HEPATITIS-B © Team GlobalNet www. Distia.co © Leading cause of transfusion-associated hepattisis Hepatitis B + Onelectron microscopy view: ‘© Acomplete virion ofhepattis B termed as Dane particle © HBsAg: Surfaceantigen Itiseitherspherigat or tubular in shape, © HBeAg: Core antigen Found in the nucleus of hepatocytes but never found n the blood. ~ However, antibody toitis seen inthe blood. © HBVDNAPCR DNA will be positive inthe early phase of disease even before HBSAg, © HBeAg: Marker for teplication or infectivity ofthe virus Presence of HBeAg is highly infectious, © Pre-core mutant: HBeAg absent > Mote Severe: ~ Replicationnot detected routinely > Seen in Russia and Central Europe © Escape mutant: Avoid neutralization with antibody to bepattis Bsurfuce antigen. + The manifestation that oceurs in this is termed oocult infection. Genome Partially SS and partially ds circular DNA (3.2 kb): Douile- shelled virionand the usual size is 42nm, Route of Transmission ‘© Most common: Percutaneous exposure Parenteral LV. drug abusers. © Blood transfusion + Fortransfusion, Blood istested for seh th 8 Dass ied Bole Ge aad fied Bue“ f lag ich, © HBV (HBsAgis Austtalian antigen) © HCV(EIAantitiCV antibody, PCR HCV RNA) © HIV (ELISA HIV, PCR HIV for earlier identification of disease especially inthe window period) Syphilis (FTA- ABS), VDRL for Neurosyphilis. and Nertical transmission (© Malaria; HRP-2 dipstick test + On Peripheral smear, banana-shaped gametocytes, and maltese cross appearance MALTESE Coss: BAPE A MickoTE ©@O00 » Differential diagnosisis babesia mierot ~ Ixodes scapularis isthe vector responsible frit > Management: atovaquone and azithral mother ta baby © Casescenario: Commercial sex worker pregnancy and her test report as: HBsAg and HBeAg, positive (© Mother has « 90% chance of transmitting to baby during, delivery baby orin pregnancy. (© Management: HB Ig LM. to be given ASAP within <12 hours To the child) + Hepatitis B vaccine 3 doses(0, 6,10, or 4 wks.) + Accidental needle stick injury ‘© Occurs mostly whilereeapping theneed. (© Chanceof transmission is 30% Srdtrimester of 389 Telegram : @teamglobalchat‘© Conjunctival inoculation; Due to splatering of blood on the face while handling trauma patient- the risk of infection present (© Management: HBIgILM.ASAP(within6 hours) ‘© Most common cause of chronic hepatitis or chronic liver isease or end-stage liver disease or orthoptc liver transplant isthohepaiisC virus. Serum markers vo HBsAg © Isttoappear: I-12 weeks o infection Earliest serological evidence of hepatitis-B infection © Precedes SGPT rise by 2-6 wk. ‘Sequence of appearance: HBsAg positive followed by ‘SGPT raised followedby jaundice. © Disappearinthereverse direction ‘© MBeAg: Never appears inthe blood. ‘© HBeAg: Replication or highly infectious ‘© HBxAg: CD 95 inhibitor, it inhibits the extrinsic pathway of apoptosis © Triggers development of Hepatocellular carcinoma (HBV >HCV) PCR HBV DNA: Quaniily the viral load E& important Information + Cut off viral load for starting treatment in chronic hepatitis B: Patient is diagnosed with a case of ehronic Hepatitis B and HBe Ag: reactive and viral loadis>2 x 10° 1Uiml with mised SGPT 2x. ‘+ Management: Tenofovir Drug of choice) or Entéeavir © Pegylated interferon +Lamivudine (Inearlertimes) Antibodies Against HepB ‘ Anli-HBsAg: Protectiveantibody Ani HBeAg © Antibody against the HBeAg. (© Initially it an IgM type of antibody later it converts into 1G. © IgM: Diagnosis of Acute viral hepatitis and Antibody present in Gup Period or Window period. © IgG: Chronic hepatitis © Anti-HBeAg: Replication rate reduces, and Infectivity reduces. (©. Jaundice will appear atlastandis the rs to disappeat, ‘© Gap period: it is the period between when the HBsAg disappears and anti-HBsAg appears © Only the IgM type of antibody against the HBeAg is resent inthis period + SGPT startsrisingbefore icterus appears + Jaundice will disappear bul SGPT wil take lime to get normal onset © Team GlobalNet www. Distia.co ‘Biochemical recovery will take time, but clinical recovery willoccurearly + HBsAg — anti HBeAg —+ SGPT elevated —+ Jaundice (but firstio disappear) orarts ‘© Above diagram shows chronic hepatitis B which leads to citthosisand ultimately develops End-Stage Liver Disease. ‘+ In chronic hepatitis B the HBs Ag remains detectible for long ime period, fo Insome cases, it shows a decline ina couple of months oF eats. ‘1g type of antibody against the core antigen persists for 2 longtime period EX Important Information ‘+ Hep B usually recovers (90%) even without any ‘medications: due to seroconversion and medication is ‘only given in ehronie phase not given in patients of Acute HepB, Case Scenario ‘+ 22-year male comes with complaints of nausea, change in olfaction and taste, Multiple episodes of vomiting, aversion lo smoking, Low-grade fever present with right upper ‘quadrant discomfort forthe past 1 week, ‘Onexamination: 1. Teterus present (usually when Serum Bilirubin > 2-3 mg/dL. Sclera (High cone of Elastin that binds with bilirubin) is yellow 2, Tenderhepatomegaly 390 Telegram : @teamglobalchat Q +Liver span increased (normals 12-15 em) (© Ifthe liver regress in next few weeks then it means it progression into Fulminant Hepatic failure. Nopedall edema Noascites Nocaput-medusae Probable diagnosis: Acute Viral Hepatitis Work-Upof Patient Let 1, Imhepatocellular jaundice © Senambilirubin f © SGOTt © SGPT 11 mostspecitic © Serumalkaline phosphatase normal Inobstructive jaundice © Serumbilirubin ¢ (conjugated) © SGOT normal © SGPT normal © Serumalkaline phosphatase increased 4 times Inhemolytic jaundice © Serumbilirubin t (unconjugated) © SGOT normal © SGPT normal (© Serum alkaline phosphatase is normal and Splenomezaly ccanalsobescen 'SGt to check echo texture and span ofliver| ‘OnUSG, Starry Sky Liverappearance'seen. Free fluid absent, Liver span tf seen in Viral hepatitis - Simplified diagnostic algorithm for finding etiology of acute virathepattis Non-A, Non-B, Non-C infection means itis either due to ep-D or E. As in India Hep Eis more common due o feeo- oral contamination Acute Hepatitis B Acute Hepatitis A Acute Hepatitis C Non-A. Non-B Non-c © Team GlobalNet www. Distia.co Treatment Acute Hepatitis B + 90% casesrecoverspontancously © Bedrest ‘+ Management: Itoprideor Mosapride for Nausea + LV. fluids (10% dextrose or NS) fordehydration. + Monitoring Pissszfaosibsfaniiicfiicnsy omes9 1M) +) Atte HepB Highly infestious + - eG + Chron Hep B - + Ge, Recovery : = WM =) Gap period E zs z = Vaccinated - = WG? Lowlevel carier! Remote infection E& important Information © HBGAg@ #1gM anti HBe: Acute Hepatitis ‘© HbsAg@ + IgGanli HBc: Chronic Hepatitis ‘¢ lgM antiliBcg preseat: Gap period ‘© AntiHbsAg> 101U/ml: Vaccinated IgG Anti Hbe:Low level carrier Treatment Chronic Hep B © HBeAg: Reactivity © PCRHBV DNAshows>2x 10‘1UDNAVml © SGPT shows doubling (© Management: Tenofovir 300 mgO.D. for 48 weeks. © Monitor KFT while Tenofoviris recommended. + Compensated cirrhosis patients. © HBeAg: Reactivity © PCRHCV DNAshows2x 10°1U DNA/ml © SGPT raised shows doubling © Drug of choice: Tenofovir 300 mg OD for 48 weeks or Entecavir Hepatitis A # ‘Transmission by the fecal-oral route ‘© Chances of fulminant hepatic filureis 0.1% # Nocarrers. + Noprogression tochronicity 391 Telegram : @teamglobalchat‘© Noprogression to carcinoma, ‘+ More symptomatic inaduls © IgManti-HAV is resent Vaccines available: inactivated vaccine presen, © Given? shots to children, Hepatitis E ‘© Most commoneause of Acute viral hepatitis © Chancesof fulminant hepatic failureis.0.1 1% ‘© Leading cause of fulminant hepatic failure in preunancy (up t030%), ‘+ Incidence of Fulminant hepatic failure associated with ~ 20 “HV. + Vaccine: Available EB important Information © Fulminant hepatic failure encountered with all Hepatotropic viruses. * Chronic hepatis isnot associated with hepatitis A, ‘© Novaccine isavailableagainst Hepatitis C. * Complete clinical and biochemical recovery isexpected in alleases of HAV, HEV in I-2 months of post-jaundice, + Prescribe high-carbohycrate and low-fat diets in all of the patients Physical findings to be evaluated in al 1. Teterusseenin sclera and frenulum, 2. Spider angiomas are dilated cutaneotis arterioles. Found in both acute and chronic liver disease, Palmar erythema TTenderhepatomegaly, Ascites: Shifting dullness andlor Fluid till, Encephalopathy; earliest feature is an alteration in sleep pattern, (© Trail aking’Number Connection Testis done 7. Caputmedusae, widened pulse pressure, 8. Hyperpigmentation in cholestatic disorders 9. Slate gray Skin in haemochromatosis, xanthoma, xanthelsina, KFring. liver diseases © Team GlobalNet www. Distia.co Extra Mile: © Triggers of Hepatic encephalopathy + Gllbleed: Blood s good eulture media which helps the bacteriato multiply > Diuretic excess: oss of water from the body will raise theammonia levels, > Loss of potassium + Dehydration > Infection Approach toderanged LFT Hepatocellular jaundice: Predominantly rise in ALT * ALTelevated. Alkaline phosphatase ¢levaied. + Testsdoneare; © IeMAni HAV © HBsAg, IgM Anti Hbe © AntiHicv 0” ANA (anti-nuelearantibody),SMA '>Ceruloplasmin, aleohol history, drughistory. Obstructive jaundice: + Alkaline phosphatase inereased by 4X, + GGTisclevated, * ALTiselevated, © ‘Tests done: 0 AMA: for primary biliary cirhos © Druginiake © USG: I investigation tobe done. © MRCP. 392 Telegram : @teamglobalchatHEPATITIS © Team GlobalNet www. Distia.co HEPATITIS-C-D AND FULMINANT Q a Hepatitis C ‘© Single-stranded RNA virus ‘© Leading cause of End-stage liver disease (ESLD) ‘© Liver Transplant (OLT)-MC cause for liver transplant inthe wworldisHCV-induced cirthosis, ‘© Most commoneause of Transmission is Parenteral © IWdrug abusers (IVDU)>B.T (Blood transfusion) Clinical Features wo9sen ‘© Extrahepatic manifestations like Arthralgia, Myalgia, Paresthesa',Prurtus and sicea syndrome-ke manifestations ‘© Other manifestations like MPON, Lichen planus, porphyria, ccutenea tarda and mixed Cryoglobulinemia canbeseen Cirrhosis © Hand sign: Palmar erythema, Duputyren's contracture leukonychia, clubbing asterixis © Ascites, pedal edema, caput medusa, oesophageal varices Workup vote © E.LA (enzyme immunoassay) anti-HCV antibody positive; can be false positive in patients with autoimmune hepatitis ‘+ Investigation of choice is PCR HCV RNA: "no cut ofP* for ‘management of patient, ‘+ LET: SGPT may be normal ot increased. All patients with chronic HCV infection, detectable HCV RNA with or without elevated ALT, atany stage of fibrosisnecds treatment | ‘Treatmentis based on Genotype Harrison 21” edition update: Treatment Naive or relapsed after prior PEG-IFN-Ribavarin Therapy Genotype 1-6: Sofosbuvir~ Velpalasvir 12 weeks Glecaprevir + Pibrentasvir 8 weeks| ‘© Objectiveistoobtaina sustained virological response>90% + Post-exposure prophylaxis: notavailable. ‘© Chances of getting infected by HCV after needle stick injury 153%, © DoPCR HCV RNA test if results are positive in within 3 ‘months: then teat as the ease of HCV positive. Hepatitis D-Delta ‘© Transmission is through Parenteral ‘+ Hepatitis Disanincomplete virus ‘© For Eradication: 100% coverage ofthe population with HBV vaccine should be done Example © LY. drug abuser, Jaundice with HBsAg +, also, IgM anti HBeAg #-in Feb 2021, He had to shift his workplace due to police patrolling. While taking IV drugs, he got delta virusin Mar2021.1gM anti-HDV Ag+ Co-Infection © IgMantiliBeAg+IgManiDVAg © Fulminant liver failure Example «IV drugabuser with HBsAg +. also, IgG anti HBeAg in Feb 2021. He had to shift hisplace due to police patrolling. He got Depatitis Din Mar 2021. IgM anti HDV Ag + Superinfeetion © IgGantiHBcAg+eMantiDVAg + Chronic HepB + Acute HepD. Chronic hepatitis Drugof Choice ona Hep D ‘anterferon Hep B Entecavir or Tenofovir Hep SotosbuvirVelpatasvir Muliple selerosis| Bimterferon Important Information ‘Most common eause of acute viral hepatitis is Hepatitis -E (P2574: Harrison 21" edition) ‘Most common cause of fulminant hepatic failure (FHE) is Hepatitis -D (20%) ‘Most common cause of FHE in pregnancy is HEY Fulminant Hepatic Failure nase Cause ‘© Toxins> Viralhepattis, Hepatitis, Hepatitis E(pregnancy) © Toxinsare: © PCMtoxici © Halothane o ATT: Pyrizinamide ‘©. Amanita (Mushroom) poisoning # Cutoff: <8 weeks ofliver insult, developmentof ‘© Coagulopathy and/or > Encephalopathy Sub fulminant cut off 8-26 weeks Features of Fulminant Hepatic Failure ons 1. Jaundice: worsens 393 Telegram : @teamglobalchatBleeding will occur because all clotting faetors are produced in liver except Factor VIII: endothelial cell, Factor IV: caleium- Epistaxis 3. Ammonia intoxication/Hepatic Encephalopathy ‘+ Theearliest manifestation of encephalopathy: A. Alteration ofsleep:awake eyele B. Euphoria, slurring of C. Construetional apraxia: Number connection test and Clockeface test yt @ - Conte 5 AA tombe fonnachi Teot Avot. NRA Anomic aphasia dificult in namingan objet. E, Most Reliable sign: Asterixis (Flapping Tremors) F. Stupor: ComaGCS<& ‘Asterixis or Flapping Tremors Extra Mile: temorseean be seen in Anxiety neurosis, © Grave's disease © Pheochromocytoma ite ofammonia (NH,) synthesis is Gt flora + Hepaticencephalopathy ean be triggered by Gl bleeding. © Internal GI bleeding can worsen hepatic encephalopathy, Thus, stabilizing coagulogram is ‘importa Work-up of FHF 1. LET © SGOTisraised, © SGPTisraised © Team GlobalNet www. Distia.co © Serum Bilirubinisraised (© Serumalkaline phosphatase is normal | BT (2-9 min) Platelets: PT (11-16 see) Extrinsic 5,7 t t © Factor 7 has the shortest 1 deranged, 3. Investigation of choice in fulminant hepatic failure is Blood animonia levels which are raised, © On EEG, Triphasic waves and-delta waves (Metabolic encephalopathy) and PT is the first to get ‘Delta wave in EEG: * Discharge frequency of delta wavesin EEGis0.S~4Hz 4. USG: LiverSpan decreases 5. KPT: Serum Creatinine levelsareraised. MELD Score: Used to registera patient for liver Transplantation, ‘We can use thi score prognosis and referral ofthe patient ‘+ The parameters include are: © Bilirubin © INRorPT © Serumereatinine ‘© If the MELD seore is more than 17 is an indication of an orthoptic live transplant Extra Mile ‘+ MELD-Na: Incorporates serum sodium used to Stratify ‘transplant candidates for organ allocation. Chitd-Pugh Score ‘© Theparameterineludes are: © Bilirubin o INR © Albumin © Ascites © Asterixis ‘+ Class A (score 5-6), class B (svore 7-9), class C: 210 score. Class B is an accepted level criterion for listing a patient for liver transplantation, ‘Management 1. Drugof choice: lactulose (Ammonia binder) 394 Telegram : @teamglobalchatE> Important Information HLA matching is not necessary in © Itgives through the Nasogastric tube © Mauses diarrhea thus, ut bacterial loads reduced. © More the gut is clear, the lesser will be ammonia production. Rifaximin (bacteriostatic antibiotic) and Neomycin © Givetlrough theNG tube. © Topreventthe multiplication of bacteria in the gut. LO.LA(L-omithine-L-aspartate) © It binds to ammonia resulting in the production of ‘glutamine. Ths glutamineisexcretedby the kidney. MARS: Molecular absorbent re-irculating system (liver dialysis) Extracorporeal hepatic support system. Care of bowel, back and bladder for a comatose patient, To prevent the bed sore inthe patient . Orthotopic liver transplant is done on the base of the MELD score ofthe patient. © HLAmatchingisnot mandatory Cornea transplant Cardiac transplant Liver transplant © Team GlobalNet www. Distia.co Discriminant function or score: Used for alcoholics hepatitis severity © Serumbilirubin © INR ‘IF the discriminant function seore >32, it is indication for ‘weatment, + Management: Prednisoloneor Methyl Prednisolone 938? 1. NAZER index Lliver Transplant in Wilson disease 2. MELD. Liver Transplant in Fulminant Hepatic Failure 3. Discriminant Alcoholic hepatitis forseverty of disease function 4, Child Pugh ForLiver Transplantation 395 Telegram : @teamglobalchat Q +45 Hepatorenal Syndrome © Itisacomplication of Decompensated circhosis. © Seeninl0% cases of advanced cirhosis + Overall poor prognosis and requires Orthoptic liver transplant ‘© Itis Characterized by oliguria followed by anuria in a patient ‘with liver failure which is primarily because of toxins and due to an imbalance between vasoconstriction and vasodilatation mechanisms the body 40% of cithosis patents haverefractory Ascites LHPE: On light Mieroscopy, glomerulus is normal ‘These patients would develop Pre-renal Acute kidney injury. Prostaglandins are important for the maintenance of the slomerular filtration rate. (© PGE, and PGI, these are vasodilatory prostaglandins. ‘© They will help increase the enal blood flow and increase ‘the glomerular filtration rate to maintain perfusion, (© In hepatorenal syndrome, Vasodilatory prostaglandins are lostin urine, (© This causes predominant renal vasoconstriction and the blood supply ofthe kidneys ishampered. ‘That is why it leads toPre-renal Acute Kidney injury. + Thedamaged cirrhotic liver will be producing endothelin- (©. This will upregulate the endothelial nitric oxide synthesis ‘enzyme in the splanchnic blood vessels, ‘© There will be more nitric oxide synthesis, wich will ‘cause splanchnic vasodilation, (© Shunting of blood away fromm the kidney cause less perfusion of Glomerulis, ‘© Onthoptic Liver Transplant (OLT) Leads to restoration of [kidney function. Revised Criteria fordiagnosis of HRS. + Refractory Ascites © Ascites persisting patients even after optimal ‘management of ascites are vulnerable to developing. hhepatorenal syndrome. ‘© Substantial ascites contributes: ~> Tenseascites > Respiratory difficulty > Hepatic hydrothorax. + Serum creatinine (© The values ofserum creatinine are progressively raised, © Thevaluesmorethan 1.Smg% (© Muscle mass in the patient is reduced. Rise in 0.3 me% ‘over the baseline valuesin hours (© Urine output is less than 0.5 ml/kg/hour over 6 hours defines kidney malfunetion © Team GlobalNet www. Distia.co HEPATORENAL SYNDROME AND HEPATOPULMONARY SYNDROME Q & + Stopdiureties (© Diuretics contsibute to dehydration which leads to kidney ‘malfunction. © Stop the diuretic for thenext2 days, + Volume expansion © UseFoley’seatheter and central line, © Do not give 1.5 liters of normal saline sometimes, it contributes to heat failure 6 Itissafertodo volume expansion with albumin, © Dose: Igike ‘©. Maximum dose tha canbe uselis ~100 grams. © Albumin contributes ton increase in oncotic pressure andreduces the amountof ascites inthe patient © Ascitesis third spaceloss. fo If we get this fluid back in the vascular compartment, theres the'chance that kidney perfusion will increase and. {urine output will also increase. © It we use foley’sors condom catheter in the patient it will help usto check heise‘ the urinary ouspat in the patient (© Central line in the patient, it will help to check the central ‘enous pressure. * Noshock ‘© Dovital monitoring toruleoutthis. + Nonephrotoxiedrug, © Ifthe patients aking any nephrotoxic drug then has to be ‘immediately stopped, + Noparenchymal disease © Itcanbe led out by doing USG. ‘© Objective evidence: no parenchymal disease as indicated by proteinuria> S00mgiday. ‘© Demonstrate tha there is no evidence of microhematuria| atleast> 50 RBC/HPE ‘© Noabnormality detected in Renal ulirasound. (Clinical Scenario + Chronic liver disease patient presents with be refractory ascites. He had 2-3 episodes of hematemesis. On examination, vitals are deranged withanuria Workup: © Urinary sodiumiselevated. (0 In Pre-renal variety of AKI the urinary sodium is ess. © In acute tubular necrosis, salt is not absorbed, so urinary sodiumishigh ‘Fraction of sodium (FeNa)> 1% 396 Telegram : @teamglobalchatDiagnosis ‘Volume depletion contributing to Acute Tubular necrosis, ‘This is not HRS which causes pre-Renal AKI and FeNa is ety ‘Types of HRS ‘Type-1 HRS ‘© Itisalsoknownas HRS acute kidney injury. «Its fast progressive in nature and develops over 1-2 weeks ‘Type-21RS *# Itisalso known as HRS Non-acute kidney injury ‘© Thisishavinga relatively better prognosis than Type 1 ‘© Thediseaseis slowly progressive, over 3-6months. ‘Treatment 1. Albumin with octreotide or midodsine, 2. Octreotide or midodrine: They cause vasoconstriction ‘mainly actingon the splanchnicarteries © Hresulisin deereasein venous flow. ‘© Manifestations will reduce due to reduction in portal hypertension ‘© It neutralizes the vasodilation occurring i 3. Subsequently this patent will require liver transplant splanchnic Hepatopulmonary Syndrome ‘Triad of HPS 1, Intrapulmonary shuoting 2, Platypneaor Orthodeoxia 3. Cirthosis = * Intra oeon ny Shonting © Thedamaged cirrhotic liver will be producing Endothelin-1 © This will upregulate the endothelial nitric oxide synthesis ‘enzyme which is present in the splanchnic blood vessels ‘and pulmonary bed ‘© There will be more nitric oxide synthesis, which will ‘cause dilation of pulmonary vessels © Ifthe blood vessel diameter ges increase. A Jot of RBCS farther away from the basement membrane might be passing through blood vessels without getting oxygenated a © Team GlobalNet www. Distia.co © So,there would be hypoxia fo Itistermedintrapulmonary shunting. ‘* Platypnea or Orthodeoxia: Duc to gravity, there is dilation of pulmonary blood vessels. © Ifthe patient is in sitting position, the Weight of dilated blood vessels will mainly affect the basal part of the lung When this patent lies down fat, the effet of gravity will, ‘become relatively equally distributed over the hing When thepatientsits up theSpO, will fallby>5% Fallin pO,> 4mm Hgon iting. Pa0.<80mmhg ‘Acagradientisraised, Extra Causes of Platypnea 1. Atrial myxoma 2. Atrial septal defect 3. Emphysema 4, ARDS Work up, 1. Investigation of Choice: Bubble contrast Echocardiography 16 Risk oir embolismisabsent © Pushing-up agitated saline into the heart Simultaneously, do echo-cardiography. ‘6: The bubble in agitated saline in size of approximately 25 © The diameter of pulmonary vessels in normal individuals are $-8microns © IF there is gross dilation in the pulmonary vessels. The agitated bubbles ean pass through it and seen into left atria © Itshows that there i intrapulmonary shunting, 2. Lungscan © With Te, labelled particles © This having particular size which cannot cross the pulmonary vesselsin normal individuals, Management ‘Supplemental oxygenis given ‘© Definite treatment: Onthoptic iver transplant, Production of NO is excess Substantial renal ‘vasoconstriction because of ‘urinary loss of prostaglandins # Decrease in systemic vascular resistance, * Intrapulmonary shunting 397 Telegram : @teamglobalchat Q +AUTOIMMUNE HEPATITIS © Team GlobalNet www. Distia.co © Alsoknownas Lupoid Hepatitis or Plasma Cell Hepatitis, * Untreated autoimmune hepatitis: mortality rate of 6 months is 40% (high) Pathophysiology worst ‘© Colkmediated damage to liver parenchyma causing interface hep Causes 1. Iopathic 2. Lossof immunological tolerance toself-liver Ag 3. Itis T8cell-mediated atack on liverantigens directly. Triggers or Conditions associated with © HAVHBV,HCV ‘+ Drugs: minocycline (used in management ofaene) ‘© Autoimmune hepatitis is associated with common auloimmune disorders like: (© Hashimoto'sthyroiditis © Celiae spruce © Uleerativecolitis (© Membrano Proliferative Glomeralo Nephitis (MPGN) Type I Autoimmune Hepatitis | Type 2 Autoimmune ‘© More common with 1» Young female ‘© Ethnicity: North America. children ‘* Ab: associated with ANA. * Mediterranean or central (whichis also seen in SLE, 80 Europe i'salso known as lupoid—# ANA absent hepatitis) ‘© Anti LKMI (can (Anti SMA (actin) contribute toa false ‘eAnti-soluble liver antigen positive diagnosis of| © Atypical p-ANCA (x- Hepatitis C) ANCA) '*Aml-liver eytosol ~ 1 Ab HLA associated with HLA associated with *HLADRS *HLADRBI HLA Dri HLA DQ BS Eb important Information # xANCA: Autoimmune Hepatitis, # c-ANCA: Wegner’s Granulomatesis + p-ANCA: Microscopic polyangitis und Churg Strauss Important Antibodies association © AnfiLKMI:HCV # AntiLKM2: Drug-induced hepatitis * AntiLKM3:HDV Clinical Feature 1. Initially, the patientisasymptomatic. © OnRoutineLET: SGPTisraised, 2, Symptomatic: Non-specific symptoms © Fatigue © Anorexia © Acne ‘© Amenorrhea }- Jaundice (waxing and waningeourse) SICCA syndrome (Dry eyes and mouth) Erythema nodosum, Cirthosis (decompensated: Ascites, pedal edema, variceal bleeding) 7. Incidence of HCCisincreased Work Up 1 Ler ©. Swblirubin: might be increased, © SGPT Significantly raised. © S.Alkaline Phosphatase normelorraised. 2. Albumin | and - globulin 7 (change in albursin plobain rato) and as no catabolism of this protein oecurs therefore it keeps onelevating. © Differential diagnosis is Multimyeloma: Albumin is ‘normaland y-globulin tt Viral serology PTisinerease. . RA factorispositive. ANA @, SMA etn), AntiLKM-I Ab, Livereytosol LA. Liver Biopsy © Interfucehepattis © Rosette formation (this indicates regenerating hepatocytes) 398 Telegram : @teamglobalchatDiagnostic Criteria ‘Diagnosis of Exclusion Excluded causes © Virus © Drugs Alcohol Genetic ds {a-1 AT def, Wilbon disease, Menke's disease} Include + Antibodics profile, Biopsy findings ‘Management Steroids Azathioprine (© Steroidused is prednisolone steroid given for a long time leads to steroid wsiciy. Cushing syndrome-like manifestation will eceur © Team GlobalNet www. Distia.co Q © Steroids will be given at lower dosages. To prevent side effets ‘Late representation: Orthopti Liver Transplantation In ease of decompensated cirshosis, © On basis of child Pugh seore: Class B Listed for liver transplantation, © Disease ean re-occur theincidence i are McQ Q. Which disease has propensity to reoccur in a transplanted liver? (Recent NET) Ans. Autoimmune Hepatitis 399 Telegram : @teamglobalchat[-q7_]| CIRRHOSIS AND ITS COMPLICATIONS 447 Introduction © Cinthosisisreversible ‘©. Hepatitis C isthe leading cause of the requirement of an orthoptic liver transplant, as it pregresses into chronic hepatitis ‘©. Hemochromatosis © Alcohol liverdisease ‘Scores determining Mortality oa MELD Score (Model for End-Stage Liver Disease) ‘© Parameters are: © Serumbilirubin o INR © Serumereatinine Lowerlimitis6 and the higher limit is 4, Higher the score enlists for an orthoptic liver transplant. United network for ongan sharing MELD Seore > 17: enlist fran orthoptic liver transplant. Ifa Liver transplant has to be done, it should be planned before the value of 20, because afer this steep ise in| morality ate oecurs {Noh Mealy 8 MELD-Na © Parameters are: © Bilirubin fo INR © Creatinine © Serumsodium © Need forkidney dialysis. © Team GlobalNet www. Distia.co No Yes Creatinine Nonm:62-115 mol. Bilirubin Norm: 5.13 +3249 mol/L INR ‘Norm: 0.8 - 1.2 Sodium Norm: 136-145 mmol. PELDscore © Parameters are: © Bilirubin © INR © Albumin © Age © Growth failure Child-Pugh score © Parameters are: © Bilirubin © INR Albumin Ascites © Asterixis Category (© A:5-6 (Compensated cirthosis) © B:7-9(Decompensated cirrhosis) © C0 + Category B list for liver transplantation Encephalopathy None Grate 1-2 Grade 3-4 (orprecipitant (or chronic) indued) Ascites None Mild to moderate Severe rete refractory) Bilirubin <2 23 23 (gal) Albumin (gidL) >35 2835 <28 INR <17 1723 >23 CChild-Turcotte-Pugh Class obtained by adding score for each parameter (total points) Class A = 5 to 6 pois (least severe liver disease) 400 Telegram : @teamglobalchat Q +is will occur the manifestation of encephalopathy andior Coagulopathy will develop. ‘+ Ifthere san internal Gi bleed it contributes to more bacterial growth, Thebacteria proliferate to produce moreammonia.in the intestine. t will precipitate hepatic encephalopathy. Invasivetest wosear Liver Biopsy © Mustalways bedone USG guided © Coagulogram should always be normalized before liver biopsy, as bleeding chances will bhighin cirmhotic patients. ‘© Preferred site:midaxillary line 7*or8* intercostal space. ‘Liver biopsy is the most accurate to assess the severity of the chronic liver disease ‘* Gradingis done by using the METAVIR or ISHAK score. FO © No fibrosis can be detected FL © Fibrosis exists with the expansion of portal zones F2 —_« Fibrosis exists with the expansion of most portal zones, and occasional bridging F3_« Fibrosis exists with the expansion of most portal zones, marked bridging, and ‘ccasional modules. FS « Presence of Cirrhosis 0 NoFibrosi ‘ibrosis expansion of some portal areas, short fibrosis septa 2 Fibrosis expansion of most portal area, short fibrosis septa 3. Fibrosis expansion of most portal areas with an ‘occasional portal to portal (P-P) bridging, 4 Fibrosis expansion of portal areas with marked bridging (P-P) as well 3s portal-central (P-C) 5 Marked bridging (P-P and/or P-C) with occasional nodules (incomplete cirrhosis) 6 Cirrhosis, probable or definite © Team GlobalNet www. Distia.co » + Transient clastography (Fibroscan): Measure the speed of shear wave whichis generated by vibration. + Fibrotst: © Bilirubin © Gamma-GGT (© Haptoglobin > Apolipoprotein A (© @Macroglobulin, ‘+ Serum electrophoresis: © ee 1 pst altonin b Legos ae ‘In Nephrotic syndrome: Albumin decreases, y Globulin decreases Dnermee aterm! ayer Y 401 Telegram : @teamglobalchatTeam GlobalNet W www. Distia.co + + Inchronic liverdisease: Albumindecressesand'yGlobulinis 2. Hepatitis BorCorD clevated 3. Autoimmune hepatitis ©. Because catabolism of globulin occurs inthe liver and if 4. Primary biliary cholangitis / Secondary theliveris damaged it willnot occur primary sclerosing cholangitis © Primary biliary cholangitis that can deteriorate into primary biliary cirhosis. © Antimitechondrial antibody was seen in Primary biliary cirshosis. © P-ANCA antibody is seen in Primary sclerosing cholangitis 5. NASH © Obesity contributes tonon-aleoholi steatohepatitis 6. Wilson orhemochromatosis ©. For Wilson disease: Done specialized test 24-hour urinary copper. + Inmultple myeloma: There is spike inthe y Globulin gives For hemochromatosis! cheek out the percentage characteristic church spire of M spike appearance iary cholangitis / saturation of transferin along with ferritin values. = © Which can be associated with both emphysema and 1 1 NERSaAyTINCTE tronehicctsis 9 The production of alpha | antitrypsin is normal but 1 1 intosis ‘excretionishampered, 6. Mwillaccumlatein he hepatocyessoitis PAS-positive Normal 1 Mutiple inclusion presenti hepatocytes imyeloma/plasmacytoma There isa special phenotype ofthis that scaled double ZZ, phenotype which has a higher incidence for the development of cirhosis. 8, Cardiaccirthosis © Thisisrae, © COPDpatients could behaving cardiac cirrhosis. © Readin pathology as nutmeg iver. 9. Cryplogeniccirthosis/ non-cirrhotic portal fibrosis (NCPE). ‘© Line diagram for cardiae cirthosis: There is patient with COPD who issulTering from pulmonary artery hypertension & Important Information + Most common cause of cintosis is Alcoholic Liver disease. ‘Most common ease of Orthoptic Liver transplant is Hepatitis. Causes of cirrhosis conus 1. Alcoholic iver disease © Aleohol once goes into the body then it will be ‘metabolized through eytosoliealcohol dehydrogenase > This is going to produce acetaldehyde which will be causing reactive oxygen species to contribute to demagetolivercal pai te + Kupfecel reas activated proding Cytokines Sel Those Cykies iach fo hte tte yy cals ht ease Hbrosis aad causing mic Nodular cinhoss, +n ihe inal part of alcoholic cirhass, ice tncrenodaar cdl + It theresa complete absence of lho his canbe reveredbacktononna -+ Thedeiiion of mcronodlar sie wouldbe es han Smilies 402 + Telegram : @teamglobalchat +© Starting from Cor Pulmonale then there was pulmonary artery hypertension, then there was a right ventricular decompensation that was oceurring in the patient and leadsto hepatomegaly. © The stellate cells cause fibrogenesis that will result in shrinkage ofthe liver. + Liverspan will egress. —> In pathology, term nutmeg liver and in medicine, the {erm cardie cethosis, Manifestation of compensated cierhosis ose 1. Spider Naevi or Spider angioma: Fine dilated cutaneous arterioles © Site: Over the shoulders and over the neck ofthe patients 0 they would be present on superior vena cava distribution ‘© Oestrogen isresponsibe frit. > Itis nota pathognomonic finding that may also be seen in pregnancy and rheumatoidarthrts. ‘©. you press on the center you see there isa central filling of arteriole © ifyou press on twill blanch, but when you leit go, it will ‘tart filling from inside to outside, 2, Palmar erythema: Areas of redness and in some areas blanching present alternatively inthe palms. 3. Duputyren's contracture: Can begin in the ring finger and then can involvethelltlefingeras well 4. Clubbing: There is release of platelet-derived growth factor causing proliferation of tissues atthe base ofthe nail leading | tobulbousappearanceof the Nail © Clubbing is also seen in inflammatory bowel disease. 5. Parotid enlargement (© There is an interstromal fatty infiltration present in alcoholic cirehosis, 6, Testicular atrophy: Gynecomastia, decreased body hair and ‘musele wasting which will be predominant proximal ‘musele wasting and temporal muscle wasting, ‘+ [the patient goes into decompensation then 509% of these patients wouldbe dead over next’ to4 years Refer Tuble 47.1 ‘+ Decompensated cirrhosis leads to development of Portal hypertension ‘© The oesophageal varies which develop initially is non- bleeding. So, we can start the patient with Propranolol oF Nadotol ‘© Once the pressure becomes substantially high usually more than 12 mm Hg these varices can burst to result in Tie threatening hematemesis and hemorrhagie shock. © Team GlobalNet www. Distia.co Q vows Portal Hypertension Exo tn Nese ‘+The Normal pressure in the poral vein i usually less than S mmHg ‘Fibrosis in the liver will increase the resistance leading to increase pressure in the portal vein © This will be transmitted to the splenic vein resulting in enlargement ofthe spleen. © Splenomegaly. is one of the carly findings of portal hypertension. ‘©. When thesize ofthe speed increases, tothe developmentofhypersplenism. The increase of the pressure in the splenic vein there is ‘tansudation of uid from the capillary bed into the peritoneal cavity there would be also the development of Ascites. 16) Puddle sign present ‘© Shifting dullness, fluid thrill present. ' Subsequentially, the pressure will increase in the superior mesenteric vein resulting inrectal hemorrhoids, + The oesophageal veins ate thin, they can rupture resulting in esophageal verties. Once itruptures cause life-threatening, hhematemesis and contributes to hemodynamic compromise cecurringinthe patent. ‘Nitric oxide is liberated from a damaged live cirrhotic liver ultimately causes vasodilation so there would be more blood flow pressure inthis entite circuit and the higher blood flow also contributes 0 an increase in hydrostatic pressure resulting inasstes Definition of portal hypertension is (© Hepatie venous pressure gradient (HVPG) of more than $ mmiig ‘© Esophageal varices when the pressure should usually be inexcessof 12 mmHg. -analso contribute Investigation of choice 1. OnUSG, the Liver span isreduced. (©. Freefluidin the peritoneal cavity 2. On the Doppler scan (investigation of choice for diagnosis) Used to measure the pressure gradient. 403 Telegram : @teamglobalchatExtra mile Porto-systemic collateral sites are 1. Esophageal veins 2, Rectal emorthoids:so fresh blood in he stool canbe present 3. Bareareaoftheliver 4, Capat medusae: This would be dilated veins present around theumbilicus a - ne) y gic Thanbare a AR, We Tromboss © Dilated veins which ate present only above the ‘umbilicus: dueto superior vena cava thrombosis. © Dilated veins which are present only below the umbilicus: dueto infer 5. Omental veins 6. Retroperitoneal veins 7. Lumbar vein vena cava thrombosis. & Important Information * Overall, the Most common cause of Hematemesis is peptic ulcer disease, + The common cause of Hematemesis with Splenomegaly isPortal hypertension Causes of Portal Hypertension ‘© Portal vein Pre-sinusoidal © Budd Chiari thrombosis © Schistosomiasis Syndrome «Splenic vein + Sinusoidal * Inferior vena ttrombesis 6 Cintsis cava webs ‘+ Banti syndrome + Post sinusoidal. © Restitive (onassive © Veno occlusive cardio splenomegaly) disease snyopathy fo Miopathicand Radiation, ® Constrctive ‘more common Herbal tea pericarditis in Japan (compliance willredueed) © Team GlobalNet www. Distia.co & Important Information Bantu syndrom ‘© FoundinAfrica * There is iton overload, Due to the consumption of local beer whichis storedin iron utensils Management For non-bleeding oesophageal varices: + Endoscopic variceal ligation or + Drugs: Nadolol or Propranolol For bleeding esophageal varices with having haemodynamic compromise: + Massive blood transfusion: Meanwhile, you can secure grey cannula access preferably putting 2 wide bore cannulas in a patient © Iisthe inital step afmaagement © Give LY, fluid which will be normal saline in decompensated shock. Drugs: LY, octreotide Upper Gl endoscopy Endoscopic variceal ligation Sclerotherapy: Sclerosing agent is Ethanolamine oleate Patient might even be having a Portal Gastropathy that is cevenif you do ligation ofall the esophageal varices, since the blood vessels ofthe stomach will also be dilated so the blood Imightbe coming fromthestomach. © So, in some cases if there is recurrent bleeding inspite of| EVL itismainly due to portal gasropathy. For recurrent variceal bleeding patients: ‘In esophagus, afer endoscopic variceal ligation there is a possibility that the new veins are developed which are arossly dilated and rupture. So, we can repeat endoscopic variceal ligation, + IFbleeding is controlled: (Child Pugh staging in the patient has tobe done) (© In Class A (compensated cirthosis): Plan a peritoneal ‘Venous shunt to decrease theportal vein pressure If the patient is not willing for it then the TIPS procedurecanbedone. © In Class B and C (decompensated cirthosis): Do ‘Transplant evaluation. Ifthe child Pugh score is more than 7 then enlist the patient for orthoptic liver transplantation + TIPS is recommended in this particular case, *# Inboth eases, Orthoptic liver transplantation isa required. Ascites ost + Amountof peritoneal fluids present in normal manismil Amount of peritoneal fluid is present in normal female isi 404 Telegram : @teamglobalchatManagement 1 © Inmideycleovulatory female: 10~20ml ‘Normal Pleural Guidis$-15 ml, © Minimalpleuralfluidefusion — Investigation ofchoice is CT chest > USG. ‘Normal Pericardial Nuids 20-50 ml ©. Pericardial efusion —> Investigation of choice is echocardiography. > 100 mlofperitoneal uid: Puddle signs detectable. > $00 mlof peritoneal fluid: Shifting dullness is detectable. Fluid thrill or fluid wave sign: There isa high chance of false positiveresults. Superficial veins of the anterior abdominal wall become ‘more prominent and gradually it becomes tortuous. ‘There is everted umbilicus which is due to an increase in ascites uid Subsequently, there is the development of Respiratory distress inthe patient, when it deteriorates into Tense Ascites Some of the fluid passes through fenestrations into the pleural cavity leading to respiratory distress results in patie hydeothorax in the patient. Minimal fluid to be present in the peritoneal fluid to be detected clinically is~ 1500 ml If we don't intervene it deteriorates into abdominal ‘compartment syndrome (pressure in the peritoneal cavity is somuch that tompressesthe veins). ‘©. Abdominal compartment syndrome is grade higher than intra-abdominal hypertension. © Ithepressuredueto ascites luid is more than 25 mm Hg. oui Salt-restrcted diet Diuretics: Spironolactone + Furosemide © Maximum amount Dosage of spironolactone given is 400 mg, © Maximum amount of Dosage of furosemide given is 160 mg, Refractory Ascites: after medical treatment there, ascites is ‘occurring again. © Spironolactone causes painful gynecomastia as a side effect. So, Amiloride instead of spironolactone is used, ‘© Midodtrine: itis vasoconstrictor, => Meagonist, > Itwill cause constriction ofthe splanchnic artery. Flow ‘othe splenic veinis also reduced, ~ Reduced portal hypertension. ~ Transudation occurring in the capillary bed around the spleenis also reduced. ~> Developmentofaseitesisalso less © Clonidine: also,a vasoconstrictor. > Iter agonist © Ifinspite of maximum dose ofthese drugs, Large volume paracentesis is recommended. © Team GlobalNet www. Distia.co Q Either, large-volume paracentesis or TIPS has to be performed. + Large volume paracentesis: Draining the fluid from the body aggravates the dehydration, © Riseofammonia concentration in the body. ‘© Substantial rise in ammonia can trigger asterexis, © IValbuminis given with LVP toneutraize. = 1Valbumin raisesthe oncotie pressure. twill eause the mobilization of Muid back nto the intravascular space + TIPS is superior o prevent the reaccumulation of fluid inthe liver + Side etfectis hepatic encephalopathy. + Mortality reduction with LVP withalbuminis equal to TIPS, +B blockers are contraindicated in refractory ascites as they will increase mortality, Notice that f blockers were DOC for Nonbleeding Ovarices ‘Serum albumin ascites gradient (SAAG) 12936 Refer Flow Chart 47,1 ‘Albumin present in the ascitic uid is subtracted fiom the Serumalbumin. Management + SerialVPwithalbumin + Petitoneo venous shuating can be done, Blackascitiefluid + Causes ‘© Pancreaticnecrosis due to hemorrhagic pancreatitis, > May be secondary to Pepticuleer disease, > SAG valueisessthan 1.1 gi Amylase inascitie Muidis aise. © Malignant melanoma racentesis, + Position: The head end is slighily elevated of the patient so ‘that fluid tendsto move down into lanks so itis easy to ta. (© Preferably Done from the left side because there is a higher incidence of gutperforation on right side. 405 Telegram : @teamglobalchat‘© Angle: Needle introduced at 45°in Z. tracking to minimize the leakage subsequent to tap. ‘© Anatomical localization: 2 em below the umbilicus in the ‘midine (Line alba) orin left lower flank as shown in diagram © Thisareaisavascular, soitissafe, © Inferior epigastric artery is present lateral to the rectus abdominis muscle, © So,Bither do the procedure medial or lateral it © Another site is4em supero medial to the anterior supetior iliac spine to avoid trauma tothe inferior epigastic artery. + Collected fluid sent for the microscopic examination, cytology, gram slain, and culture is done, ‘© Sugarand protein valuchas tobe checked. + InSecondary peritonitis, sugar values re very low and LDH values are raised, ‘© Dark brown: Seen in biliary tract perforation. ‘© Coagulopathy isarelative contraindication, ‘+ Always perform Paracentesis USG guided, to avoid medico legal issues Spontaneous abdominal bacterial peritonitis (SBP) o:sias ‘© Case scenario: Patient having refractory ascites presents with breathing difficulty. On examination patients is grossly ‘malnourished with leoholic liver disease, ‘SBP be prevented by performing Paracentess in a patient within 12 hours of admission, ‘+ Bacteria (coli) present in the gut exhibit transmigration ot translocation o lymph nodes then disseminate o peritoneal uid This leadsto peritonitis, (Clinical features 1. Fever 2. Nausea or vomiting 3. Worsening ofascites/Incteasein girthof thesbdomen. 4. Encephalopathy Diagnostic ascitic tap © Fluids Turbid in nature, ‘© More than250 PMN cells/cubie mm. + Dogramstainandeulture, Management + [Vcefotaxime ‘© Toprevent the patient from developing SBP: Norfloxacin is recommended. Systemicinflammatory response syndrome (SIRS) © Presents ith © Fever © TLCRaised © Increased respiratory rate (© Increased pulse rate. © Team GlobalNet www. Distia.co Q + For diagnosis of SIRS: 2 out of the above 4 have to be present. + Ifnot intervened daring thisit will lead to sepsis. ‘Sepsis: SIRS with positiveblood culture report Septie shock. + Iwill decrease the perfusion of the kidney leading to acute tubularneerosis, ‘© Sepsis criteria will be satisfied with hypotension Jess than 90/60 mm Figinspite ofusing vasopressor. + Vasopressorof choice in septic shock is Norepinephrine. Extra mile Summary onstas ‘= Bleeding esophageal varices: Endoscopic variceal ligation, © Nadolol or propranolol ‘= Refractory ascites: LVP with albumin o TIPS ‘© Spontaneous bacterial peritonitis: cefotaxime (© Primary prevention: Norfloxacin, ‘© Hepato renal syndrome: Albumin with octreotide or smidodeine, ‘© Hepato= pulmonary syndrome: oxygen supplementation. + Bleeding or coagulopathy: Fresh frozen plasma. ‘Encephalopathy: lactulose © Rifaximin © Neomycin Malnutrition Osteoporosis Hematological: macrocyticansemia ZIEVE syndrome: severealcoholichepatts, © Acanthocytes and spur cells preseat. Orthopticliver transplant (OLT) + HLAmatching is not mandatory. + UWsolution coldischemia time~20 hours. © Mealis~12hours. (© Components: lactobionate > Raffinose. + Donoradult:Right lobe * Donoradult forthe child: Left lateral lobe, + Theremightbe postoperativebiliary complications, 406 Telegram : @teamglobalchat© Team GlobalNet www. Distia.co ‘Table 47.1 Stage Stage 1 Stage? Stage 3 Clinical No Varices Varces + Ascites Bleeding +/- No Ascites No Ascites Varices + Ascites + Death (att year) % % 20% 51% Flow Chart 47.1 rs J > LigiaL, <1 wil Ascitic fla protein <2.5gidL >25yaL B-BNP.T Cimthosis (MC) + CCHE © BeBe leak BCS (ate) + B-BCS (Eauly) ‘+ N-Népluotc Syndrome Liver mets = Eve 1+ P-Penioneal Carcinomalosis obstruction Causes are # Sinusoidal Mesouhetioma/Sarcoma obstriction © CaOvary © CaStomach © Ca Breast © CaLung ++ P-Pencreattis © TB Tubercles 407 Telegram : @teamglobalchatlas 1] + 48 PBC AND PSC © Team GlobalNet www. Distia.co Primary Biliary Cholangitis, so0s8 ‘© Autoimmune disorder: due to Anti Mitochondrial Antibody (ama) Its primarily an intrahepatic lesion, ‘© Damagetocholangiocytes > Initial manifestations features of cholestasis © There is development of granuloma formation and ductsopenia > Lasteitthosis occurs. > Micronodular (Most common) > Mactonodulae EE important Information ‘+ Antimicrosomal antibody is responsible in Hashimoto thyroiditis Staging ‘Stages Portal stage of Ludwig + Stage Periportaldamage Stage Il Septal damage + StageIV-Cirthosis Clinical features ceurin 40-60 yearsge group. © Most common in female igue: Most commonandearliest manifestation, 2. Pruritus ollowedby Lichenification ofskin ‘©. When there is liver damage, there is the production ofthe endogenous opioid peptides responsible for pris, Jaundice and hepatomegaly Palmar Erythema Spidernacvi ‘Temporal andl proximal muscle wasting, Ascites, caputmedusae Collaterals present ‘Xanthoma or xanthelasma (over eyelids): LDL is elevated in these patents. Receptors of LDL uptake are hampered, © Initially, LDLand HDL valuesare elevated © Ina laterstage, LDL values ae elevated and HDL value reduced. 10,Steatorthea, deficieney of fit-soluble vitamins, 11.Osteopenia: Due to vitamin D deficiency. 12SICCA syndrome manifestations present. (Dry eyes, dry mouth) 13.Kayser- Fleischer Ring: arare manifestation ofthe disease © Bilirubin: itkeeps fluctuating over the course of time. © SGOT, SGPT isnormal or maybe slightly increased. (© Serum Alkaline phosphatase: elevated (shows up to 4) timeselevation), AMAispositive. (© Mitochondrial antigens (M,—M). ANA positive in~20~ 50% of the patients Prothrombin time iselevated INR increased. Platelet count is reduced: Hypersplenism due to portal hypertension, Deranged Lipid profile. USG: Torule outany extrahepatic cause. 9. Liver biopsy: Investigation of choice Management vorras * UDCA (Ursodeoxysholie acid): Stow the progression ofthe disease, ‘Obetocholie acid: ifthereis intolerance to UDCA. Cholestyramine: isasequestrant “Antibistaminies ‘Dronabinol: neutralizes the endogenous opioids peptide. Naltrexone Plasmapheresis: Given in case the patient deteriorates in spite of medical treatment, + Treatment of choice isan Orthoptic liver teansplant. Differential diagnosis 1. Autoimmune hepatitis 2. Primary sclerosing cholangitis 3. Sarcoidosis Primary Sclerosing Cholangitis (PSC) oats 408 Telegram : @teamglobalchat(On ERCP in patients: multiple strictures present in the extrahepatic biliary pathway give a characteristic beaded appearance. Characteristic onion skin appearance of the bile duct on | biopsy. ItIsa multifactorial disease ‘© Autoimmuneisoneof the components. ‘© Genetic predispositions play an important role in the evelopment ofthe disease. Riskof cholangiocareinoma inciddnee increased ‘©. Because of PSC association with ulcerative colitis, © Ulcerative colitis, is a premalignant condition and predisposes toColonCa, When cholestasis is present in the patient conjugated bilirubinis not get excreted. 409 © Team GlobalNet www. Distia.co Clinical features 1 2. 3 4. Workup, Management Prusitis Teterus: Waxing and weaning course values may touch normally in between. Steatorthea| Vitamin deficiency manifestation occurs: Osteopenia, LPT deranged ‘Serum Alkaline Phosphatase elevated up to4 times. GammaGTP elevated. Hypergammaglobulinemia (IgM) is elevated. PTisinereased Albumin value is reduced. PANCA\s postive in 65% of total cases. ‘50%4o total cases: are associated with Ulcerative colitis. Investigation ofchoice is MRCP > ERCP. (© OnERCP: Beaded appearance or multiplestrictures Liver biopsy: Onion skin appearance UDCA ERCP: dilation of strictures © Balloon dilatation Orthoptic-liver transplant (OLT): is the mainstay of treatment. Telegram : @teamglobalchat Q +© Team GlobalNet www. Distia.co 49 | WILSON DISEASE AND HEMOCHROMATOSIS @ In Wilson disease, absomption of copper from the gut is absolutely normal-This copper binds to a protein named metallothionine inthe liver, ‘© Fromthe liver copper in excreted and is transported to the ‘tissues bound to ceruloplasmin, IPR gee, cle ener -. In Wilson disease hepatobiliary excretion of copper is less ‘causing accumulation of eopper in Hepatocytes. Ceruloplasmin levels are reduced in Wilson disease Ceruloplasmin i also reduced in: 1, Alcoholic cirrhosis 2. Nephrotic Syndrome 3. Kwashiorkor Hence Ceruloplasmin has less uly for diagnosis Inthe Body, total Coppers S0-100 mg, Consumption of Cu 2-5 mg/day. ‘The problem in Wilson disease i defective biliary excretion of copper due toa defect inthe ATP7B gene on chromosome 13) Itisautosomal recessive in inheritance. There is P-type ATPase enzyme activity linked to this gene and this activity is defective, ‘Amount of copper in the liver increases leading to the development of cirrhosis. ® Git + Subsequently, decompensated cirrhosis and portal hypertension will beoccurring. Extra mile: ‘= ATP7A: Menkes disease (Cu deficiency) (© Case scenario: A boy with mental retardation with kinky xine Clinical Features Occurin = yearsand<50 years of age. 1. Inthe liver: chronic hepatitis manifestations develop © Ieterus © Deranged liverenzyme. © Viral markers arenegative © Copper will damage the hepatocytes resulting in cirhosis, © Initially, itis compensated later it becomes: ‘decompensated. © Child presents with history of repeated hospitalization, © Compensated cihosis > Spider naevi > Palmer erythema © Decompensated “> Portal hypertension > Splenomegaly > Refiactory aseites 2. Neuropsychiatriemanifestation © Damage to Basal genglia: Lenticularnucleus © Midbrainand Cerebellum may also et involved. © Earliest neurologic manifestation: Resting tremor, postural, kinetic. > Parkinsonism-like manifestations > Dysarthria, rigidity related tothe vocal cord as well. + Drooling of saliva > Incoordination Psychiatrie manifestations > lumpulsiveness, disinhibition, > Emotional lability: child eries without any reason. > Migraine likeheadaches (Pulsatile) > Temper tantrums 3. Hemolyticanemia ‘© Freecopper inthe blood is toxic to RBCs. ‘© Copper evelis increased Hemolytic episode. ‘© In the late phase, the serum copper is less because itis deposited inthe tssuesandexcreted ino the urine. 4, Joint: premature osteoarthritis (© Because copper gets deposited inthe synovium. 5. KF Ring: itispresent as 10- 12 years of age but not visible. 410 Telegram : @teamglobalchat Q +(© KF ring initially, develops into the upper part of the (© Upper 2 mm isnot visible to the naked eye because it is ‘coveredtby eyelids, © Usually, by 15 years of age or older the lower part ofthe ‘cornea gets involved, f© Increases in the circum tial fashion involves the © Descemet membrane gets involved. (Examined under sit amp). © Greenish brownish ring 6. KF ring with neuropsychiatric features are diagnostic accuracy of 95-98%, 17. Kidney: Urinary copper is significantly increased leading to damage to PCT and glomerulus, (© Which leads to gross hematuria, Fanconi syndrome, Renal glycosuria, and aminonciduria. 8, Otherrareclinical features seen in Wilson disease: © Azute nail: lunule is blue in color and present inthe nail bed. © In females: amenorrhea, cholelithiasis, nephrolithiasis. 9. Cardia involvement: there isa conduction defect in the form of bradyarthythmia and tachyarthyitumia ‘Radiological finding on MRI Head face of giant pands. E& important Information 3 commonmovernent disorders een in Wilson disease are 1. Tremors 2, Dystonia 3. Incoordination ‘Organsinvolved in Wilson disease Liver: Cinthosis(mostcommon) © RBC: Her © Basal ganglia; Tremors olytic anemia © Team GlobalNet www. Distia.co Q © Nails: Avurelunalae + Kidneys: RTAtype 2 fo Renal tubular acidosis type PCT damage. © Bye:KPring 2s the alternative name for EE important Information Inhemochromatosis ing the fb cells of the pancreas rest insulinopenia, So type I DM is one of the manifestations termedasbronze diabetes, + Itinvolvesskin: slate gray. © Due tooverexpression of melanin, ‘© Because iron triggers the melanoeytes, © Liver: contributes tcirhosis. 9 Higher incidence of HCCispresent # Iron dama Workup vores 24-hour Urinary copper value (screening test) 10 Elevated more than 100 meg/day. D-Penicillamine challenge test Liver Biopsy fo Itistheinvestigation ofchoice, © Useforstimation of Hepatic Copper ©, Content:>250meg/g ofthe dry weight ofthe liver 1 Stains used ar > Rhodamine > Rubeanicacid 3. Serum ceruloplasmin: <20mew/dl fo Itisuseful if there is positive family history 4. MRIHead: Faceof panda appearance © Pantsinvolvedare > Tegmentum > Parsteticuatis of substantia Nigra > Superior colliculusisalso involved. Panda Sign: Sen in gallium scan: In sarcoidosis patients Panda Sign or Raccoon eyes: Seen in Anterior Cranial fossa Slit lamp biomieroscopic examination, 6. DNAtesting for ATP 7B gene (0 Haplotypetesting in siblings. Management vows2 1, Ifthe patient is having hepatitis-compensated cihosis: Zine tate © Mode of actin: it competitively inhibits the absorption of copper from the gut, » Inducer of metallothionein 411 Telegram : @teamglobalchat> Copper is a teratogenic so give zine acetate in pregnancy. 2. In case of hepatic decompensation (it means portal hypertension already ooeurred): Trientene > D penicillamine. 3. 1FCNS manifestations present: Terathiomolybdate Liver manifestations and severity of manifestation will be reduced after medical management ‘In 2" year the KF ring will remain static but later reduced as well ‘+ Need forliver transplantation is evaluated by: © NAZERindex: © Parametersare 1, Serum bilirubin 2. INR 3. SGOT > >9: Register for orthoptc liver transplant Hemoehromatoss a ogee a coat w 59 6 vee ey Fama mm n> ‘Total amount of ion content in body is3—4 um%. + In hemochromatosis iron content is increased to 20 ~ 25 grams. + Duodenumis the main ite forthe absorption of iron, © Divalent Metal Transporter it helps In the transport of Fe" into enteroeytesin Fe’ form, © Duodenal Cyt helps inthe reduction of Feo Fe + Ferroportin: Importer of iron from enterocyte into circulation, + Ceruloplasmin converts Fe" into Fe” and Fe” binds to ‘Transfer for transport to bone marrow, «Ferritin is the storage form of ion, This wll help with RBC synthesis + Allihisis regulated by hepciin Etiology. # Aceruloplasminemia: Defi ‘Conversion into Fe” will not occur © So Fe" gets deposited in tissue and causes Neurodegeneration ey of ceruloplasmin © Team GlobalNet www. Distia.co ‘+ Subsequent damage to the liver: Leads to inability to produce ceruloplasmin and transferrin Hereditary Hemochromatosis + Thereis Hepeidindeficieney. + Unregulated irons absorbed in excess and will gt deposited inthe tissues. ‘© Etiology: Gene mutation: HFE gene present on chromosome {6p.€282Y homozygosity (most common) (© Hemojovilinmutation © Transferrinmutation © Ferroportin mutation Typeofhemochromatosis “Type | ‘Chronic liver disease © HIFE gene 6p, AR, © Alcohol coR2Y © Hepatitis C (MCC) Homozygosity © NASH (Non acholic steatohepatitis) © Cirthosis ‘Type ‘Thalassemia (transfusion © Hepciin’ related iron overload) Hemojuyelin mutation 0 Myelodysplastic © Aplastic anaemia © Sideroblastic anemia © Chronic hemolytic ‘Type IL © Transferrin Receptor ‘mutation ‘Type 1V © Ferroprotein mutation (Cinkcal features oasis ‘© Most common symptom: Arthralgia > Pigmentation ofskin ‘Most common organ involved: Liver. ‘+ Most common presentation: Hepatomegaly ge. NT eer the Mam el Cue aR tonne Wate Potente He vee Crane S\N este oe deta 4 Goge Sma yoy dalvtin 412 Telegram : @teamglobalchat Q +Pituitary damage: hypogonadotropic hypogonadism (LH and FSH decreased) fo Testosterone and estrogen decreases (© Decrease of Libido © Brectle dysfunction © Amenorhea © Lossofbody air © Testicularatrophy Hear © Congestive Hear failure (Right sided) © RCM (Restrictive cardiomyopathy) © Arhythmia (lachyarshythmia orbradyarshythmia) © AVDloek Pancreas (© Insulinopenia: Type~ 1 DM © Bronze diabetes (because there is also bror along with diabetes) Arthritis: Joints: 2nd and 3rd MCP joints (Most common) would be erosive arthritis. © Persists inspite of treatment © Ulnardeviation of fingers Skin: production of melanin is increased (Hyperpigmentation) © Slatcor gray discolouration of skin Livers inetease in liverspanhepstomegaly. Asthe disease progresses, Stigmata of development of spider ‘nacvi, Dupuytren contracture and palmer erythema. In later stage, caput medusae, variceas or variceal bleeding nd refractory ascites developed in patients Most common cause of death in hemochromatosis: Congestive Heart failure of skin © Team GlobalNet www. Distia.co Q Workup, oxo 1. Ironstudies ‘Serum Iron TBC TT Percentage saturation of Transferrin: raised > 45% (Screening) © Serum Ferritin is elevated >1000-6000 micro gmvdl (Screening) Liver Biopsy with an estimation of Iron content is the investigation of choice, © Hepatic Iron! 2. HbAIC 3. Echocardiography 4. Xorayhand| ‘Treatment Phlebotomy ‘© Deferoxamineis theron chelator. Given as Subeutuneous infusions. + Deferasitox(ora) © Sideeffects represent + Defriprone (oral) Death is generally veto: Heart Failure» Liver failure» HCC 413 Telegram : @teamglobalchat© Team GlobalNet www. Distia.co ALCOHOLIC HEPATITI $50] ate s Q a Introduction coms ‘© Marker for determining heavy aleohol consumption is Garr. ‘© Best test for determining alcoholic hepatitis is SGOT/SGPT ratioismorethan |. ‘© Alcohol is the third largest disease burden in world after CAD&DM ‘© Male: 40-80 grams per day: fatty liver and 160 gt forover 10-20 years develop alcoholic hepatiti ‘+ Female: 20 grams perday © Female is having higher chance of developing alcoholic liver disease, ‘©. Because BML is less so alcohol distribution per unit body ‘weightismore, © Estrogenisalso responsible fori ‘© There is 50 % chances that patient of alcoholic hepatitis is deteriorates intoalcoholie cirrhosis, © 60 % monlity rate over the next 4 years once aleoholic cimhosisoccurs ‘© Binge drinking cause sudden cardiac death due to Atrial fibrillation. msperday Risk factors for Alcoholiceirrhosis = HCV * Geneties © NAFLD + Obesity Spectrum ‘Fatty iveriscausedby aleohol is reversible. ‘© Fatty liver deteriorates intoalcoholic hepatitis. oss Pathological Features ‘Thereis ballooning of hepatocytes. Macro vesicular at deposition cam beeen. Neutrophil infiltrate canbe seen. ‘Spoily necrosis once this appear. ALihis stage sellatecellsin liverbecomes active. ‘© Mallory denk bodies: not seenby low magnification, ‘Causes of Mallory Denk bodies (I Will Break Alcoholic Status) Clinical features, 1 2 3 4 5 Work up 1 6 1 Maddrey’/ Discriminant Function/ Score 414 Telegram : @teamglobalchat Indian childhood cirshoss: increase in copper (due 1 use of copper utensils) Wilson Disease Primary Biliary cihosis (Anti mitochondrial Ab) Alcoholic Hepatitis Non-Aleoholic Steatohepatitis (NASH) Asymptomatic Right upper quadrant discomfort Nausea Vomiting Jaundice Absent initially. Hf patient continues to drink and damage inereasesthen Bil canrise. ‘Tender Hepatomegaly History of heavy alcohol intake AC patient presents late palmer erythema, spider naevi, 32; indicationto start the treatment,© Q Team GlobalNet www. Distia.co + LD Parameters o240s Management oa © SerumBilirubin 1. Alcohol Deaddiction: Naltrexone, Acamprosate + INR 2. Daug of choice for Alcoholic Hepatitis: Predaisolone 32 mg) # SerumCreatinine day ford weeks + IfM.E.L.D score> 21: indication To start the treatment 3. TNF-cinhibitor Pentoxyphylline (Efficacy not proven) Extramile ‘Glasgow Alcoholic Hepatitis Score oars Age Sac ‘Test Comment © BUN AST Increased two to sevenfold, <400 1U/L, + Bilirubin sreaterthan ALT opr Aur Increased two to sevenfold, <4001U/L ASTALT Usually> 1 corp Not spesfié to alcoho, easily inducible, I >15(-2) Index) ELF Enhanced Liver ‘Age, Hyallronic acid, MMP-3 217 293 Fibrosis) [Metalloproteinase], TIMP (Tissue Inhibitor of Metalloproteinase) FIB—4 ‘Age, AST, ALT, platelet count Plas 23.25 Fibro Test Haptoglobin, 02- macroglobulin, >0.45 2003 apolipoprotein A1,y GT, totalbilirubin ‘TE (Transient Measure speed of a shear wave >73kPa S15 KPa Elastography/Fibrosure) generate by vation through er eee ARFT [Acoustic Radiation _—- Measure speed of shear wave S13 ms S187 mis force imaging} generated by acoustic radiation force through liver tissue 415 + Telegram : @teamglobalchat +Hereditary Hyperbilirubinemia a3 © Hemolysis © Unconjugated Bilirubin is produced © It can cross blood brain barrier causing kernicterus! bilirubin encephalopathy ‘© Nonmally, not transported in fee form, (© It binds to plasma proteins and does not cause encephalopathy LIGANOIN © Gilbert Syndrome ‘© IF promoter molecule mutation is present: Autosomal Recessive ‘+ 1fMissense Mutations present: Autosomal Dominant ‘+ UDPGT(UDP-glucuronosyliransferase) activity is 10-20% in comparison with normal person + Fluctuating/inereased levels of unconjugated bilirubin maybedueto stress, medications + Phenobarbitone ‘© Enzyme inducer activity is used to promote the enzyme activity of UDP- glucuronosyltransferase © Sothatitcan normalize the Serum Bilirubin evels ‘© Elevated Unconjugated Bilirubin most of the times is incidental diagnosis or seen afler fasting, febrile illness andphysicalexhausation, Crier NiatiaeSyadrams © Leading cause of kemicterus + Complete absence/Profound reduction of UDP- _lucuronosyltransferase enzyme activity in Type 1, UGTIA 1 _gonedefec. ‘© Increase in unconjugated Bilirubin which can cross BBB if ‘exchange transfusion is not done. The sequelae of Basal ‘ganglia damage is Athetoid cerebral palsy © Team GlobalNet www. Distia.co 31 || HEREDITARY HYPERBILIRUBINEMIA & Clinical Features 1, Neonateon day 0 © Palms/ soles start turning yellow causing Pathological jaundice (© In these patients despite giving Phototherapy and Exchange Transfusion, the levels of bilirubin remain high ‘and tends to cause: Bilirubin encephalopathy © The bilinubin damages Putamen and it leads to sequelae called as Athetoid cerebral palsy (© Most common cause of pathological jaundice is Rh incompatibility © There is no effect of phenobarbitone because there is completeabsence ofenzyme 2. Opisthotonusand Séizares may develop 3. Delayedmilestones DubinJonhson Gene defect: ABCC? ‘Protein defect: MRP-2 (Multi drug resistance associated protein 2)- Canalicular protein responsible for excretion of| conjugated bilirubin Clinical Features 1. Obstructive jaundice occurs but since the bile salt / acid handling isnormal, so pruritusis absent. 2, Increased conjugated bilirubin [Bilirubinemia] — Bilirubinuria—» Mustard yellow urine 3. Ioterus developing after Pregnancy, Oral Contraceptive Pills, stress Workup 1. LPT: Serum Conjugated Bilirubin > bilirubin © SGOT,SGPT:Nonmal © SAP (Serum alkaline phosphatase) and Sinucleotidase, ‘normal 15% of Total serum MRCPimaging: Normal 3. Investigation of Choice (© Bromsulphalein (BSP)Test + Normally dye is cleared, excreted into bile by MRP-2 protein but in DIS —as the defect lies inthe transporter protein- BSP regurgitatesback into blood stream 4. Urine total copropomhyrin value- Normal, but ratio of ccoproporphyrinis changed, ‘© Innormal person the value of Coproporphyrin 11> A16 Telegram : @teamglobalchat +© Team GlobalNet www. Distia.co © InDIS values of Coproporphyrin 1>11T 5, Oralcholecystography ~Gall Bladder not visualized 6. Black liver because of accumulation metabolites epinephrine Rotor Syndrome eos 1, Autosomal Recessive 2. Defectin OATPB, -organicaniontransport protein B, 3. Liver Color: Normal, Echotexture also remain normal 4, Gall Bladder ean be visualized because the choleeystography dyeis excreted by MRP-2 and not excreted by OATPB 4. Total urinary coproporphyrin increased: significantly elevated 5. Coproporphyrin III raiois more (11) 417 Telegram : @teamglobalchat52 | Introduction Single Acute Actaminophen Overdose Nomogram ‘Acetaminophen Plasma Concentration © RumackMathew line © Mthe values are at least 100 Hg/mL. at 8 hours and 200 g/ml. at hours: doan orthoptic liver transplant (© AF the values are lower than this: N-tcelyleysteine is aiven, ‘© Areaingrey denotes that we have todo an intervention. © Treatment Hine threshold kept 25% lower than the ‘Rumack Mathew ine: ‘© Ir'the persons falls to left side of the treatment line: Only ‘conservative treatment is requied. ‘© Safe dose of paracetamol: 3 gramsperday ‘+ Safe dose inthe alcoholic patient:2 gramsperday ‘© PCM in combination with opioid tablets: because of dependence there sa risk of causing paracetamol toxicity ‘+ Todecrease the risk of toxicity the maximum dose of PCM as ‘marketed is325 mg tablet + 10-15 grams perday: requires Hospitalization, ‘© >25gramsper day: has higher chances of fatality, © Antidote: N-acetyl eysteine ‘© indicated the patient start within hours of intake and canbe given up 0 24-36 hs. (©. Thereisareduction inmortality. ‘+ Blood PCM>300 g/ml at 4 hours ofingestion: High chance ofliver damage, ‘© Blood PCM < 150 jgiml at 4 hours of ingestion: damage is unlikely. © Team GlobalNet www. Distia.co PARACETAMOL TOXICITY/ ACETAMINOPHEN TOXICITY Metabolism of PCM ‘© Metabotized by phases | and of metabolism, InPhaset * Cytochrome P4S0: Produces NAPQ I (N-acetyl-P- ‘benzoquinone Lmine) neutralized by Glutathione. ‘+ Low glutathione levels inperson (Inaleoholies,instarvation, consumption of anti-TB drugs (INH) and Barbiturates: Ability to cause more damageto the liver. ‘© N-acetyl eysteine generates glutathione InPhase2 # IcInvolves conjugation: Responsible for © Sulfate moiety (© Glucuronidation Clinical features vous? 1. AL4-12 hours: Nausea, vomiting, disthea, abdominal pain and shock. 2. A124-48 hours: Fulminant hepatic failure a ‘= Blgeding fiom gums and Altered sensorium progressing Nose ete. to Coma 3. Acutekidney injury: Oligurisoranuria 4, Myocardial injury: Troponins are elevated, ‘Treatment vos 1. Gastric lavage (not effective afterhalfan hour of intake) 2. Cholestyramine 3, N-Acetyleysteine <8 hr Antidote ‘© MOA: replenish feves of glutathione which will neutralize NAPQ [by providing sulfhydryl groupsto glutathione 4. If Fulminant hepatic failure is already present: An ortboptic liver transplant is done Iron toxicity + Leading cause of poisoning in children less than 6 years, Stages © Stage-I: Nausea, vomiting, hemorrhagic manifestation in conjunction with diarrhea and shock. + Stage-2 Latent phase): Deceptive phase (© Occursin~6~12 hours. Patientappears relatively beter * Stage—3: Metabolicacidosis cause cardiac depression, © Themost common cause of deaths iron toxicity. + Stage 4: Darnage tothe liver. 418 Telegram : @teamglobalchat Q +y Paci} g es © Cougulopathy ‘©. Encephalopathy ‘© Stage-s (Delayed phase):searring ofthe gut (©. Thepatientsend up with gastric outlet obstruction, Management © LY.fids ‘© Oxygensupplementation Team GlobalNet W www. Distia.co + Noroleforactivated charcoal andipecae. + Deferoxamine: chelate the iron. © Indications: > 350,g/dl: toxicity (6 7500 pid: inespectiveof toxicity ‘© Deferasirox oral: used in chronic overload and ~2-year ‘thalassemia transfusion dependent © Primary bronve diabetes 419 Telegram : @teamglobalchat© Team GlobalNet www. Distia.co BUDD CHIARI SYNDROME Q a ‘© Normally: In the Portal vein there is ammonia rich blood: © Inliver.NH,+CO,—+ Urea In Budd Chiari syndrome: there would be, Thrombus formation in hepatic veins ‘© Secondary to obstruction there is congestion developing in theliver. ‘There will be the development of centrilobular necrosis ‘resulting in fulminanthepatie failure. © Bitherhaveacute orchronic manifestations. ‘© ItisPosthepaticcause of Portal hypertension Definition ‘© Thrombosis of22 hepatic veins ‘© Usually, Caudate lobe drains in IVC. If thrombus involves ING, then on USG, with help of doppler Enlargement ofthe caudate lobe is seen, ‘+ Hydatideystisa secondary cause of Budd Chiari syndrome Liver tumor: it could be HCC or multiple metastases pressing on multiple hepatic veins. Causesof BCS 1, Hematological disorders © Polyeythemia vera JAK 2mutation) + Etythrocytosis explains the sluggish circulation inthe liver and thrombosis © Paroxysmal nocturnal Hemoglobinuria (PLG.A gene defect) + Defect in CD 59 which s present on the platelets andit will nbibit the platelet aggregation, > Thrombus formation chances will increase. Deep vein thrombosis 3. Thrombotic dathesis © Protein C, S and AT IT all decreases it contributing to a hhypercongulablestate. ©. Factor V Leiden mutation: defective binding with protein C leads o extrinsic system activation and initiates the clot. 4, Postpartum, OCPs: High estrogen leads toa hypercoagulable state 5. Tumors: Hepatocellular carcinoma, RCC, Wilm’s tumor (TUMOR PRESSING HEPATIC Vein) Infections: Hydatideyst 7. Connective tissue disorders: Anti-phospholipid antibody syndrome © Anti 2 glycoprotein antibody stimulates the Intrinsic Systemofclottingandtumsinto automatic mode, ‘May ormay not be associated with SLE. © Sarcoidosis © Bechet'sdisease 8, Membranous defects in VC or portal Vein 9, Total parenteral ntstion: IVC catheter Clinical Features of BCS voasas 1. Triad © RUQpain Suddenonset) (© Ascites (due to portal HTN) © Hepatomegaly: the gross detention of the liver and liver spanisincreased. 2. Jaundice due to necrosis in the central lobular part of the liver. 3. Hepatorenal Syndrome © Splanchnie vasodilation: in the kidney, there is apparent hypovolemia + Inthekidney theres hypoperfusion > Acutekidney-fike manifestation occurs. 4. Pedaledema InChronic presentation: Caput Medusae orascites 420 Telegram : @teamglobalchatTeam GlobalNet W www. Distia.co + Workup oni98 1, USG with doppler: Helps to rule out gallstones and cholelithiasis. 2. Ascii Tap: SAAG> 1.1 caudate lobe may beenlarged In the initial phase, the Ascitic fluid protein can be more than 2.5 grams, 3. LFT ‘Serumbilirubin values can be increased, SGOT and SGP Tare grossly elevated, 4. MRIabdomen 5. Investigation of choice is Hepatic venography Treatment + EASL Guidelines for Acute Budd Chiari syndrome Balloon angiog © Anticoagulation ‘© InChronic Budd Chiari syndrome TIPS, © Spironolactone with furosemide Onthoptic Liver Transplantationin ESLD sty 421 + Telegram : @teamglobalchat +