USMLE Crap Pages: 241-244 Hemochromatosis Hemosiderosis

1
USMLE Crap

• Pages 241-244

2
Hemochromatosis

• Hemosiderosis deposition of hemoisoderin (IRON)
• Hemochromatosis the DISEASE caused by this
  deposition
• Total body iron may reach 50 g enough to set off
  the metal detectors at airports
• Treat with PHLEBOTOMY
• Can be 1 (autosomal recessive) or 2 due to
  chronic transfusion therapy

3
Classic Triad of Hemochromatosis

• Micronodular cirrhosis
• Pancreatic fibrosis
• Skin pigmentation

Excess iron is stored in body tissues, especially
the liver, heart, and pancreas.
4
Jaundice

• UN-conjugated bilirubin is UN-soluble (this is a
  Jones-ism, I cant/dont want to take credit for
  it)

5
Jaundice
6
Jaundice
7
Hereditary Hyperbilirubinemias

• Gilberts Syndrome
• Crigler-Najjar Syndrome, type I
• Dubin-Johnson Syndrome
• Gilbert is better off because he doesnt have a
  hyphenated name (Gilberts is asymptomatic)

8
Gilberts Syndrome

• Decreased UDP-glucuronyl tranferase
• Asymptomatic
• Unconjugated bilirubin is elevated
• MILD

9
Crigler-Najjar SyndromeType I

• Absent UDP-glucuronyl transferase
• Presents early, die early
• Jaundice, kernicterus, increased unconjugated
  bilirubin
• SEVERE
• Type II less severe and responds to phenobarbital

10
Dubin-Johnson Syndrome

• Conjugated hyperbilirubinemia due to defective
  excretion
• Black Liver
• Benign
• Rotors syndrome is similar, but milder and no
  black liver

11
Primary Sclerosing Cholangitis

• Inflammation and fibrosis of bile ducts
• Alternating strictures and dilation with
  beading on ERCP
• Can lead to 2 biliary cirhosis
• Associated with Ulcerative Colitis
• Remember that autoimmune disorders tend to be
  associated with one another

12
Primary Sclerosing Cholangitis
13
Biliary Cirrhosis

• Primary
• Autoimmune Antimitochondrial antibodies
• Obstructive jaundice, itching, hypercholesterolemi
  a
• Secondary
• Extrahepatic biliary obstruction
• Increased pressure injury/fibrosis
• Often complicated by ascending infection, bile
  stasis and bile lakes.

14
Biliary Cirrhosis
15
Hepatocellular Carcinoma

• Most common 1 malignant tumor of the liver in
  adults
• Assoc. with chronic liver diseases (ie. Hep B,
  Hep C, Wilsons disease, Hemochromatosis, alpha1
  antitrypsin, alchoholic cirrhosis and carcinogens
• Elevated alpha-fetoprotein

16
Hepatocellular Carcinoma
17
Reyes Syndrome

• Rare, often fatal childhood hepatoencephalopathy
• Fatty liver, hypoglycemia, coma
• Viral infection ( maybe salicylates)

18
Reyes syndrome



19
Gallstones

• 4 Fs
• Female, Fat, Fertile, Forty
• Charcots Triad Epigastric/RUQ pain, fever,
  jaundice
• Treat with cholecystectomy, diagnose with
  ultrasound

20
Gallstones - Types

• Cholesterol stones associated with obesity,
  Chrones, cystic fibrosis, age, etc
• Mixed stones (radiolucent) have both
  cholesterol and pigment most common
• Pigment stones (radiopaque) seen in chronic RBC
  hemolysis, alcoholic cirrhosis, age, biliary
  infection

21
Acute Pancreatitis

• GET SMASHeD
• Gallstones, Ethanol, Trauma, Steroids, Mumps,
  Autoimmune disease, Scorpion sting,
  Hyperlipidemia, Drugs
• Epigastric pain radiating to back, anorexia,
  nausea
• Elevated amylase, lipase (higher specificity)

22
Acute Pancreatitis Cont.

• Leads to DIC, ARDS, diffuse fat necrosis,
  hypocalcemia, pseudocyst formation
• Chronic pancreatitis strongly assoc with
  alcoholism

23
Pancreatic Adenocarcinoma

• BAD prognosis aggressive 6 mos to live
• Abd pain radiating to back, wt loss, migratory
  thrombophlebitis (trousseaus syndrome),
  Obstructive Jaundice with palpable gallbladder
  (Corvouisiers sign)

24
Pancreatic Adenocarcinoma
25
Obstructive Lung Disease

• Obstruction of air flow, air trapping
• Decreased FEV1/FVC ratio (hallmark)

26
4 Types COPD

• Chronic Bronchitis blue bloater productive
  cough for gt3 consec months in 2 or more years.
  Smokers. Mucous. Wheezing, crackles, cyanosis
• Emphysema pink puffer enlargement of air
  spaces and decreased recoil. Smokers and alpha1
  anti-trypsin deficiency. Dyspnea, Dec. breath
  sounds, tachycardia
• Asthma bronchial hyperresponsiveness causes
  reversible bronchoconstriction. Viral URIs,
  allergens, stress. Cough, wheezing, dyspnea, etc
• Bronchiectasis chronic necrotizing infection of
  bronchi. Dilated airways, purulent sputum,
  recurrent infections, hemoptysis. Bronchial
  obstruction, CF, poor ciliary motility and
  Kartageners syndrome

27
Restrictive Lung Disease

• Restricted lung expansion causes dec lung volumes
  (dec VC and TLC)
• FEV1/FVC ratio gt80

28
Types of Restrictive Lung Disease

• Poor breathing mechanics (extrapulmonary)
• Poor muscular effort (polio, myasthenia gravis)
• Poor apparatus (scoliosis)
• Interstitial lung diseases (pulmonary)
• Adult Respiratory Distress Syndrome (ARDS)
• Neonatal Respiratory Distress Syndrome (hyaline
  membrane disease)
• Pneumonconiosis (coal miners silicosis,
  asbestosis)
• Sarcoidosis
• Idiopathic Pulm Fibrosis
• Goodpastures syndrome
• Wegeners granulomatosis
• Eosinophilic granuloma

29
Lung Physical Findings
30
Obstructive vs. Restrictive Lung Disease
31
(No Transcript)
32
(No Transcript)
33
(No Transcript)
34
(No Transcript)
35
(No Transcript)
36
(No Transcript)