end stage liver failure

Indications for liver transplantation in children

SYMPOSIUM: HEPATOLOGY

End stage liver failureJane L Hartley

Deirdre A Kelly

AbstractAdvances in surgical techniques and immunosuppressive agents have led to

paediatric liver transplantation being a standard treatment option for both

acute and chronic liver failure with good long term outcome and quality of

life. It is important to recognise the early clinical presentation and aetiology

of hepatic disease so that accurate diagnosis and management may be initi-

atedand referral to a specialist centremade. Childrenwith liver disease should

have shared care between a regional and specialist centre with timely referral

for transplant assessment and furthermanagementwhen complications arise.

Keywords ascites; encephalopathy; liver failure; liver transplant;

nutrition; portal hypertension; pruritis; sepsis; varices

Introduction

End stage liver failure is rare in childhood. With advances in

diagnosis, treatment options, nutritional support and liver trans-

plantation the natural history of chronic liver disease in children

has changed. It is important that paediatricians are aware of the

causes of chronic liver disease so that children can be managed

with share care between a regional and specialist centre.

Liver failure is a loss of the synthetic properties of the liver

and also the development of complications of cirrhosis such as

malnutrition, bleeding oesophageal varices, ascites, encepha-

lopathy and hepatorenal syndrome. It may occur due to the

progression of chronic liver disease or secondary to acute hepa-

tocellular necrosis in acute liver failure.

The primary indications for liver transplantation vary with

age and are shown in Figure 1 with biliary atresia being the most

common indication for liver transplantation in childhood. Tables

1 and 2 provide a list of the more common causes of chronic liver

failure and acute liver failure.

Chronic liver disease presenting in infancy

Biliary atresia (BA)

BA is the most common cause of chronic liver disease worldwide

and is also the most common indication for liver transplantation

Jane L Hartley MBChB MRCPCH MMedSc is a Consultant Paediatric Hepatolo-

gist, The Liver Unit, BirminghamChildren’s Hospital NHS Trust Steelhouse

Lane,Birmingham,B46NH, and,MRCResearch TrainingFellow,University

of Birmingham, Vincent Drive, Edgbaston, Birmingham, B15 2TT, United

Kingdom.

Deirdre A Kelly BA MD FRCPI FRCP FRCPCH is a Consultant Paediatric

Hepatologist, The Liver Unit, Birmingham Children’s Hospital NHS Trust,

Steelhouse Lane, Birmingham, B4 6NH, and, Professor of Paediatric

Hepatology, University of Birmingham, Vincent Drive, Edgbaston,

Birmingham, B15 2TT, United Kingdom.

PAEDIATRICS AND CHILD HEALTH 20:1 30

in childhood accounting for 74% of children transplanted aged

less than 2 years. The pathology is unknown but infection,

autoimmunity and genetic factors have been implicated. The

clinical presentation of BA is that of a well grown baby with

prolonged conjugated jaundice and pale stools due to destruction

of the extrahepatic and intrahepatic biliary ducts. This leads to

cholestasis, fibrosis and cirrhosis. The surgical removal of the

fibrosed biliary tree and formation of a Roux-en-Y anastomosis

(Kasai portoenterostomy) is a palliative procedure, which ach-

ieves biliary drainage in 60% of infants. It is more likely to be

successful if carried out at a younger age (6e8 weeks) and in

experienced paediatric units. Medical management consists of

prevention of cholangitis with rotating low-dose oral antibiotics

(eg, three months each of amoxycillin, 25 mg/kg/day; cephalexin

12.5 mg/kg/day; and trimethoprim 2 mg/kg/day), and nutri-

tional and family support. If the Kasai is unsuccessful and the

jaundice is not cleared, or recurrent cholangitis develops, chronic

liver failure with the development of cirrhosis and portal

hypertension are inevitable.

a1-antitrypsin deficiency

This is the most common inherited disorder causing liver failure

in childhood. It usually presents in the neonatal period with

failure to thrive, jaundice, hepatomegaly and vitamin deficiency.

Although it can present in a similar way to BA it is diagnosed by

measuring the level of a1-antitrypsin (low level <0.9 g/l) and

identifying the protein phenotype PiZZ or PiZS. The liver biopsy

may show features similar to BA but in addition a1-antitrypsin

granules may be seen. 30% of babies recover fully with no

ongoing liver disease, 30% will develop compensated liver

disease requiring medical support and 40% will develop chronic

liver failure and require transplantation.

0–2 years 2–15 years

76%

2%

9%

10%

3%

47%

4%

25%

14%

10%

Cholestatic disease

Cancer

Metabolic disease

Acute hepatic failure

Cirrhosis

Figure 1 The indications for liver transplantation in children. Although the

indications differ by age group, cholestatic liver disease is the predomi-

nant indication with biliary atresia being the most common aetiology.

European liver transplant registry.

� 2009 Elsevier Ltd. All rights reserved.

Aetiology of chronic liver failure in children

Cholestatic liver disease Biliary atresia

Alagille’s syndrome

Familial intrahepatic cholestasis

Idiopathic neonatal hepatitis

Metabolic liver disease a1-Antritrypsin deficiency

Tyrosinaemia type 1

Wilson’s disease

Cystic fibrosis

Glycogen storage disease type IV

Chronic hepatitis Autoimmune þ/�sclerosing cholangitis

Post viral (hepatitis B and C)

Fibropolycystic liver disease

Primary immunodeficiency

Non-alcoholic steatohepatitis

Table 1

Aetiology of acute liver failure in children. The aetiologyof acute liver failure varies with age

Neonates Infectious Herpes viruses

Echovirus,

Adenovirus,

Hepatitis B

Metabolic Galactosaemia

Tyrosinaemia

Neonatal haemochromatosis

Mitochondrial disease

Ischaemic Congenital heart disease or cardiac

surgery

Myocarditis

Asphyxia

Hepatitis A

Older

Children

Infectious Hepatitis B

Non AeG hepatitis

Herpesviruses

Sepsis

Drugs Valproate

Isoniazid

Paracetamol

Carbamazepine

Toxins Carbon tetrachloride

Amantia phalloides

Metabolic Wilson’s disease

Hereditary fructose intolerance

Autoimmune Hepatitis

Ischaemic Congenital heart disease or surgery

Asphyxia

Budd-Chiari syndrome

Table 2



Alagille syndrome

This autosomal dominant condition is caused by mutations in

JAG1 and NOTCH2 which are important in the foetal develop-

ment of bile ducts. It is a multisystem disorder resulting in typical

facial features, cardiac defects, renal anomalies, ocular defects

and skeletal abnormalities. The expression is variable with some

only having facial features and for parents this may be the first

indication that they have also have Alagille syndrome. The

severity of the liver involvement is variable with 50% regaining

normal liver function. Indications for transplantation in Alagille

syndrome would be progression to cirrhosis however severe

vitamin deficiency, poor nutrition and pruritis resulting in a poor

quality of life are more common indications.

Familial intrahepatic cholestasis (FIC)

The FIC disorders are an abnormality in the transport of bile salts

from the hepatocyte into the bile ducts. Three different types

have been described (1,2 and 3). Types 1 and 2 are associated

with a low gamma glutamyl transpeptidase (GGT) whilst type 3

has a raised GGT. These conditions require intensive nutritional

and vitamin supplementation. Pruritis is often a problem which

can be treated medically (table) or by biliary diversion tech-

niques including nasobiliary drainage. Liver transplantation is

often needed in childhood for chronic liver failure, poor nutrition

and pruritis.

Chronic liver disease presenting in older children

Autoimmune hepatitis (AIH)

AIH is the most common liver disease seen in older children. It

may present with acute liver failure or develop slowly with

fatigue, intermittent jaundice and weight loss. Chronic liver

failure may develop. The diagnosis is made by identifying

raised auto antibodies, elevated immunoglobulins and low

complement levels. The liver histology typically shows inter-

face hepatitis and fibrosis. Immunosuppression initially with

prednisolone and azathioprine should be used. Indications for

liver transplant are a poor response or intolerable side effects to

immunosuppression, or the development of end stage liver

failure.

Cystic fibrosis (CF)

Over 20% of children with cf develop liver disease which pres-

ents with hepatomegaly and splenomegaly and is diagnosed by

elevated transaminases, fatty irregular or nodular liver on

ultrasound and steatosis and fibrosis on biopsy. Portal hyper-

tension and bleeding from oesophageal varices are a risk. Liver

transplant is needed in those with hepatic decompensation prior

to deterioration in lung function.

Wilson’s Disease (WD)

Abnormal copper metabolism results in WD. The most common

age of presentation is 6e12 years although it can present as

young as 3 years. WD may present with liver disease or in older

children and adults, with neurological involvement such as

deteriorating school performance, mood swings, slurred speech

and tremor. The hepatic manifestations are wide ranging from

acute liver failure, cirrhosis and portal hypertension, chronic

hepatitis or as an incidental finding of increased transaminases

and hepatomegaly.


Recommended doses of fat soluble vitamins

Vitamin A 5e15,000 IU/day

Vitamin D (alphacalcidol) 50 ng/kg/day

Vitamin E 50e200 mg/day

Vitamin K 2.5e5 mg/day

Table 4


In general there is a low caeruloplasmin level, a raised urinary

copper especially after a penicillamine challenge and a raised

liver copper on biopsy. Mutations may be detected in the gene

ATP7B. In children older than 10 years Kayser-Fleischer rings

may be seen around the cornea.

Copper chelation therapy treats WD with Penicillamine and

zinc being the most common used. Despite chelation therapy, in

those children presenting with acute liver failure a liver trans-

plant may be required. In asymptomatic siblings mutation

detection will enable zinc to be utilised early so preventing the

onset of hepatic or neurological symptoms.

Non-alcoholic fatty liver disease (NAFLD)

The increased prevalence of childhood obesity and the recogni-

tion of inherited disorders of insulin resistance (Alstrom

syndrome, Bardet-Biedl syndrome) have led to a rising incidence

of NAFLD being diagnosed in children. It presents in as mildly

elevated transaminases and hepatomegaly which on ultrasound

and histology shows features of fatty infiltration. If on histology

there is inflammation and fibrosis with the steatosis it is known

as non-alcoholic steatohepatitis (NASH). The natural history of

the disease is not fully elucidated but there have been reports of

cirrhosis developing in childhood.

NASH is a frequent indication for liver transplantation in

adults.

A healthy lifestyle of diet and exercise should be emphasised

as there may be an improvement with weight loss.

Complications of chronic liver failure and how to prevent them

Nutrition

Recommended medication to treat pruritis

Drug name Dosage

Cholestyramine Under 6 years 2 g (1/2 sachet) day

Over 6 years 4 g (1 sachet) day

Increase dose according to response

Give as single dose or in up to 4 divided doses

Other drugs should be given 1 hour before or

6 hours after

Poor nutrition is common in children with chronic liver disease it is

essential to provide nutritional support and prevent the develop-

ment of malnutrition. There is often fat malabsorption and a high

catabolic rate therefore providing adequate nutrition can be chal-

lenging especially when fluid restriction is a necessary intervention.

Table 3 provides the recommended nutritional intake for

a child with chronic liver disease.

Over night nasogastric feeding or continuous nasogastric

feeding is often needed to provide sufficient energy. Concen-

trated formula or modular feeds may be necessary in infants to

provide sufficient calories in small volume.

In children who are cholestatic it is essential they receive fat

soluble vitamin supplementation and recommended doses are

given in Table 4.

The recommended nutritional intake of a child withchronic liver failure

Energy intake 110e160% of recommended

allowance

Carbohydrate 15e20 g/kg/day

Fat 8 g/kg/day with 50% as

medium chain triglyceride

Protein e not encephalopathic 3e4 g/kg/day

Protein - encephalopathic 1e2 g/kg/day

Table 3


Metabolic bone disease may also occur resulting in patho-

logical fractures. Bisphosphonate infusions may be beneficial.

Pruritis

The accumulation of bile salts is a predominant clinical feature of

children with biliary hypoplasia such as in Alagilles syndrome. It

can be extremely difficult to treat often necessitating multiple

medications and a list of medications is provided in Table 5. In

addition a cool room to sleep in and ensuring the skin is mois-

turised will provide some symptomatic relief.

Portal hypertension

Increasing hepatic fibrosis with the development of portal

hypertension is inevitable in chronic liver disease. Portal

hypertension results in increasing splenic size which may cause

hypersplenism, the development of varices which may be

detected either on ultrasound scan around the splenic hilum or

oesophageal varices identified on endoscopy, and the develop-

ment of ascites. Good synthetic hepatic function and nutrition

can be maintained despite portal hypertension therefore portal

hypertension and the development of oesophageal varices if

adequately controlled are not an indication for transplantation.

Rifampicin 3e10 mg/kg once a day

Phenobarbitone 3e5 mg/kg daily

Trimeprazine 250 microgram/kg 6 monthse1 year 3e4

times/day

2.5 mg 1e2 years 3e4 times/day

5 mg 2e12 years 3e4 times/day

10 mg 12e18 years 2e3 times/day

Ursodeoxycholic

acid

5e7 mg/kg 2e3 times a day

Ondansetron 2e4 mg bd up to 12 years

4e8 mg bd 12e18 years

Naltrexone 6e20 mg/day

Table 5



Oesophageal varices

Varices are most commonly seen in the oesophagus but gastric

varices, rectal varices or varices around a stoma sites can occur.

Current practise is to only treat varices which have previously bled.

Acute variceal bleed management: The initial management is

fluid resuscitation with colloid and packed red cells. Intravenous

vitamin K will correct vitamin K dependent coagulopathy. FFP

and platelets may also be required.

H2 blocker such as ranitidine or a proton pump inhibitor such

as omeprazole should be give intravenously.

Medications to control the portal pressure and reduce

bleeding are given in Table 6.

When the child is cardiovascularly stable the child should

have an endoscopy to identify the site of bleeding and treat the

bleeding point.

If bleeding continues a Sengstaken-Blakemore tube should be

passed on intensive care with protection of the airway.

The insertion of a transhepatic portal vein stent between the

hepatic and portal veins can be inserted to reduce portal pressure

if there is continued variceal bleeding. This procedure can also be

used electively to control varices in children who have otherwise

normal synthetic function and compensated liver disease such as

in cystic fibrosis liver disease.

Ascites

Extravascular fluid retention occurs due to an increased capillary

hydrostatic pressure from portal hypertension, decreased plasma

A treatment plan for acute oesophageal varicealbleeding

Drug Dosage

Ranitidine Dose: 1e3 mg/kg/dose tds IV (maximum

50 mgs IV tds)

Octreotide Dose: 3e5 mg/kg/hour

Maximum dilution 25 mg/ml

Make 1250 mg octreotide in 50 mls N saline

i.e. concentration 25 mg/ml

Run at wt � 0.12 mls/hr ¼ 3 mg/kg/hr

Run at wt � 0.2 mls/hr ¼ 5 mg/kg/hr

Starting dose 3 mg/kg/hr

Glypressin 0.01 mg/kg bolus 4e6 hrly for 24e48 hours

0.05 mg/kg infusion over 6 hours for

24e48 hours

Vasopressin 0.33 units/kg over 30 minutes followed by

0.33 units/kg/hr

If bleeding continues the dose may be

progressively increased to a maximum of

1 unit/kg/hour

GTN Dose: 1e10 mgm/kg/min

Compatible with 0.9% or 5% dextrose

50 mg GTN with 50 mls of solution (1 mg/ml)

Run at wt(kg) � 0.06 mls/hr ¼ 1 mg/kg/min

Run at wt(kg) � 0.6 mls/h ¼ 10 mg/kg/min

Table 6


colloid oncotic pressure from reduced albumin synthesis and

increased sodium and water retention. Low serum sodium may

develop due to a dilutional effect and additional sodium should

not be given as this will perpetuate fluid retention.

Presentation: Ascites presents with abdominal distension and an

everted umbilicus or inguinal hernias may develop. The enlarged

abdomen causes discomfort and can lead to respiratory

compromise especially in younger children or those with already

compromised ventilation as in cystic fibrosis. Pitting oedema of

the peripheries and the formation of pleural effusions may also

occur.

Treatment: The management of ascites is summarised in Table 7

and depends on severity. If it is mild then fluid restriction and the

avoidance of excess salt may be sufficient. If treatment is

necessary the aldosterone antagonist spironolactone can be used

to create a diuresis with the addition of a frusemide if required.

To reduce ascites quickly when the serum albumin is low an

infusion of 20% Human albumin solution (20% HAS) will

provide symptomatic relief.

Hypersplenism

The increased splenic size as a result of portal hypertension

results in the sequestering of platelets and white cells resulting in

low white cell count and thrombocytopenia which may adversely

affected haemostasis in the event of variceal bleeding.

Encephalopathy

Encephalopathy can be defined as any brain dysfunction

secondary to hepatic dysfunction. It may be exacerbated by:-

Variceal bleed (which increases the gastrointestinal protein load)

Hypovolaemia

Sepsis

Use of sedatives

Electrolyte disturbance

Anaesthetic

High protein meal

Portosystemic shunting

The pathogenesis is related to the interaction of increased

nitrogenous metabolites such as ammonia with altered cerebral

neurotransmitter function.

A recommended management plan for the treatment ofascites

1 Fluid restrict

but maintain

nutritional

support and avoid

excess sodium

80% maintenance

2 Spironolactone 1e9 mg/kg per day in 2 divided doses

3 Frusemide 0.5e2 mg/kg twice daily

4 20% Human

albumin solution

10 mls/kg over 4 hours

Followed by 2 mg/kg frusemide

Table 7



All degrees of encephalopathy can be reversed by liver

transplantation.

Diagnosis: It can be difficult to diagnose in young infants and

children therefore there needs to be a high index of suspicion.

Young children may become increasingly irritable with poor

sleeping in the initial stages followed by drowsiness and leth-

argy, high pitched screaming. With further progression coma,

seizures, decerebrate posturing and loss of brainstem reflexes

develop. An EEG may show slow wave activity but is not always

a sensitive or specific test. In older children other clinical signs

which can be elicited include regression of skills, astrixis in

children older than 8 years, inattentiveness and apraxia, poor

writing and colouring skills. In late stages of encephalopathy in

older children there is often extreme agitation and anger.

Management: The mainstay of treatment is identifying and

treating the precipitating factors whilst avoiding sedatives and

reducing the nitrogen load within the intestine.

The intestinal nitrogen can be reduced by lactulose and enteral

antibiotics such as vancomycin to reducenitrogen producing bacteria.

Coagulopathy

Coagulation disorders in liver disease are common due to malab-

sorption of vitamin K, reduced synthesis of vitamin K dependent

coagulation proteins II, VII, IX and X, hypofibrinogenaemia and

thrombocytopenia secondary to hypersplenism.

Management

� All children who are cholestatic should have prophylactic

vitamin K (2.5e5 mg/day orally)

� Children with prolonged prothrombin time should have

intravenous vitamin K (2e10 mg daily)

� Fresh frozen plasma (FFP) (10 mls/kg), cryoprecipitate

(5 mls/kg) and platelet transfusions (10 mls/kg) can be

used as a temporary measure to improve clotting parameters

for procedures or emergency treatment of bleeding but

the effects are short and therefore should not be given

routinely.

� When there is a severe coagulopathy (prothrobin time >40

seconds or platelets <20 � 109) in a child that has been listed

for transplantation, FFP and platelet transfusions will reduce

the risk of haemorrhage.

� If bleeding continues following correction with blood prod-

ucts then desmopressin will increase factors VIII and IX, and

activated factor VII (80 mg/kg) can be used.

Hepatorenal syndrome

The pathogenesis of the progressive renal failure which develops

in the setting of severe liver disease is poorly understood but it is

likely to be due to abnormalities in renal cortical blood flow.

Acute renal failure may also occur due to pre renal failure due

to reduced intravascular circulating volume and improves with

volume expansion such as 20% Human albumin solution.

Liver transplant successfully reverses hepatorenal syndrome

but without transplantation there is a 90% mortality.

Hepatorenal syndrome may occur acutely with when there is

a sudden deterioration such as a gastrointestinal bleed or

a sudden deterioration in liver function.


Treatment

� Liver transplantation will reverse hepatorenal syndrome

� Regular albumin infusions to maintain a good circulating

volume

� Octreotide 3e5 mg/kg/day

� Dialysis may be necessary

Hepatopulmonary syndrome

This is a triad of:-

Liver dysfunction

Intrapulmonary arteriovenous shunts

Hypoxia - oxygen saturations less <90% in air

This may develop unrelated to the severity of liver disease.

Clubbing of the fingers is usually present. It is important to

ensure there is no underlying cardiac defect.

Diagnosis is made by

Hypoxia on transcutaneous oxygen measurements

Contrast ECHO showing contrast within the left ventricle due

to shunting

Ventilation perfusion scan will be abnormal with isotope

within the cerebral circulation secondary to shunting

Normal lung function tests

Treatment

� Supplemental oxygen will improve the hypoxaemia

� Liver transplantation successfully reverses hepatopulmonary

syndrome although recovery can be slow and there is reduced

tolerance to anaesthetic. In the post transplant period there

can be a transient worsening of hypoxaemia which may be

improved with nitric oxide

Sepsis

Children with cirrhosis are at an increased risk of bacterial

infection. Infection may also precipitate other complications such

as encephalopathy, ascites, oesophageal variceal bleeding and

hepatorenal syndrome. Both ascending cholangitis in children

with biliary atresia and spontaneous bacterial peritonitis is

children with ascites are common. The clinical features of

spontaneous bacterial peritonitis are:-

Ascites

Fever

Abdominal distension and pain with rebound tenderness on

palpation

Vomiting and diarrhoea

Bowel sounds may be reduced

The diagnosis is made on ascetic tap where the white cell count

is greater than 250/mm and the protein content is low <20 g/l.

Broad spectrum antibiotics should be instigated such as

cefotaxime (20 mg/kg/dose three times daily),

amoxicillin (25 mg/kg/dose three times daily) and

metronidazole (8 mg/kg/dose three times daily)

are usually effective. In those with recurrent infection a prophy-

lactic oral antibiotic can be used such as ciprofloxacin.

Hepatocellular carcinoma (HCC)

HCC is a recognised complication of long standing cirrhosis.

Specifically it has been identified in children with hepatitis B,

tyrosinaemia and familial intrahepatic cholestasis type 2. It may

present with an abdominal mass and a raised serum alpha feto-

protein. It is occasionally an incidental finding at transplantation.



Management of complications in acute liver failure

The complications pertaining to chronic liver failure are also

important in acute fulminant hepatic failure. Additional compli-

cations which occur in the setting of acute liver failure are:-

Hypoglycaemia

Cerebral oedema

Hypoglycaemia

Hypoglycaemia occurs due to

Failure of hepatic glucose synthesis

Hyperinsulinism

Increased glucose need

It may cause a reduction in conscious level. Blood glucose

should be maintained using intravenous infusions.

Cerebral oedema

Practice points

C Biliary atresia and a1-antitrypsin deficiency are the most

common causes of chronic liver failure in infants

C Chronic liver failure in older children is most commonly caused

by autoimmune hepatitis and cystic fibrosis

C Due to increasing prevalence of childhood obesity the inci-

dence of non-alcoholic fatty liver disease is rising and may

lead to cirrhosis in childhood

C Nutritional support and management of medical complications

improves the outcome of children with end stage liver failure

C Liver transplantation for chronic liver failure in childhood,

permits an excellent quality of life with over 90% transferring

to adult service

C The management of children with liver disease should be

a shared care approach between a regional and specialist

paediatric hepatology and liver transplant centre

Cerebral oedema is often the cause of death in acute liver failure.

Once cerebral oedema develops the prognosis is poor and

therefore attempts should be made to avoid it. It develops quickly

usually in the later stages of encephalopathy and is initially

identified by abnormal neurological signs e unequal pupils or

abnormally reacting pupils, focal seizures, clonus and decere-

brate posturing. Cranial imaging is not useful in making an early

diagnosis but with progression there are signs of increased

intracranial pressure. Brainstem coning is the final event.

The cause is unknown but contributory factors may be:-

Fluid overload from treatment of coagulopathy

Inadequate blood sugar management

Inadequate systemic blood pressure resulting in cerebral

ischaemia

Management

� Fluid restrict to 75% maintenance and maintain the circu-

lating volume using colloid

� intravenous mannitol (0.5 g/kg every 4e6 hours). Monitor

serum osmolality to ensure it does not exceed 320 mosmol/l

� elective ventilation with adequate anaesthetic to avoid

causing any rise in intracerebral pressure

� maintain cerebral perfusion pressure (cerebral perfusion

pressure ¼ mean arterial pressure e intracerebral pressure)

using inotropes such as adrenaline or noradrenaline

� controlled hypothermia may be of benefit

� seizures should be treated promptly with phenytoin (10 mg/kg

intravenously) or phenobarbitone (10e15 mg/kg intrave-

nously). If seizures continue then an infusion of thiopentone

should be used.

Liver transplantation

The development of successful paediatric liver transplantation

has changed the natural history of many conditions and liver

transplant is now a standard therapy for acute and chronic liver

failure. This has been achieved through better management of

end stage liver failure, the development of innovative surgical

techniques to expand the donor pool for smaller children and

improvements in immunosuppressive therapy.

A transplant assessment includes a review of renal and cardiac

function, imaging to assess anatomy, neurodevelopmental

assessment, dental assessment as well as nutritional support,

management of complications and counselling for the family and


age appropriate play therapy for the child. The child should receive

all routine immunisations and in those older than 6 months with

chronic liver failure the MMR should also be offered. Hepatitis A

and B should also ideally be given prior to transplant.

The one year survival for a child transplanted for chronic liver

disease is 90e95%. An excellent quality of life can be achieved

and it is expected that children will return to nursery, school and

college. Growth is improved, pubertal development achieved and

many successful pregnancies reported.

Conflict of interest statement

No conflict of interest from either author affecting the content of

this article. A

FURTHER READING

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endoscopic plus pharmacologic treatment for acute variceal bleeding:

a meta-analysis. Hepatology 2002; 35: 609e15.

European transplant registry; 2006. Available at: www.eltr.org

Kelly DA. Disease of the liver and biliary system in children. Chichester,

West Sussex, UK: Wiley-Blackwell; 2008.

McDiarmid SV, Anand R, Lindblad ASPrincipal Investigators and Institu-

tions of the Studies of Pediatric Liver Transplantation (SPLIT) Research

Group. Development of a pediatric end-stage liver disease score to

predict poor outcome in children awaiting liver transplantation.

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Palma DT, Fallon MB. The hepatopulmonary syndrome. J Hepatol 2006;

45: 617e25.

Plauth M, Cabre E, Riggio O, et al. ESPEN guidelines on enteral nutrition:

liver disease. Clin Nutr 2006; 25: 285e94.

Ramsey G. Treating coagulopathy in liver disease with plasma trans-

fusions or recombinant factor VIIa: an evidence-based review. Best

Pract Res Clin Haematol 2006; 19: 113e26.

Salerno F, Gerbes A, Gines P, Wong F, Arroyo V. Diagnosis, prevention and

treatment of hepatorenal syndrome in cirrhosis. Gut 2007; 56: 1310e8.

Vieira SM, Matte U, Kieling CO, et al. Infected and noninfected ascites in

pediatric patients. J Pediatr Gastroenterol Nutr 2005; 40: 289e94.


http://www.eltr.org

end stage liver failure

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