1
Q
Fever (Pyrexia) characteristics
A
- equal or above 37.3°C in morning, equal or above 37.8°C in evening
- common
- may mask malignant or non- malignant haematology disorder
2
Q
Pathophysiology of fever
A
- febrile response to endogenous pyrogens (cytokines) in response to exogenous pyrogens (micro- organisms or toxins)
- endogenous pyrogens act on thermo sensitive neurons in hypothalamus, upgrade set point via prostaglandins
- body reacts with heat production
- chemotaxis of neutrophils, adhesion and diapedesis of vessel endothelium, proliferation t-lymphocytes, release cytokines, histamine, leukotrienes, prostaglandins, tissue necrosis (eventually)
3
Q
Pel- Ebstein Fever
A
- condition in Hodgkin’s lymphoma
- fever that cyclically decrease and increase over average of 1-2 weeks
- B symptoms
4
Q
What is a major cause of pyrexia?
A
• infectious diseases in immunosuppressed haematology patients
5
Q
What do immunosuppressed haematologic patients with pyrexia need for immediate treatment?
A
empiric broad spectrum antibiotics
6
Q
Where do Infectious diseases commonly occur?
A
- Neutropenic patients
- Lymphoma- Mutliple Myeloma
• non- malignant haematology disorders (thalassemia- sickle cell disease)
7
Q
What is impaired in infectious diseases in immunosuppresed haematologic patients
A
- cellular response (decreases granulocytes)
* immune response (decreased immunoglobulin levels)
8
Q
What does Chemotherapy/Radiotherapy do to normal tissue
A
• destruction of normal mucosal tissue in gastro- intestinal, sinuses and urinary tract systems
9
Q
What are drugs that lead to immunosuppresion
A
- corticosteroids
- Fludarabine (CLL treatment)
- Idarubicine- cytarabine ( AML treatment)
- high dose methotrexate (Lymphoma treatment)
10
Q
if AFP increased, what could be suspected?
A
cancer in liver
11
Q
what is AFP?
A
- α fetoprotein
- protein made in liver of developing baby
- used to detect birth defects and genetic disorders (down syndrome, neural tube defects)
12
Q
Portal hypertension
A
• increase in blood pressure around liver
→ portal venous system
→ veins from stomach, intestine, spleen, pancreas merge into portal vein → then branch into liver
13
Q
What Hepatic Vein Pressure Gradient indicates Portal Hypertension?
A
if more than 5mm Hg
14
Q
How is the Wedge HVP, Free HVP and HVPG in Posthepatic portal hypertension?
A
WHVP increased
FHVP increased
HVPG normal
15
Q
What is HVPG?
A
- Hepatic Vein Pressure Gradient
* difference between wedged and free hepatic venous pressures
16
Q
What are esophageal varices??
A
- extremely dilated sub mucosal veins in lower third of esophagus
- consequence of portal hypertension due to cirrhosis
- can lead to severe bleeding in affected patients
17
Q
What are causes of portal hypertension?
A
- ↑atrial pressure (constrictive pericarditis)
- IVC → webs, tumor onvasion, thrombosis
- Hepatic veins → large due to thrombosis, web, tumor invasion
- portal veim & splenic vein thrombosis or invasion, comrpession by tumor
- incr blood flow → splenomegaly, idiopathic, arteriovenous fistula
- Post-sinusoidal, sinusoidal, pre sinusoidal
18
Q
Posthepatic diseases
A
- Budd-Chiari syndrome
- Constrictive pericarditis
- inferior vena cava obstruction
- right sided heart failure
- severe tricuspid regurgitation
19
Q
Intrahepatic diseases
A
• presinusoidal → idiopathic portal hypertension → Primary biliary cholangitis → sarcoidosis → Schistosomiasis • sinusoidal → alcoholic cirrhosis → alcoholic hepatitis → cryptogenic cirrhosis → postnecrotic cirrhosis • Postsinusoidal → sinusoidal obstruction syndrome
20
Q
Prehepatic
A
• portal vein or splenic vein thrombosis
21
Q
Cirrhosis?
A
- diffuse process with fibrosis, nodule formation replacing normal hepatic parenchyma
- end result of fibrogenesis → occurs with chronic liver injury
22
Q
Etiology cirrhosis
A
- US → alcohol, hepatitis C
- 27.000 death / year
- reduced life expectancy
- obesity, drugs, age, male, chronic HBV / HCV, iron (hemachromatosis), α-1-antitrypsin, metabolic syndrome, drugs
23
Q
Causes of Clubbing
A
• thoracic and non thoracic
• thoracic: bronchitis, empyema, lung abscess, cystic fibrosis, lung Ca, Esophageal Ca, mesothelioma, Bac. Endocarditis
→ interstitial lung disease: asbestosis, fibrosis alveolitis
→ vascular causes: AV malformation, cyanotic heart disease
• non thoracic: hepatic cirrhosis, ulcerative colitis, crohn’s disease
24
Q
What are complications of cirrhosis?
A
- ascites
- spontaneous bacterial peritonitis
- hepatorenal syndrome
- variceal hemorrhage
- hepatic encephalopathy
- hepatocellular carcinoma
25
Ascites?
* accumulation of fluid within peritoneal cavity
* common
* 2 yr survival of patients with ascites approx 50 percent
* Grade I: mild, detectable
* Grade II: moderate, symmetrical distention
* Grade III: large gross ascites with marked abdominal distension
26
Puddle Sign
• prone 3-5 min, then rises to all fours
• stethoscope on area of abdomen
• flicking finger over localized flank area
→ move stethoscope to opposite flank
→ sudden increase in intensity is positive sign
27
Stages of ascites
* No ascites
* uncomplicated ascites
* ascites and hyponatraemia
* refractory ascites
* hepatorenal syndrome
28
Spontaneous bacterial peritonitis (SBP)
most common bacterial infection on hospitalized cirrhotic patients
29
Unconjugated Hyperbilirubinemia Causes
* ↑bilirubin production (prehepatic → hemolysis, ineffective erythropoiesis, blood transfusion, resoprtion of hematomas)
* ↓ hepatocellular uptake of unconjugated bilirubin (drugs → rifampin, cyclosporine A)
* ↓ bilirubin conjugation (autosomal inherited disorders)
30
How does the bilirubin transport by hepatocyte occur?
??
31
What is Chlestasis
* stagnation, or marked reduction in bile secretion and flow
* can be due to functional impairment of hepatocytes in secretion of bile and/ or due to obstruction at any level of excretory pathway of bile
* from level of hepatic parenchymal cells at basolateral membrane of hepatocyte to ampulla of vater in duodenum
* marke bile acidemia, normal to slightly elevated bilirubin
32
name the two type of gallstones and describe them
• cholesterone stone
→ more than 80%
→ cholesterol monohydrate crystals
→ obese, women, race, estrogen, age
• pigment stones
→ 20%
→ black pigment stone ( calcium bilirubinate polymer, formed in gallbladder, chronic hemolysis (sickle cell anemia), crohn's disease (ileal resection)
→ brown pigment stone ( cholesterol/fatty soap/ calcium bilirubinate, bile duct, chronic biliary tract infection, bacterial β-glucoronidase deconjugates bilirubin, biliary stasis)
33
What is biliary colic?
* symptoms of gallbladder stones
* episodic attacks of severe pain in RUQ or epigastrium for at least 15-30min, radiating to right back or shoulder
* pos reaction to analgesics
→ NSAISs, spasmolytics and opioids
34
Cholecystitis
• inflammation of gallbladder
35
what are signs and symptoms of chelcystitis
* severe pain in RUQ or center abdomen
* pain that spreads to right shoulder or back
* tenderness over abdomen when touched
* nausea
* vomiting
* fever
* often after meal, esp. large and fatty
36
Cholangitis
* inflammation of bile duct system
| * inflammation and fibrosis pf hepatobiliary system characterized by eventual narrowing and obstruction of bile ducts
37
Into what categories is cholangitis divided into
* primary sclerosing cholangitis
* secondary cholangitis
* immune cholangitis
38
What sign and symptom has a high specificity for presence of acute cholangitis
Charcot's triad
→ jaundice
→ fever with or without rigors
• RUQ abd pain
39
What is included in the Reynolds pentad
• charcot's triad, confusion, shock
40
With what value of HVPG do varices develop?
> 10mmHg
41
With what value of HVPG does one have a high risk of variceal bleeding?
> 12mmHg
42
With what value of HVPG does one have a high risk of death
> 16mmHg
43
With what value of HVPG does one have a high risk of treatment failure and rebleeding?
> 20mmHg
44
With what value of HVPG does one have a 3% increase of death for the following 19month?
1mmHg
45
What portal circulation is located in the retroperitoneum?
mesenteric veins
46
What portal circulation is located in the umbilical (caput medusa)
• left portal via recannulated umbilical vein
47
What portal circulation is located in the rectum?
• superior hemorrhoidal veins
48
What portal circulation is located in the gastroesophageal junction?
• short gastric and left gastric (coronary) veins
49
What does alcohol lead to in male patients?
* in Testes: inhibition retinol to retinal, inhibition testosterone synthesis, down regulation LH and GABA receptors
* Periphery: increased hepatic conversion to active estrogen forms, relative incr
50
what is Sialadenosis?
* uncommon benign non inflam and non neoplastic enlargement of salivary gland, usually parotid
* associated with diabetes mellitus, malnutrition, liver cirrhosis due chronic alcoholism, hyperlipidemia, acromegaly
51
Hepatic Encephalopathy
decline in brain function as result to severe liver disease, liver can't remove toxins from blood toxin build up
52
Pathophysiology of Hepatic Encephalopathy
```
• Cerebral vasomotor dysfunction
• oedema sec to ammonia toxicity
• Inflam due to SIRS
• Putative benzodiazepine- like molecules
• Ammonia thought to be main factor
→ Ammonia converted to glutamine by liver, because ammonia clearance impaired
→ astrocyte swelling (↑Glu and Gln)
→ astrocyte dysfunction
→ neuronal dysfunction (↑GABA)
→ hepatic encephalopathy
```
53
Stages of Hepatic Encephalopathy West Haven Scale:
0 Asterixis absent
I asterixis can be detected, lack awareness, hypersomnia
II obvious asterixis
III asterixis generally absent, bizarre behaviour, gross disorientation, stupor
IV coma
54
What is feta hepaticus?
* sour, musty, feculent smell of breath
* presence of mercaptans (byproduct methionine metabolism)
* liver trouble filtering toxins
55
What is the role of the liver?
* release of HDLP, VLDLP, Phospholipids, blood glucose, urea, uric acid, amino acids, coenzymes(: NAD, NADP, FMN, FDP, HS CoA, PALP), Bile, cholesterol, bile acids, bilirubin, blood plasma proteins
* takin in of lactate, monosaccharides, AA, Lipids, Fatty acids, HDLP, Bilirubin,...
56
Celiac disease
• gluten sensitive enteropathy or celiac sprue
Semiology
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