Pediatric Current Medical Diagnosis & Treatment – Chapter 22: Liver & Pancreas | Verified Questions and Answers

PEDIATRIC CURRENT MEDICAL
DIAGNOSIS AND TREATMENT :CH22:
LIVER & PANCREAS WITH
QUESTIONS AND ANSWERS WRITTEN
CORRECTLY


• What is upper intestinal obstruction? - correct answer.💖💖💖💖✔<<<<<Obstruction in the
upper part of the intestines.



• What are some causes of indirect hyperbilirubinemia in newborns? - correct
answer.💖💖💖💖✔<<<<<Duodenal atresia, annular pancreas, pyloric stenosis.



• What is the mechanism behind indirect hyperbilirubinemia in newborns with high intestinal
obstruction? - correct answer.💖💖💖💖✔<<<<<Unknown.



• What is found on liver biopsy in pyloric stenosis? - correct
answer.💖💖💖💖✔<<<<<Diminished levels of hepatic glucuronyl transferase.



• What genetic condition may be indicated by indirect hyperbilirubinemia in newborns with high
intestinal obstruction? - correct answer.💖💖💖💖✔<<<<<Gilbert syndrome.



• What is the treatment for upper intestinal obstruction? - correct
answer.💖💖💖💖✔<<<<<Usually surgical.



• When does jaundice disappear in cases of upper intestinal obstruction? - correct
answer.💖💖💖💖✔<<<<<Once adequate nutrition is achieved.

,• What is Gilbert syndrome? - correct answer.💖💖💖💖✔<<<<<A common form of familial
hyperbilirubinemia.



• What percentage of the population is affected by Gilbert syndrome? - correct
answer.💖💖💖💖✔<<<<<3%-7%.



• What is the association of Gilbert syndrome with hepatic bilirubin uridine diphosphate-
glucuronyl transferase activity? - correct answer.💖💖💖💖✔<<<<<Partial reduction.



• What symptoms may affected infants with Gilbert syndrome experience in the newborn period?
- correct answer.💖💖💖💖✔<<<<<More rapid increase in jaundice.



• What type of jaundice is accentuated in infants with Gilbert syndrome? - correct
answer.💖💖💖💖✔<<<<<Breast milk jaundice.



• What symptoms may individuals with Gilbert syndrome experience during puberty and beyond?
- correct answer.💖💖💖💖✔<<<<<Mild 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? - correct answer.💖💖💖💖✔<<<<<Reduced activity of enzymes involved in
heme biosynthesis.



• What treatment has been used to reduce hyperbilirubinemia in patients with Gilbert syndrome?
- correct answer.💖💖💖💖✔<<<<<Administration of phenobarbital.



• Is phenobarbital therapy necessary for patients with Gilbert syndrome? - correct
answer.💖💖💖💖✔<<<<<No, it is not needed.



• What is the disease inherited as? - correct answer.💖💖💖💖✔<<<<<An abnormality of the
promoter region of UGT1A1.

,• What is UGT1A1? - correct answer.💖💖💖💖✔<<<<<Uridine diphosphate-glucuronyl
transferase-1.



• What is necessary for disease expression? - correct answer.💖💖💖💖✔<<<<<Another
factor.



• What are the common states of the disease? - correct
answer.💖💖💖💖✔<<<<<Homozygous (16%) and heterozygous (40%).



• Which gender is affected more often? - correct answer.💖💖💖💖✔<<<<<Males (4:1).



• What is the typical range of serum unconjugated bilirubin? - correct
answer.💖💖💖💖✔<<<<<Less than 3-6 mg/dL.



• Can the level of unconjugated bilirubin exceed 8 mg/dL? - correct
answer.💖💖💖💖✔<<<<<Yes, in unusual cases.



• What are the findings on liver biopsy and most LFTs? - correct
answer.💖💖💖💖✔<<<<<Normal.



• What increase in unconjugated bilirubin level after a 2-day fast is consistent with the diagnosis
of Gilbert syndrome? - correct answer.💖💖💖💖✔<<<<<1.4 mg/dL or more.



• Can Gilbert syndrome occur following liver transplantation? - correct
answer.💖💖💖💖✔<<<<<Yes, if conferred by the donor liver.



• Is genetic testing for Gilbert syndrome commonly needed? - correct
answer.💖💖💖💖✔<<<<<No, it is rarely needed.



• Is treatment necessary for Gilbert syndrome? - correct answer.💖💖💖💖✔<<<<<No
treatment is necessary.

, • What is another name for Crigler-Najjar Syndrome? - correct answer.💖💖💖💖✔<<<<<E.
Crigler-Najjar Syndrome.



• What is the article reference for more information on inherited disorders of bilirubin transport
and conjugation? - correct answer.💖💖💖💖✔<<<<<Erlinger 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? - correct answer.💖💖💖💖✔<<<<<Kathemann 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? - correct answer.💖💖💖💖✔<<<<<Travan 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? - correct answer.💖💖💖💖✔<<<<<A genetic disorder
characterized by severe unconjugated hyperbilirubinemia.



• What are the neurologic consequences of Crigler-Najjar syndrome? - correct
answer.💖💖💖💖✔<<<<<Kernicterus.



• How is the deficiency in UGT1A1 inherited? - correct answer.💖💖💖💖✔<<<<<Autosomal
recessive.



• Is consanguinity often present in Crigler-Najjar syndrome? - correct
answer.💖💖💖💖✔<<<<<Yes.



• What is the definitive test for Crigler-Najjar syndrome? - correct
answer.💖💖💖💖✔<<<<<Genetic testing of UGT1A1.