Chapter 22: Pediatric Current Medical Diagnosis &
Treatment: Liver & Pancreas.
100% Certified Questions and Answers.
Latest Updated 2025/2026.
What is upper intestinal obstruction? - ansObstruction in the upper part of the intestines.
What are some causes of indirect hyperbilirubinemia in newborns? - ansDuodenal atresia,
annular pancreas, pyloric stenosis.
What is the mechanism behind indirect hyperbilirubinemia in newborns with high intestinal
obstruction? - ansUnknown.
What is found on liver biopsy in pyloric stenosis? - ansDiminished levels of hepatic
glucuronyl transferase.
What genetic condition may be indicated by indirect hyperbilirubinemia in newborns with
high intestinal obstruction? - ansGilbert syndrome.
What is the treatment for upper intestinal obstruction? - ansUsually surgical.
When does jaundice disappear in cases of upper intestinal obstruction? - ansOnce adequate
nutrition is achieved.
What is Gilbert syndrome? - ansA common form of familial hyperbilirubinemia.
What percentage of the population is affected by Gilbert syndrome? - ans3%-7%.
What is the association of Gilbert syndrome with hepatic bilirubin uridine diphosphate-
glucuronyl transferase activity? - ansPartial reduction.
What symptoms may affected infants with Gilbert syndrome experience in the newborn
period? - ansMore rapid increase in jaundice.
What type of jaundice is accentuated in infants with Gilbert syndrome? - ansBreast milk
jaundice.
What symptoms may individuals with Gilbert syndrome experience during puberty and
beyond? - ansMild fluctuating jaundice, especially with illness and vague constitutional
symptoms.
What is thought to cause shortened red blood cell survival in some patients with Gilbert
syndrome? - ansReduced activity of enzymes involved in heme biosynthesis.
What treatment has been used to reduce hyperbilirubinemia in patients with Gilbert
syndrome? - ansAdministration of phenobarbital.
Is phenobarbital therapy necessary for patients with Gilbert syndrome? - ansNo, it is not
needed.
What is the disease inherited as? - ansAn abnormality of the promoter region of UGT1A1.
What is UGT1A1? - ansUridine diphosphate-glucuronyl transferase-1.
What is necessary for disease expression? - ansAnother factor.
What are the common states of the disease? - ansHomozygous (16%) and heterozygous
(40%).
Which gender is affected more often? - ansMales (4:1).
What is the typical range of serum unconjugated bilirubin? - ansLess than 3-6 mg/dL.
Can the level of unconjugated bilirubin exceed 8 mg/dL? - ansYes, in unusual cases.
What are the findings on liver biopsy and most LFTs? - ansNormal.
,Chapter 22: Pediatric Current Medical Diagnosis &
Treatment: Liver & Pancreas.
100% Certified Questions and Answers.
Latest Updated 2025/2026.
What increase in unconjugated bilirubin level after a 2-day fast is consistent with the
diagnosis of Gilbert syndrome? - ans1.4 mg/dL or more.
Can Gilbert syndrome occur following liver transplantation? - ansYes, if conferred by the
donor liver.
Is genetic testing for Gilbert syndrome commonly needed? - ansNo, it is rarely needed.
Is treatment necessary for Gilbert syndrome? - ansNo treatment is necessary.
What is another name for Crigler-Najjar Syndrome? - ansE. Crigler-Najjar Syndrome.
What is the article reference for more information on inherited disorders of bilirubin transport
and conjugation? - ansErlinger S et al: Inherited disorders of bilirubin transport and
conjugation: new insights into molecular mechanisms and consequences. Gastroenterology
2014 Jun;146(7):1625-1638 [PubMed: 24704527].
What is the article reference for more information on Gilbert syndrome in children after liver
transplantation? - ansKathemann S et al: Gilbert syndrome—a frequent cause of
unconjugated hyperbilirubinemia in children after orthotopic liver transplantation. Pediatr
Transplant 2012;16:20 [PubMed: 22360405].
What is the article reference for more information on severe neonatal hyperbilirubinemia and
UGT1A1 promoter polymorphism? - ansTravan L et al: Severe neonatal hyperbilirubinemia
and UGT1A1 promoter polymorphism. J Pediatr 2014 Jul;165(1):42-45 [PubMed:
24726540].
What is Crigler-Najjar syndrome? - ansA genetic disorder characterized by severe
unconjugated hyperbilirubinemia.
What are the neurologic consequences of Crigler-Najjar syndrome? - ansKernicterus.
How is the deficiency in UGT1A1 inherited? - ansAutosomal recessive.
Is consanguinity often present in Crigler-Najjar syndrome? - ansYes.
What is the definitive test for Crigler-Najjar syndrome? - ansGenetic testing of UGT1A1.
What is the recommended treatment for Crigler-Najjar syndrome? - ansExchange
transfusions followed by phototherapy.
Does phenobarbital administration significantly alter the findings of Crigler-Najjar
syndrome? - ansNo.
Does phenobarbital lower serum bilirubin levels in Crigler-Najjar syndrome? - ansNo.
What combination of treatments may keep bilirubin levels below 25 mg/dL in Crigler-Najjar
syndrome? - ansAggressive phototherapy and cholestyramine.
Can orlistat therapy decrease bilirubin in Crigler-Najjar syndrome? - ansYes, in a subset of
patients.
Is liver transplantation curative for Crigler-Najjar syndrome? - ansYes.
Can liver transplantation prevent kernicterus in Crigler-Najjar syndrome? - ansYes, if
performed early.
,Chapter 22: Pediatric Current Medical Diagnosis &
Treatment: Liver & Pancreas.
100% Certified Questions and Answers.
Latest Updated 2025/2026.
What is the limitation of hepatocyte transplantation for Crigler-Najjar syndrome? -
ansRequirement of multiple infusions of cells over time.
What are the current clinical trials for Crigler-Najjar syndrome? - ansAdeno-associated virus
vector-mediated gene therapy and mesenchymal stem cell therapy.
What is the milder form of Crigler-Najjar syndrome? - ansType 2.
How is the milder form of Crigler-Najjar syndrome inherited? - ansBoth autosomal dominant
and recessive.
Are neurologic complications common in the milder form of Crigler-Najjar syndrome? -
ansRarely.
How does hyperbilirubinemia in the milder form of Crigler-Najjar syndrome compare to the
severe form? - ansLess severe.
What is the response to phenobarbital treatment in the milder form of Crigler-Najjar
syndrome? - ansLowering of serum bilirubin levels.
Are liver biopsy findings and liver function tests normal in both types of Crigler-Najjar
syndrome? - ansYes.
What is drug-induced hyperbilirubinemia? - ansElevation of bilirubin levels caused by certain
medications.
Which medication can elevate indirect bilirubin levels by causing hemolysis? - ansVitamin
K3 (menadiol).
Which medication can be safely used in neonates to prevent hyperbilirubinemia? -
ansVitamin K1 (phytonadione).
Which medication can cause conjugated hyperbilirubinemia in infancy? - ansCarbamazepine.
Which medications may cause unconjugated hyperbilirubinemia? - ansRifampin and
antiretroviral protease inhibitors (PIs; atazanavir).
Which drugs have been implicated in causing neonatal jaundice? - ansPancuronium bromide
and chloral hydrate.
Which drugs may displace bilirubin from albumin, increasing the risk of kernicterus? -
ansCeftriaxone and sulfonamides.
What is conjugated noncholestatic hyperbilirubinemia? - ansA condition characterized by
elevated conjugated bilirubin levels.
What are the two types of conjugated noncholestatic hyperbilirubinemia? - ansDubin-Johnson
Syndrome and Rotor Syndrome.
What are the suspected diagnoses when persistent or recurrent conjugated hyperbilirubinemia
and jaundice occur with normal LFTs? - ansDubin-Johnson syndrome and Rotor syndrome.
What is the basic defect in Dubin-Johnson syndrome? - ansImpaired hepatocyte excretion of
conjugated bilirubin into bile due to a defect in the multiple organic anion transport protein 2
(MRP2) of the bile canaliculus.
, Chapter 22: Pediatric Current Medical Diagnosis &
Treatment: Liver & Pancreas.
100% Certified Questions and Answers.
Latest Updated 2025/2026.
What is the basic defect in Rotor syndrome? - ansDeficiency in hepatic uptake and storage of
bilirubin due to a deficiency in OATP1B1 and OATP1B3 transporters.
How is Dubin-Johnson syndrome transmitted? - ansAutosomal recessive.
What is the appearance of the liver in Dubin-Johnson syndrome? - ansDarkly pigmented on
gross inspection and may be enlarged.
What is the appearance of the liver in Rotor syndrome? - ansNormal.
What is the microscopic examination finding in Dubin-Johnson syndrome? - ansNumerous
dark-brown pigment granules consisting of polymers of epinephrine metabolites, especially
in the centrilobular regions.
Is the amount of pigment in the liver consistent within families in Dubin-Johnson syndrome?
- ansNo, it varies within families and some jaundiced family members may have no
demonstrable pigmentation in the liver.
What is the histological appearance of the liver in Dubin-Johnson syndrome? - ansOtherwise
normal.
What is the appearance of the gallbladder in Dubin-Johnson syndrome on oral
cholecystography? - ansIt fails to visualize.
What is the appearance of the gallbladder in Rotor syndrome on oral cholecystography? -
ansNormal.
How can Dubin-Johnson syndrome and Rotor syndrome be distinguished? - ansBy
differences in the excretion patterns of bromosulfophthalein, results of HIDA
cholescintigraphy, urinary coproporphyrin I and III levels, and the serum pattern of
monoglucuronide and diglucuronide conjugates of bilirubin.
Is clinical genotyping available for Dubin-Johnson syndrome, Rotor syndrome, and their
associated transporters? - ansYes, clinical genotyping of MRP2, SLCO1B1, and SLCO1B3 is
available.
Is treatment needed for Dubin-Johnson syndrome or Rotor syndrome? - ansNo treatment is
needed for either condition.
Can choleretic agents be used to reduce cholestasis in infants with Dubin-Johnson syndrome?
- ansYes, choleretic agents such as UDCA may reduce cholestasis in infants with Dubin-
Johnson syndrome.
What is HAV? - ansHepatitis A virus
What is HBV? - ansHepatitis B virus
What is HBcAg? - ansHBV core antigen
What is Anti-HBs? - ansAntibody to HBsAg
What is Anti-HBc IgM? - ansIgM antibody to HBcAg
What is HCV? - ansHepatitis C virus
What is HDV? - ansHepatitis D (delta) virus
What is HEV? - ansHepatitis E virus
Treatment: Liver & Pancreas.
100% Certified Questions and Answers.
Latest Updated 2025/2026.
What is upper intestinal obstruction? - ansObstruction in the upper part of the intestines.
What are some causes of indirect hyperbilirubinemia in newborns? - ansDuodenal atresia,
annular pancreas, pyloric stenosis.
What is the mechanism behind indirect hyperbilirubinemia in newborns with high intestinal
obstruction? - ansUnknown.
What is found on liver biopsy in pyloric stenosis? - ansDiminished levels of hepatic
glucuronyl transferase.
What genetic condition may be indicated by indirect hyperbilirubinemia in newborns with
high intestinal obstruction? - ansGilbert syndrome.
What is the treatment for upper intestinal obstruction? - ansUsually surgical.
When does jaundice disappear in cases of upper intestinal obstruction? - ansOnce adequate
nutrition is achieved.
What is Gilbert syndrome? - ansA common form of familial hyperbilirubinemia.
What percentage of the population is affected by Gilbert syndrome? - ans3%-7%.
What is the association of Gilbert syndrome with hepatic bilirubin uridine diphosphate-
glucuronyl transferase activity? - ansPartial reduction.
What symptoms may affected infants with Gilbert syndrome experience in the newborn
period? - ansMore rapid increase in jaundice.
What type of jaundice is accentuated in infants with Gilbert syndrome? - ansBreast milk
jaundice.
What symptoms may individuals with Gilbert syndrome experience during puberty and
beyond? - ansMild fluctuating jaundice, especially with illness and vague constitutional
symptoms.
What is thought to cause shortened red blood cell survival in some patients with Gilbert
syndrome? - ansReduced activity of enzymes involved in heme biosynthesis.
What treatment has been used to reduce hyperbilirubinemia in patients with Gilbert
syndrome? - ansAdministration of phenobarbital.
Is phenobarbital therapy necessary for patients with Gilbert syndrome? - ansNo, it is not
needed.
What is the disease inherited as? - ansAn abnormality of the promoter region of UGT1A1.
What is UGT1A1? - ansUridine diphosphate-glucuronyl transferase-1.
What is necessary for disease expression? - ansAnother factor.
What are the common states of the disease? - ansHomozygous (16%) and heterozygous
(40%).
Which gender is affected more often? - ansMales (4:1).
What is the typical range of serum unconjugated bilirubin? - ansLess than 3-6 mg/dL.
Can the level of unconjugated bilirubin exceed 8 mg/dL? - ansYes, in unusual cases.
What are the findings on liver biopsy and most LFTs? - ansNormal.
,Chapter 22: Pediatric Current Medical Diagnosis &
Treatment: Liver & Pancreas.
100% Certified Questions and Answers.
Latest Updated 2025/2026.
What increase in unconjugated bilirubin level after a 2-day fast is consistent with the
diagnosis of Gilbert syndrome? - ans1.4 mg/dL or more.
Can Gilbert syndrome occur following liver transplantation? - ansYes, if conferred by the
donor liver.
Is genetic testing for Gilbert syndrome commonly needed? - ansNo, it is rarely needed.
Is treatment necessary for Gilbert syndrome? - ansNo treatment is necessary.
What is another name for Crigler-Najjar Syndrome? - ansE. Crigler-Najjar Syndrome.
What is the article reference for more information on inherited disorders of bilirubin transport
and conjugation? - ansErlinger S et al: Inherited disorders of bilirubin transport and
conjugation: new insights into molecular mechanisms and consequences. Gastroenterology
2014 Jun;146(7):1625-1638 [PubMed: 24704527].
What is the article reference for more information on Gilbert syndrome in children after liver
transplantation? - ansKathemann S et al: Gilbert syndrome—a frequent cause of
unconjugated hyperbilirubinemia in children after orthotopic liver transplantation. Pediatr
Transplant 2012;16:20 [PubMed: 22360405].
What is the article reference for more information on severe neonatal hyperbilirubinemia and
UGT1A1 promoter polymorphism? - ansTravan L et al: Severe neonatal hyperbilirubinemia
and UGT1A1 promoter polymorphism. J Pediatr 2014 Jul;165(1):42-45 [PubMed:
24726540].
What is Crigler-Najjar syndrome? - ansA genetic disorder characterized by severe
unconjugated hyperbilirubinemia.
What are the neurologic consequences of Crigler-Najjar syndrome? - ansKernicterus.
How is the deficiency in UGT1A1 inherited? - ansAutosomal recessive.
Is consanguinity often present in Crigler-Najjar syndrome? - ansYes.
What is the definitive test for Crigler-Najjar syndrome? - ansGenetic testing of UGT1A1.
What is the recommended treatment for Crigler-Najjar syndrome? - ansExchange
transfusions followed by phototherapy.
Does phenobarbital administration significantly alter the findings of Crigler-Najjar
syndrome? - ansNo.
Does phenobarbital lower serum bilirubin levels in Crigler-Najjar syndrome? - ansNo.
What combination of treatments may keep bilirubin levels below 25 mg/dL in Crigler-Najjar
syndrome? - ansAggressive phototherapy and cholestyramine.
Can orlistat therapy decrease bilirubin in Crigler-Najjar syndrome? - ansYes, in a subset of
patients.
Is liver transplantation curative for Crigler-Najjar syndrome? - ansYes.
Can liver transplantation prevent kernicterus in Crigler-Najjar syndrome? - ansYes, if
performed early.
,Chapter 22: Pediatric Current Medical Diagnosis &
Treatment: Liver & Pancreas.
100% Certified Questions and Answers.
Latest Updated 2025/2026.
What is the limitation of hepatocyte transplantation for Crigler-Najjar syndrome? -
ansRequirement of multiple infusions of cells over time.
What are the current clinical trials for Crigler-Najjar syndrome? - ansAdeno-associated virus
vector-mediated gene therapy and mesenchymal stem cell therapy.
What is the milder form of Crigler-Najjar syndrome? - ansType 2.
How is the milder form of Crigler-Najjar syndrome inherited? - ansBoth autosomal dominant
and recessive.
Are neurologic complications common in the milder form of Crigler-Najjar syndrome? -
ansRarely.
How does hyperbilirubinemia in the milder form of Crigler-Najjar syndrome compare to the
severe form? - ansLess severe.
What is the response to phenobarbital treatment in the milder form of Crigler-Najjar
syndrome? - ansLowering of serum bilirubin levels.
Are liver biopsy findings and liver function tests normal in both types of Crigler-Najjar
syndrome? - ansYes.
What is drug-induced hyperbilirubinemia? - ansElevation of bilirubin levels caused by certain
medications.
Which medication can elevate indirect bilirubin levels by causing hemolysis? - ansVitamin
K3 (menadiol).
Which medication can be safely used in neonates to prevent hyperbilirubinemia? -
ansVitamin K1 (phytonadione).
Which medication can cause conjugated hyperbilirubinemia in infancy? - ansCarbamazepine.
Which medications may cause unconjugated hyperbilirubinemia? - ansRifampin and
antiretroviral protease inhibitors (PIs; atazanavir).
Which drugs have been implicated in causing neonatal jaundice? - ansPancuronium bromide
and chloral hydrate.
Which drugs may displace bilirubin from albumin, increasing the risk of kernicterus? -
ansCeftriaxone and sulfonamides.
What is conjugated noncholestatic hyperbilirubinemia? - ansA condition characterized by
elevated conjugated bilirubin levels.
What are the two types of conjugated noncholestatic hyperbilirubinemia? - ansDubin-Johnson
Syndrome and Rotor Syndrome.
What are the suspected diagnoses when persistent or recurrent conjugated hyperbilirubinemia
and jaundice occur with normal LFTs? - ansDubin-Johnson syndrome and Rotor syndrome.
What is the basic defect in Dubin-Johnson syndrome? - ansImpaired hepatocyte excretion of
conjugated bilirubin into bile due to a defect in the multiple organic anion transport protein 2
(MRP2) of the bile canaliculus.
, Chapter 22: Pediatric Current Medical Diagnosis &
Treatment: Liver & Pancreas.
100% Certified Questions and Answers.
Latest Updated 2025/2026.
What is the basic defect in Rotor syndrome? - ansDeficiency in hepatic uptake and storage of
bilirubin due to a deficiency in OATP1B1 and OATP1B3 transporters.
How is Dubin-Johnson syndrome transmitted? - ansAutosomal recessive.
What is the appearance of the liver in Dubin-Johnson syndrome? - ansDarkly pigmented on
gross inspection and may be enlarged.
What is the appearance of the liver in Rotor syndrome? - ansNormal.
What is the microscopic examination finding in Dubin-Johnson syndrome? - ansNumerous
dark-brown pigment granules consisting of polymers of epinephrine metabolites, especially
in the centrilobular regions.
Is the amount of pigment in the liver consistent within families in Dubin-Johnson syndrome?
- ansNo, it varies within families and some jaundiced family members may have no
demonstrable pigmentation in the liver.
What is the histological appearance of the liver in Dubin-Johnson syndrome? - ansOtherwise
normal.
What is the appearance of the gallbladder in Dubin-Johnson syndrome on oral
cholecystography? - ansIt fails to visualize.
What is the appearance of the gallbladder in Rotor syndrome on oral cholecystography? -
ansNormal.
How can Dubin-Johnson syndrome and Rotor syndrome be distinguished? - ansBy
differences in the excretion patterns of bromosulfophthalein, results of HIDA
cholescintigraphy, urinary coproporphyrin I and III levels, and the serum pattern of
monoglucuronide and diglucuronide conjugates of bilirubin.
Is clinical genotyping available for Dubin-Johnson syndrome, Rotor syndrome, and their
associated transporters? - ansYes, clinical genotyping of MRP2, SLCO1B1, and SLCO1B3 is
available.
Is treatment needed for Dubin-Johnson syndrome or Rotor syndrome? - ansNo treatment is
needed for either condition.
Can choleretic agents be used to reduce cholestasis in infants with Dubin-Johnson syndrome?
- ansYes, choleretic agents such as UDCA may reduce cholestasis in infants with Dubin-
Johnson syndrome.
What is HAV? - ansHepatitis A virus
What is HBV? - ansHepatitis B virus
What is HBcAg? - ansHBV core antigen
What is Anti-HBs? - ansAntibody to HBsAg
What is Anti-HBc IgM? - ansIgM antibody to HBcAg
What is HCV? - ansHepatitis C virus
What is HDV? - ansHepatitis D (delta) virus
What is HEV? - ansHepatitis E virus
