent PLTx at a median glomerular filtration rate of 65 (range 27-98) mL/min/1.73 m/day (Hoppe et al., Pediatr Nephrol 1996; 10: 488), and three patients still have sufficient residual renal function after a follow-up of median 11.6 years. Seven patients with ESRD received a combined LKTx, including four with infantile oxalosis, and three weighing less than 10 kg. There was no mortality and catch-up growth was observed in most patients. CONCLUSION: In summary and conclusion, transplantation procedures are challenging in PH1, but our results including growth data are encouraging. PLTx remains an option despite the difficulties in timing the procedure. LKTx is indicated for patients with ESRD and is possible even in patients with infantile oxalosis and may improve longitudinal growth.",
author = "Florian Brinkert and Rainer Ganschow and Knut Helmke and Egmond Harps and Lutz Fischer and Bj{\"o}rn Nashan and Bernd Hoppe and Stephanie Kulke and M{\"u}ller-Wiefel, {Dirk E.} and Kemper, {Markus J.}",
year = "2009",
language = "Deutsch",
volume = "87",
pages = "1415--1421",
journal = "TRANSPLANTATION",
issn = "0041-1337",
publisher = "Lippincott Williams and Wilkins",
number = "9",

}

RIS

TY - JOUR

T1 - Transplantation procedures in children with primary hyperoxaluria type 1: outcome and longitudinal growth.

AU - Brinkert, Florian

AU - Ganschow, Rainer

AU - Helmke, Knut

AU - Harps, Egmond

AU - Fischer, Lutz

AU - Nashan, Björn

AU - Hoppe, Bernd

AU - Kulke, Stephanie

AU - Müller-Wiefel, Dirk E.

AU - Kemper, Markus J.

PY - 2009

Y1 - 2009

N2 - BACKGROUND: Cure of the metabolic defect in primary hyperoxaluria type 1 (PH1) is possible with liver transplantation (LTx). Preemptive LTx (PLTx) was promoted to prevent chronic kidney disease due to nephrocalcinosis and urolithiasis. However, timing of this procedure is difficult in view of the heterogeneity of PH1 and effective conservative treatment. Combined liver-kidney transplantation (LKTx) is able to cure metabolic defect and replace renal function at the same time and is effective and indicated for patients with or approaching end-stage renal disease (ESRD). Sometimes a sequential approach for LKTx (first liver, then kidney) has been recommended. METHODS: We report on 13 patients with PH1 since 1995 who underwent transplantation procedures in our center for PH1 at a median age of 4.7 (range 1.4-8.9) years. RESULTS: The first two patients, planned for a sequential strategy, died early after LTx because of infectious complications. Four patients underwent PLTx at a median glomerular filtration rate of 65 (range 27-98) mL/min/1.73 m/day (Hoppe et al., Pediatr Nephrol 1996; 10: 488), and three patients still have sufficient residual renal function after a follow-up of median 11.6 years. Seven patients with ESRD received a combined LKTx, including four with infantile oxalosis, and three weighing less than 10 kg. There was no mortality and catch-up growth was observed in most patients. CONCLUSION: In summary and conclusion, transplantation procedures are challenging in PH1, but our results including growth data are encouraging. PLTx remains an option despite the difficulties in timing the procedure. LKTx is indicated for patients with ESRD and is possible even in patients with infantile oxalosis and may improve longitudinal growth.

AB - BACKGROUND: Cure of the metabolic defect in primary hyperoxaluria type 1 (PH1) is possible with liver transplantation (LTx). Preemptive LTx (PLTx) was promoted to prevent chronic kidney disease due to nephrocalcinosis and urolithiasis. However, timing of this procedure is difficult in view of the heterogeneity of PH1 and effective conservative treatment. Combined liver-kidney transplantation (LKTx) is able to cure metabolic defect and replace renal function at the same time and is effective and indicated for patients with or approaching end-stage renal disease (ESRD). Sometimes a sequential approach for LKTx (first liver, then kidney) has been recommended. METHODS: We report on 13 patients with PH1 since 1995 who underwent transplantation procedures in our center for PH1 at a median age of 4.7 (range 1.4-8.9) years. RESULTS: The first two patients, planned for a sequential strategy, died early after LTx because of infectious complications. Four patients underwent PLTx at a median glomerular filtration rate of 65 (range 27-98) mL/min/1.73 m/day (Hoppe et al., Pediatr Nephrol 1996; 10: 488), and three patients still have sufficient residual renal function after a follow-up of median 11.6 years. Seven patients with ESRD received a combined LKTx, including four with infantile oxalosis, and three weighing less than 10 kg. There was no mortality and catch-up growth was observed in most patients. CONCLUSION: In summary and conclusion, transplantation procedures are challenging in PH1, but our results including growth data are encouraging. PLTx remains an option despite the difficulties in timing the procedure. LKTx is indicated for patients with ESRD and is possible even in patients with infantile oxalosis and may improve longitudinal growth.

M3 - SCORING: Zeitschriftenaufsatz

VL - 87

SP - 1415

EP - 1421

JO - TRANSPLANTATION

JF - TRANSPLANTATION

SN - 0041-1337

IS - 9

M1 - 9

ER -