Hepatology dewdrops

 PBC (M rule):
 Lethargy followed by pruritus+ Middle aged female +Jaundice (cholestatic)+
Hyperpigmentation+ xanthoma+ Positive AMA(95%, initial test)+ Dyslipidemia
 Common association: Sjogren’s syndrome, celiac disease, systemic sclerosis,
sicca syndrome
 ANA=IgM
 Genetic association: HLAD8
 Main symptom: Pruritus
 Most common symptom: lethargy
 First line: UDCA
 M2 subtype
 PSC:
 Male+25-40 years of age+lethargy, itching, RUQ pain+ Increase ALP+ P-ANCA
positive+ H/O ulcerative colitis/ IBD/Cholangiocarcinoma+ Hepatosplenomegaly.
 Biopsy: Onion skin fibrosis
 Initial/ key investigation: MRCP
 Cancer screening: Ca gall bladder, colorectal cancer.
 Autoimmune hepatitis:
 Female+ Jaundice+ secondary amenorrhea+ hepatosplenomegaly+ ANA (IgG)
80% positive+ 70% ASMA positive
 Genetic association: HLA D3,4.
 Association: Autoimmune thyroiditis, hypergammaglobunemia
 Liver biopsy (confirmatory): portal lymphoplasmacytic hepatitis, interphase
hepatitis± cirrhosis.
 IgG-4 associated cholangitis:
 Obstructive jaundice+ PSC ± hilar cholangiocarcinoma
 Liver biopsy: Lymphoplasmacytic infiltrate with IgG4-positive plasma cells
 Autoimmune cholangitis: AMA negative PBC.
 NAFLD:
 Obese type II diabetes/ metabolic syndrome+ Abnormal liver function test+
fatigue+ mild RUQ discomfort+ 50-60 years of age
 Initial test: LFT
 Steatosis: Fat infiltration>5%± mild inflammation
 NASH: ALT>AST [Steatosis+ necroinflammation(ballooning, Mallory bodies,
megamitochondria)]
 Cirrhosis: AST>ALT
 Gold standard investigation: Liver biopsy (Perisinusoidal fibrosis)
 3 lesion; Steatosis, hepatocellular injury, inflammation.
 Alcohlic liver disease:
 H/O alcohol for 10/15 years+ features of cirrhosis (Palmar erythema, tender
hepatomegaly, Dupuytren’s contracture, Gynaecomastia, florid telangectasia)+
investigation=Macrocytosis.
 AFLD:
 No hepatomegaly
 AST>ALT
 MCV more increased
 GGT increased
 ALP-Normal
 Good prognosis
 Alcoholic hepatitis:
 Jaundice+ tender hepatomegaly
 (Balloning degeneration of hepatocyte, neutrophil infiltration,Mallory’s hyaline,
pericellular fibrosis)
 DF score (PT+ Bilirubin)>32=poor prognosis
 Mildly elevated ALT, AST/ALT>1.5-2
 Most important collaterals=Esophagus, stomach
 Most important consequence of pulmonary hypertension=Variceal bleeding
 Most common cause of portal HTN=Schistosomiasis
 Most useful investigation of portal HTN=Endoscopy
 Alcoholic cirrhosis=Ascites+ Variceal hemorrhage+ features of cirrhosis (Here
increase risk of HCC)
 Most significant bedside examination of hepatic encephalopathy=Flapping tremor
 Degree of liver damage is assessed by= S. bilirubin.
 Synthetic function of liver best assessed by=Albumin & PT
(Albumin is for chronic liver injury, PT is for acute liver injury+ prognosis of acute and
chronic liver failure)
 Increase AST condition:
 Alcoholic hepatitis
 Drug induced hepatitis
 Liver cirrhosis
 Wilson’s disease (Increase more AST then ALT)
 Increase ALT condition:
 Fatty liver
 Viral hepatitis
 NASH
 Hepatorenal syndrome:

Type I Progressive oliguria+ Rapid rise of S. creatinine+ Poor prognosis+

usually no proteinuria+ Urinary Na<10 mmol/24 hour , Urine/ plasma
osmolarity ratio> 1.5+ Hemodialysis not improve the outcome.
Treatment: Albumin infusion/ IV terlipressin.
Type II Patient with refractory ascites, moderate and stable elevation of S.
creatinine. Better prognosis.
 Hepatopulmonary syndrome:
 Clubbing+ Cynanosis+ Spider naevi+ decrease arterial oxygen saturation+
Orthodeoxia.
 Treatment: Liver transplantation
 Hemochromatosis:
 Age> 40 years+Male+Pituitary disfunction+ Arthralgia/ arthritis+ DM+
hepatomegaly (cirrhosis) with normal LFTs+ Cardiomyopathy+ Increase S.
ferritin and transferrin saturation
 Mutation in HFE gene [C282Y (chromosome 6) and H63D]
 Liver biopsy-Perl’s stain, iron in hepatocyte.
 Initial investigation=Transferrin saturation
 Confirmation=Liver biopsy
 Family member screening=HFE gene
 General member screening=Transferrin saturation> ferritin
 First iron deposition in liver=periportal hepatocyte
 Joint X ray=Chondrocalcinosis
 Leaden Gray skin pigmentation
 Most striking feature=Pigmentation
 Screening test of iron overload (absence of inflammation)=S. ferritin
 Suggestive of secondary iron overload=Accumulation of iron in RE cell.
 Reversible with treatment=Cardiomyopathy and skin pigmentation.
 Malignancy screening=HCC
 Wilson’s disease:
 Young adult+ neuropsychiatric symptoms+ family history of liver disease
 ATP7B, Chromosome 13.
 Changes in body parts:
Liver Recurrent hepatitis
Eye Kayser-Fleischer ring (most important
single clinical clue)
kidney Fanconi’s syndrome
Blood Hemolysis
Brain Chorea, Parkinsonism
 Single best laboratory clue to diagnosis=Low serum ceruloplasmin
 Useful confirmatory test=24 hours urinary copper while giving D-penicillamine
(diagnostic)
 Drug of choice=D-penicillamine
 Alpha1- AD:
 Emphysema+ Jaundice+ Ascites+ Liver biopsy+ PAS positive, diastase-resistant
globules in periportal hepatocyte
 Mutation in Pizz gene
 Gilbert’s syndrome:
  Jaundice specially with fasting+ normal LFT+ Normal Hb/PBF+ Isolated raised
bilirubin+ Inter-current illness+ family history
 Mutation in UGT1A1
 Most common form of non hemolytic hyperbilirubinemia
 Budd Chiari syndrome:
 Sudden abdominal pain+ Tender hepatomegaly+ Ascites+H/O pulmonary
embolism/ pregnancy
 Very sensitive and initial investigation=Doppler ultrasound
 CT=Enlargement of caudate lobe of liver..
 Treatment=Thrombolysis
 Biopsy: Centrilobular congestion with fibrosis
 Gold standard for diagnosis of sphincter of Oddi dysfunction=Sphincter of Oddo
manometry.
 Liver abscess: high grade fever with chills and rigor+ RUQ pain +Jaundice
 HCC: Hepatic rub+ Raised AFP+ Abdominal bruit.
 Ampullary tumour: Obstructive jaundice+ Occult blood test positive
 Most convenient method of demonstrating obstruction to the common bile
duct=Transabdominal USG.
 Hepatitis:
HAV Children mostly affected
HBV HBV DNA most sensitive, Initial test-HBsAg, vertical transmission is
most common here.
HCV Initial test=HCV antibody, more causes cirrhosis
HDV May cause aggressive hepatitis combined with HBV
HEV High mortality in pregnant lady
 Hepatitis:
 Prevented by vaccine=BAD
 Post exposure prophylaxis=ABC
 Transmitted by saliva=ABC
 Sexual transmission=BD, ACE (uncommon)
 Feco-oral route-AE
 Pre-core mutant HBV related hepatitis: HBsAg (+ve)+ HBeAg(+ve)+ Raised HBV
DNA
 Association:
HBV Membranous GN, Polyarteritis nodusa, Lichen planus
HCV Porphyria cutanea tarda, Cryoglobulinemia,Messangio-capillary GN,
Lichen planus
 Liver anatomy:
Structural unit Hepatic lobule
Nutritional unit Portal lobule
Functional/ metabolic unit Hepatic acinus
 Liver biopsy finding:
 Alcoholic hepatitis Pericellular fibrosis
NASH Perisinusoidal fibrosis
PSC Periductal onion skin fibrosis
Budd Chiari syndrome Centrilobular fibrosis
Autoimmune hepatitis Interface hepatitis
Alpha 1 AD PAS positive, Diastase resistant globules in periportal
hepatocyte
PBC Granulomatous dysfunction in intrahepatic bile duct
 Cirrhosis of liver with hepatomegaly:
i) Alcoholic cirrhosis
ii) Wilson’s disease
iii) Hemochromatosis
iv) PBC
 Isolated elevation of GGT:
i) Alcohol
ii) NAFLD/ enzyme inducing drug
 Poor prognosis of liver cirrhosis=hyponatremia
 Indicator of impaired liver function: PT
 Poor marker of ongoing alcohol consumption-macrocytosis
 Pyogenic liver abscess:
 Most common organism: S. milleri
 Most common symptom: Abdominal pain
 Confirmatory test: Needle aspiration under USG.
 Usual cause: Biliary obstruction (Cholangitis)
 Main factor thought to be of pathophysiology of CLD=Splanchnic vasodilation
 One of the most important clinical tools for assessment of hepatocyte function=PT
or INR
 First line USG=Gallstone, Biliary obstruction or thrombosis in the hepatic
vasculature.
 Cardinal feature of portal hypertension: Splenomegaly
 Portal HTN:
 Primary or secondary prevention: Beta-blocker (if not tolerated then
prophylactic banding)
 Best suited to the treatment of esophageal varices=Banding
 Cardinal feature of acute liver failure: Hepatic encephalopathy and/ or cerebral
edema
 Best screening test for acute hepatitis B infection=Hepatitis B core IgM antibody
 Poor indicator of the degree of liver fibrosis in hepatitis C=S. transaminase level
 Most common benign liver tumour=Hemangioma
 Most common antibiotic to cause abnormal LFTs=Co-amoxiclav
 Best known drug of hepatocellular necrosis=paracetamol
 Granuloma in liver injury=Allopurinol
 Microvesicular hepatic fat deposition=Tetracycline, sodium valproate
 Macrovesicular hepatic fat deposition=Tamoxifen, amiodarone