Scribd Logo
Upload

Welcome to Scribd!

  • Jaundice and Hepatomegaly

    Uploaded by

    Rudra Verma
    112 views6 pages
    Medicine topic for dental students

    Copyright

    © © All Rights Reserved

    Available Formats

    PDF or read online from Scribd

    Did you find this document useful?

    Is this content inappropriate?

    Report this Document
    Medicine topic for dental students

    Copyright:

    © All Rights Reserved
    Download as PDF or read online from Scribd
    Flag for inappropriate content
    112 views6 pages

    Jaundice and Hepatomegaly

    Uploaded by

    Rudra Verma
    Medicine topic for dental students

    Copyright:

    © All Rights Reserved
    Download as PDF or read online from Scribd
    Flag for inappropriate content
    of 6
    Jaundice and Hepatom epaly fa ‘= Jaundice: definition, bilirubin synthesis, metabolism and excretion. + Classification and causes of jaundice ‘* Clinical and biochemical analysis of a patient with jaundice ‘+ Hepatomegaly ~ definition, types and causes savrorce {¥* .) Definition Jaundice or icterus refers to yellow discoloration of skin, ‘mucous membrane, sclera and conjuctiva due to raised serum bilirubin. It is first detected in sclera which has an affinity to bind with bilirubin and also provides white background. Yellow discoloration of skin but not of sclera could be due to hypercarotenemia. Normal serum bilirubin concentration ranges from 0.3-1.2 mg/dl and most of it is unconjugated (80% approx.). Jaundice becomes lnicaly detectable when serum bilirubin is raised to 2.5, mg/dl or more. Jaundice is called subclinical if serum bilirubin is raised more than normal but remains below 2.5 mg/dl. Greenish yellow discoloration in a patient ‘with jaundice is due to conversion of bilirubin to biliverdin (Green pigment). It is commonly seen in surgical jaundice due to obstruction of biliary tract. However, it can also ‘occur due to prolonged cholestasis of any origin leading to conjugated hyperbilirubinaemia and oxidation of bilirubin to biliverdin, ~ Bilirubin synthesis, metabolism and excretion Bilirubin is synthesised normally (80-85%) from destruction of old or senescent RBCs in the reticuloendothelil system daily; while rest of 15% comes from destruction of maturing erythroid cells in the bone marrow called ineffective erythropoiesis. Haemoglobin is catabolised to haem and globin. The globin is reused for its synthesis. The haem part is converted into biliverdin by hae oxygenase Bilirubin is formed from biliverdin by biliverdin reductase, The unconjugated bilrubin after its formate y to albumin and cteuates inthe blood a albumin complex, which Is taken up by the lier for mente” Bilirubin metabolism involves three steps = 2m conjugation and biliary excretion The last sep limiting step, is impaired when liver cells are ce 2 Uptake of unconigaed birbinalbumin conpin the liver cel is followed by dissociation of aun storage of unconjugated bilirubin as bound to oy proteins and anions. This binding prevents back diss, of bilirubin in plasma. Conjugation in liver cels conan the unconjugated, water insoluble bilirubin to conjrsey water soluble derivative so that itis easly excreted ine te bile. Conjugation takes place in the endoplasmic reticle of hepatocytes by glucuronyl transferase to glucuronide salls, Excretion of conjugated bilinubin into the bil by the liver cells is energy dependent and rate limiting step the metabolism of bilirubin. Inthe event of obstruction to the excretion of bilirubin, it requrgitates back into blood stream to be excreted through kidneys. If there is no obstruction, the conjugated bilirubin enters the duodenum and small intestines in the form of bile salts, takes part in emulsification of fats, then deconjugated by intestinal bacteria to be excreted through stools as stercobilin and in urine as urobilinogen. The urobilinogen is formed in the intestine by the action of bacteria on conjugated bilirubin, then enters the enterohepatic circulation and some of it is excreted as urobilinogen in the urine. Normally, urine contains no bilirubin but urobilinogen is present in small amounts to be detected by 1:10 dilution of Ehrlch’s aldehyde reagent. Large excretion of urobilinogen and Presence of bilirubin and bile salts in urine are abnormal and indicative of liver disease. / , Classification of jaundice It is classified in many ways: A. Based on coloration of sclera T” Medical jaundice (yellow coloration) 2. Surgical jaundice (greenish yellow coloration) B. Based on aetiology of jaundice i 1, Haemolytic or prehepatic (excessive destruction of RBCs) 2. Hepatic (cause lies inside the liver) ® Scanned with CamScanner sear an Heatrnaaly o (couse 1 outside the liver) sa ure of bilfrubin Sasol hyperbilinubinaemia’ WS cent hyperbilinibinaemia — (conjugated 2 Fn 50% of total bliin) Set off limit for conugated. hyperbilrubingemia { r Sx more, caries clinical significance because Sho osrstion ether nse oF outside the live. haemoltic: is predominantly unconjugated hepatic jaundice could fall in either or may 18 yal characters of hoemobss and abstraction, The jaundice is mainly of conjugated type, vet of jaundice irrespective of its type or occurs either due to excessive destruction of SS sth overproduction of bilirubin or due to decreased * conuagation in the liver or due to impaired ‘een through liver. The causes are tabulated (Table i) The characteristics of two types of bilirubin are Served in Tole 3:32. ith Predominantly. Unconjugated petbilirubinaemis trnay result from different mechanisms: 1. Overproduction of bilirubin: This occurs due to cessive destruction of RBCs in peripheral blood or enthroid cells in the bone marrow due to any cause. This is reflected in the blood as unconjugated ‘A Haemolysis (excessive destruction of RBCs with ‘overproduction of unconjugated bilirubin) — « Intracorpuscular or extracorpuscular defects. U + Drugs induced especially in patients with G6PD efciency ‘Infections, eg., malaria, viral + Autoimmune B. Decreased uptake of bilirubin * Drugs Sepsis '* Congenital (Gilbert's syndrome) * Hepatitis (acute or chronic), decompensated cirrhosis ©. Decreased conjugation,of bilirubin. (decreased activity of glucurony! transferase) + Neonatal jaundice * Gibert's syndrome lajar (Type Land 1) syndrome c hhyperbiliubinaeria but levels usuolly remains below 5.0 mg%, There is akways slight increase in conjugated bilirubin also. 2, Decreased uptake of bilirubin by the liver cells results in unconjugated hyperbilirubinaemia which is induced by certain drugs and also occurs in congenital Gilbert's syndrome, 3. Impaired conjugation of bilirubin: Low glucuronyl transferase activily in newborn babies is a cause of neonatal jaundice seen at 2-5 days after birth Conjugation is defective in Gilbert's syndrome cither due to deficiency of glucurony! transferase or impaired uptake but in Criggler-Najar syndrome, there is deficiency (lype Itautosomal dominant) or absence (type Fautosomal recessive) of the glucuronyl transferase enzyme, Conjugating capacity of the liver may be impaired in certain acquired disorders such as hepatitis and cirthosis of the liver. Jaundice with Predominantly Conjugated Bilirubin in the Serum This occurs due to impaired excretion of bilirubin by the liver cells (Dubin-Johnson syndrome, cholestasis of pregnancy, benign intrahepatic cholestasis) or due to obstruction to the flow of bilirubin in bile canaliculi (intrahepatic cholestasis due to drugs, hepatitis or cholangitis, biliary cirrhosis) and ducts (extrahepatic biliary obstruction, e.g.. stone, stricture, tumour). The obstruction to the flow of bilirubin A. Intrahepatic cholestasis (obstruction to canaliculi or ductules) + Congenital (Dubin Johnson and Rotor syndrome) + Benign intrahepatic cholestasis (idiopathic recurrent Jaundice) + Chokestatic jaundice of pregnancy 4 + Drugs and alcohol i + Hepatitis (acute and chronic), decompensated civhots, + Primary biliary cinhosis 5 + Hodgkin's lymphoma + Postoperative + Bilary stricture s * Parasite (round worm) umour (bile duct, pancreas, duodenum) + Secondaties at portahepatis or mphnodes + Pancreatitis + Choledochal eyst f Ss e te: Vit epatis usually preduces predominant ncongsted but conned or med yperirutinaemia can oc Scanned with CamScanner PRP occsoasat Siren ela Water sohibility Nil + ASfnity for lipids + Nil © Renal excretion Nil + Van den Bergh reaction Indirect Direct High Low Reversibility of binding to albumin inside oF outside the liver leads to its back diffusion into blood resulting in conjugated hyperbilirubinaemia. ical and Biochemical Analysis of a Patient with Jaundice The clinical workup of a case with jaundice is depicted in Table 3.3.3 and important points to be asked in history and signs to be seen on examination are depicted in Box 1. All the three main types of jaundice are individually discussed below. BOX 1 CLINICAL EVALUATION OF JAUNDICE. History + Duration of jaundice. * Presence and character of abdominal pain, fever, and ‘ther symptoms of inflammatory disease. + Any change in appetite, taste, weight and bowel habits ‘+ Any history of blood transfusions, LV. injection, ‘unprotected sexual activity with partner other than wile, alcohol use. + Detailed drug history. + Prurtus or itching * Colour of urine and stools. + History of pregnancy. + Epistaxis, haematemesis or bleeding tendency, + Symptoms of encephalopathy. Physical examination Look for the following: ‘Anaemia Scratch marks ’ Pulse rate 50% of the total {anbin). Serum cholesterol and alkaline phosphatase Jes are taised. Serum transaminases (SGOT/PT) are ‘@her normal or mildly raised. USG of abdomen shows sation of intrahepatic and extrahepatic biliary system! * Hepatocellular jaundice bis associated with hepatomegaly, which may be tender in hepatitis or malignancy. Jaundice is usually a mixed type with conjugated and unconjugated bilirubin being ssised equally or either may be dominant depending on tbe cause, Urine may be dark coloured but stool is not cay coloured. Pruritus is present in those cases where there is intrahepatic cholestasis. Similarly, if cholestasis 's present in the liver, biochemically it will have same uracterstc features as discussed in obstructive jaundice But if hepatocellular jaundice is not associated with cholestasis, then serum transaminases would be highly raised with normal alkaline phosphatase. Prothrombin tine (PT, PTTK) may be increased. Serum albumin level may fall. Serum cholesterol is normal or low. USG liver shows enlarged liver with normal echopattern. Haemolytic jaundice Patient complains of jaundice with dark coloured stools and tine. Anaemia is present. There is no pruritus or itching, ‘The liver and spleen may be enlarged due to anaemia and Shu! * ; haemolysis. There may be history of a precipitating event such as fever (malaria, viral) or intake of drugs or heavy metals. In some cases, no factor may be found out. Blood examination is essential because peripheral ‘blood may show anisocytosis, poikilocytosis, increased reticulocytes with presence of abnormal RBCs {spherocytes, ovalocytes, sickle shaped cells). Anaemia is usually normocytic normochromic. Urine and stools show increased amount of urobilinogen and stercobilinogen respectively. Bilirubin is mildly elevated (< 5.0 mg%) with predominant unconjugated fraction, SGOT may be elevated but SGPT remains normal. Red cell fragility test, Van den Bergh reaction, Coombs test, test for cold agglutinins will establish the diagnosis in case of doubt. Based on the investigations, the main three types of jaundice are compared in Table 3.3.4. Congenital (Familial) Hyperbilirubinaemia Itis of two types: |. Familial unconjugated hyperbilirubinaemia (impaired/ defective conjugation in the liver due to decrease in activity of glucoronyl transferase enzyme) *Gilbert’s syndrome (mild decrease in activity of enzyme) * Crigler-Najjar syndrome (moderate to severe deficiency of enzyme} Type | autosomal recessive with complete absence of enzyme. Type Il autosomal dominant with partial absence of enzyme (decreased activity) 7 * Petipheral blood film ‘Abnormal RBCs (spheroeytes, N N covalo cytes) may be present in ‘congenital haemolytic anaemia 42 Urine for urobilinogen Present in 1:40 dilution or more Present in 1:20-40 dilution (+) (4) = positive: | | Gort 6 12 Stacl for sterecbin 7 \ (tt oy be (9 a 0) * Urine for bile salts and pigments (-) frvahopaie choles ° | 5:15 ma% 10-20 ma : eee 35 ma a |! ee disse eee Mixed or ether dominant Conjugated type . | ene ei a Nort 11 300 KA unt) \} | Atatine phosphatase N t fof senm cholesterol ie t | ox SGOT maybe slighty raised N ) 677 Sern Warsaminases SGOT/?PT - N or SGOT May be abnormal N i + PTVPTTK Liver enlarged. Liver-enlarged | isciane (Oe Eehutnire (ord. dled sand | Vv dtl iene 1 ncrensd; 11 = mately ierensed | a a ! Scanned with CamScanner @ _ = 1. Farnial Conjugated byperblirubinaemia (Defective excretion) * Dubin Johnson (autosomal recessive) syndrome —= Unit I: Diseases A Hepatchitn L Spry enlarged Iner because if liver is displaced dows becomes palpable. {Ther Arians, relore, before commenting ¢ enlargement of the Jiver. its upper border ra. tut be defo * Rotor's (autosomal dominant) syndrome by percussion in middsdcular ling Sicrtnaly, lee deca © Benign recurrent intrahepaiechoestassrecument ars nih tera space bat may be gy dee familial intrahepatic cholestasis. dovmvards in hypoasthenic patients) Liver enlaraennes | « Progressive Joniial Intrahepatle cholestasis ay be limited to one lobe (ight lobe) on beth va eet | syndromes and lel) or left lobe, Left lobe is normally papas | + Intrahepatic cholestasls of pregnancy eg epigastrium, in 21 t2 7 In case of enlargement of liver cn sheald lo \] Gilbert's syndrome _ (1) exdent of enlargement, (2) tenderness. (3) dae | 1} This most Commod familial unconjugated +? or blunt) (4) surface (smooth or nodular) Gy ecness =? hyperbilirubinaemla “that occurs due to defective» (fren, soft or hard) Massive enlargement of iver irene Conjugation, (Most patents avo have reduced levels of * plata, halazar. amoebic abscess, malignancy fect) (HVG.Br 1) encoding this enzyme This abnormality (mild deficiency) appears 10 be ae for this enzyme but is not in itself sufficient for the phenotypic expression of this syndrome. , 4 Most pallents are asymptomatic [A ver icon tests are normal apd there are no signs of chronic liver diseases, ‘About 25% patients give family history of jaundical, Rise 2) “if bilirubin Is an incidental finding, detected on routine _ check up oF during any illness. The normal reticulocyte count excludes haemolysis. No treatment ‘is required, / Crigler-Najar syndrome ip ‘This is very sara,” Two types aréTecognised depending Tie plpble ner does not mean) ‘and supine _positio lucoronyl transferase activity—the enzyme that conjugates _~‘Constrictive pericarditis, h bilirubin with glucuronic acid) Mutations occur in the gene 2,_¢nlargement is seen in feukaemias, congesti, Z 7 The causes of hepatomegaly are given in Table 3.3.5, tis, hepatic vein thrombosis ve heart fa: reliculoendothelal malignancy, hepatitis, des is seen in typhoid fever, anaemia, toxaema cr sept and haemolysis, Tender hepatomegaly occurs in Fepatt malignancy, hepatic vein thrombosis, congestive car failure, constrictive pericarditis,, The soft liver indicates congestive heart failure, hepatitis, leukaemia, heemolytic anaemia Firm hepatomegaly occurs in malaria, kala-anaz lymphoma, postnecrotic cirthosis,|Hard nodular lier is due to malignancy whether primary or secondary. Causes con whether the enzyme glucuronyl transferase is absent EEenOr enn (type 1) or deficient (type 2). There—is_unconjugated “ 1, Vascular (a) Congestive heart failure (CHF) hyperbilirubinaemia. Mutation of HVG-Br I gene for the

    You might also like