Pediatric liver transplantation: where do we stand? Where we are going to?

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Date: Sept. 2013
Publisher: Expert Reviews Ltd.
Document Type: Clinical report
Length: 9,953 words
Lexile Measure: 1350L

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Author(s): Denis Devictor [*] 1 , Pierre Tissieres 2 3

Keywords

acute liver failure; biliary atresia; children; cholestatic diseases; immunosuppression; liver graft; liver transplantation; metabolic diseases; neonates; outcome; pediatrics; surgery; results

Pediatric liver transplantation (LT) is one of the most successful solid organ transplants [1-3] . It has become a well-established strategy in treating children with end-stage liver disease as well as children with irreversible acute liver failure (ALF), with excellent success and limited mortality. In most centers, the 1-year actuarial survival rate is higher than 90% in elective patients and higher than 70% in children with ALF [4] . Long-term survival is also excellent, more than 80% of children will survive to become teenagers and adults with excellent health-related quality of life [5-7] . Many aspects have contributed to improved survival in children post-LT, especially advancements in pre-, peri- and post-transplant management [8] . The development of surgical techniques, such as reduction hepatectomy, split-LT and the introduction of living related LT, has extended LT to infants under the age of 1 year and weighing less than 8 kg, which has effectively reduced the waiting list mortality from 25 to 5%. Nowadays, pediatric LT are routinely performed in all developed countries across the world [9] . Reports of experience from single centers provide are certainly encouraging, and the databases of the Studies in Pediatric Liver Transplantation (SPLIT group) and of the pediatric acute liver failure study group (PALF group) are also invaluable sources demonstrating these marked improvements in outcome [5,10-12] . For instance, the data of the SPLIT group allows analysis of currently more than 4000 North-American children who have undergone LT [13-16] . Similarly, the PALF group provides considerable data to improve our understanding on the treatment and outcome of children with ALF and to identify factors to predict need for LT in children with ALF [11] . This article will review the recent advances and will consider the future in pediatric LT.

Prior to the operation

This step includes the evaluation of indications and contraindications for LT, the selection, evaluation and preparation of the candidates and their prioritization.

What are the main indications for LT?

The main indications for LT in the pediatric population can be broadly separated in four groups: cholestatic liver diseases, ALF, metabolic liver disease, and liver tumors (Tables 1 & 2) [1,17] .

Table 1.

Indications for liver transplantation in children and outcomes.

Diagnosis Frequency (%) SPLIT registry [dagger] Bicêtre

Year of study

1995-2002

1986-2002

Patients (n)

1092

568

Number of transplantations

NA

648

Cholestatic liver disease

66

77

Biliary atresia

42

53

Others

14

34

Alagille syndrome

6

Sclerosing cholangitis

3.5

Progressive familial intrahepatic cholestasis

8

Alpha-1-antitrypsin deficiency

4.5

Acute liver failure

13

11

Metabolic diseases

12

9

Others

13

3

Liver graft survival

75 [double dagger]

65 §

Patient survival

69 [double dagger]

83 §

[dagger]

Data taken from [3] .

[double dagger]

15-year outcome.

§

3-year outcome.

NA: Not available; SPLIT: Studies of Pediatric Liver Transplantation.

Table 2.

Patient characteristics of 2982 children who underwent a first liver transplantation registered in SPLIT from 1995 to 2008.

Characteristics Patients (N = 2982) n % Age at transplant

Missing

1

0

0-6 mo

260

8.7

6-12 mo

725

24.3

1-5 y

962

32.3

5-13 y

616

20.7

13+ y

418

14

Race

Missing

47

1.6

White

1668

56.3

Black

464

15.6

Hispanic

494

16.6

Other

299

10

Sex...

Source Citation

Source Citation   

Gale Document Number: GALE|A343898795