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GI > Portal HTN/Chronic Liver Failure/Last Nichols > Flashcards

Portal HTN/Chronic Liver Failure/Last Nichols Flashcards

1
Q

Liver Blood Flow

A
  • 30% Hepatic Artery

- 70% Portal Vein (Splenic & Splanchnic)

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2
Q

Pressure=

A

flow x resistance

  • cirrhosis
  • fibrosis w/distortion of vasculature
  • sinusoids to capillaries
  • inc. blood flow to stomach & intestines
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3
Q

Portal HTN

A
  • venous collaterals form from distal esophagus to rectum
  • anterior collaterals via umbilical vein
  • posterior collaterals via retroperitoneal vains, splenorenal shunts
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4
Q

Varices

A
  • tortuous venous collaterals under high pressure (high pressure bleeding)
  • 50% of newly diagnosed cirrhotics
  • inc. up to 8%/year
  • 32% bleed within 2 years
  • major cause of death
  • thrombocytopenia
  • coagulopathy (synthetic impairment of clotting factors)
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5
Q

Treatment of Portal Vein HTN

A
  • volume resuscitation
  • correction of coagulopathy
  • splanchnic vasoconstriction
  • dec. blood flow to stomach and intestines
  • dec. blood flow via collaterals
  • Vasopression
  • Somatostatin (block vasodilators)
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6
Q

Natural History of Variceal Hemorrhage

A
  • mortality 42% in 6 weeks
  • 60% of early deaths due to bleeding
  • approximately 1/3 of patients had rebleeding within 6 weeks
  • 1 year survival 34%
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7
Q

Treatment of Variceal Hemorrhage

A
  • endoscopic therapy to sclerose or band the varices
  • decrease portal pressure (beta blockers, transjugular intrahepatic portosystemic shunt, liver transplantation, surgical portosystemic shunt)
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8
Q

Child Pugh Classification

A
  • albumin
  • prothrombin time
  • bilirubin
  • ascites
  • encephalopathy
  • Child A, B, C
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9
Q

Ascites

A
  • inc. resistance to portal venous flow
  • inc. flow to portal vein
  • inc. lymphatic flow
  • leakage of lymphatic flow from liver and intestines
  • inc. Portosystemic shunting of vasodilators
  • Systemic vasodilation
  • dec. renal perfusion
  • inc. renal vasoconstriction
  • inc. Renin-Angiotension activity
  • Inc. Sodium reabsorption
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10
Q

Ascites Causes?

A
  • decreased renal clearance
  • severe liver disease is associated with sodium retention & decreased creatinine clearance
  • Hep C can also cause renal disease
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11
Q

Why is Ascites important?

A
  • tense ascites, pressure on diaphragms & stomach, difficulty breathing and eating
  • hepatic hydrothorax
  • spontaneous bacterial peritonitis
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12
Q

Spontaneous Bacterial Peritonitis

A
  • large amount of undrained fluid
  • low protein ascites
  • low complement
  • bacterial translocation from intestines to blood
  • transient bacteremia infects the ascites
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13
Q

Treatment of Ascites

A
  • sodium restriction
  • diuretics
  • treat the liver disease
  • large volume paracentesis
  • correct the portal HTN
  • transugular portosystemic shunt
  • surgical portosystemic shunt
  • liver transplantation
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14
Q

Portal HTN causes?

A

life threatening complications

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15
Q

Chronic Liver Failure (TYPES)

A
  • hepatic encephalopathy
  • hepatorenal syndrome
  • IgA nephropathy
  • other renal complications with liver disease
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16
Q

Hepatic Encephalopathy

A

-acute liver failure
-portosystemic shunt without liver failure
-chronic liver failure:
precipitated
spontaneous
recurrent

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17
Q

Mechanisms of Hepatic Encephalopathy

A
  • ammonia, nitrogenous wastes
  • inc. intracellular gluatamine
  • astrocyte swelling, Cerebral edema
  • Inflammatory cytokines alter blood-brain barrier
  • Inc. Benzodiazepine receptors
  • inc. Neurosteroids, inc. GABA receptor activity
  • Manganese - neurotoxin which deposits in basal ganglia
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18
Q

Acute Hepatic Encephalopathy

A
  • acute liver failure: coagulopathy and altered mental status within 2 weeks of jaundice
  • alteration of BBB
  • associated with acute cerebral edema
  • cerebral edema results in cerebral herniation
  • major cause of death from acute liver injury
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19
Q

Chronic Hepatic Encephalopathy

A
  • slow & subtle onset
  • often noticed by family members
  • milder symptoms frequently missed by coworkers or physicians
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20
Q

Stages of hepatic encephalopathy

A
  • Grade I: irritability, insomnia, agitation
  • Grade II: indifferent, personality change, short term memory impairment, mildly disoriented about time or place
  • Grade III: drowsy but arousable, significantly confused & disorented to time & place
  • Grade IV: coma
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21
Q

Physical Exam for Hepatic Encephalopathy

A
  • neuro exam
  • alert, orientation to time, place, date, President’s name, family members Bday, maiden names
  • Asterixis: hyperextend wrists and observe for repetitive movement “flap”, inability to perform sustained grip of hand
  • myoclonus: with hyperrextension of ankles
  • absence of sensory, motor or cerebellar deficit to suggest another etiology for altered mentation
  • absence of autonomic hyperactivity, tachycarida, HTN
  • absence of sensory, motor or cerebellar deficit to suggest another etiology for altered mentation
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22
Q

Chronic Hepatic Encephalopathy Outcome

A
  • generally reversible with treatment
  • with long standing chronic hepatic encephalopathy: permanent brain damage can occur
  • mild decrease in mentation, calculation
  • rarely results in irreversible dementia
  • rarely permanent movement disorders
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23
Q

Treatment for Hepatic Encephalopathy

A

Treat the precipitating conditions:

  • Hypovolemia: correct causes-diuretics, medications which caused excessive diarrhea
  • Hypokalemia
  • GI bleeding
  • Prescribed medications, sedatives, substance abuse
  • Infection: evaluate for common systemic infections, meningitis
  • Exclude intracranial hemorrhage, falls with thrombocytopenia, coagulopathy (inc. INR)
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24
Q

Treatment for Hepatic Encephalopathy: Lactulose

A
  • poorly absorbable sugar
  • cathartic
  • decreased intestinal pH
  • decreases glutamine absorption
  • reduces synthesis & absorption of NH3
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25
Q

Treatment for Hepatic Encephalopathy: Zinc Sulfate

A
  • zinc is cofactor in NH3 metabolism, zinc deficiency is common in liver disease
  • correction of zinc deficiency is part of the treatment of encephalopathy
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26
Q

Treatment for Hepatic Encephalopathy: antibiotics

A
  • alter intestinal flora
  • decreased NH3
  • decrease intestinal mucosal glutaminase
  • decrease coliform bacterial which produce urease and convert urea to NH3
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27
Q

Mechanisms of Treatment for Hepatic Encephalopathy

A
  • nutrition improves the underlying liver disease
  • skeletal muscle metabolizes NH3
  • malnutrition is common in liver patients
  • protein restriction is not helpful
  • high vegetable proteins with increased branched chain amino acids advised
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28
Q

Nutrition & Hepatic Encephalopathy

A
  • protein restriction was not recommended in 1963

- it should not be part of orders today

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29
Q

Renal Complications associated with Liver Disease

A
  • hepatorenal syndrome
  • IgA nephropathy
  • membranoproliferative glomerulonephritis
  • membranous glomerulonephritis
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30
Q

Hepatorenal Syndrome

A
  • most feared complications of acute liver failure or cirrhosis
  • liver failure cause renal arterial vasoconstriction and renal failure
  • not associated with underlying renal parenchymal abnormality
  • generally reversed with correction of liver failure such as with transplantation
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31
Q

Hepatorenal Syndrome: What Happens?

A
  • cirrhosis & ascites
  • serum creatinine >1.5mg/dL
  • no imporvement (decrease 500mg protein per day)
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32
Q

Diagnosis of Hepatorenal Syndrome

A
  • exclusion

- lack of return of renal function with intravascular volume repletion

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33
Q

Hepatorenal Syndrome Type I

A
  • rapid, worsening

- creatinine >2.5mg/dL or decrease CrC <20ml/min in 2 weeks

34
Q

Mechanism of Hepatorenal Syndrome

A
  • peripheral artery vasodilaton
  • stimulation of renal sympathetic nervous system, renin-angiotension-aldosterone system
  • cardiac dysfunction, impaired contractility
  • cardiac dysfunction (impaired contractility)
  • cytokines & vasoactive mediators
35
Q

Hepatorenal Syndrome Type II

A

-slow progression, often with worsening liver disease

36
Q

Treatment for Hepatorenal Syndrome

A
  • IV volume repletion
  • treat infection
  • avoid meds that worsen renal function (NSAIDS)
  • avoid contrast (renal injury)
  • optimize renal perfusion w/Midodrine & Octreotide
  • Liver transplant*
  • Hemodialysis until liver transplant*
37
Q

IgA Nephropathy

A
  • liver disease most common cause of secondary
  • inc. deposition of IgA, C3, and other immunoglobuliins in 35-90% of cirrhosis patients
  • dec. hepatic clearance of IgA with cirrhosis and portal hypertension (with collateral blood flow around liver)
38
Q

Membranoproliferative Glomerulonephritis

A
  • associated with chronic Hep. C
  • cryoglobulinemia- abnormal protein in the blood which precipitates with cold temperatures
  • initially associated with Hep. C
  • attributed to immune complex formation with chronic Hep. C
39
Q

Inborn Errors of Metabolism

A
  • involve liver
  • babies: often 24-72 hrs: poor feeding, lethargy, vomiting, seizures, shock
  • later presentations: often >28 days, failure to thrive, developmental delay, vomiting, respiratory or pyschomotor
40
Q

Tryosinemia Urine Odor

A

boiled cabbage

rotten eggs

41
Q

Phenylketonuria Urine Odor

A

mousy or musty

42
Q

Trimethylaminuria Urine Odor

A

rotting fish

43
Q

Isovalaric acidemia Urine Odor

A

sweaty feet

44
Q

Maple Syrup urine disease Urine Odor

A

Maple Syrup

45
Q

Do you have to smell patients urine?

A

no, sometimes emanated from breath or sweat

-majority of patients with odor do not have inborn errors of metabolism

46
Q

History for Inborn Errors of Metabolism

A
  • family history (autosomal recessive)
  • Dietary history (specific sugary, proteins, can cause manifestations)
  • Poor feeding + lethargy + seizures suggests hypoglycemia or hypocalcemia
  • older children may present with episodic acute manifestations of disease
47
Q

Tests for Inborn Errors of Metabolism

A

ammonia, bicarb, pH, glucose, electrolytes, bilirubin, lactate, CBC, urine dipstick, urine “clinitest” for reducing substances

  • if ammonia, bicarb, pH normal = aminoacidopathy
  • episodic: lab tests normal between
  • freeze liver biopsy for enzyme assays
48
Q

Tryosinemia Type I

A
  • aminoacidopathy due to fumaryl-aceto-acetase deficiency
  • prevalent in French Canadians
  • Autosomal recessive
  • backup of metabolism of tyrosine leads to buildup of metabolites that are toxic to liver & kidney (mutagenic in liver)
  • liver toxicity manifested by steatosis, cholestasis, nodular regeneration with cholangiolar proliferation, cirrhosis,dysplasia and (37%) hepatocellular carcinoma
49
Q

Tyrosinemia Type I: gross pathology

A
  • cirrhosis with very large regenerative nodules

- yellow-orange due to steatosis & cholestasis

50
Q

Tyrosinemia Type I: microscopic pathology

A
  • steatosis & cholestasis (dark bile plugs “black”)

- dysplasia

51
Q

Tyrosinemia Type I: Classic Presentation

A

-first few months of life, failure to thrive, vomiting, diarrhea (bloody), jaundice, lethargy, coma, death (fulminant hepatic failure)

52
Q

Tyrosinemia Type I: Diagnosis

A
  • high urine succinylacetone

- DNA test for specific mutation (100% french canadians, 28% worldwide)

53
Q

membranous glomerulonehritis

A

associated with C & D

54
Q

Tyrosinemia Type I: Treatment

A
  • low-tyrosine diet, herbicide called NTBC (inhibits upstream enzyme that causes tyrosinemia type II)
  • liver thansplantation
55
Q

Tyrosinemia Type I: Prognosis

A

much better if treated early

56
Q

Ornithine Transcarbamylase (OTC) Deficiency

A
  • most common urea cycle disorder
  • X-linked
  • patients normal at birth until fed protein, then develop irritability, poor feeding, vomiting, lethargy, respiratory distress, hypotonia, seizures, coma and respiratory arrest due to hyperammonemia
57
Q

Ornithine Transcarbamylase (OTC) Deficiency: Diagnosis

A

blood & urine amino acid levels
liver enzyme assay
DNA test

58
Q

Ornithine Transcarbamylase (OTC) Deficiency: Treatment

A

emergency: IV benzoate, phenylacetate & citrulline

long term: protein restriction, liver transplant

59
Q

Gaucher’s Disease

A
  • beta-glucocerebrosidase deficiency
  • adult form = the most common lysosomal storage disease
  • autosomal recessive (Jews)
60
Q

Gaucher’s Disease: Microscopic

A

-Kupffer cells and macrophages with expanded crinkled cytoplasm

61
Q

Signs of Gaucher’s Disease

A
  • splenomegaly=most common initial sign

- pancytopenia, bone pain

62
Q

Gaucher’s Disease: Diagnosis

A

-assay of beta-glucosidase in white blood cells

63
Q

Gaucher’s Disease: Treatment

A

enzyme replacement where available ($)

64
Q

Glycogen Storage Disease Type I (Von Gierke)

A

deficiency of glucose-6-phosphatase (catalyzing conversion of glucose-6-phosphate to glucose), resulting in decreased hepatic glucose production and accumulation of glycogen in liver, kidney and intestine

65
Q

Glycogen Storage Disease Type I (Von Gierke): Classical Presentation

A
  • in first year of life: marked hepatomegaly & hypoglycemia

- lactic acidosis, hyperlipidemia, hyperuricemia

66
Q

Glycogen Storage Disease Type I (Von Gierke): Diagnosis

A

DNA test

67
Q

Glycogen Storage Disease Type I (Von Gierke): Microscopic pathology

A
  • cytoplasmic glycogen accumulation

- hepatomas

68
Q

Porphyrias

A
  • diverse group of inborn errors of metabolism involving enzymes in heme synthesis (not all in bone marrow, 20% liver)
    1. acute
    2. cutaneous
69
Q

Porphyrias: Clinical Presentation

A
  • porphyria cutanea tarda (heal by scaring)
  • onset after 20 years old
  • skin vesicles and bullae with sun exposure, attributed to formation of reaction oxygen species from porphyrin compounds, especially uroporphyrinogen
70
Q

Acute Intermittent Porphyria

A
  • autosomal dominant
  • porphobilinogen-deaminase (PBGD) deficiency
  • body’s ability to effectively supply heme is altered, alteration can create shortages in times of need
  • abnormal accumulation/build-up of bio-chemicals in body
  • 5x more female
71
Q

Acute Intermittent Porphyria: Symptoms precipitated by?

A

-drugs, alcohol, smoking, steroids, oral contraceptives

72
Q

Acute Intermittent Porphyria: Symptoms

A
  • abdominal pain
  • vomiting
  • tachycardia
  • constipation
  • neck, back, head pain
  • paresis
  • mental
73
Q

Acute Intermittent Porphyria: Urine

A

-dark, even purple with standing

74
Q

Acute Intermittent Porphyria: Diagnosis

A

urine test for porphobilinogen (PBG)

75
Q

Acute Intermittent Porphyria: Treatment

A

IV heme

76
Q

Acute Intermittent Porphyria: Prevention

A

-avoiding precipitants

77
Q

Pyloric Stenosis

A 
uncommon (0.35% births), 5x males
-genetic predisposition & environmental triggers
200x if monozygotic twin
4.62x more with bottle-feeding
erythromycin associated
78
Q

Pyloric Stenosis: Pathology

A

hypertrophy of pyloric muscle

79
Q

Pyloric Stenosis: Presentation

A

3-6 week old baby

  • immediate postprandial, non-bilioius, projectile vomiting & demands to be re-fed soon after
  • emaciated & dehydrated with “olive-like” mass at lateral edge of rectus abdominus muscle in right upper quadrant of abdomen (92%)
  • peristaltic waves seen from left to right just before emesis
80
Q

Pyloric Stenosis: Diagnosis

A

H&P +/- radiology

81
Q

Pyloric Stenosis: Treatment

A

surgery (pyloromyotomy)

GI (19 decks)

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