jaundice - glocal university

JAUNDICE

BY: ANJALI NAUDIYAL

B.Sc (N)Glocal College of Nursing

I T I S T H E Y E L L O W I S H D I S C O L O R A T I O N O F T H E TI S S U E S D U E T O D E P O S I T I O N O F B I L I R U B I N

W H I C H O C C U R S I N P R E S E N C E O F H Y P E R B I L I R U B I N E M I A

JAUNDICE

Normal serum bilirubin level - 0.3-1.0 mg/DL

Conjugated - 0.1-0.3 mg/DL

Unconjugated - 0.2-0.7 mg/dl

Clinically jaundice is evident when serum bilirubincrosses 3 mg/DL

Jaundice is latent I.e., clinically non evident (onlydetected by serum analysis) when serum bilirubinis in between 1 - 3 mg/DL.

Unconjugated hyperbilirubinemia – when direct bilirubin level is less than 15% of total serum bilirubin.

Conjugated hyperbilirubinemia – when direct bilirubin level is greater than 15%

Sites to be examined

Upper bulbar conjuctiva (contains elastin which has hisj affinity for bilirubin)

Base of tongue

Mucous membrane of palate (specially soft palate)

Palms and soles

General skin surface

Differential diagnosis

Carotenoderma (discoloration is limited to palms, sole, forehead, nasolabial fold with sparing of sclera)

intake of drug quinacrine (only skin, urine and eyes are yellow)

excessive exposure to phenols.

Formation of bilirubin

BILIRUBIN METABOLISM

Types of jaundice

Pre-hepatic / Hemolytic jaundice

Hepatic jaundice

Post-hepatic / Obstructive/

Surgical jaundice

Hemolytic Jaundice/Pre hepatic Jaundice

Excess production of bilirubin due to excess breakdown of hemoglobin

Indirect bilirubin (insoluble in water since unconjugated).

Causes of pre hepatic jaundice

HEMOLYTIC DISORDERS

1. Inherited

a. Spherocytosis, elliptocytosis- Hereditary condition, with defect or

absence of RBC membrane protiens.

b. glucose 6 phosphate dehydrogenase- Most common cause of enzyme

deficiency hemolysis.

- autosomal recessive condition

triggered by certain certain food,

drugs, etc. Avoiding such triggers

is advised.

c. pyruvate kinase deficiency- second most common cause of enzyme

deficiency hemolysis.

b. sickle cell anemia- Abnormal haemoglobin synthesis leading to

sickeling under low oxygen condition. Incresed hemolysis.

2. Acquired

a. Microangiopathic haemolytic anemias

b. Paroxysmal nocturnal hematuria.

c. Spur cell anemia.

d. Immune haemolysis.

e. Parasitic infections-

- Malaria- patient presents with fever chills rigor, hepatosplenomegaly

- Babesiosis

INEFFECTIVE ERYTHROPOIESIS1. Cobalamin, Folate deficiency- Megaloblastic anemia

2. severe iron deficiencies- Microcytic hypochromic anemia

3. Thalassemia

• INCREASED BILIRUBIN PRODUCTION1. Massive blood transfusion

2. Resorption of hematoma.

• DRUGS1. Rifampicin, ribavirin, Probenecid.

Hepatic Jaundice

Liver’s ability to conjugate or excrete bilirubin is affected

Increased level of conjugated and unconjugated bilirubin present

Causes of hepatic jaundice

VIRAL HEPATITIS

features HAV HBV HCV HDV HEV

transmission Feco-oral

Parentral,sexual,perinatal

parentral Parentral, sexual, perinatal

Feco oral

Carrier none .1-30% 1.5-3.2% variable none

chronicity none occasional common common none

cancer none + + +- none

prognosis excellent Worsen with age

moderate Acute-good Chronic-poor

good

• Other viruses- Epstein barr, CMV, HSV

• ALCOHOLIC HEPATITIS- Balloon degeneration, fibrosis of

parenchymal tissue.

- 160g/day for 10-20yrs(women are more

susceptible)

• DRUG TOXICITY

1. Acetaminophen- Predictable, Dose dependent

2. Isoniazid- Unpredictable, Idiosyncratic.

• ENVIRONMENTAL TOXINS

- Vinyl chloride, Jamaica bush tea-pyrrolizidine alkaloids , kava kava, Wild mushrooms.

WILSON’S DISEASE- hepato lenticular degeneration

due to copper accumulation in liver tissue.

AUTOIMMUNE HEPATITIS

INHERITED CONDITIONS-

Indirect hyperbilirubinemia

- Gilbert syndrome- Enzyme(UDP glucoronyl transferase) deficiency- 10-33%

activity only.

- Crigler-najjar syndrome type 1- Enzyme – absent

type2- enzyme activity- 0-10%

Direct hyperbilirubinemia

- Dubin Johnson syndrome- mutation in MRP2 gene. Defect in canalicular

transport of organic anions

- Rotor syndrome- defect in bilirubin storage.

Obstructive Jaundice

Bilirubin formation rate is normal

Conjugation is normal = direct bilirubin

Any intrahepatic or

extrahepatic condition leading

Obstruction to the flow of bile.

Causes of post hepatic / obstructive jaundice

INTRAHEPATIC CAUSES

1. Viral hepatitis-

a. Fibrosing cholestatic hepatitis- hep.B and C

b. hep A, Epstein barr, cytomegalovirus infection.

2. Alcoholic hepatitis

3. Drug Toxicity-

a. Pure cholestasis- anabolic and contraceptive steroids

b. Choleststic hepatitis- chlorpromazine, erythromycin estolate

c. Chronic cholestasis- prochlorperazine and chlorpromazine

4. Primary biliary cirrhosis5. Primary sclerosing cholangitis6. Vanishing bile duct syndrome

- chronic rejection of liver transplants- sarcoidosis- drugs

7. Congestive hepatopathy and ischemic hepatitis

8. Inherited conditions-- progressive familial intrahepatic cholestasis- Benign recurrent cholestsis

9. Cholestasis of pregnancy10.Total parenteral nutrition11. Non hepato biliary sepsis

12. Benign post operative cholestasis

13. Paraneoplastic syndrome

14. veno-occlusive disease

15. Graft versus host disease

16. Infiltrative disease-- tuberculosis, amyloidosis, and lymphomas

17. infections--malaria, leptospirosis

EXTRAHEPATIC CAUSES1. Malignant conditions

- Cholangiocarcinoma- Gallbladder cancer- Pancreatic cancer- Ampullary carcinoma- malignant involvement of porta hepatis lymph nodes

2. Benign conditions

- Choledocolithiasis

- post operative biliary strictures- Primary sclerosing cholangitis- Chronic pancreatitis- AIDS Cholangiopathy- ascariasis

Sign & Symptoms

Early features- Yellowish discolouration(skin, sclera, etc)

- Pale/Clay coloured stool

- Dark Urine

- Pruritis

Late Features- Xanthelasma and Xanthomas

- Malabsorption- weight loss, steatorrhea,

Osteomalacia,Incresed bleeding

Tendency

Fever, Rigor, pain (features of cholangitis)

INVESTIGATION

OFJAUNDICE

Investigation

Depends on aetiology –that can be concluded by :-1.history, 2.C/F, 3.Clinical Ex.Jaundice- cause by rise in blood plasma of bilirubin

Normal= <1 mg/dL---------- 1. Unconjugated/Indirect= 0.2-0.7 mg/dL2.Conjugated/Direct =0.1-0.4 mg/dL

If bilirubin value is– 1. >1mg/dL, -Hyperbilirubinemia2. >2-2.5mg/dL, -Start diffusing into tissues3. ~3mg/dL, -Clinically jaundice detectable

The typical investigation will include blood levels of enzymes found primarily from the liver, such as the aminotransferases (ALT, AST), and alkaline phosphatase (ALP); bilirubin (which causes the jaundice); and protein levels, specifically, total protein and albumin. Other primary lab tests for liver function include gamma glutamyl transpeptidase (GGT) and prothrombin time (PT)

Pre-hepatic Jaundice

Cause- Mainly by haemolysis of RBCDetoxification Function Test –

- Serum:- Increase unconjugated bilirubin-Urine:- Bilirubin= Absent (unconjugated bilirubin is not water soluble)

-Urobilinogen= Increases (Increase in 6x function of Normal liverto cope with load of unconjugated bilirubin)

Stool:- Fecal Urobilinogen increases

Rest of parameter usually remains normal.

Hepatocellular Jaundice

Detoxification Function Test:-1. Serum bilirubin- conjugated and unconjugated both increased

2. Urine -Bilirubin – Present (Conjugated Bilirubin is water soluble) Urobilinogen- decreased

3. Fecal stercobilinogen/Fecal Urobilinogen- decreased

Enzymatic test:-1. AST,ALT – highly raised (due to lysis of liver parenchymatic cells)2. ALP, GGT – is slightly raisedAST and ALT rise is significantly higher than the ALP and GGT rise

Plasma albumin level is low but plasma globulins are raised due to an increasedformation of antibodies

Disorders Bilirubin Aminotransferases Alkaline phospha. Albumin Prothrombin time

Post Hepatic/Obstructive Jaundice

Detoxification test1. Serum bilirubin – Direct(conjugated)– increased2. Urine – Bilirubin- Present

- Urobilinogen – absent3. fecal stercobilinogen- trace to absent

Enzymatic Test1. AST,ALT – Slightly increase2. ALP, GGT- Highly Incresed

If the ALP (10–45 IU/L) and GGT (18–85) levels rise proportionately about as high as the AST (12–38 IU/L) and ALT (10–45 IU/L) levels, this indicates a cholestatic problem

Disorder Bilirubin Aminotransferases Alkaline phosphatase Albumin Prothrombin Time

Radiological Investigation

Plain radiographs -are of limited utility as Frequently, calculi are not visualized because few are radiopaque.

Ultrasonography (USG)- most sensitive technique for visualizing the biliary system, particularly the gallbladder.

Procedure of choice for the initial evaluation of cholestasis and for helping differentiate extrahepatic from intrahepatic causes of jaundice

CT Scan -helps visualize liver structures more consistently than USG. CT scan has limited value in helping diagnose CBD stones because many of them are radiolucent and CT scan can only image calcified stones ( in such situation CT cholangiography by the helical CT technique is used)

Radiological Investigation-Continue

MRI- MRCP (Magnetic resonance cholangiopancreatography)type is used to visualize the hepatobiliary tree.

It helps in detecting biliary and pancreatic duct stones, strictures, or dilatations within the biliary system. It is also sensitive for helping detect cancer. MRCP combined with conventional MR imaging of the abdomen can also provide information about the surrounding structures (eg, pseudocysts, masses).

Biopsy• Usually done at last in series of investigation to establish the cause of

Jaundice.• In patients with apparent intrahepatic cholestasis, the diagnosis is often

made by serologic testing in combination with percutaneous liver biopsy.• to assess the condition of the liver tissue if it may have been damaged by a

condition such as cirrhosis or liver cancer.

Treatment of Jaundice

General Treatment

Treatment of pre hepatic causes

• G6PD deficiency - mostly people recover on their own

o if progresses to hemolytic anaemia, oxygen therapy or blood transfusion may be required.

• Spherocytosiso These infants should be treated with phototherapy

and/or exchange transfusion as clinically indicated.o Folic acid is required to sustain erythropoiesis.

o Patients with HS are instructed to take supplementary folic acid for life in order to prevent a megaloblastic crisis.

o Splenectomy is the definitive treatment for HS

• Sickle cell anaemiao Treatments may include medications to reduce pain and

prevent complications, blood transfusions and supplemental oxygen, as well as a bone marrow transplant.

o Antibiotics -Children with sickle cell anemia may begin taking the antibiotic penicillin when they're about 2 months of age and continue taking it until they're at least 5 years old.

• Immune related hemolysis – corticosteroids, folic acid is main line of treatment

• Parasitic Infections like malaria are treated with antimalarial drugs like chloroquine, artesunate, lumefantrine,amodiaquine

• Ineffective erythropoiesis- iron and folic acid supplementation, vit B12 tablets given and repeated blood transfusions

Treatment of hepatic causes

• Viral hepatitisHepatitis A is mostly self limiting no treatment is

required, but in some cases 0.02 ml/kg administration of anti-HAV Ig can be given.

Hepatitis B treated with combination of HBIG and Hep b vaccines.

Recombivax and Engerix-B are 2 vaccines for hepatitis BHepatitis C is treated with interferons.• Other Viral infections like EBV, CMV, HSV are treated

with Antiviral medications like acyclovir , ganciclovir and foscarnet.

• Alcoholic hepatitis

Discriminant function - determines the prognosis of the person suffering from alcoholic liver disease. Given by Maddrey.It is calculated by a simple formula:

(4.6 x (PT test - control))+ S.Bilirubin in mg/dl A value more than 32 implies poor outcome.

MELD – model for end stage liver disease

scoring system for assessing the severity of chronic liver diseaseMELD uses the patient's values for serum bilirubin, serum creatinine, and the

international normalized ratio for prothrombin time (INR) to predict survival.

• Wilsons disease- pharmacologic treatment with chelating agents such as D-penicillamine and Other agents include sodium dimercaptosuccinate, dimercaptosuccinic acid

Treatment of obstructive jaundice

SUPPORTIVE MANAGEMENT44

Preoperative biliary decompression (ERCP or PTC)

Intravenous admistration of 5% dextrose saline followed by 10%mannitol

or loop diuretics to prevent hepatorenal syndrome/ renal failure(12 to 24

hours prior to surgery)

catheterization to monitor output

Broad spectrum antibiotic prophylaxis with 3rd generation cephalosporins

Parenteral vitamin K +/- fresh frozen plasma

Need careful fluid balance to correct dehydration

Correction of hypokalemia and other electrolyte imbalance.

Cholestyramine and antihistamine for symptomatic relief of pruritis

Definitive treatment depends upon the cause-

• Choledocholithiasis- cholecystectomy is done

• Carcinoma of head of pancreas- whipple resection done

• Ca gall bladder- whipple resection done but if unoperable radiological stenting is done.

• Choledochal cyst- excision of the cyst with reconstruction of extrahepatic biliary tree.

• Stricture- endoscopic stenting. Standard care is surgery by roux-en-y choledocojejunostomy.

SUMMARY46

THANK YOU!