Tag: Hyperbilirubenimia

Hyperbilirubenimia

Hyperbilirubinemia

Q. 1

Unconjugate hyperbilirubinemia is seen in

 A

Physiological jaundice

 B

Breast milk jaundice

 C

Gilbert syndrome

 D

All

Q. 1

Unconjugate hyperbilirubinemia is seen in

 A

Physiological jaundice

 B

Breast milk jaundice

 C

Gilbert syndrome

 D

All

Ans. D

Explanation:

Ans. is ‘a’ i.e., Physiological jaundice; ‘b’ i.e., Breast milk jaundice; ‘c’ i.e., Gilbert syndrome


Q. 2

Unconjugated hyperbilirubinemia is seen in

 A

Rotor syndrome

 B

Dubin-Johnson syndrome

 C

Biliary atresia

 D

Crigler-Najjar syndrome

Q. 2

Unconjugated hyperbilirubinemia is seen in

 A

Rotor syndrome

 B

Dubin-Johnson syndrome

 C

Biliary atresia

 D

Crigler-Najjar syndrome

Ans. D

Explanation:

Ans. is ‘d’ i.e., Crigler-Najjar syndrome

Predominantly Unconjugated fliperbilirubinenrict

Excess production of bilirubin Hemolytic anemias

Resorption of blood from internal hemorrhage (e.g. alimentary

tract bleeding, hematomas)

Ineffective erythropoiesis syndromes (e.g. pernicious anemia, thalassemia)

Reduced hepatic uptake

Drug interference with membrance carrier systems

Some cases of Gilbert syndrome Impaired bilirubin conjugation

Physiologic jaundice of the newborn (decreased UGT I A I activity,

decreased excretion)

Breast milk jaundice (b-glucrurondases in milk)

Genetic deficiency of UGT 1 AI activity (Crigler-Najjar syndrome types I and II)

Gilbert syndrome (mixed etiologies)

Diffuse hepatocellular disease (e.g. viral or drug-induced hepatitis, cirrhosis)

Predominantly conjugated hyperbilirubinemia

Deficiency of canalicular membrane transporters (Dubin-Johnson syndrome, Rotor syndrome) Impaired bile flow.


Q. 3

Causes of conjugated hyperbilirubinemia is ‑

 A

Rotor syndrome

 B

Breast milk jaundice

 C

Crigler najjar

 D

Gilbert syndrome

Q. 3

Causes of conjugated hyperbilirubinemia is ‑

 A

Rotor syndrome

 B

Breast milk jaundice

 C

Crigler najjar

 D

Gilbert syndrome

Ans. A

Explanation:

Ans. is ‘a’ i.e., Rotor syndrome

Quiz In Between


Q. 4

Indirect hyperbilirubinemia are seen in

 A

Dubin-Johnson syndrome

 B

Rotor syndrome

 C

Gilbert syndrome

 D

Gallstone

Q. 4

Indirect hyperbilirubinemia are seen in

 A

Dubin-Johnson syndrome

 B

Rotor syndrome

 C

Gilbert syndrome

 D

Gallstone

Ans. C

Explanation:

Ans is ‘c’ i.e., Gilbert syndrome


Q. 5

Unconjugated hyperbilirubinemia in neonate is seen in all of the following except –

 A

Physiological jaundice

 B

Dubin johnson syndrome

 C

Hypothyroidism

 D

Hemolytic anemia

Q. 5

Unconjugated hyperbilirubinemia in neonate is seen in all of the following except –

 A

Physiological jaundice

 B

Dubin johnson syndrome

 C

Hypothyroidism

 D

Hemolytic anemia

Ans. B

Explanation:

Ans. is ‘b’ i.e., Dubin Johnson Syndrome


Q. 6

Autosomal dominant familial nonhemolytic hyperbilirubinemia occurs in all except –

 A

Crigler-Najjar syndrome

 B

Dubin – Johnson syndrome

 C

Gilbert syndrome

 D

Cryoglobulinemia

Q. 6

Autosomal dominant familial nonhemolytic hyperbilirubinemia occurs in all except –

 A

Crigler-Najjar syndrome

 B

Dubin – Johnson syndrome

 C

Gilbert syndrome

 D

Cryoglobulinemia

Ans. B

Explanation:

Ans. is ‘b’ i.e., Dubin-Johnson syndrome

Dubin-Johnson syndrome is an autosomal recessive disorder.

o Note sure about option d.

Quiz In Between


Q. 7

Hyperbilirubinemia in a child can be due to

 A

Breast milk jaundice

 B

Cystic fibrosis

 C

Fanconi’s syndrome

 D

All

Q. 7

Hyperbilirubinemia in a child can be due to

 A

Breast milk jaundice

 B

Cystic fibrosis

 C

Fanconi’s syndrome

 D

All

Ans. A

Explanation:

Ans. is ‘a’ i.e., Breast milk jaundice


Q. 8

Following are causes of unconjugated hyperbilirubinemia, except:

 A

Hemolytic anemia

 B

Large hematoma

 C

Rotor syndrome

 D

Megaloblastic anemia

Q. 8

Following are causes of unconjugated hyperbilirubinemia, except:

 A

Hemolytic anemia

 B

Large hematoma

 C

Rotor syndrome

 D

Megaloblastic anemia

Ans. C

Explanation:

Answer is C (Rotor syndrome)

Rotor’s syndrome is an Autosomal recessive inherited disorder characterized by a deject in biliary excretion leading to conjugated hyperbilirubinemia:

Indirect hyperbilirubinemia                                                              

Direct hyperbilirubinemia

A.   Hemolytic disorders

A.   Inherited conditions

1.    Inherited

1.     Dubin-Johnson syndrome

a.   Sperocyteosis, elliptocytosis

2.     Rotor’s syndrome

Glucose-6-phosphate dehydrogenase and pyruvate kinase deficiencies

b. Sickle cell anemia

 

2.    Acquired                         _

a. Microangiopathic hemolytic anemias

b. Paraoxysmal nocturnal hemoglobinuria

c. Immune hemolysis

 

B.    Ineffective erythropoesis

 

1.    Cobalamin, folate, thalassemia, and severe iron deficiencies

 

C. Drugs

 

1.    Rifampicin, probenbecid, ribavirin

 

D.   Inherited conditions

 

1.    Crigler-Najjar types I and II

 

2.    Glibert’s syndrome

 


Q. 9

A patient presents with unconjugated hyperbilirubinemia and presence of urobilinogen in urine. Which amongst the following is the least likely diagnosis:

 A

Hemolytic jaundice

 B

Crigler Najjar syndrome

 C

Gilbert’s syndrome

 D

Dubin Johnson syndrome

Q. 9

A patient presents with unconjugated hyperbilirubinemia and presence of urobilinogen in urine. Which amongst the following is the least likely diagnosis:

 A

Hemolytic jaundice

 B

Crigler Najjar syndrome

 C

Gilbert’s syndrome

 D

Dubin Johnson syndrome

Ans. D

Explanation:

Answer is D (Dubin Johnson Syndrome)

Dubin Johnson syndrome is associated with conjugated hyperbilirubinemia & not unconjugated hjperbilirubinemia. Dubin Johnson Syndrome results from a hereditary defect in excretion of conjugated bilirubin across the canalicular membrane and leads to conjugated hyperbilirubinemia.

Dubin Johnson syndrome is an inherited disorder charachterized by defective excretion of conjugated bilirubin from hepatocytes into biliary canaliculi. It thus presents with a clinical picture similar to obstructive jaundice with conjugated hyperbilirubinemia and absence of urobilinogen in urine.

Hemolytic Anemia typically presents with unconjugated hyperbilirubinemia and elevated urinary urobilinogens. Gilberts syndrome and Cri2ler Najjar syndrome also present with unconjugated hyperbilirubinemia. Urinary Urobilinogens are however not elevated in these conditions. Urobilinogen may never the less be present in urine (N or in these conditions

Quiz In Between


Q. 10

Conjugated hyperbilirubinemia is seen in all EX­CEPT:

March 2013

 A

Dubin Johnson syndrome

 B

Rotor syndrome

 C

Gilbert syndrome

 D

None of the above

Q. 10

Conjugated hyperbilirubinemia is seen in all EX­CEPT:

March 2013

 A

Dubin Johnson syndrome

 B

Rotor syndrome

 C

Gilbert syndrome

 D

None of the above

Ans. C

Explanation:

Ans. C i.e. Gilbert syndrome

Gilbert syndrome presents with unconjugated hyperbilirubinemia


Q. 11

Unconjugated hyperbilirubinemia is seen in all of the following except:   

March 2010

 A

Crigler Najjar Syndrome

 B

Physiological jaundice

 C

Dubin-Johnson syndrome

 D

Gilbert syndrome

Q. 11

Unconjugated hyperbilirubinemia is seen in all of the following except:   

March 2010

 A

Crigler Najjar Syndrome

 B

Physiological jaundice

 C

Dubin-Johnson syndrome

 D

Gilbert syndrome

Ans. C

Explanation:

Ans. C: Dubin-Johnson Syndrome

Dubin-Johnson syndrome is an autosomal recessive disorder that causes an increase of conjugated bilirubin without elevation of liver enzymes (ALT, AST).

This condition is associated with a defect in the ability of hepatocytes to secrete conjugated bilirubin into the bile.

The conjugated hyperbilirubinemia is a result of defective endogenous and exogenous transfer of anionic conjugates from hepatocytes into the bile.

Pigment deposition in lysosomes causes the liver to turn black.

Other causes of conjugated/direct hyperbilirubinemia:

  • Hepatocellular diseases:

– Hepatitis:

  • Neonatal idiopathic hepatitis
  • Viral (Hepatitis B, C, TORCH infections)
  • Bacterial (E. colt, urinary tract infections)

–        Total parenteral nutrition

–        Hepatic ischemia (post-ischemic damage)

–        Erythroblastosis fetalis (late, “Inspissated Bile Syndrome”)

Metabolic disorders:

  • Alpha-1 antitrypsin deficiency
  • Galactosemia, tyrosinemia, fructosemia
  • Glycogen storage disorders
  • Cystic fibrosis

Biliary tree abnormalities:

–         Extrahepatic biliary atresia: In first 2 weeks, unconjugated bilirubin predominates; elevated conjugated bilirubin is late.

–        Paucity of bile ducts

–        Choledochal cyst

–        Bile plug syndrome

Causes of unconjugated/indirect hyperbilirubinemia:

  • Increased lysis of RBCs (i.e., increased hemoglobin release)

–        Isoimmunization (blood group incompatibility: Rh, ABO and minor blood groups)

–        RBC enzyme defects (e.g., G6PD deficiency, pyruvate kinase deficiency)

–        RBC structural abnormalities (hereditary spherocytosis, elliptocytosis)

–        Infection (sepsis, urinary tract infections)

–        Sequestered blood (e.g., cephalohematoma, bruising, intracranial hemorrhage)

–        Neonatal Jaundice

–        Polycythemia

–        Shortened life span of fetal RBCs

Decreased hepatic uptake and conjugation of bilirubin

–        Immature glucuronyl transferase activity in all newborns: term infants have 1% of adult activity, preterm infants have 0.1%.

–        Gilbert Syndrome

–        Crigler Najjar Syndrome (Non-hemolytic Unconjugated Hyperbilirubinemia): inherited conjugation defect (very rare)

–        Breastmilk Jaundice (pregnanediol inhibits glucuronyl transferase activity)

Increased enterohepatic reabsorption

–        Breastfeeding jaundice (due to dehydration from inadequate milk supply)


Q. 12

Conjugated hyperbilirubinemia

 A

Dubin johnson syndrome

 B

Criggler naj jar syndrome

 C

Breast milk jandice

 D

Gilbert syndrome

Q. 12

Conjugated hyperbilirubinemia

 A

Dubin johnson syndrome

 B

Criggler naj jar syndrome

 C

Breast milk jandice

 D

Gilbert syndrome

Ans. A

Explanation:

Ans. is ‘a’ i.e., Dubin johnson syndrome

Breast milk jaundice –

  • Decrease bilirubin uptake across hepathocyte membrane.
  • Entero-hepatic recirculation.
  • Leads to indirect hyperbilirubinemia.

Crigler naj jar & Gilbert syndrome (deficiency of glucuronyl transferase)

  • Decrease conjugation leads to Indirect hyperbilirubinemia.
  • Defect in hepatocyte secretion of conjugated bilirubin.
  • Leads to direct hyperbilirubinemia

Quiz In Between



Hyperbilirubenimia

Hyperbilirubenimia


HYPERBILIRUBINEMIAS

JAUNDICE

  • Sclera icterus indicates bilirubin >3mg/dL.
  • Sclera icterus in fetus bilirubin >15mg/dL

 Congenital Hyperbilirubinemia

  • Unconjugated hyperbilirubinemias
  1. Glibert’s disease
  2. Crigler- Najjar syndrome
  3. Physiological Jaundice
  4. Breast Milk Jaundice
  5. Lucey Driscoll Syndrome

Conjugated Hyperbilirubinemias

  • Dubin Johnson’s syndrome- caused due to mutation of gene encoding MRP2.
  • Rotor syndrome- defective bile excretion
  • Benign recurrent intrahepatic cholestatsis
  • Progressive Familial intrahepatic cholestatsis

ACQUIRED HYERBILIRUBINEMIA

  • Hemolytic jaundice
  • Hepatic jaundice
  • Obstructive jaundice 

Exam Important

  • Sclera icterus in fetus bilirubin >15mg/dL

Congenital Hyperbilirubinemia

  • Unconjugated hyperbilirubinemias
  1. Glibert’s disease
  2. Crigler- Najjar syndrome
  • Physiological Jaundice
  • Breast Milk Jaundice
  • Lucey Driscoll Syndrome

Conjugated Hyperbilirubinemias

  • Dubin Johnson’s syndrome- caused due to mutation of gene encoding MRP2.
  • Rotor syndrome- defective bile excretion
  • Benign recurrent intrahepatic cholestatsis
  • Progressive Familial intrahepatic cholestatsis
Don’t Forget to Solve all the previous Year Question asked on Hyperbilirubenimia

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