Studydroid is shutting down on January 1st, 2019

by mtoom

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What is the pathophysiology of Wilson's disease?
Excess of copper causing accumulation and damage to organs
What organs does Wilson's disease affect?
  • Liver disease (hepatitis, cirrhosis)
  • Eyes
  • CNS
  • Renal
What do you see in Wilson's disease in the eyes?
Kayser-Fleischer rings
What do you see in Wilson's disease if the CNS is affected?
  • (hint: symptoms are similar to what disease?) 
Extrapyramidal symptoms like Parkinson's disease
How do you diagnose Wilson's disease? (3)
  • (hint: 2 blood tests, 1 tissue test) 
  • liver enzymes
  • serum ceruloplasmin
  • Biopsy of copper-infiltrated tissue
How do you treat Wilson's disease? (1)
  • D-penicillamine
  • Zinc
  • Liver transplant 
What type of disease genetically is Wilson's disease?
Autosomal recessive
  • What causes hemochromatosis?
  • What accumulates in this disease?
  • What is the result of the accumulation? 
Hemochromatosis is caused by accumulation of iron, which leads to fibrosis due to free radicals (as a result of the iron).
What very serious complication are we concerned about in hemochromatosis?
HCC (hepatocellular carcinoma)
  • 200 times more likely 
What are symptoms of hemochromatosis? (6)
  • Symptoms of liver disease
  • Fatigue
  • Arthritis
  • Impotence/amenorrhea
  • Abdominal pain
  • Cardiac arrythmias
What organs are affected in hemochromatosis? (7)

Primarily liver
  • Liver → cirrhosis
  • Pancreas → DM
  • Skin → Hyperpigmentation
  • Thyroid (hypo)
  • Heart → cardiomyopathy
  • Joints → arthritis
  • Gonads → impotence, amenorrhea
What is the gold standard for diagnosis of hemochromatosis?
  • What are other blood tests to check in hemochromatosis
Liver biopsy, but also:
  • ↑serum iron
  • ↑serum ferritin
  • ↑iron saturation
  • ↓TIBC
What is the treatment for hemochromatosis?
Repeated phlebotomies (ie. remove blood)
What type of disease genetically is hemochromatosis?
Autosomal recessive
What is the most common cause of primary liver cancer?
HCC (hepatocellular carcinoma)
What are risk factors for HCC? (5)
  • Cirrhosis
  • Chemical carcinogens
  • AAT deficiency
  • Hemochromatosis, Wilson's
  • Cigarettes
What are symptoms of HCC? (4)
  • Abdominal pain
  • Symptoms of chronic liver disease
  • Weight loss, anorexia, fatigue
  • Paraneoplastic syndromes
What blood test is useful in trying to diagnose HCC? (1)
AFP (α-fetoprotein)
What diagnostic tests are used in HCC? (3)
  • Liver biopsy (definitive)
  • Lab tests (Hep serology, LFTs, coags)
  • Imaging (CT, US, MRI)
How do you treat HCC? (2)
  • Resection
  • Transplant
What is the pathology in Budd-Chiari syndrome?
  • What does this lead to?
Occlusion of hepatic venous outflow
  • Hepatic congestion
  • Subsequent microvascular ischemia
What are causes of Budd-Chiari syndrome? (1)
  • Idiopathic
  • Hypercoagulable states
  • Myeloproliferative disorder
  • Pregnancy
  • Chronic inflammatory disease
  • Infection
  • Trauma
What are symptoms of Budd-Chiari syndrome? (5)
  • Hepatomegaly
  • Ascites
  • Abdominal pain (RUQ)
  • Jaundice
  • Variceal bleeding
How do you diagnose Budd-Chiari syndrome?
Hepatic venography
What is the treatment in Budd-Chiari? (2)
  • Surgery
  • Liver transplant
Describe the pathogenesis of acute appendicitis in detail?
  • Lumen of appendix is obstructed (lymph, stool, foreign body)
  • Obstruction leads to stasis which promotes bacterial growth, leading to inflammation
  • Inflammation can lead to distension and ischemia
  • Necrosis and perforation can occur
Describe the symptoms of acute appendicitis in chronological order. (3)
  • Abdominal pain begins in the epigastrium and moves toward the umbilicus and then to the RLQ
  • Parietal peritoneum becomes irritated leading to sharp pain
  • Anorexia is always present
  • Nausea and vomiting occur
What are the signs of acute appendicitis? (5 general symptoms, 3 specific tests)

General symptoms:
  • Tenderness in RLQ
  • Rebound tenderness (at McBurney's)
  • Guarding
  • Low-grade fever
  • ↓bowel sounds

Specific tests:
  • Rovsing's: Deep LLQ palpation causes RLQ pain
  • Psoas sign: RLQ pain when thigh is extended as patient lies on left side
  • Obturator sign: RLQ pain when flexed thigh is internally rotated while patient is supine
How is acute appendicitis diagnosed? (3)
It is a clinical diagnosis, however:
  • Mild leukocytosis may be seen
  • Imaging studies are sensitive:
    -CT scan (98% sensitive)
    -US (90% sensitive) 
What is the treatment for acute appendicitis?
Laparoscopic appendectomy
Where is Crohn's disease found?
  • What is the hallmark location? 
Anywhere from "gum to bum"
  • Terminal ileum (hallmark location)
  • However, can any of the following three types: Ileum, ileocolic or colon
Describe the pathology of Crohn's disease. (6)
  • Skip lesions
  • Fistulae
  • Luminal strictures
  • Non-caseating granulomas
  • Transmural thickening and inflammation
  • Mesenteric "fat creeping"
Describe the course of Crohn's disease.
Flares and remissions
  • Medications become less effective
  • Surgery becomes more necessary 
What are the clinical features of Crohn's disease? (6)
  • Diarrhea (without blood)
  • Malabsorption and weight loss
  • Abdominal pain (usually RLQ)
  • Nausea/Vomiting
  • Fever/Malaise
  • Extraintestinal manifestations
What are the extraintestinal manifestations of Crohn's disease? (8)
  • Arthritis
    -ankylosing spondylitis
  • Eye lesions
  • Skin lesions
    -erythema nodosum
    -puoderma gangrenosum 
  • Oral ulcers
    -aphthous oral ulcers
  • Stones
What populations get IBD? (2)
  • Whites (especially Jews)
  • Younger people (ages 15-35)
How is Crohn's disease diagnosed?
Endoscopy with biopsy
What symptoms might patients have for years before Crohn's is diagnosed? (2)
  • Diarrhea
  • Vague abdo pain
What are complications of Crohn's? (8)
  • Fistulae
  • Anorectal disease
  • Small bowel obstruction
  • Malignancy (↓risk as compared to UC, but still some risk)
  • Toxic megacolon (less common than UC)
  • Malabsorption of B12 and bile cids
  • Cholelithiasis/nephrolithiasis
  • Growth retardation
What is the medical treatment for Crohn's? (3)
  • Sulfasalazine (5-ASA)
  • Metronidazole
  • System corticosteroids (acute exacerbations)
  • Immunosuppressants (azathioprine, 6-mercaptopurine)
  • Bile acid sequestrants

Do not use anti-diarrheal agents
Describe the role of surgical treatment in Crohn's.
  • Surgery is used to correct complications such as obstruction, fistulae, perforation or abscess
  • Surgery involves segmental resection and recurrence is common
What is cholelithiasis?
  • What is obstructed in this condition? 
Gall stones (in the gall bladder)
  • Cystic duct is obstructed 
What are the types of stones in cholelithiasis? (3)
  • Cholesterol
    -fat, female, forties, fertile, fair
    -whites, natives
    -obesity, diabetes, ↑lipids 
    -↑pregnancies, OCPs
    -Crohns disease
    -CF, cirrhosis 
  • Pigment
    -associated with hemolysis
    -alcoholic cirrhosis 
  • Mixed
    -majority of stones
    -both cholesterol and pigment 
Describe biliary colic. (3)
  • When?
  • Where?
  • Radiation 
  • Pain associated with meals and at night
  • Pain is in the RUQ
  • Pain radiates to the R shoulder (Boas sign)
What is the main complication of cholelithiasis?
  • Acute Cholecystitis
  • Choledocholithiasis
  • Gallstone ileus
  • Malignancy
How is cholelithiasis diagnosed? (2)
  • RUQ Ultrasound (high sens/spec)
  • CT scan & MRI
How is cholelithiasis treated? (2)
No treatment (if Pt is asymptomatic)
  • Cholecystectomy if recurrent bouts of biliary colic 
What is acute cholecystitis?
Acute inflammation of gall bladder
  • Usually as a result of chronic cholelithiasis (10% of Pts with gall stones lead will get acute cholecystitis)
What are symptoms of acute cholecystitis? (4)
  • Continuous RUQ/epigastric pain (which may radiate to R shoulder)
  • Nausea
  • Vomiting
  • appetite
What are signs of acute cholecystitis? (5)
  • RUQ tenderness (ie. rebound tenderness in RUQ)
  • Murphy's sign (inspiratory arrest with deep palpation of RUQ; specific, but not sensitive)
  • bowel sounds
  • Low-grade fever, leukocytosis
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