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Pediatr Nephrol. 2016 Sep;31(9):1517-29. doi: 10.1007/s00467-016-3324-6. Epub 2016 Apr 8.

Pediatric combined liver-kidney transplantation: a single-center experience of 18 cases.

Author information

1
Centre de Référence des Maladies Rénales Rares Néphrogones, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, Bron, France. remi.duclauxloras@gmail.com.
2
Université Claude-Bernard Lyon 1, Lyon, France. remi.duclauxloras@gmail.com.
3
Hépatologie Gastro-Entérologie et Nutrition Pédiatrique, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, 59 boulevard Pinel, 69677, Bron cedex, France. remi.duclauxloras@gmail.com.
4
Centre de Référence des Maladies Rénales Rares Néphrogones, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, Bron, France.
5
Université Claude-Bernard Lyon 1, Lyon, France.
6
Information Médicale Evaluation Recherche, Hospices Civils de Lyon, Lyon, France.
7
Hépatologie Gastro-Entérologie et Nutrition Pédiatrique, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, 59 boulevard Pinel, 69677, Bron cedex, France.
8
Chirurgie Pédiatrique, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, Bron, France.
9
Réanimation Pédiatrique, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, Bron, France.
10
Anatomie Pathologique, Groupement Hospitalier Est, Hospices Civils de Lyon, Bron, France.
11
Chirurgie Urologique et chirurgie de la transplantation, Hôpital Edouard-Herriot, Hospices Civils de Lyon, Lyon, France.

Abstract

BACKGROUND:

Experience in combined liver-kidney transplantation (CLKT) in children is limited.

METHODS:

We conducted a retrospective study of all pediatric CLKTs performed at our medical institution between 1992 and 2013.

RESULTS:

We identified 18 pediatric patients (9 girls) who had undergone CLKT at our institution during the study period. The median age [range] and body weight [range] of this patient group was 3.6 [1.0-18.6] years and 13 [10-40] kg, respectively; 11 patients weighed <15 kg at the time of CLKT. Indications for CLKT were primary hyperoxaluria (PH1; n = 14), association of hepatic fibrosis and end-stage renal disease (n = 3) and methylmalonic acidemia (n = 1). In the early postoperative period, eight patients required dialysis. Median stay in the pediatric intensive care unit was 10 [6-29] days. One patient died from cardiovascular disease 10 years after CLKT. There were no liver graft losses despite six acute liver rejection episodes, whereas four kidney grafts were lost. At last follow-up (6 [0.5-21] years) for patients with a functioning renal graft, the glomerular filtration rate was 71 [26-146] mL/min/1.73 m(2). In PH1 patients, urine oxalate normalized in six patients within 3 years after CLKT, but three patients still presented with elevated oxaluria at 1, 2 and 3 years after CLKT.

CONCLUSIONS:

Pediatric CLKT provides encouraging results in the long term, even in the youngest patients.

KEYWORDS:

Boichis syndrome; Child; Combined liver–kidney transplantation; Methylmalonic acidemia; Primary hyperoxaluria type 1

PMID:
27060059
DOI:
10.1007/s00467-016-3324-6
[Indexed for MEDLINE]

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