ACUTE LIVER FAILURE

JM JM male 10yrs referred from Kanyama health

centre for further management of a patient with gen body swelliing and abd distension

Presented with Headache 3/52 Yellow discoloration of eyes 3/52 Fever 2/52 Diarrhea (yellow stool) 3/7 and vomiting 5/7 ago Abd pains+ Appetite +- Swelling of feet 2/7 NO H/O TRAVEL

Review of Systems

GUT – frequencyo, dysuriao

CNS – seizureso, personality changeso

Resp – cougho, difficulties breathingo

CVS – palpitations Skin – no h/o itchy skin

PMHx and Drug Hx

Past hx unremarkable – DMo, asthmao, TBo, RVDo, epilepsyo

Treated with tricholine citrate syrup and ampicillin capsules

No known drug allergies

Family and Social Hx

1st born in a family of 3. No h/o paed death.

No FHx of DMo, asthmao, TBo, RVDo, epilepsyo

Child is in grade 3 Mom is a domestic worker and dad

is a bus-driver.

Physical exam

Generally ill-looking, with a puffy face Severely jaundiced, pale, not cyanosed Afebrile Not in Resp distress Vitals: temp: 36.4 HR: 80bpm. RR: 20/min RBS 3.9mmol/l Chest: clear CVS: S1S2 normal P/A: distended, soft, non-tender, fluid-

thrill positive. Lo So

Urinalysis – bilirubin +++, glucose neg, SG 1020, pH 6.0

U/S from local clinic suggested cholecystitis, fatty liver and ascites

RDT – (for malaria) negative

IMPRESSION: Typhoid fever r/o SCD r/o EPTB

Plan

ADMIT FBC/DC, solubility test, LFTs, lipid

profile, group & save, HBsAg Repeat U/S scan CXRay Urine M/C/S Stool M/C/S Antibiotics- xpen, ciprobid Mebendazole, multivit, FA

Day 2 - 6

Fevers +++ Stools not pale but quite bulky Xmatched and received BT on day 4 Ciprobid continued

Day 7

UTH u/s done: incompletely done Patient noted to be in shock in the

afternoon. Responded to normal saline and dextrose

??hypoglycaemia….Noted absence of glucostix on the ward

Day 8

Noted conjugated hyperbilibunaemia and rise in hepatic enzymes > 5 times

∆∆ r/o malignancy: lymphoma or hepatoma

Suspected hepatic failure and encephalopathy

Ordered: 10% dextrose infusion, low protein diet, oral gentamycin, peripheral smear, clotting profile, α-feto protein, repeat LFTs, CT scan abd, surgical input after CT.

Day 10 – consultant’s notes Noted poor improvement in clinical

condition Patient responding to name No hepatic flap Heard a grade 4 murmur loudest

ULSB with lod P2 Impression: Hepatitis with CHD

with ?SABE Ordered repeat U/s, f/up HBsAg,

HCV, LFTs. To do ECG and Echo

Day 9 - 10

On day 9 Patient sent back from CT because was not prepared. Condition noted to be bad too – restless, irritable and not able to communicate verbally

Day 10: Patient started bleeding early hours of the morning

Certified dead at 07:55hrs

Lab results15/12/2014

18/12/201

23/12/2014

27/12/2014

Hb 12.7 10.6 9.8Plt 152 85 101WCC 6.30 9.40 6.80

HBsAg NRBS No MPS

seen

Urea 2.3Creatinine

25

Bilirubin (D)

281

Bilirubin (T)

382 95

Enzymes AST/ALT

658/502 396/253

Albumin 30 25

ACUTE LIVER FAILURE

DEFINITION

Acute liver failure is defined as "the rapid development of hepatocellular dysfunction, specifically coagulopathy and mental status changes (encephalopathy) in a patient without known prior liver disease".

ALF indicates that the liver has sustained severe damage (loss of function of 80-90% of liver cells).

One scheme defines "acute hepatic failure" as the development of encephalopathy within 26 weeks of the onset of any hepatic symptoms.

This is sub-divided into: “fulminant hepatic failure", which requires

onset of encephalopathy within 8 weeks "subfulminant", which describes onset of

encephalopathy after 8 weeks but before 26 weeks.

Another scheme defines "hyperacute" as onset within 7 days, "acute" as onset between 7 and 28 days, "subacute" as onset between 28 days

and 24 weeks

Functions of the liver

Is the largest gland in the body Produces bile that enters the

duodenum via bile duct Synthesize Proteins and clotting factors Synthesizes cholesterol Regulates blood glucose level in body Metabolic function- glycogenesis,

glycogenolysis, gluconeogenesis)

Deamination of amino acids and converts ammonia to urea

Detoxifying chemical agents and poisons

Conjugates bilirubin Immunological function

AETIOLOGY

parac

etamol

drugs

hep b

hep a

autoi

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ischa

emia

wilsons

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ancy

other

unkn

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050

100150200250300 262

7647

22 35 29 15 8 4 22

90

USA registry N=610

Clinical features Recent viral hepatitis or recent drug/toxic

ingestion Lethargy, nausea, vomiting, fever, abd pains,

anorexia Jaundice Hepatic encephalopathy (minor beh, motor

problems then confused, slurred speech then deep coma

Episodes of bleeding Cardiac arrhythmias and hypotension Rapidly decreasing hepatic size is ominous

PATHOGENESIS

Impaired hepatocyte regeneration, altered parenchymal perfusion, endotoxemia, and decresed hepatic reticuloendothelial function

Hepatocyte necrosis is the common pathway with effects on hepatic synthetic function, excretory and detoxifying functions

Hepatic encepalopathy – multifactorial…. Ammonia theory Synergism theory False neurotransmitter theory GABA (gamma-amino butyric acid )

neurotransmission theory

Labs….

Elevation of both conjugated and unconjugated bilirubin

Aminotransferases raised Indices of hepatic function are

altered (PT>50 secs or INR>4 have been assoc with poor prognosis)

Thrombocytopaenia

Treatment

Mainly supportive Coagulopathy: vit k, platelets, ffp, blood

and H2-receptor blockers Prophylax against bacterial and fungal

infections Electrolyte imbalance Dextrose reducing ammonia load (reduce protein

load, sterilize the gut) Reducing increased ICP

Treating hepatic coma

ICU care Endotracheal intubation and mech

ventilation Electrolytes and glucose by IV

Liver transplant

Indications Acute liver disease Chronic liver disease Primary biliary cirrhosis Autoimmune hepatitis Alcoholic liver disease Primary metabolic conditions (wilsons,

haemochromatosis, a antitrypsin deficiency

Contraindications to liver transplant… Active sepsis Malignancies spread beyond the liver Patient not psychologically ready Metabolic condition

Relative contraindications Age>65yrs Anatomical considerations Hepatocellular carcinoma:

PROGNOSIS Without liver transplant, mortality is greater than 80%, but

with transplant some series reporting a survival rate of approximately 60%.

The risk of mortality increases with complications, which include cerebral edema, renal failure, adult respiratory distress syndrome (ARDS), coagulopathy, and infection.

The etiologic factor and the development of complications are the main determinants of outcome in acute liver failure.

ALF caused by acetaminophen has a better prognosis

Patients with stage 3 or 4 encephalopathy have a poor prognosis.