Medical Archive

C381 Morphogenesis of the Liver and Biliary System

C382 Manifestations of Liver Disease

C383 Cholestasis

C383.1 Neonatal cholestasis

Evaluation

Intrahepatic cholestasis

• Neonatal hepatitis
• Disorders of Transport, Secretion, Conjugation, and Biosynthesis of Bile Acids

Bile acid–coenzyme a ligase deficiency

Disorders of embryogenesis

Biliary atresia

• Pathogenesis
  • Extrahepatic bile duct fibrosis
• Clinical findings
  • Infants age 2-8 weeks
  • Jaundice, acholic stools, dark urine, hepatomegaly
• Diagnostic evaluation
  • Direct hyperbilirubinemia
  • Ultrasound
    • Absent/abnormal gallbladder &/or CBD
  • Liver biopsy
    • Intrahepatic bile duct proliferation
    • Portal tract edema
    • Fibrosis
  • Intraoperative cholangiography (GOLD STANDARD)
    • Biliary obstruction
• Treatment
  • Immediate surgical hepatoportoenterostomy (Kasai procedure) ? reestablish bile flow to the small intestine.
  • Liver transplant ? most will eventually require.

C383.2 Cholestasis in the older child

Biliary cyst

• Epidemiology
  • Most commonly seen in patients of Asian descent and
• Clinical findings
  • Cholestatic jaundice and, if left untreated, increases the risk of cholangitis and cholangiocarcinoma.
  • In rare cases, a biliary cyst may involve the cystic duct and result in the gallbladder dilation
• Abdominal ultrasonography
  • Dilation of the gall bladder and a fusiform dilation of the extrahepatic bile duct
  • Dilated masses that communicate with the biliary tree.
• Treatment
  • Surgical excision of the cyst, followed by the creation of a biliary-enteric anastomosis with a Roux-en-Y hepaticojejunostomy.

C384 Metabolic Diseases of the Liver

C384.1 Inherited De?cient Conjugation of Bilirubin (Familial Nonhemolytic Unconjugated Hyperbilirubinemia)

Gilbert syndrome

Deficiency of serum glucuronosyltransferase

Criggler-Najjar syndrome type 1

Criggler-Najjar syndrome type 2

Inherited conjugated hyperbilirubinemia

Dubin-Johnson syndrome

• Discovered in puberty or early adulthood
• Episodes of jaundice can be triggered by intercurrent illness, pregnancy, or the use of oral contraceptives.
• Lab findings
  • Normal LFTs
  • Urine: bilirubinuria
  • Liver biopsy
    • Grossly black liver
    • Dark, lysosomal pigments on histology

Rotor syndrome

C384.2 Wilson disease

<Harrison> P12 C408 Wilson’s Disease

C385 Viral Hepatitis

Issues common to all forms of viral hepatitis

Hepatitis A

Hepatitis B

Perinatal hepatitis B infections

• Epidemiology
  • 90% risk of vertical transmission without prophylaxis
  • <2% risk after prophylaxis
• Risk factors
  • High maternal viral load
  • Maternal HBeAg+
• Transmission
  • Perinatal exposure to genital secretions (m/c)
  • Transplacental (rare, 2.5%)
  • Not transmitted by breastfeeding.
• Clinical features
  • Viral replication occurs rapidly in infected infants d/t immune system immaturity in newborns.
  • Despite this high level of replication, infants eneter an immune-tolerant phase
• Prevention
  • HBV vaccine + HBIG
  • Must be administered together, within 12 hours of birth, regardless of the infant’s birth weight or clinical condition

Hepatitis C

Hepatitis D

Hepatitis E

Approach to acute or chronic hepatitis

C386 Liver Abscess

C387 Liver Disease Associated With Systemic Disorders

C388 Mitochondrial Hepatopathies

Primary mitochondrial hepatopathies

Secondary mitochondrial hepatopathies

Reye syndrome

• Epidemiology & etiology
  • In the past, the m/c secondary mitochondrial hepatopathy, the prevalence of which peaked in the 1970s and had a mortality rate of >40%
  • Fulminant liver failure and encephalopathy in children with viral illness who take aspirin
• Clinical features
  • N/V, hypoglycemia, acute liver failure
  • Encephalopathy
  • May progress to coma and death
• Lab findings
  • ?AST, ALT, PT, INR, PTT
  • ?NH3 – neurotoxic
• Pathology
  • Sinusoidal congestion and. hemorrhagic necrosis
  • LM: Microvesicular steatosis (cf. macroveisicular: AH, NAFLD)
  • EM: swelling, a decreased number of mitochondria and glycogen depletion

Treatment

• No effective therapy.
• Current treatment strategies are supportive and include the infusion of sodium bicarbonate for acute metabolic acidosis, low carbohydrate diet may decrease the lactic acidosis, transfusions for anemia and thrombocytopenia, and exogenous pancreatic enzymes for pancreatic insufficiency.
• ?? ??? ?? ??
  • ??? ??, ??? (? ???? ????), mannitol, Pb
• ??? ??: 10-15% dextrose solution
• ??? ??: vitamin K, FFP
• ?? ????: neomycin, lactulose ??
• Avoid aspirin in children, except in those with Kawasaki disease

C389 Autoimmune Hepatitis

C390 Drug- and Toxin-Induced Liver Injury

C391 Acute Hepatic Failure

C392 Cystic Diseases of the Biliary Tract and Liver

C393 Diseases of the Gallbladder

C394 Portal Hypertension and Varices

C395 Liver Transplantation