ACUTE LIVER FAILURE

1. ACUTE LIVER FAILURE

2. 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

3. Review of Systems • GUT – frequencyo, dysuriao • CNS – seizureso, personality changeso • Resp – cougho, difficulties breathingo • CVS – palpitations • Skin – no h/o itchy skin

4. PMHx and Drug Hx • Past hx unremarkable – DMo, asthmao, TBo, RVDo, epilepsyo • Treated with tricholine citrate syrup and ampicillin capsules • No known drug allergies

5. 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.

6. 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

7. 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

8. 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

9. Day 2 - 6 • Fevers +++ • Stools not pale but quite bulky • Xmatched and received BT on day 4 • Ciprobid continued

10. 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

11. 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.

12. 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

13. 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

14. Lab results

15. ACUTE LIVER FAILURE

16. 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).

17. 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.

18. 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

19. 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)

20. Deamination of amino acids and converts ammonia to urea • Detoxifying chemical agents and poisons • Conjugates bilirubin • Immunological function

21. AETIOLOGY

22. 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

23. 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

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

25. 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

26. 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

27. Treating hepatic coma • ICU care • Endotracheal intubation and mech ventilation • Electrolytes and glucose by IV

28. 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

29. 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:

31. 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.