CASE PRESENTATIONDr.Usman Ali,

PGR West Medical Ward,Mayo Hospital, Lahore .

Patient’s Bio data

Name:Shahbaz s/o Abdul Majeed Age: 18 YearsSex: MaleOccupation: works in a factory which makes lead based batteriesAddress: SHAMKE,KALA SHAH KAKU,SHEIKHUPURA.

D.O.A: 10/10/14M.O.D: Emergency DepartmentEthnicity: PunjabiLanguage: Punjabi, Urdu

Religion: Islam

Presenting complaintsFever-7daysJaundice-3daysvomiting+pain abdomen-3daysASOC-6hrsHistory of presenting illness:My patient who is neither hypertensive nor diabetic was in usoh 7 days ago when he started having fever which was high grade(102f-103f) not associated with rigors or chills and continous (no

diurnal variation) ,relieved upon taking medication.

He went to a local quack who gave him some injections(unknown to attendants) and after that he felt better-fever reduced.The following day he started having yellowing of eyes and dark colored urine.this was of sudden onset and also accompanied by abdominal pain which was severe and generalized.vomiting also started the same day several episodes containing food particles but no blood.he remained in that condition for 2 days and took medication prescribed by Dr at mayo ER until this morning when he became uncosious.asoc was of gradual onset not preceded by headache or any focal neurological deficit.There is no history of diarrhoea/constipation,sore throat /cough ,burning micturition.no h/o fits,sob ,bleeding or any other complaint

•Past History:Suffered from viral encephlitis 2 months back for which he was treated at NMW and recovered completely .No H/O surgery, allergies,psychiatric illness,TB,Asthma or any traumaDrug History:.Rx for Encephlitis at NMW(Aclova,ctrox,vanco)Discharged on PPI,Surbex-z,gravinate Family History:No h/o jaundice,viral hepatitis,TB or any serious infection.

Personal History :no h/o alcohol,smoking or any other drug of abuse.Socioeconomic History : He belongs to poor class . Lives in a joint family of 12 persons in a 10 Marla house .works at factory making lead based batteries.

Systemic Review Skin: No H/O Rash, redness or itching or any wound yellow tinge+Eyes: No H/O Eye pain redness, dryness or recent changes in vision.Ears: No H/O hearing loss, discharge, dizziness or ringing in ear.Nose: No recent H/O runny nose, stuffy nose or nose bleeds.Throat: no h/o sore throat,cough,discharge.

Neck: No H/O soreness, stiffness , enlarged nodes or lumps on neck.Heart: No known H/O of heart problems, hypertension, high cholesterol, chest pain, palpitations, SOB, orthopnea, PND or lower extremity edema.LUNGS: No H/O lung disease, asthma, TB or TB contact, pneumonia. NO recent H/O cough, sputum, wheezing, SOBGIT: vomiting+,abd pain+,NO diarrhoea/constipation , hematemesis or blood in stool/malena .

Urinary: No H/O change in frequency, burning or painful urination and other lower urinary tract symptoms.Urine color became dark with onset of jaundiceGenital: Sexually contact -ve. No H/O penile sores, discharge, testicular pain or enlargement.Musculoskeletal: No H/O muscle or joint pain, cramps or stiffnessNeurologic: H/O seizures 2 months back, asoc +,no numbness, tingling, shooting pain or tremors

Endocrine: No H/O diabetes, thyroid problems, increased thirst, urination, heat or cold intolerance.Psychiatric: no h/o any psychiatric disesase

SummaryAn 18 yr old male presented with high grade fever for 7 days followed by sudden onset jaundice,vomiting ,abd pain for 3 days and asoc for 6 hrs.

2 months back had viral encephlitis which resolved after 2 wk treatment at NMW,MHL.

Differential Diagnosis:Drug/Toxin induced hepatic failureAcute viral hepatitisLEPTOSPIROSISLYMPHOPROLIFERATIVE DISORDER HIVCerebral MalariaHemolysis leading to pre hepatic jaundiceAutoimmune hepatitisSepsis leading to organ failure (liver

PHYSICAL EXAMINATION

General Survey:The patient is a normal build, young male who appears his stated age of 18 and is very irritable. He is drowsy and poorly oriented to person,place and time. Patient is lying down but can ambulate with some difficulty . He appears not so well groomed,dirty clothes and has difficulty communicating with other people but now better responding well HR: 90/mint, BP: 120/80,RR:17/mit, Temp: 99 FHeight: 5‘6“, wt: 51 kg

SKIN:Pallor +veJaundice +ve Cyanosis –vePalmar erythema -veslightly warm, humid, normal turgor, . No apparent Skin Lesion in the exposed area from waist upwards and feet.NAILS: Clubbing –veCapillary refill 3 secondsNo Nails deformity or pathology in both

hands and feet .

Head - normocephalic, no masses /lesions, malar flushing .Eyes - visual fields intact, PERRLA , conjunctival palor, sclera icteric, no ptosis Ears – Hearing appears Normal. No external lesion noted. Nose - nares patent, no deformity, septal deviation or perforation.

Throat – Pharanx normal, palate rises symmetrically, gag present.MOUTH – poor oral hygeine,tongue having yellow tinge.no other lesion apparentNeck & Axilla – no LN enlargement,or thyromegaly/focal lump,carotid pulses 2+ , no bruits, neck supple , trachea midline.

Back, Thorax & Lungs – N abdomino-thoracic breathingChest expansion symmetric, Clear to A&P, Normal vesicular breathing, eupnoea, no added soundsCardiovascular – JVP at 450 not raised,

lInspection: normal,no scars,marksno lifts , heaves or thrill. Palpation:

.apex beat palpable in 5th ICS, MCL.Auscultation: S1- heard best at apex, nl intensity S2- heard best at base, nl splitting, A2 > P2 no murmurs/S3/S4/friction rub

ABDOMENObservation: Normal boat shaped abdomen, umbilicus inverted and situated centrally, no stria, scar marks.Auscultation: bowel sounds 4-5 cycles/min, no bruits Palpation: Superficial- tenderness in general,voluntary guarding +ve, no masses. Deep- Tenderness more in rt hypochondrium, rebound tenderness negative, Liver Palpation-

liver edge not palpablePercussion - Size-12 cm in R midclavicular line Spleen not palpable, B/L Kidneys not palpableFemoral Pulses: bil equal, no bruit

musculoskeletal - gait normal; joints and muscles symmetric, noswelling, masses, deformity or tenderness to palpation; no heat or swelling of joints; musclestrength 5/5- able to flex against resistance & w/o tenderness.

Nervous – impaired consciousness,Irritable, not very cooperative previously but now better, sensory - normal, motor - no atrophy, weakness, tremors or clonus; tone normal .DTR's - all + n; Babinski; toes downgoing. gaze normal;hmf cant be assesed Cranial Nerves:All cranial nerves are Intact

Differential Diagnosis:

• •Acute viral hepatitis•Sepsis causing organ failure•Hemolysis d/t disseminated infection/drug/toxin•Autoimmune hepatitis

LABORATORY FINDINGS

CBC:wbc 6.3*103

Hb 10.1 mg/dlHCT 33 %MCV 84 fLMCH 26 pgPLT 157 *103/ ulLymp 21 %

Neut 75%

LFT’s:Ast: 570/619/332ALT: 664/886/256TBil: 7.9/13.7 /8.1/DBIL:1.7T.P: 6.5Alb: 3.7HCV+RFT’S:urea: 43Creat: 1.2S/E :Na+ : 133K+ : 3.9

Pt/aptt:normalHAV BORDERLINE/HEV: NON REACTIVECPK:NLDH :slightly raised-617U/E : unremarkableCxray: NormalABG: NUltrasound Abdomen : Normal

Further Evaluation:

REPEAT HAV SEROLOGYBLOOD CULTURES

PERIPHERAL SMEARLIVER BIOPSY

Final Diagnosis:ACUTE FULMINANT VIRAL HEPATITIS

Treatment Given in the ward:INJ RISEK 40MG iv odinj flagyl 500mg iv tdsinj 10% d/w+2 amp hepamerz bdinj gravinate iv tdsmucolator sachet 40 stat then 20sachet6 hrly till upto 17 doses

Discussion:

Introduction:Acute liver failure is an uncommon but serious condition.

The presentation is with progressive deterioration

in liver function and mental changes progressing from

confusion to coma. The syndrome was originally defined

further as occurring within 8 weeks of onset of the

precipitating illness, in the absence of evidence of preexisting

liver disease. This distinguishes it from instances

in which hepatic encephalopathy represents a deterioration

in chronic liver disease

ACUTE FULMINANT HEPATIC FAILUREMassive hepatic necrosis with impaired consciousness occurring within 8 weeksof the onset of illness.

Causes Infections [viral, including HAV, HBV, HCV (rarely), HDV, HEV;bacterial, rickettsial, parasitic],Drugs and toxins, ischemia (shock), Budd-Chiarisyndrome, Idiopathic chronic active hepatitis, acute Wilson’s disease, microvesicularfat syndromes (Reye’s syndrome, acute fatty liver of pregnancy).

Classification of acute liver failureTime: jaundice to encephalopathy:Hyperacute < 7 days, Cerebral oedema CommoncausesViral,

Acute 8–28 days CerebraloedemaCommoncauses paracetamolCryptogenic

Subacute 29 days–12 weeks Cerebraloedema Uncommon drugsCryptogenic,drugs

Clinical Manifestations Neuropsychiatric changes—delirium, personality change,stupor, coma; Cerebral edema—suggested by profuse sweating, hemodynamic instability, tachyarrhythmias, tachypnea, fever, papilledema, decerebrate rigidity(though all may be absent); Deep jaundice, coagulopathy, bleeding,Renal failure,Acid-base disturbance, hypoglycemia, Acute pancreatitis, Cardiorespiratoryfailure,Infections (bacterial, fungal)

Monitoring in acute liver failure

•Cardiorespiratory• •Pulse

• •Blood pressure• •Central venous pressure

• •Respiratory rate•Neurological• Intracranial pressure

monitoring (specialist units,• Conscious level

• •Temperature

Fluid balance• Hourly output (urine, vomiting, diarrhoea)• Input: oral, intravenousBlood analyses• Arterial blood gases• Peripheral blood count (including platelets)• Sodium, potassium, HCO3−, calcium, magnesium• Creatinine, urea• Glucose (2-hourly in acute phase)• Prothrombin timeInfection surveillance• Cultures: blood, urine, throat, sputum, cannula sites• Chest X-ray• Temperature

23.10 Investigations to determine the cause ofacute liver failure

• •Toxicology screen of blood and urine• •HBsAg, IgM anti-HBc

• •IgM anti-HAV• •Anti-HEV, HCV, cytomegalovirus, herpes simplex, Epstein–

•Barr virus• •Caeruloplasmin, serum copper, urinary copper, slit-lamp eye

•examination• •Autoantibodies: ANA, ASMA, LKM, SLA

• •Immunoglobulins• •Ultrasound of liver and Doppler of hepatic veins

Adverse Prognostic Indicators• Age <10 or >40,Certain causes (e.g., halothane,hepatitis C),Duration of jaundice <7 d before onset of encephalopathy,Serum bilirubin > 300 μmol/L (>18 mg/dL), Coma (survival <20%), Rapid reduction in liver size,Respiratory failure,Marked prolongation of PT, Factor V level< 20%. In acetaminophen overdose, adverse prognosis is suggested by blood pH< 7.30, serum creatinine > 266 μmol/L (>3 mg/dL), markedly prolonged PT.

Acute Hepatic Failure TreatmentEndotracheal intubation often required. Monitor serum glucose—IV D10 or D20 as necessary. Prevent GI bleeding with H2-receptor antagonists and antacids(maintain gastric pH ≥ 3.5). In many centers intracranial pressure is monitored—more sensitive than CT in detecting cerebral edema. Value ofdexamethasone for cerebral edema unclear; IV mannitol may be beneficial.Liver transplantation should be considered in pts with grades III–IV encephalopathyand other adverse prognostic indicators.

VIRAL HEPATITISAcute viral hepatitis is a systemic infection affecting the liver predominantly.Clinically characterized by malaise, nausea, vomiting, diarrhea, and low-grade fever followed by dark urine, jaundice, and tender hepatomegaly; may be subclinical and detected on basis of elevated aspartate and alanine aminotransferase (AST and ALT) levels.

CONTINUED:Hepatitis B may be associated with immune-complex phenomena,including arthritis, serum sickness–like illness, glomerulonephritis, and a polyarteritis nodosa–like vasculitis. Hepatitis-like illnesses may be caused not only by hepatotropic viruses (A, B, C, D, E) but also by other viruses (Epstein-Barr, CMV,coxsackievirus, etc.), alcohol, drugs, hypotension and ischemia, and biliary tract disease

TOXIC AND DRUG-INDUCED HEPATITISDose-Dependent (Direct Hepatotoxins) Onset is within 48 h, predictable, necrosis around terminal hepatic venule–e.g., carbon tetrachloride, benzene derivatives,mushroom poisoning, acetaminophen, or microvesicular steatosis (e.g.,tetracyclines, valproic acid).Idiosyncratic Variable dose and time of onset; small number of exposed persons affected; may be associated with fever, rash, arthralgias, eosinophilia. In many cases, mechanism may actually involve toxic metabolite, possibly determined on genetic basis—e.g., isoniazid, halothane, phenytoin, methyldopa, carbamazepine,diclofenac, oxacillin, sulfonamides

Toxic and Drug-Induced Hepatitis TreatmentSupportive as for viral hepatitis; withdraw suspected agent, and include use of gastric lavage and oral administration of charcoal or cholestyramine. Liver transplantation if necessary. In acetaminophen overdose, more specific therapyis available in the form of sulfhydryl compounds (e.g., N-acetylcysteine).These agents appear to act by providing a reservoir of sulfhydryl groups to bind the toxic metabolites or by stimulating synthesis of hepatic glutathione.Therapy should be begun within 8 h of ingestion, but may be effective even if given as late as 24–36 h after overdose

Complications of acute liver failure

Encephalopathy andcerebral oedema• Hypoglycaemia• Metabolic acidosis• Infection (bacterial, fungal)• Renal failure• Multi-organ failure(hypotension andrespiratory failure)

QUIZ: