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Parenchymal GI Glands: Liver

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Pathology of Childhood and Adolescence

Abstract

The liver, as one of the most central glands of the body, is the most driving force in the development of the embryo and fetus. The study of the primitive biliary system is crucial for the detection and correct management of numerous cholestasis syndromes of neonatal age and infancy. The explanation of the slowdown period of bile duct morphogenesis between 22 and 32 weeks gestation as well as ductal plate malformation will be an integral part of this chapter. The clarification of the neonatal iron storage disorder as gestational alloimmune liver disease and the identification of numerous molecular mechanisms for familiar intrahepatic cholestasis have been the pillars in the last decade to address the management of these infants. The presentation of several cholestatic liver diseases as well as metabolic disorders is in this chapter and will be accompanied by the introduction of benign, malignant tumors that may target the liver in childhood and youth.

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  1. University of Alberta, Edmonton, AB, Canada

    Consolato M. Sergi

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Multiple Choice Questions and Answers

Multiple Choice Questions and Answers

  • HEP-1 Cytoskeleton is made up of microtubules, actin filaments, and intermediate filaments and plays a significant role in the cell. These structures frame the cell shape and help organize the cell compartments as well as provide a basis for cellular movement and cell division. Cytokeratins are an essential component of intermediate filaments of epithelial tissue. Which of the following statement is TRUE?

    1. (a)

      Epithelial cells forming bile ducts express CK-8 and CK-18 in addition to CK-7 and CK-20, the latter two being also positive in normal adult hepatocytes.

    2. (b)

      Epithelial cells forming bile ducts express CK-7 and CK-20 in addition to CK-8 and CK-18, the latter two being also positive in normal adult hepatocytes.

    3. (c)

      Epithelial cells forming bile ducts express CK-7 and CK-19 in addition to CK-8 and CK-18, the latter two being also positive in normal adult hepatocytes.

    4. (d)

      Epithelial cells forming bile ducts express CK-7 and CK-20 in addition to CK-5 and CK-6, the latter two being also positive in normal adult hepatocytes.

  • HEP-2 Which of the following statements regarding fibrocystin is NOT correct?

    1. (a)

      The PKHD1 gene provides a protein called fibrocystin (aka polyductin).

    2. (b)

      More than 270 mutations in the PKHD1 gene have been identified in individuals with polycystic kidney disease, which is autosomal dominant inherited.

    3. (c)

      The PKHD1 gene is located on 6p12.3-p12.2.

    4. (d)

      PKHD1 is present in fetal and adult kidney cells, the liver, and the pancreas.

    5. (e)

      Fibrocystin may act as a receptor, interacting with extracellular molecules.

    6. (f)

      Fibrocystin is also found in primary cilia.

  • HEP-3 The paucity of the interlobular bile ducts (PIBD) can be part of a familial syndrome of cholestasis named Alagille syndrome (AGS) or occurs as non-syndromic PIBD. Which of the following conditions is NOT presenting as PIBD, non-syndromic type?

    1. (a)

      Kartagener syndrome

    2. (b)

      Progressive intrahepatic familial cholestasis (PFIC)

    3. (c)

      Maternal use of progesterone during pregnancy

    4. (d)

      Hemophagocytic lymphohistiocytosis

    5. (e)

      Congenital pancreatic hypoplasia

  • HEP-4 Which material is NOT required in the pediatric pathologist’s liver biopsy cart?

    1. (a)

      Upright light microscope

    2. (b)

      Inverted light microscope

    3. (c)

      Containers of 10% buffered formalin, EM grade 2.5% glutaraldehyde in 0.1M buffer glutaraldehyde, and Hank’s Balanced Salt Solution with a sterile urine specimen cup containing two drops of Hank’s medium

    4. (d)

      Sterile scalpel blades, aluminum foil, the plastic mold containing OCT (optimal cutting temperature) compound, and Dewar flask of liquid nitrogen

    5. (e)

      Glass slides, Coplin jars of Diff-Quik staining solutions, and large-caliber squeeze-bulb soft plastic pipettes

  • HEP-5 What is the most common cause of neonatal cholestasis?

    1. (a)

      Neonatal sclerosing cholangitis

    2. (b)

      The paucity of the interlobular bile ducts

    3. (c)

      Biliary atresia

    4. (d)

      Progressive familial intrahepatic cholestasis

    5. (e)

      Cystic fibrosis-related hepatopathy

  • HEP-6 Which of the following etiologies of conjugated hyperbilirubinemia is NOT correct?

    1. (a)

      Cholelithiasis

    2. (b)

      Primary biliary cirrhosis

    3. (c)

      Hepatitis B Virus (HBV) hepatitis

    4. (d)

      Rotor syndrome

    5. (e)

      Crigler-Najjar syndrome type I

  • HEP-7 Autoimmune hepatitis (AIH) is a relapsing and remitting immune-mediated liver disease that most commonly affects females in their 20s and 40s, but it may be seen in children and adolescents as well. It should be diagnosed following exclusion of other etiologies (e.g., viral hepatitis, drug reaction). What are the histologic hallmarks of AIH?

    1. (a)

      Hepatitis dominated by plasma cells and interface activity potentially accompanied by emperipolesis and pseudorosettes

    2. (b)

      Hepatitis with pericellular fibrosis and lobular disarray

    3. (c)

      The paucity of interlobular bile ducts with spotty necrosis in the liver lobules

    4. (d)

      Hepatitis with bile duct proliferation with emperipolesis and pseudorosettes

  • HEP-8 Computed tomography scan of the abdomen in a young patient with a liver neoplasm shows a central scar inside of the mass. In addition to a fibrolamellar carcinoma of the liver, which condition should be considered in the differential diagnosis?

    1. (a)

      Mesenchymal hamartoma of the liver

    2. (b)

      Focal nodular hyperplasia

    3. (c)

      Nodular regenerative hyperplasia

    4. (d)

      Scirrhous variant of conventional hepatocellular carcinoma

    5. (e)

      Metastatic rhabdomyosarcoma

  • HEP-9 Aflatoxins are a family of toxins produced by certain fungi, mostly Aspergillus flavus and Aspergillus parasiticus, that are found on farming crops such as maize (corn), peanuts, cottonseed, and tree nuts. Which neoplasm is associated with exposure to aflatoxins?

    1. (a)

      Mesenchymal hamartoma of the liver

    2. (b)

      Cholangiocellular carcinoma

    3. (c)

      Hepatocellular carcinoma

    4. (d)

      Embryonal sarcoma of the liver

    5. (e)

      Hepatoblastoma

  • HEP-10 Which molecular biological abnormality is associated with the fibrolamellar carcinoma of the liver?

    1. (a)

      MYCN amplification

    2. (b)

      DNAJB1–PRKACA fusion transcript

    3. (c)

      BRCA2 amplification

    4. (d)

      t(2;5)(p23;q35)

    5. (e)

      NPM-ALK fusion protein

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Sergi, C.M. (2020). Parenchymal GI Glands: Liver. In: Pathology of Childhood and Adolescence. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-59169-7_4

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