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liver , gallbladder & exocrine pancrease Flashcards

1
Q

cause Jaundice

A

retention of bile
1) Predominantly Unconjugated Hyper-bilirubinaemia
* Excess Production of Bilirubin
* Reduced Hepatic Uptake
* Impaired Bilirubin Conjugation

2) Predominantly Conjugated Hyper-bilirubinaemia:
* Decreased Hepatocellular Excretion (decreased clearance)
* Impaired Intra- or Extra-hepatic bile flow

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2
Q

patho of Jaundice

A

Disruption of the equilibrium between Bilirubin
production and clearance; Bilirubin is the end
product of haeme degradation

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3
Q

CM of Jaundice

A

Patients with a yellow discolouration of skin and sclerae (icterus)

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4
Q

cause of Neonatal Jaundice

A

Due to immaturity of the liver in conjugating and
excreting Bilirubin –> Development of transient
and mild unconjugated hyper-bilirubinaemia

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5
Q

state the diffrence between UnConjugated/Conjugated Bilirubin of Jaundice

A

–> Unconjugated Bilirubin:
* Insoluble in water at physiologic pH
* Exists in tight complexes with serum albumin
* Cannot be excreted in the urine
* Small amount of unconjugated Bilirubin, present as an albumin-free anion in plasma
* Increase of this unbound fraction (e.g. in haemolytic disease of the newborn [erythroblastosis fetalis]) can lead to accumulation of unconjugated Bilirubin in the brain –> Kernicterus

–> Conjugated Bilirubin:
* Water-soluble
* Non-toxic
* Loosely bound to albumin
* Can be excreted in urine
* Bilirubin delta fraction: With prolonged conjugated hyper-bilirubinaemia, a portion of circulated pigment becomes covalently bound to albumin

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6
Q

state the 3 mechanisms that disrupt the clearance and production of Bilirubin -> which casue Unconjugated Hyper-bilirubinaemia

A
  1. Excessive extra-hepatic production of Bilirubin
  2. Reduced hepatocyte uptake
  3. Impaired conjugation
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7
Q

state the 2 mechanisms that disturb the clearance and production of Bilirubin -> which casue conjugated Hyper-bilirubinaemia

A
  1. Decreased hepatocellular excretion
  2. Impaired bile flow
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8
Q

cause of Crigler-Najjar Syndrome

A

Mutations in the UGT1A1 gene –> Deficient
bilirubin uridine diphosphate glucuronosyl transferase
(bilirubin-UGT) enzyme

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9
Q

Types of Crigler-Najjar Syndrome

A

– Type 1 (CN1): Very severe; no enzyme function; affected individuals can die in childhood due to Kernicterus
– Type 2 (CN2): Less severe; <20% of normal enzyme’s function; people with CN2 are less likely to develop Kernicterus, and most affected individuals survive into adulthood

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10
Q

CM of Crigler-Najjar Syndrome

A

– Jaundice , at birth or in infancy
– Severe unconjugated hyper-bilirubinaemia –> Kernicterus

*Kernicterus –> brain damage due to high levels of bilirubin in a baby’s blood

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11
Q

Clinical picture of babies with Kernicterus

A
  • Extremely tired (lethargic)
  • Weak muscle tone (hypotonia)
  • Episodes of increased muscle tone (hypertonia) and arching of the backs
  • Involuntary writhing movements of the body
    (choreo-athetosis)
  • Hearing problems, or intellectual disability
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12
Q

casue of Dubin-Johnson Syndrome

A

Absence of the canalicular protein, multidrug
resistance protein 2 ([MRP2]–> (responsible for the transport of bilirubin glucuronides & related organic anions into bile)

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13
Q

patho of Dubin-Johnson Syndrome

A

Defect in hepatocellular excretion of
bilirubin glucuronides across the canalicular membrane

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14
Q

CM of Dubin-Johnson Syndrome

A

Chronic or recurrent Jaundice of
fluctuating intensity

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15
Q
  • Macroscopic findings: Darkly pigmented liver
  • Microscopic findings: Coarse pigmented granules within the cytoplasm of hepatocytes

Are the features of what type of Syndrome?

A

Dubin-Johnson Syndrome

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16
Q

cause of Rotor Syndrome

A

unknown

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17
Q

patho of Rotor Syndrome

A

Multiple defects in hepatocellular
uptake and excretion of Bilirubin pigments

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18
Q

CM of Rotor Syndrome

A

Jaundice, but otherwise
normal lives

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19
Q

Micro findings of Rotor Syndrome

A

Normal liver

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20
Q

cause of Cholestasis

A

Extra-hepatic or intra-hepatic obstruction of
bile channels or Defects in hepatocyte bile secretion

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21
Q

CM of Cholestasis

A

– Jaundice
– Pruritus
– Skin Xanthomas (focal accumulations of
Cholesterol)

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22
Q

Patho/Morphology:
1. Accumulation of Bile pigment within Hepatocytes –> fine, foamy appearnace (feathery degeneration)
2. Dilated canalicular spaces
3. Apoptotic cells
4. Kupffer cells with regurgitated bile
pigments (green-brown)
5. Bile ductular proliferation
6. Bile pigment retention
7. Swollen and degenerated Hepatocytes

–> There is also oedema and neutrophilic infiltrates

Feaures of what type of syndrome ?

A

Cholestasis

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23
Q

Factors that influence the development and severity of alcoholic liver disease (Renal Failure)

A

1) Gender: W>M
2) Ethnic differences
3) Genetic factors (Genetic polymorphisms in
detoxifying enzymes and some cytokine promoters)
4) Co-morbid conditions: Iron overload and infections (HBV and HCV) –> Increase in the severity of alcoholic liver disease

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24
Q

Complications of short-term alcohol consumption on the liver

A

mild, reversible Hepatic Steatosis

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25
Macroscopic features: – Large, heavy (4-6 kg) and soft liver (**Hepatomegaly**) – **Yellow and greasy organ** Microscopic findings: – Micro-vesicular **lipid droplets in hepatocytes** – Chronic alcohol intake: * **Clear** macro-vesicular **globules** --> Compression and displacement of the hepatocyte nucleus to the cell periphery * Fibrous tissue around terminal hepatic veins with extension to adjacent sinusoids Are the features of what type of Alcoholic liver disease?
Hepatic Steatosis (Fatty Liver Disease)
26
The 3 distinctive forms of Alcoholic liver disease
1. Hepatic Steatosis (Fatty Liver Disease) 2. Alcoholic Hepatitis 3. Cirrhosis
27
Microscopic findings : – **Hepatocyte swelling (ballooning*) and necrosis** – Cholestasis and Haemosiderin deposition in hepatocytes and Kupffer cells – **Mallory bodies**: Eosinophilic cytoplasmic clumps in hepatocytes – Neutrophilic infiltrates around degenerating hepatocytes. **Accumulation of lymphocytes and macrophages within parenchyma** – Activation of sinusoidal stellate cells and portal tract fibroblasts --> Fibrosis (mostly sinusoidal and peri-venular) Are the features of what type of Alcoholic liver disease?
Alcoholic Hepatitis
28
casues of Cirrhosis
– Chronic viral infections – Alcoholic or Non-Alcoholic Steato-Hepatitis – Autoimmune diseases – Iron overload
29
Main characteristics: 1) **Fibrous septa**: Delicate bands or broad scars around multiple adjacent nodules 2) **Parenchymal nodules**: * Encircled by the fibrous septa * Size: Small (<3mm) to large (>1cm) * Hepatocytes within nodules (i. Pre-existent, long-lived, ii. Newly formed and capable of replication) Are the Characteristics of what type of Alcoholic liver disease?
Cirrhosis
30
Morphology: – Yellow-tan, fatty and enlarged liver (>2kg) – **Brown, shrunken, non-fatty organ** (<1kg) [over the span of years] – Regenerative activity of entrapped parenchymal hepatocytes --> Uniform micro-nodules --> **diffuse Nodularity** becomes more prominent --> Scattered larger nodules (**“hobnail” appearance** on the surface of the liver) – Engulfment of parenchymal islands by wide bands of fibrous septa --> Mixed micro- & macro-nodular pattern – Ischaemic necrosis and fibrous obliteration of nodules --> Development of broad expanses of **tough, pale scar tissue** (**“Laennec Cirrhosis”**) Are the features of what type of Alcoholic liver disease?
Cirrhosis
31
Clinical features of Cirrhosis
– Anorexia – Weight loss – Weakness – Frank debilitation (advanced disease) – Incipient or Overt Hepatic Failure
32
Conditions with an end-result of Fatal Cirrhosis
1. Progressive Liver Failure 2. Portal Hypertension 3. Hepatocellular Carcinoma
33
Morphology: – **Steatosis** – Multifocal **parenchymal inflammation** (neutrophils) – **Mallory bodies** – **Hepatocyte** death (**balooning** degeneration and apoptosis) – **Fibrosis** (sinusoidal, but also within portal tracts and around terminal hepatic venules) of what type of Non-alcoholic fatty liver syndrome?
Non-Alcoholic Steato-Hepatitis (NASH)
34
Types of NAFLD (non-alcoholic fatty liver disease)
– Simple Hepatic Steatosis – Steatosis accompanied by minor non-specific inflammation – Non-Alcoholic Steato-Hepatitis (NASH)
35
What are the 2 sequential events of the **"two-hit** model of NAFLD pathogenesis
1. **Hepatic fat accumulation** and 2. **Hepatic oxidative stress**; The oxidative stress acts upon the accumulated hepatic lipids, resulting in lipid peroxidation and the release of lipid peroxides, which can produce reactive oxygen species (ROS)
36
Morphology: Large (macro-vesicular) and small (micro-vesicular) droplets of fat (predominantly triglycerides), within hepatocytes Are the features of what type of NAFLD?
Steatosis
37
Macro/micro feature diffrence btw NASH(non-alcoholic steato Hepatitis) and Steatosis
38
Clinical features of NAFLD
1) Individuals with **Simple Steatosis**, generally **asymptomatic** 2) Some patients show **general symptoms**, such as **fatigue and right-sided abdominal discomfort caused by Hepatomegaly** 3) Patients with **NASH: Cardiovascular Disease --> Death**
39
Imaging studies of NAFLD?
fatty liver
40
Labratory findings of NAFLD
1) Elevated serum AST and ALT (90% of patients with NASH) – AST/ALT ratio: * Non-Alcoholic Steato-Hepatitis (NASH): <1 * Alcoholic Steato-Hepatitis: >2.0 to 2.5
41
Managment of NAFLD
Goal of treatment: **Reversion of Steatosis and prevention of Cirrhosis** --> --> **Correction** of the underlying **risk factors: Obesity and Hyperlipidaemia** and --> **Treatment of Insulin resistance**
42
Portal Hypertension --> is the result of increased resistance to portal blood flow **state the casues**
– **Pre-Hepatic** – **Intra-Hepatic** (**Mainly: Cirrhosis**; Less frequent: Massive Fatty Change, Granulomatous Diseases) – **Post-Hepatic**
43
How does Cirrhosis result in portal hypertension?
– Increased resistance to portal flow at the level of sinusoids – Compression of Central Veins by peri-venular fibrosis and expanded parenchymal nodules –**Anastomoses between the arterial and portal system in the fibrous bands --> Portal Hypertension** – Hyper-dynamic circulation, resulting from arterial vasodilation in the splanchnic circulation, due to increased production of NO in the vascular bed
44
Four major Clinical Consequences of Portal Hypertension:
– Ascites – Formation of PortoSystemic Venous Shunts – Congestive Splenomegaly – Hepatic Encephalopathy
45
complications of the **Acute** setting of hepatic Encephalpathy
Elevation in blood Ammonia --> Impairment of neuronal function --> **Generalised Brain Oedema**
46
complications of the **Chronic setting** of hepatic Encephalpathy
**Deranged neuro-transmitter production** (especially in mono-aminergic, opioidergic, GABAergic and endo-cannabinoid systems) --> **Neuronal dysfunction**
47
CF of Hepatic Encephalopathy
– Spectrum of **brain dysfunction**, ranging from subtle behavioural abnormalities --> **Marked Confusion and Stupor**--> **Deep Coma and Death** – **Rigidity, Hyper-reflexia, EEG changes** (non-specific), Seizures (rarely) – **Asterixis (Flapping Tremor)**: Non-rhythmic, rapid extension-flexion movements of the head & extremities
48
----------------:Collection of excess fluid in the peritoneal cavity
Ascites
49
mechanisms responsible for the development of Ascites
1) **Sinusoidal Hypertension and Hypo-Albuminaemia** (--> Increased movement of intravascular fluid into the extravascular space of Disse) 2) **Leakage of fluid from the liver into the peritoneal cavity; Cirrhosis** 3) **Renal retention of Na and H2O**(, due to secondary Hyper-Aldosteronism)
50
loc. of porto-Systemic Shunt
Veins around and within: 1) The Rectum (Haemorrhoids) 2) The Cardio-Oesophageal junction (OesophagoGastric varices) 3) The Retro-Peritoneum and the Falciform ligament of the Liver (peri-umbilical and abdominal wall collaterals) --> **“Caput medusae”**
51
clinical features of Hepato-renal Syndrome
Decreased Urine output
52
Laboratory findings of Hepato-Renal Syndrome
1) **↑Urea,Nitrogen and Creatinine in blood** 2) **Hyperosmolar Urine** (devoid of proteins and abnormal sediment), with **↓ sodium levels*** (unlike in Renal Tubular Necrosis)
53
Treatment of Hepato-renal syndrome
Liver transplantation
54
Porto-Pulmonary hypertension involves
1) Portal Hypertension (cirrhotic or non-cirrhotic cause) 2) Excessive pulmonary vasoconstriction 3) Vascular remodeling
55
Clincial features of Porto-pulmonary Hypertension
– Dyspnoea on exertion – Clubbing of the fingers – Palpitations – Chest pain
56
clincial features of Heptao-pulmonary Syndrome
1) **Platypnoea** (easier breathing in a horizontal position) 2) **Orthodeoxia** (fall of arterial blood Oxygen with upright posture)
57
Phases of Acute **Symptomatic** viral hepatitis w/ recovery
1. Incubation period 2. Symptomatic pre-icteric period 3. Symptomatic icteric phase 4. Convalescence (Recovery)
58
epi of Chronic Viral Hepatitis
– High frequency: HCV-infection --> Chronic Hepatitis – Small number of HBV-infection cases --> Chronic Hepatitis
59
clincial features of Chronic Viral Hepatitis
– Fatigue – Malaise – Loss of appetite – Occasional bouts of mild Jaundice
60
Clinical findings: – Spider Angiomas – Palmar Erythema – Mild Hepato-Splenomegaly – Hepatic Tenderness Are the features of what type of Syndrome?
Chronic Viral Hepatitis
61
Labratory findings: – Prolongation of prothrombin time – Hyper-globulinaemia – Hyper-bilirubinaemia – Mild elevations in Alkaline Phosphatase levels are the LF of?
Chronic Viral Hepatitis
62
Chronic Viral Hepatitis **Complications** in cases of HBV and HCV
* Vasculitis * Glomerulonephritis * Cryoglobulinaemia
63
Microscopic features: * Diffuse swelling (**“ballooning degeneration”**) * **Cholestasis**, with **bile plugs in canaliculi** and brown pigmentation of hepatocytes * **fatty change** (Hepatitc C) * **Apoptosis** * **peioportal necrosis**Confluent necrosi * Hypertrophy and hyperplasia of Kupffer cells, often laden with lipofuscin pigment * Mixed **inflammatory cell infiltrate** in the portal tracts Are the feature of Acute/Chronic viral Hepatits?
Acute Viral Hepatits
64
microscopic features: infected hepatocytes with intra-cytoplasmic sphaeres and tubules, giving the characteristic appearance of **“ground-glass hepatocytes”** is the feature of what type of syndrome?
Viral Hepatits associated w/ **HBV** *HBV: Hepatitis B Virus
65
Microscopic features: infected livers with **lymphoid aggregates within portal tracts** and focal lobular regions of hepatocyte **Macro-Vesicular Steatosis** Are the features of what type of Condition?
Viral Hepatitis associated w/ **HCV** *HCV : Hepatitis C Virus
66
What is the Histological Hallmark of chronic Viral hepatitis B (HBV) infection?
**"Ground glass Hepatocyte"**
67
cause of liver Abscess
– Parasitic infections (developing countries) – Complication of a bacterial infection elsewhere in body (Western world)
68
Macroscopic features: – **Solitary or multiple lesions** – Size: From **tiny to massive lesions** Microscopic findings: – **Liquefactive necrosis** – Abundant neutrophils Are the features of what type of condition?
Liver Abscess
68
Clinical manifestaions of Liver Abscesses
– Fever – Right upper quadrant pain – Tender Hepatomegaly – Jaundice (in cases of biliary obstruction)
69
Tx of Liver Abscesses
– Antibiotic therapy and – Percutaneous or surgical drainage
70
patho+ triggers of Autoimune Hepatitis
– **T-Cell mediated autoimmunity** – **Hepatocyte injury caused by IFN-γ** (produced by CD4 and CD8 T-cells) **and CD8 T-cell-mediated cytotoxicity** – **Defect in regulatory T-cells** --> Uncontrolled activation of pathogenic self-reactive lymphocytes Triggers: - Viral infections, certain drugs (e.g. Atorvastatin, Simvastatin, Methyldopa, Interferons etc.), herbal products
71
Laboratory findings: – Absence of serologic evidence of viral infection – Elevated serum IgG – High titers of autoantibodies (80% of cases) – Autoantibodies: Detected by Immuno-Fluorescence (IF) or Enzyme-Linked Immunosorbent Assays (ELISA) * Antinuclear Abs * Anti-Smooth Muscle Abs * Liver/Kidney microsomal Abs * Anti-soluble Liver/Pancreas Abs Are the findings of whattype of condition ?
Autoimune Hepatitis
72
Microscopic findings: – Severe **hepatocyte injury** --> Confluent necrosis – Simultaneous, **marked inflammation** and advanced scarring – **Burned-out Cirrhosis** (No histological hallmarks present) – Abundant plasma cells Are the features of what type of conditio?
Autoimmune Hepatitis
73
CM of Autoimmune Hepatitis
Mild to severe Chronic Hepatitis
74
Clinical features of Haemochromatosis
– Hepatomegaly – Skin pigmentation – Diabetes mellitus – Cardiac dysfunction – Atypical arthritis
75
Diagnosis of Haemochromatosis
– High levels of serum Iron and Ferritin – Exclusion of secondary causes of Iron overload (for Hereditary Haemochromatosis)
76
Management of Haemochromatosis
**Phlebotomy; Use of Iron Chelators* -> Decrease total body Iron** *(Chelators: Agents that are able to bind metal ions for drastic reduction in their reactivity)
77
Secondary Iron Overload -> Acquired forms of Iron accumulation; known sources of excess Iron?
– Multiple transfusions – Ineffective Erythropoiesis (β-Thalassaemia and Myelodysplastic Syndromes) – Increased Iron intake
78
Wilson disease is Charactersied by?
accumulation of toxic levels of Copper in many tissues and organs (Liver, Brain and Eye)
79
casue of Wilson disease
Loss-of-function mutations in ATP7B gene (Chromosome 13); ATP7B gene encodes an ATPase metal ion transporter, localised to the Golgi region of hepatocytes
80
Clinical features of Wilson disease
– Acute or Chronic Liver Disease – Neuropsychiatric manifestations (mild Behavioural Changes, frank Psychosis, Parkinson disease-like syndrome)
81
Treatment of Wilson disease
Long-term therapy with Copper Chelators (D-Penicillamine) and Zinc salts
82
Wilson disease is charactersised by Progressive accumulation of Copper in Hepatocytes -->Toxic injury. what are the 3-step mechanism of this process?
1. Induction of free radical formation 2. Binding to sulfhydryl groups of cellular proteins 3. Displacement of other metals in hepatic metalloenzymes
83
Wilson disease may mimic?
1) Fatty Liver Disease (with mild to moderate Steatosis, Steato-Hepatitis and similar patterns of Scarring) 2) Acute Hepatitis-like injury: In its most severe form, manifests as Fulminant Hepatic Failure 3) Chronic Hepatitis-like picture, with coexistence of Fatty Liver Disease features; Progression of Chronic Hepatitis --> Development of Cirrhosis
84
Special stains used to identify Copper deposits in Wilson Disease
i. Rhodanine stain for Copper and ii. Orcein stain for Copper-associated protein)
85
fxn of α1-Antitrypsin
Inhibition of neutrophil elastase released at sites of inflammation
86
complications of AAT (α1-Antitrypsin) Deficiency
1) Hepatocellular Carcinoma 2) Pulmonary Emphysema
87
Morphology: – Hepatocytes with round to oval cytoplasmic globules (PAS+ and PASd+ [DD: Glycogenosis of Diabetic Hepatopathy, and Glycogen Storage Diseases]) – Patterns of Hepatocyte injury associated with PiZZ homozygosity: * Marked Cholestasis with Hepatocyte necrosis in newborn * Childhood Cirrhosis * Smoldering Chronic Hepatitis * Cirrhosis (apparent late in life) Are the features of what type of syndrome ?
α1-Antitrypsin Deficiency
88
CM of α1-Antitrypsin Deficiency
– Cholestasis: 10-20% of newborns with AAT deficiency – Chronic Hepatitis – Cirrhosis or – Pulmonary disease
89
Treatment of α1-Antitrypsin Deficiency
Orthotopic Liver transplantation

Pathology (11 decks)

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