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Peds GI RC > Liver > Flashcards

Liver Flashcards

1
Q

List 8 Liver Manifestations of CF

A 
Cholestasis
Hepatic steatosis
Bridging Fibrosis
Focal Biliary Cirrhosis (** pathognomonic)
Mulitlobular cirrhosis
Portal Hypertension
Cholelithasis (up to 10% of pts)
Extrahepatic strictures (beading similar to PSC)
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2
Q

List 10 genetic causes of Hepatic Steatosis

A 
NAFLD
FAOD
Mitochondrial Disorder
Citrin Deficiency
Wilson's disease
DM
Lipodystrophy
LAL deficiency (Wolman dz)
Familial Hyperlipidemia
Abeta/hypobeta lipoproteinemia
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3
Q

List 7 medications that cause Hepatic Steatosis

A 
Amiodarone
Steroids
Methotrexate
Antipsychotics
Antidepressants
HAART
VAP
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4
Q

List 4 Dietary causes of Hepatic Steatosis

A

Malnutrition
ETOH
Rapid weight loss
PN

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5
Q

What infection can cause hepatic steatosis

A

Hep C genotype 3

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6
Q

What are factors affecting the prognosis of BA and Kasai

A

Age at Kasai

  • 80% survival if w/n 60 days
  • 20% survival if > 90 days
  • size of ducts in the porta hepatis
  • bacterial cholangitis
  • intrhepatic PV thrombosis
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7
Q

List 5 risk factors for fractures posit liver transplant

A
  • Older age at transplant
  • Male Sex
  • fracture prior to Ltx
  • low body weight
  • cumulative steroid dose
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8
Q

Indications for bisphosphonates post liver txt

A

-low bone mass with vertebral fracture, a lower extremity fracture or 2 upper extremity fractures

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9
Q

Which vessel supplies blood to the bile ducts

A

The hepatic artery

-GGT, bili and ALP are sensitive indicators of HAT

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10
Q

How is Alagille Syndrome Inherited and which genes are involved

A

Autosomal Dominant
JAG1 (95%)
NOTCH2 (5%)

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11
Q

What are the clinical features of Alagille Syndrome (6)

A

Ductopenia on liver bx
Characteristic Alagille facies (broad forehead, small pointy chin)
Posterior embryotoxin
Butterfly vertebrae
Renal disease
Cardiac defects - peripheral pulmonic stenosis or TOF

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12
Q

Precipitants for Hepatic Encephalopathy (10)

A

i. Nitrogenous:
- uremia
- azotemia
- excessive dietary protein
- GIB
- infection (SBP)
- TIPS
- dehydration (aggressive diuretics)
- metabolic alkalosis
- hypokalemia
- constipation
ii. Non-nitrogenous:
- sedatives (benzodiazepine, barbiturates)
- hypoxia
- hypoglycemia
- hypothyroidism
- anemia

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13
Q

Complications of Choledochal Cyst (4)

A
  • Spontaneous perforation of the CBD in infants
  • biliary cirrhosis
  • end stage liver failure
  • recurrent cholangitis
  • increased risk of cholangiocarcinoma
  • recurrent pancreatitis
  • fat malabsorption
  • portal hypertension
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14
Q

Treatment for Portal Gastropathy

A
  • beta-blockers
  • somatostatin analogue (octreotide)
  • iron supplementation (chronic blood loss)
  • TIPS (Transjugular Intrahepatic Portosystemic Shunt)
  • blood transfusions
  • liver transplant
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15
Q

Hepatobiliary Complications of TPN

A 
●	Steatosis
●	Steatohepatitis
●	Cholestasis
●	Fibrosis
●	Micronodular cirrhosis
●	Phospholipidosis
●	Biliary sludge
●	Cholelithiasis
●	Acalculous cholecystitis
Infants
●	Cholestasis
●	Fibrosis
●	Cirrhosis
●	Hepatocellular carcinoma
●	Biliary sludge
●	Abdominal pseudotumor
●	Cholelithiasis
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16
Q

Acquired metabolic diseases associated with NASH

A
  • insulin resistance
  • diabetes mellitus type 2
  • dyslipidemia
    • hypertriglycidemia
      • high LDL
      • low HDL
  • obesity
  • systemic hypertension
  • thyroid disorders
  • atherosclerosis
  • PCOS
  • cirrhosis
  • hepatocellular carcinoma
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17
Q

DDX for Non-excreting HIDA

A
  • biliary atresia
  • choledochal cyst
  • alagille syndrome
  • Rotor Syndrome (defect in glutathione s transferase - GST - urine coproporphyrin ratio of I:III < 80%, )
  • Dubin-Johnson syndrome (defect in MRP2 - poor excretion of bile acids into bile ducts, urine coproporphyrin ratio of I:III > 80%; black liver) - lipofuscian)
  • neonatal sclerosing cholangitis
  • obstruction of cystic duct by stone
  • false positive: hyperbilirubinemia (bile doesn’t make it down - why we give phenobarb, prolonged fasting, TPN, severe parenchymal disease - don’t uptake
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18
Q

Lab Findings Associated with Refractory Ascites

A

Labs (Paracenthesis):

  • protein<2.5g/dL,
  • neutrophils (PMNs) <250cells/mm3, mostly lymphocytes
  • low glucose, increased LDH, increased amylase

Serum:

  • hypoalbuminemia
  • hyponatremia (high ADH)
  • thrombocytopenia (from splenomegaly)
  • anemia (EV bleeding)
  • increased INR
  • Increased Globulins - Globulins tend to be increased in patients with cirrhosis. This may be secondary to shunting of bacterial antigens in portal venous blood away from the liver to lymphoid tissue which induces immunoglobulin production
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19
Q

Treatment options for Ascites

A
  • diuretics with Spironolactone
  • Restricted Na diet (lower limit of normal for age eg. 2mmol/kg/day)
  • fluid restriction
  • colloid replacement with 25% albumin
  • paracentesis
  • Transjugular Intrahepatic Portosystemic Shunt (TIPS)
  • Shunt from PV to HV , therefore risk of hepatic encephalopathy
  • Rex Shunt for PV thrombosis (aka mesoportal shunt)
  • Shunt from mesenteric vein (or low portal vein) to left portal vein, low risk of encephalopathy
  • transplantation
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20
Q

Risk factors of Esophageal Variceal Bleed (8)

A
  • thrombocytopenia*** (C Gana & SC Ling published in 2011)
  • splenomegaly*** (C Gana & SC Ling published in 2011)
  • PV:HV gradient >12mmHg
  • Large, tense varices
  • Red wale marks, red spots on varices
  • More severe liver disease (Child Pugh C) (from sleisenger Ch 90)
  • Total bili >170
  • Sepsis
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21
Q

Causes of Biliary Stricture Post Liver Txt (5)

A
  • history of graft rejection
  • recurrent PSC
  • anastomotic stricture
  • complication of recurrent cholangitis
  • history of HAT
  • ischemia
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22
Q

Diagnostic Criteria for AIH

A

Constellation of findings – AASLD Criteria:
- Abnormal liver biochemistry
- Elevated IgG
- Positive ANA/ASMA/ALKM Ab
- liver biopsy consistent with AIH (interface hepatitis,
lobular necrosis, lymphocytic infiltration)
- other diseases on differential ruled out (hepatitis
viruses, wilson disease, a1At…)
- minimal drug use / alcohol consumption
- family history of autoimmune diseases

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23
Q

Four Medications Used for AIH

A
  • prednisone/prednisolone
  • azathioprine
  • mycophenolate mofetil
  • budesonide

Other treatments that have been proposed include mycophenolate mofetil, budesonide, cyclosporine A, tacrolimus, 6-MP, methotrexate, ursodeoxycholic acid, rapamycin and rituximab although experience with all these agents is limited”

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24
Q

Treatment for Hepatorenal Syndrome (4)

A
  • liver transplantation
  • hemodialysis
  • avoidance of nephrotoxic drugs (eg aminoglycozides)
  • low protein diet
  • midrodrine & octreotide & terlipressin
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25
Q

Pathophysiological Mechanisms of NAFLD

A
  • Insulin resistance from excessive accumulation of FFA
  • Elevated glucose activates carbohydrate response element-binding protein and elevated insulin levels promote de novo lipogenesis (DNL)
  • Leads to increased secretion of proinflammatory cytokines (TNF alpha, IL6, resistin, visfatin, plasminogen activatory inhibitor and decreased secretion of anti-inflammatory cytokine adiponectin (reduced in obesity, DM, insulin resistance
  • Increased availability of Fatty acids results in accumulation of intrahepatic TG and steatosis, over secretion of VLDL, various forms of dyslipidemia and increased glucose production
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26
Q

Pathophysiology of Steatohepatitis

A
  1. Direct cytotoxic effects of ↑FFA
  2. Impaired beta-oxidation of FFA
  3. Oxidative stress and lipid peroxidation
  4. Mitochondrial damage: ↑ROS, altered ATP homeostasis, structural abnormalities
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27
Q

Liver Histological Findings of GVHD

A
  • endothelialitis,
  • lymphocytic infiltration of the portal areas,
  • pericholangitis and
  • bile duct destruction: ductopenia, duct obliteration, duct fibrosis,
  • cholestasis

GI Histological findings of GVHD
The histologic hallmark of gastrointestinal GVHD is epithelial cell apoptosis. The grade of GVHD traditionally has been assigned based on the amount of apoptosis and epithelial loss, with grade I—increased epithelial cell apoptosis, grade II—single gland/crypt dropout, grade III—confluent gland/crypt dropout, and grade IV—ulceration and extensive loss of glands/crypts. With the exception of grade IV acute GVHD, histologic grading has not been shown to correlate with outcome; however, Paneth cell loss may predict outcome.

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28
Q

Causes of Low Grade SAAG (<11g/L)

A
  • Biliary ascities
  • Bowel obstruction/infarction
  • Nephrotic syndrome
  • Pancreatic ascites
  • Peritoneal carcinomatosis
  • Postoperative lymphatic leak
  • Serositis in connective tissue diseases
  • Tuberculous peritonitis
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29
Q

Causes of High Grade SAAG > 11g/L

A

-Alcoholic hepatitis
-Budd-Chiari syndrome
-Cardiac ascities
-Cirrhosis
-Fatty liver of pregnancy
Fulminant hepatic failure
-massive liver metases (mixed ascities)
-PHTN
-Portal vein thrombosis
-Sinusoidal obstructive syndrome

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30
Q

Extrahepatic Manifestations of Hepatitis B

A
  • Polyarteritis nodosa
  • Glomerular disease (membranous nephropathy and, less often, membranoproliferative glomerulonephritis)
  • Serum sickness-like syndrome manifested as fever, skin rashes, arthralgia, and arthritis, which usually subsides with the onset of jaundice.
  • Aplastic anemia
  • Gianotti Crosti: acrodermatitis + lymphadenopathy
  • Cryoglobulinemia
31
Q

Mechanisms of Action of Urso

A
  • Inhibiting absorption of toxic, hydrophobic, endogenous bile salts;
  • Stabilizing hepatocyte membranes against toxic bile salts;
  • Replacing endogenous bile acids, some of which may be hepatotoxic, with the nonhepatotoxic UDCA;
  • Reducing expression of major histocompatibility complex (MHC) class I and class II antigens
  • Reduces cholesterol levels
  • Ursodeoxycholic acid and/or chenodeoxycholic acid reduces cholesterol secretion and fractional reabsorption of cholesterol
  • They also increase bile acid concentration in bile, promoting dissolution of cholesterol stones
•	Ursodiol
o	Inhibiting HMG-CoA reductases
o	Blocks intestinal absorption of cholesterol
o	Reduces lipid peroxidation 
o	Reduces mucin secretagogue activity 
o	Diarrhea and hepatotoxicity are rare
•	Only for cholesterol stones
32
Q

Proven Indications for URSO

A
  • PBC
  • Post functional Kasai (after Kasai to improve drainage) versus if Kasai is not functioning well
  • Dissolution of radiolucent cholesterol gallstones
  • CF related disease
  • ? PSC (adults shown 30mg/kg harmful)
33
Q

Treatment for Hemochromatosis in Children

A

Symptomatic Kids: weekly phlebotomy 5-8 cc/kg until ferritin <300 then 2-4 times per yr

34
Q

Complications of ERCP (5)

A
  • sedation related
  • perforation
  • pancreatitis
  • bleeding
  • infection
35
Q

Name 2 primary bile acids

A

Cholic acid

Chenodeoxycholic acid

36
Q

How do secondary bile acids differ from primary bile acids in their structure?
Where does this happen?

A

Become deconjugated by bacterial to become more hydrophobic

In the intestines

37
Q

Risk of HBV transmission if mom is

a) HBeAg positive
b) HBeAg negative
c) if baby receives vaccine

A

a) 90%
b) 20%
c) 2%

38
Q

How to prevent transmission of Hep B to baby if Mom is HBV positive

A
  • vaccine within 24 hours, HBIG given within 7 days
39
Q

What is the management for PTLD

A
  • An immediate decrease or discontinuation of immunosuppression
  • Institution of anti-EBV therapy (ganciclovir & cytogam).
  • If tumor cells express B-cell marker CD 20 at histology, the anti-CD 20 monoclonal antibody rituximab has been used with increasing success.
40
Q

What are the secondary bile acids

A
  • lithocholic acid
  • deoxycholic acid
  • ursodeoxycholic acid
41
Q

Where does Urso come from?

A
  • secondary bile acid produced synthetically
  • large quantities in bear bile
  • small quantities in body (<5%)
  • produced by bacterial epimerization of C-7 hydroxyl group of CDCA to from ursodeoxycholic acid (UCDA)
42
Q

What are absolute and relative contraindications to transplantation?
According to AASLD 2014 guildelines

A

Absolute Contraindications:

  • HCC with extrahepatic disease and rapid progression
  • generalized extrahepatic malignancy (exception: hepatoblastoma with isolated pulmonary mets)
  • uncontrolled systemic infection
  • severe multi system mitochondrial diseases
  • valproate induced liver failure
  • niemann-pick disease type C
  • severe portopulmonary HTN not responsive to medical therapy

Relative Contraindications:

  • HCC with: venous invasion, rapid progression despite chemo
  • high certainty of non-adherence despite multidisciplinary interventions and support
  • HLH
  • critical circumstances not amenable to psychosocial intervention
43
Q

Diagnosis of SBP

A

Ascitic fluid: > 250 neutrophils/mm3 has highest sensitivity for diagnosis of SBP
-peritoneal bacterial cultures only positive in ~60% of cases

44
Q

Organisms causing SBP and Antibiotics for treatment

A
  • Ecoli
  • Klebsiella
  • GAS
  • Enterococcus

Abx: cefotaxime

45
Q

Secondary proph for SBP

A

-66% preventin of recurrence with nofloxacin, or septra or cipro

46
Q

What are the limitations of capsule endoscopy

A
  • Cannot provide therapeutic intervensions
  • Difficult to know exact localization of lesion
  • Time consuming to evaluate
  • Very long to perform
  • Very long to read results
  • Possibility of retained capsule/obstruction and need for surgical removal
47
Q

Syndromes/conditions associated with Hepatoblastoma (11)

A
  • increased in premature infants,
  • Beckwith Weideman syndrome,
  • FAP
  • Downs, T21
  • T18
  • Goldenhar,
  • Prader-Willi syndrome
  • GSD 1a
  • Meckel’s Diverticulum
  • tyrosinemia
  • alpha 1 antitrypsin deficiency
48
Q

Imaging findings associated with Hepatoblastoma

A
  • U/S: it usually appears as a solitary lesion, although it can be multifocal. The lesion has well defined margins and shows minimal increased echogenicity;
  • Unenhanced CT (UECT): calcification is seen in half of the cases
49
Q

Conditions associated with Hepatic Adenoma

A
  • young women
  • on OCP
  • pregnancy
  • glycogen storage disease
50
Q

Extrahepatic Manifestations of Wilson’s Disease

A
  • Ocular: Kayser-Fleischer ring
  • PSYCHIATRIC PRESENTATION (depression)
  • Neurologic presentation: movement disorder or rigid dystonia, seizures
  • Hematologic: Episodes of hemolytic anemia can result from the sudden release of copper into the blood.
  • Psychosocial - school dysfunction
  • Renal disease: mainly Fanconi’s syndrome, may be prominent. Findings include microscopic hematuria, aminoaciduria, phosphaturia, and defective acidification of the urine. Nephrolithiasis has also been reported.
  • MSK: Arthritis, affecting mainly the large joints, may occur as a result of synovial copper accumulation, Other musculoskeletal problems include osteoporosis and osteochondritis dissecans. Vitamin D–resistant rickets may develop as a result of the renal damage.
  • More MSK: Copper deposition in skeletal muscle can cause rhabdomyolysis.
  • Cardiac: Copper deposition in the heart can lead to cardiomyopathy or cardiac arrhythmias. Sudden death in Wilson disease has been attributed to cardiac involvement but is rare.
  • Endocrine: Hypoparathyroidism has been attributed to copper deposition. Amenorrhea and testicular problems appear to result from Wilson disease itself, not from cirrhosis.
    Infertility or repeated spontaneous abortion may be a sign of Wilson disease.
  • GI: Pancreatitis, possibly resulting from copper deposition in the pancreas, may also occur.
51
Q

Local Risk Factors of PVT

A

Cancer
-any abdominal organ

Focal Inflammatory Lesions

  • neonatal omphalitis, umbilical vein cath
  • diverticulitis, appendicitis
  • pancreatitis
  • duodenal ulcer
  • cholecysitis
  • tuberculous lymphadenitis
  • CD, UC
  • CMV hepatitis

Injury to the portal venous system

  • splenectomy
  • colectomy, gastrectomy
  • cholecystectomy
  • liver txt
  • abdominal trauma
  • surgical portosystemic shunting, TIPS,

Cirrhosis

  • preserved liver fxn with precipitating factors (splenectomy, surgical portosystemic shuntint, TIPS dysfunction, thrombophila)
  • Advanced disease in the absence of obvious precipitating factors
52
Q

Risk factors of Acalculous cholecystitis

A

a. Sepsis
b. Gastroenteritis
c. Abdominal trauma or surgery
d. Extensive burns
e. Shock, cardiac resuscitation
f. IV nutrition and prolonged fasting
g. Systemic inflammatory diseases – SLE, Kawasaki disease, polyarteritis nodosa
h. Malignancy
i. Congestive heart failure
j. cytotoxic drugs (chemo)
k. infections (AIDS, CMV, EBV, Salmonella, Staph Aureus)

53
Q

US Findings of Acalculous cholecystitis (6)

A

1) Gallbladder distension
2) Gallbladder wall thickening
3) Sludge but not stones
4) Pericholecystic fluid
5) Sonographic Murphy sign
6) Intramural gas sometimes occurs
7) Abscess and perforation rare

54
Q

Long term effects of immunosuppression post Liver TXT despite weaning

A
  1. HTN
  2. Renal impairment
  3. PTLD
  4. Risk of infection
  5. Dyslipidemia
  6. Risk of PRESS
  7. Hyperglycemia/diabetes
  8. New food allergies
55
Q

Diagnostic Criteria for Hepatorenal syndrome (5)

A

-cirrhosis with ascities
serum creatinine level >/ 1.5 mg/dL (133umol/L)
-no or insufficient improvement in serum Cr, 48 hours after withdrawing diuretics and adequate volume expansion with IV Alb
-absence of shock
-No evidence of recent use of nephrotoxic agents
-absence of intrinsic renal disease

Major criteria
§ Chronic / acute liver disease with advanced hepatic failure and portal HTN
§ ↓GFR (↑Cr >132, ↓creatinine clearance)
§ Absence of Rx with nephrotoxic drugs, shock, infection, substantial recent fluid losses
§ Lack of sustained improvement in renal function following diuretic withdrawal and volume expansion with 1.5L NS
§ Urinary protein <500mg/dL and lack of US evidence of parenchymal renal disease or obstruction

·     Minor criteria
§  Urine volume <500ml/d
§  Urine Na <10mEq/L
§  Urine osmolality > plasma osmolality
§  Urine RBC < 50 per HPF
§  Serum Na < 130
56
Q

Components of bile

A
  • bile acids
  • phospholipids
  • bilirubin
  • proteins (Ig’s)
  • GGT
  • ALP
  • metals (copper, iron, zinc, manganese)
  • cholesterol
  • drug metabolites (xenobiotics)
  • Cl
  • H20
  • Na
57
Q

List 3 canalicular transports and the diseases which they are associated with

A

FIC1: PFIC1 / FIC1 deficiency - ATP8B1
- defect in canalicular surface protein FIC1

BSEP: PFIC2 / BSEP deficiency - ABCB1
-mutation in bile transport into the canaliculus

MDR3: PFIC3 / MDR3 deficiency -ABCB4
- mutation in gene coding transporter MDR3 - affecting phosphatidylcholine secretion into bile canaliculus

58
Q

Risk factors for development of TPN Cholestasis

A
  1. Prematurity
  2. Soybean lipid source
  3. Repeated sepsis
  4. Poor bowel function/bowel resection/short bowel
  5. Longer duration of TPN
  6. Low birth weight
  7. Carbohydrate excess
  8. Amino acid excess
  9. High Mn & Copper levels
  10. Carnitine deficiency
59
Q

Mechanisms of TPN Cholestasis

A
  • Enteral starvation results in decreased gut motility, mucosal atrophy, and bacterial overgrowth. The result may be production of hepatotoxic agents such as bacterial by-products, endotoxin, and lithocholate and translocation of bacteria and bacterial products into the portal circulation
  • Systemic infections that predispose to cholestasis occur frequently. Patients are exposed to hepatotropic viruses through blood product transfusions and receive potentially hepatotoxic drugs. The infusate may contain potentially toxic materials or may be deficient in specific nutrients
  • In infancy, the immaturity of the enterohepatic circulation is a major factor. The driving force for bile flow is bile secretion; this requires effective hepatocyte uptake, orderly intrahepatocyte processing, and canalicular excretion of bile acids. This physiologic immaturity results in a decreased bile pool size, elevated serum bile acid, and decreased intraluminal bile acid concentrations
  • Intravenous infusions, particularly amino acids, result in decreased bile output in animal studies; a smaller decrease follows intragastric infusion. Parenteral nutrition also alters bile composition in newborn animals
  • Sepsis is also a critical factor. Sepsis exacerbates the cholestatic state in the low birth weight infant. Endotoxins directly damage hepatocytes and induce the release of toxic free radicals and proinflammatory cytokines.
  • Nutrient deficiencies or excesses are potential causes
  • Parenteral nutrition solutions often do not contain carnitine. Low serum and tissue carnitine concentrations have been reported in infants and children receiving long-termparenteral nutrition. Premature infants appear to be particularly susceptible. Carnitine deficiency may result in steatosis and hyperbilirubinemia.
  • Choline deficiency has been suggested as a cause of hepatic steatosis
60
Q

Conditions associated with Iron Overload

A
  • Hemochromatosis
  • GALD (neonatal hemochromatosis,)
  • aceruloplasminemia,
  • atransferemia
  • heavy chain ferritin disease
  • transfusion associated
  • alcoholic liver disease
  • NAFLD
  • CF
  • porphyria
  • cutanea tarda
  • chronic viral hepatitis
61
Q

Complications for PSC

A
  • cholestasis
  • malabsorption of fat-
    soluble vitamins (due to
    reduced intestinal
    concentrations of
    conjugated bile salts)
  • pruritus
  • metabolic bone disease
  • cholangiocarcinoma
  • biliary stones
  • coagulopathy
  • adenocarcinoma
  • end stage liver disease
  • complications of portal
    HTN
62
Q

Treatments for Pruritis from Cholestasis

A
  • skin emollients, keep fingernails short, light-weight clothes, unscented soap
  • Antihistamines (sedation)
  • Urso (choleretic; effective in cholestasis of pregnancy but not shown to improve pruritus in PBC)
  • Rifampin (inhibits uptake of bile acids into the hepatocyte; effective to reduce pruritus in PBC & pediatric cholestatic d/o, but potential for hepatotoxicity)
  • Cholestyramine (bile acid binding resin)
  • Phenobarbital (may be effective in individual patients, but poorer than other meds overall in efficacy; not great long-term choice in pediatrics – effects on developing brain)
  • UV light
  • Biliary diversion
  • Naltrexone (opiate withdrawal sx)
  • transplant
63
Q

Liver Biopsy Risks

A 
Minor: 
pain                                                                                                                                      	 Major: 
pneumothorax,
 hemothorax, 
hemoperitoneum, 
hemobilia, 
significant 
intrahepatic hematoma,
gallbladder perforation, 
biopsy of wrong organ (kidney, pancreas), 
death, 
increased infection risk
64
Q

Histological findings in BA

A

bile duct proliferation
canalicular and cellular bile stasis
portal and perilobular edema and fibrosis
bile plugs in bile ducts - relatively specific but only found in 40%

65
Q

Histological findings of neonatal hepatitis

A
  • giant cell transformation
  • ballooning/degeneration
  • neutrophil infiltration
  • intralobular inflammation
  • Kupffer cell hyperplasia
66
Q

Acute hepatitis histological findings

A
  • inflammation predominantly w/n in the lobule
  • hepatocellular injury is more pronounced (edema and necrosis of individual hepatocytes and macrophages are enlarged and numerous)
67
Q

Chronic Hepatitis histological findings

A
  • inflammation w/n in the portal tracts

- variable degree of parenchymal loss, scarring, parenchymal regeneration and cirrhosis

68
Q

Autoimmune Hepatitis histological findings

A
  • dense mononuclear and plasma cell infiltration of the portal areas which expands into the liver lobules
  • destruction of the hepatocytes at the periphery of the lobule
  • erosion into the limiting plate (interface hepatitis)
  • hepatic regeneration with rosette formation
69
Q

Drug-induced liver injury histological findings

A
  • prominence of neutrophils and eosinophils among inflammatory cells - hepatocellular injury can be spotty, affecting single hepatocytes or can be confluent, affecting groups of hepatocytes
  • confluent necrosis can be zonal or nonzonal depending on offending agent
  • zonal necrosis: characteristic of compounds with predictable, dose-dependent, intrinsic toxicity such as halothane (zone 3), carbon tetracholaride (zone 3) and acetaminophen (zone 3)
  • obliterative atrophy of septal or trabecular ducts proximal to sites of large duct scarring (progressive ductal lesions)
  • focal concentric edema and fibrosis (onion skinning) around intralobular bile ducts
70
Q

NAFLD histologic findings

A
  • ballooning degeneration of hepatocytes
  • macrosteatosis
  • perisinusoidal fibrosis
71
Q

Wilsons disease histological findings

A
  • early stage: marked steatosis of hepatocytes, mild chronic hepatitis, portal tract fibrosis and scant stainable copper
  • characteristic ultrastructural lesions on EM: heptocellular mitochondria are pleomorphic and abnormally large and show widen intracristal spaces, increased matrix density and large granules
72
Q

Reye syndrome histology findings

A
  • microvesicular steatosis in the absence of hepatic inflammation or necrosis
  • characteristic swelling and pleomorphism of mitochondria on EM
73
Q

Alpha1-Antitrypsin deficiency histology findings

A
  • PAS-positive diastase-resistent globules in the ER of hepatocytes
  • variable degree of hepatocellular necrosis, inflammatory cell infiltration, periportal fibrosis or cirrhosis
74
Q

Metabolism of Bilirubin

A

i. In RES: Heme oxygenase degrades heme-containing compounds to biliverdin
ii. Biliverdin reductase coverts biliverdin to bilirubin
iii. In circulation: Bilirubin is bound to albumin for transport
iv. Bilirubin dissociates from albumin, taken up into hepatocyte by carrier mediated transport (Cl exchange)
v. In hepatocyte: Bilirubin binds glutathione S transferase (GST) [Rotor]
vi. In ER: Bilirubin conjugated by bilirubin UDP glucuronosyltransferase (BUGT) to bilirubin glucuronide [Crigler Najjar, Gilbert]
vii. Conjugated bilirubin secreted at canalicular membrane by canalicular multispecific organic anion transporter (cMOAT) into bile [Dubin Johnson]
viii. Intestinal lumen: bacteria reduces and oxidizes conjugated bilirubin to urobilinoid, excreted in stool

Peds GI RC (12 decks)

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