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262 Cards in this Set
- Front
- Back
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Describe Right to left shunt and the disorders generally that cause
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Blue Babies-
The "T" Syndrome issues • Tetralogy of Fallot(1*) • Transposition of vessles • Total anomalous pulmonary venous connection (TAPVC) • Tricuspid atresia • Persistent truncus arteriosus |
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Describe left to right shunt and the disorders generally that cause...
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Blue Kids (later in life, not a birth)
The D-Disorders VSD - most common ASD -causes loud S1 with long S2 split PDA- close with indometacin |
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What is Eisenmengers syndrome?
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Uncorrected Left to Right shunt VSD,ASD,PDA that leads to progressive pulmonary HTN and eventually revers the L->R to a R-> which is what causes the cyanosis latter in life
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What are the four componets of Tetraology of Fallot?
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PROVe
(sub-)Pulmonary stenosis (most important) Right Ventricular Hypertrophy Overriding Aorta (Overrides the VSD Ventricular Septal Defect -Boot shaped heart on CXR due to enlarged RV |
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What causes Tetraology of Fallot?
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anteriosuperior displacement of the infandibular septum
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Give the defect that goes with the disorder: 22q11 syndromes
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Truncus Arteriosus
Tetraolgy of Fallot |
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Give the defect that goes with the disorder: Downs Syndrome
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ASD & VSD
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Give the defect that goes with the disorder: Congenital Rubella
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Septal Defects, PDA
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Give the defect that goes with the disorder: Turners Syndrome
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Coarction of the Aorta
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Give the defect that goes with the disorder: Marfans Syndrome
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Aortic insufficency
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Give the defect that goes with the disorder: offspring of Diabetic mother
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Transpostion of the great vessles
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Describe the findings seen with coarction of the aorta.
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In adults you see notching of the ribs, hypertension in the upper extermities, and weak pulses (hypotension) in the lower extremities
Murmer throguh systole with thrill Cardiomegaly In Infants steosis is proximal to ductis, in ADults Distal to Ductus |
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What type of arterosclerosis would you expect to see with Eisenmengers syndrome?
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Hyperplastic due to the diastolic pressure being greater than 120
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What are the complications of congenital heart disease?
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Cardiac Hypertophy and dialation
Failure to Thrive Infective endocarditis Complications form infections in childhood |
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What are the features of ASD?
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Pulmonic Valve Murmer thorugh systole
Left to Right shunt Pulmonary HTN is uncommon |
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What is the most common type if ASD?
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Septum Secundum Type 1*
(Septim primum is 2nd) If fixed then no change in survival |
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What are the frequency, most common location
and outcomes of VSD? |
20-30% isolated defect
can be membrabnous or muscular: membranous most common |
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Why does cyanosis developing babies with large persistent VSD?
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Pulmonary hypertension reverses the Left to Right shunt to a Right to Left one
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Where is an ifracrystal ventricular septal defect?
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in the muscle
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How does a patent ductus arteriosus present?
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Machine like constant murmur with normal cardiac function at birth
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How do you maintian patentcy in a Patent Ducuts Arteriosus? How would you close it?
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To keep open: Proataglandin E
To close: indomethacin or ibuprofen |
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what is the embryology of an Atrioventricular septal defect? What is the most common association/occurrence of this related to
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Failure of fusion of endocardial cushions
Assocuated with DOWNS Syndrome |
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What gene is indicated in tetralogy of fallot?
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NOTCH
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Define transposition of the great arteries
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Aorta arises from right ventricle and is anterior and right of pulmonic artery: pulmonic arter arises from the left ventricle
its a "ventriculoarterio' discordance |
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What is the difference between a stable and unstable transpostion?
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Stable has a VSD which "fixes" the problem and
Unstable: incompatible with life and requires creation of an intimidate shunt |
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What is a persistant truncus arteriosis?
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When there is a Single great artery overiding both ventricles. It is associated with a VSD
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What disorder do you see a persistant truncus?
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22q112 deletion
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What is tricuspid atresia?
When is it most often seen? |
Occlusion of tricuspid orfice
assocoated with hypoplasia of the right ventricle and is due to uneven division of the AV canal |
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What is a Total anomalous pulmonary venous connection?
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When the pulmonary VEINS don't enter the left atrium
Volume and pressure hypertrophy of right heart • Left atrium is hypoplastic |
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What are some clinical associations with coarction? *(Bolded)
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Valve defects: bicuspid aortic
Berry aneurysm, circle of Willis Rib notching Lots of collaterals (can have aching legs when walking, systolic murmur) |
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What murmer is heard with aortic stenosis?
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Prominent crescendo, decresendo systolic ejection murmur with thrill
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Define hypoplastic left heart syndrome.
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• Severe stenosis or atresia of aortic valve with an Underdeveloped left ventricle and aorta
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What Gene is associated with Williams Syndrome?
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Elastin (ELN) gene
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List the features of Williams Syndrome
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Deletion of chromosome 7 takes our ELN gene
Thickend ascending aorta causing supravalvular stenosis Facial abnormality elfin like with flat nose, always smiling Hypercalcemina in infancy causing colic, vomiting and metastatic calcification risk |
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Pro-Platelet Aggregation agonists
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ADP
Thrombin TxA2 Collagen Epinepherine PAF (from basophils and Mast cells) |
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What are the biological variations that impact lab test resuults..
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TIME, both cercadian and in relation to other activites like eating or medication use
AGE GENDER PREGANCNY POSTURE EXERCISE |
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What will EtOH influence on labs?
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TAGS
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What will caffeine influence on labs?
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Increased catecholamines, glucose
And Elevated lipids w/ chronic consumption |
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What important lab value changes for Kiddos? Elderly?
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Kiddos: Lymphocytes must exceed 2500/mm3
Alkaline phosphatase due to bone growth |
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What important lab value changes for Elderly?
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Albumin, creatnine and lymphocytes are all reduced
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What are the gender lab variations?
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Hormone/Fe/Hemoglobin
Less muscle means lower: AST, BUN, CK and others |
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What lab variations would you expect with a marathon runner?
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lower mean cell hemoglobin, glucose, and WBC
Higher billirube and BUN Higher muscle enzymes and muscle byproduct |
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What is the guassian distribution?
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usual interval is 2 SD from test mean; ‐95% of persons w/o disease will have
analyte result in this range |
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What is incidence?
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Incidence: number of patients in whom the disease develops in a 1 year period
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What does BUN measure?
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Reflects the breakdown of amino acids in liver that is excreted from the kidenys, thus:
IT is a marker for decreased GLOMULAR FILTRATION |
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What are the gender lab variations?
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Hormone/Fe/Hemoglobin
Less muscle means lower: AST, BUN, CK and others |
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What lab variations would you expect with a marathon runner?
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lower mean cell hemoglobin, glucose, and WBC
Higher billirube and BUN Higher muscle enzymes and muscle byproduct |
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What is the guassian distribution?
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usual interval is 2 SD from test mean; ‐95% of persons w/o disease will have
analyte result in this range |
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What is incidence?
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Incidence: number of patients in whom the disease develops in a 1 year period
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What does BUN measure?
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Reflects the breakdown of amino acids in liver that is excreted from the kidenys, thus:
IT is a marker for decreased GLOMULAR FILTRATION by itself can indicate decreased profution, dehydration or heart failure |
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What is Azotemia?
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High BUN and CREATNINE usally indicates some form of renal failure
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What would cause a low BUN lab?
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-Low protein diet
-Advanced Liver disease |
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Are there non-reanal reasons why BUN would be elevated?
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yes.
GOI Bleed or Catabolism such as: Fever Burns Diabetes and Intensive exercise |
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What does Creatinine measure?
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it is the catabolic end product of sk muscle that yielded ATP
Insensitve but SPECIFIC for renal imparment |
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What is Indirect bilirubin?
Direct? |
Unconjugated and not H2o soluble
Direct is conjugated in the liver |
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What causes Prehepatic Jaundice? Intrahepatic jaundice?
Posthepatic Jaundice? |
Pre-Hemolysis
Intra: Liver disease Post- Gallstone in bile duct |
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What is the alkaline phosphatase test (ALP) used for? When might you see higher then expected results that are non-pathologic?
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The alkaline phosphatase test (ALP) is used to help detect liver disease or bone disorders.
Will be higher in pregnancy and kiddos |
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What does Lactate Dehydrogenase testing show you?
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•Used as a cardiac (MI) indicator Significant plasma elevation occurs with small amounts of tissue injury
• High plasma levels associated with break down of erythrocytes (hemolysis); platelets |
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What is AST and what does it measure?
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aspartate aminotransferase
seen in wide variety of tissues including liver, skeletal and heart |
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What is ALT?
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(alanine aminotransferase)
More specific for liver B6 Def can show falsely low ALT |
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What enzymes/markers are elevated in an acute MI?
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Creatine Kinase (and isoenzyme MB)
AST Lactate Dehydrogenase Myoglobin Troponin I and T |
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What enzymes are increased in liver disease?
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AST
ALT Alkaline phosphatase Gamma glutamyl transferase‐ GGT LH or LDH ‐lactate dehydrogenase |
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What enzymes are elevated wtih muscle diseases?
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‐ CK (creatine kinase)
‐ AST ‐ LD Aldolase ‐ Nonenzyme proteins: myoglobin |
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What enzymes are high in bone disease
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Alkaline phosphatase
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What doe the total plasma proteins consist of?
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Albumin and globulin
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how is globulin calcualted?
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Total plasma - albumin
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What does globulin include? Where are the componets made?
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alpba, beta in liver
gamma from plasma cells (major component of globulin |
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What does prealbumin measure?
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Nutrition status (short term, only 2 day half-life)
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Describe job and concentration of albumin
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2/3 plasma protein concentration
It is a carrier protein that maintains INTRAVASCULAR oncotic pressure 17 day half life |
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What causes high albumin labs?
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dehydration (infrequent finding)
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What causes decreased albumin?
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Much more common lab finding:
1) Decreased synthesis from malnutrtion, decreased liver finction or chrinic inflammation 2) loss from renal disease, acites or protein‐losing gastroenteropathy |
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What is the major a-1 globulin? When is it increased? Decreased?
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α‐1 antitrypsin
Decreased with Pulmonary Emphysema and liver cirrhosis it is a protease inhibitor that counters the effects of leukocyte elastase |
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What is CRP?
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C-reactive Protein
Sensitive indicator of tissue necrosis, acute infection and inflammation; itis an acute phase reactant Cardiac risk factor |
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What is the significance of increased a-2 globulins?
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High levels indicates acute damage- they are the clean up crew
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What are the Acute Phase Reactions/Reactants?
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‐Neutrophilia ‐Thrombocytosis (platelets increase
Fibrinogen increased ‐Alpha‐2‐macroglobulin increased ‐C‐reactive protein increased ‐Haptoglobin increased ‐Albumin decreased Erythrocyte sedimentation rate increased |
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What does ESR measure?
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increased ESR is a nonspecific indicator
of inflammation |
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Where are Gamma globulins, immune globulin, IG all produced?
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in/by plasma cells
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What are the three types of Increased immunoglobulin?
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Polyclonal gammopathy:
Monoclonal Oligocolona |
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What causes Polyclonal gammopathy?
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autoimmune disease; chronic infections
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What causes Monoclonal gammopathy?
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plasma cell malignancy or
premalignancy or lymphoma |
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What causes oligocolonal gammopathy?
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Multiple Sclerosis and other CSF indicated diseases
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What pathway and factors does the PT test assess?
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Extrinisic pathway
Facotrs 2,5,7,10 and fibrinogen |
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What clotting factors are NOT activated with the intrinsic pathway?
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7 & 13
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What pathway and factors does the PIT test assess?
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Partial Thromboplastin Time
Intrinsic pathway 2,5,8,9,10,11,12 |
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What is the most important fibrinolytic protease?
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Plasmin. it splits fibrin
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What factor conversion to its activated form links the fibrinolytic, coagulation, compliment and kinin systems?
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Factor 12 to 12a
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What are thrombolytic disorders?
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Disorders that are antithrombolytic (hemmorrhagic) or prothrombolytic (hypercoaguable)
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What labs are increased with exudates?
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WBC and lactate dehydrogenase
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What are most common cuases of exdates
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Infections/inflammation
repain/angiogenisis (new vessles are leaky) Malingnancy which also causes angiogenisis |
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What are the lab values for tansudates?
Causes |
low protein LD does not exceede plasma
Hydrostatic pressure increase Oncotic pressure decrease Lympoh obstruction increases sodium intake |
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What are the causes of local edema due to decreased colloid osmotic pressure?
How does it frequently present? |
periorbital edema
1) Protein loss 2) Decerase protein production 3)movement of fluid into tisse exacerbated by renin/angiotens/aldo system 4)Generalized Edema |
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What is lymphedema?
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imparedlymoh drainage leading to edema
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What are the 1* and 2* causes of lymphedema
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1* Milroys, or inherited defect of lymph vessles or nodes
2*Aquired, trauma, mets. |
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how does lymphedema often present?
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Localized, unilateral, often extremities
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What is dependent edema?
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Edama that that is gravity/hydrostatic related
Like pregnancy, RV heart failure, air travel |
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What is anasarca?
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severe generalized edema
caused by CHF, shock, and nephrotic syndrome |
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Signs of heart failure?
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Rales
Edema JVD Icterus Hepatomegaly |
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What causes pulmonary edema?
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Left heart failure!!! This is a question!
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What would you expect to see microscopically in chronic pulmonary edema?
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siderophages coming to gobble up the iron from rupture RBCs since the pressure is so high
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what is the 1* cause of edema inthe brain
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hypertensie crisis...
Exhibited by paplidema, confusion, etc. Gyri get smashed |
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What are serous effusions?
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edema in serous cavities that are within membrane surfaces in the abdomen and chest.
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What is present in chlyous effusions
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Triglycerides and chilomicrons... this gives milky color
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What is Hyperemia?
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Consider ACTIVE
local arteriolar dialation (blushing, inflammation) |
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What is congestion?
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Passive hypermia or passive
congestion Caused by obstructed venous return or increased back pressure from CHF |
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What are causes of acute passive congestion?
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Shock
Acute Inflammation or Sudden Right Sided Failure |
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What are the causes of chronic passive congestion?
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In Lung
- Left sided heart failure or mitral stenosis (will see heart failure cellls and brown induration) In Liver and Lower Exteremities -Right sided heart failure -(can see nutmeg liver) |
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What causes hepatic congestion
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Right sided heart failure
will see nutmeg liver ewlevated ALT, AST, LD and siderophores that can cause fibrosis |
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What are the causes of hepatosplenomegaly?
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Right HF
Portal vein occlusion hepatic cerrhosis |
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What is brown induration?
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Brown= hemosideran laden macrophages
Induration = hardening (due to fibrosis) Venous insufficiency due to bad deep valves. complications include stasis ulceration |
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True or false:
Thrombus is always pathological |
True
It is an overextension of the bodys function |
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What are lines of Zahn
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The striped features of a thrombus higlighting alternating pale pink bands of platelets with fibrin and red bands of RBC's forming a true thrombus
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Where do internal hemorrhages occur?
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Brain
Lung |
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Where are external hemorrhages?
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GI
GU Ducts/Skin |
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Blunt force injury that damages vessels without disruption of tissue
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Contusion
(bruise, echimosis) |
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What do you call petechia of the intestines?
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punctuate hemmorhages
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What is the difference between Purpura and Ecchymoses?
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Eccymoses are larger
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What causes hepatic congestion
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Right sided heart failure
will see nutmeg liver ewlevated ALT, AST, LD and siderophores that can cause fibrosis |
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What are the causes of hepatosplenomegaly?
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Right HF
Portal vein occlusion hepatic cerrhosis |
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What is brown induration?
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Brown= hemosideran laden macrophages
Induration = hardening (due to fibrosis) Venous insufficiency due to bad deep valves. complications include stasis ulceration |
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True or false:
Thrombus is always pathological |
True
It is an overextension of the bodys function |
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What are lines of Zahn
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The striped features of a thrombus higlighting alternating pale pink bands of platelets with fibrin and red bands of RBC's forming a true thrombus
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Where do internal hemorrhages occur?
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Brain
Lung |
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Where are external hemorrhages?
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GI
GU Ducts/Skin |
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Blunt force injury that damages vessels without disruption of tissue
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Contusion
(bruise, echimosis) |
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What do you call petechia of the intestines?
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punctuate hemmorhages
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What is the difference between Purpura and Ecchymoses?
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Eccymoses are larger than 1-2 cm
blue/purple to blue green to brown/yellow |
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What are petechia or puntuate hemorrhages associated with?
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Thromobocytopenia
(Low or dysfunctional platelets) and suffocation |
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What are purpura?
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Confluent petechia greater than 3mm caused by low or dysfunctioanl platelets (throbocytopenia) and vascular inflammation or trauma
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Where do you find bridging strands of fibrous tissue or blood vessles?
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in laceration wounds (Blunt force trauma)
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What type of skin injury usually heals without a scar?
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Abrasion
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Hemoptysis;
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Coughing up blood
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Excessive menstrual bleeding is called?
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Menorrhagia
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Signs of Primary Hemostasis Disorders
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excessive beleeding gums, nosebleeds, gi bleeds, cuts, and bruises
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What is primary hemostasis?
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it is the intimidate response to a vascular injury.
Endothelium + Platelet component |
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What is secondary hemostasis?
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Permanent clot/healing components
Involves: procoagulants + anticoagulants + fibrinolytic agents |
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What are the prothrombolytic effects/componentsof the endothelium?
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vWBF stored there
Procoagulants like tissue factor and 9a & 10a and antifibrionolyic plasminogen activating inhibotor made here |
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What are in the a-granules of platelets?
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P Selectin
Fibrinogen/Fibrnectin Factors V and VIII Heparin binding PF 4 Growth Factors PDGF, TGF-B |
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What are in the dense granules of platelets?
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Metabolism and recruitment factors
ADP Seratonin Epinpherine Calcium |
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What two disease can cause adhesion dysfunction?
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von Willebrand disease
Bernard-Soulier syndrome GP1B deficienct which is on platelets |
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GpIIb GpIIIa have what function? What is the defect in this refered to as?
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Forms fibrinogen bridge aiding in aggregation of platelets in 1* homeostasis.
Glanzmann's thrombasthenia |
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What are the antiplatelet drugs?
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ADP receptopr inhibitors (Plavix)
NSAIDS (block TxA2) GIIbIIIa Inhibitors |
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What is the role of prostacyclin, PGI2?
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it is a vasodialator and strong platelet aggregate inhibitor
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What are the causes of thrombocytopenia and what is it?
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It is a low platelet disorder
caused by bone not making enough or too many being destroyed in periphery like in autoimmune and HIV disorders |
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What are the lab tests for platelet disorders?
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Bleeding time
Platelet function analyzers (neasures bleeding time) Thromboelastograph |
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When are bleeding times prolonged?
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in von willebrand and platelet dysfunctions
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what platelet disorder causes storage pool defects? what are they?
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Glansmann Thrombasthenia
decreased a-granules and dense bodies |
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What therapy is monitored with PT?
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coumadin
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What therapy is monitored with aPPT?
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unfractionated heparin
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What clotting factor deficiencies are X linked? How do you test?
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Factors 8 & 9
Tested via aPTT test |
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What clotting factor disease are usually autosomal dominant?
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vWB disease (rarely recessive)
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How are most of the clotting factor deficiencies pass on?
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autosomal Recessive
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What role does vitamin k have in anticoagulation?
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allows calcium binding for procoagulants protein C and protein S
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What role does vitamin k have in procoagulation?
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it is mostly PRO coagulation
Allows for ca binding for facttors 2, 7, 9 10 |
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What factors are vitamin K dependent?
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2,7,9,10
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How does coumadin/warfarin work as a blood thinner?
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it is a vitamin K antagonist so it hinders clotting/ factors assocaited with vitamin k
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People with homoplillia a and b have what prolonged lab?
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aPTT
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What are the natural anticogualants?
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Antithrombin III
Protein C Protein S |
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How does antithrombin III work?
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It is activated by binding to heparin like molecuels on endothelial cells
The complex then inhibits thrombin and factors 9,10 and 11 |
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What does activated protein c & s incavtivate?
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5a and 8 a
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What are the activators of the fibrinolytic cascade/
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Factor 12a dependent
tPA Uroklinase Streptokinase |
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What are the the products of the fibrinolyitc cascade?
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fibrin degradation products and d-dimer
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What are the components of Virchows Triad?
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Endothelial Injury
Stasis or turbulance of blood hypercoaguability Pathogenisis of throimbosis |
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What injurious agents to the endothelium can cause thrombosis?
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Cigarette smoke
Sepsis (acitvates endotelium) Malignancy |
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What are the most to least common thrombophillias?
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Factor V Liden mutation
Prothombin 20210A Mutation _____________ Proteins C, S And antthrombin III _____________ Hyperhomocysteinemia (very rare!) |
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Describe Factor V Liden Mutation
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Most frequent cause of hereditary thrombophillia
Mutation causes factor Va to be resistant to digestion of activated protein C |
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60% of patients with recuttent DVT have what? how can you test?
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Factor V Leiden
Tested acitvated protein c resistance |
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Describe prothrombin20210A
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2nd most common hereditary thrombophillia
gene mutationresults in 3x the amount of prothrombin being made and therefore more thrombosis |
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What is the cause of hyperhomocystinemia?
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mutation in meythyhydrofolate reductase which causes increase in homocystine which can cause thrombosis
Can be reduced by B6 and B12 suppliments |
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What is the most typical cause of Secondary Hypercoaguability
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bedrest/immobilization
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What is HIT?
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heparin induce thrombocytopenia
Consequence of high Molecular weigh heparin where antibodies to heparin/platelet complex can cause thrombosis |
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Describe Antiphospolipid antibody syndrome
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prothrombic disorder wehre autoantibodies complex with antigens on phospolipids. results in
-Fetal loss thromboembolism -thromboCYTOPENIA AND NEUROLOGICAL CONDITIONS |
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Libman-Sacks Endocarditis is associated with what thrombolytic disorder
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Antiphospholipid antibody syndrome
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What factor results in along PTT but has no risk for bleeding or clotting?
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XII
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Superficial saphenous ithrombophlebits usually cause what complications?
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Venous insufficent and vericose ulcers of the skin but NOT (rarely) embolous
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What lab test EXCLUDED DVT?
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Normal D-Dimer
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What are the most common causes of arterial thrombi?
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Athrosclerosis
-Turbulance -Endothelial injury |
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What are the fate of thrombi?
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Embolize
Dissolve Propagate (grow bigger) Scarrin- Organization and recanilization (vessle can look occluded) |
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Types of Emboli
-Which is most common? |
FAT BAT
Fat Air Thrombous (Most Common) Bacteria Amniotic Fluid Tumor |
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What are the most common source of pulmonary emboli?
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95% in deep veins of the leg above the popiliteal fossa
Second most common are right mural thrombi |
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What pulomnary Emboli are fatal?
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Saddle
they cause obstructive shock |
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What gross or microscoopic feature may be seen with Pulmonary artery thrombosis
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Web Formation ???
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What is the predominat source of arterial thrombi?
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Mural thrombi (80%) for left ventrical with infarct or left atrium with fibrilation
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What are other non mural sources of arterial thrombi?
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Aortic aneurism
ulcerated atherosclerosis vegetation paradoxical embolism |
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Describe Trousseaus syndrome
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Some malignancies, especially adenocarcinomas of the pancreas and lung, are associated with hypercoagulability. Can result in DIC or other clots
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What causes 99% of all infarcts?
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Thromboembolytic diseases
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Where do you tend to see whit infarcts?
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Heart kidney Spleen
There are not collaterals to provide revascualriazation |
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Where do you tend to see red infarcts?
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Lung
testes ovaries intestines Multiple blood source |
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How long can neurons survive infarct? Cardian myocytes? Fiibroblasts?
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Neurons 3-4 minutes
Myocytes 30-60 minutes Fibroblasts and skeletal muscle can tolerate hours |
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What is the job of endothelial lipoprotein lipase?
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Extracts TAG from VLDL for storage in fat and use in muscle
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Where do intermediate density lipoproteins go/
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It has two options...
1)Uses LDL receptor to get to liver (50%) or other organs 2)Metabolized to LDL in the liver where excess gets eaten by mactophages that turn into foam cels |
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What is Neiman Pick type C disorder
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Problem with NPC tranport protin that takes cholesteol out of the lysozyme for use by the cells ????/
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What is 7 HMG Co-A Reductase used for?
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it is the RLS for cholesterol synthesis
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What does cholesterol acitvate and inhibit?
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Activates ACAT which favors intercellular cholesterol storage and down regulation of LDL receptor syntehsis
It inhibits HMG Co A reductates at that just makes more cholesterol |
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What is the most common cause of Famillal Hyperlipidemia?
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LDL Receptor defects
Autosomal Dominant |
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Accumulation of cholesterol in the vessel walls is called ___________ while accumlation in the skin is called ___________.
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atherosclerosis
xanthomas |
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What is difference between heterozygous and homozygous with familial hypercholesterolemia?
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Herterozygoes have 2-3x the amount and develop sx in early adulthood
Homo zygote have total cholesterol >500 and exhibit problems as young children |
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What are the three steps in healing damaged intima?
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Recruitment of smooth muscle
smooth muscle mitosis elaboration of ECM |
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What are the 2 factors regulating blood pressure?
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Cardiac output and TPR
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Hypertensive criteria
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140/90 or greater
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What is difference between essential secondary and benign htn?
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Most common is Essential (90-95%) Idiopathic
Secondary usually due to a kidney proble, Benign means that is is modest and has been fairly stable for several years |
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What is malignant HTN?
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>200/120
Will cause death within 2 years if untreated |
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What are two types of arteriolosclerosis and what are they associated with?
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– Hyaline arteriolosclerosis (benign <120 dyastolic)
– Hyperplastic arteriolosclerosis (malignant >120 diastolic) Looks like inions |
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What disease is common in hyaline arteriolosclerosis?
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Diabetes melitus
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What are the three patterns of
aterioslcerosis? Which is most important? |
Atherosclerosis (dominant pattern) ‐
Monckeberg’s medial calcific sclerosis ‐ Arteriolosclerosis ‐ Proliferation or hayline thicking of small vessles |
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What is the lesion that characterizes
atherosclerosis? |
Lesion is an atheroma or fibrofatty plaque
Raised focal plaque within intima with a lipid core and covering fibrous cap |
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Constitutional Risk factors of Ischemic heart disease?
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Increaseing Age
Male Genetics Family History |
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Modifiable Risk factors of Ischemic heart disease?
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Hyperlipidemia
Hypertension DM Cigarette smotking CRP |
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Describe the response to injury hypothesis of vasculture
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Endothelial injury
Accumulation of lipoproteins Monocyte adhesion to endothelium,migration into intima, transform into macrophages and foam cells – Platelet adhesion Factor release from activated platelets, macrophages and vascular wall cells –Smooth muscle cell proliferation and ECM p production –Lipid accumulation |
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Two biggest factors in endothelial injury
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Hemdynamic disturbances and Hypercholesterolemia
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What happens with Chronic hyperlipidemia?
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Lipoproteins accumulate in intima – Lipds are oxidized by oxygen free radicals
Oxidized LDL ingested by macrophage through scavenger receptor Accumulates in phagocytes = foam cell – Oxidized LDL stimulates release of factors = monocyte recruitment into lesion Oxidized LDL cytotoxic to endothelium – |
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Dysfunctional endothelial cells axpress what adhesion molucule?
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VCAM 1
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When do fatty streaks appear?
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Cna be before 1 year, but usually before 10 years
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What is the hallmark of atherosclerosis?
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– Atheromatous plaques
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What are the components of an Atheromatous plaque?
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Cells SMC’s macrophages T lymphocytes
– ECM – Lipids ‐ intra and extracellular |
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When does acthrosclerosis become "critical"?
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With a 70% occlusion
Angina may be felt |
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What are the complications of atherosclerotic occlusions?>
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– Mesenteric occlusion = bowel ischemia
Chronic ischemic heart disease – Ischemic encephalopathy – Intermittent claudication |
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What are the three categories of acute Plaque change?
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Rupture/fissuring
Erosion/ulceration Hemorrhage into the atheroma |
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Consequences of atherosclerosis major
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Myocardial infarction (heart attack)
Cerebral infarction (stroke) Aortic aneurysms Peripheral vascular disease (gangrene of legs) |
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False Aneurism
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– Extravascular hematoma that
communicates with intravascular space |
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List Disorders what can contribute to aneurysm and the component that goes with...
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Marfan syndrome – defective Fibrillin
Loeys‐Dietz syndrome- abnormalities of elastin & collagen Ehlers‐Danlos syndrome- Defective collagen type III synthesis Vitamin C deficiency- Altered collagen cross‐linking |
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Most common predisposing factors for aneurysms
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Atherosclerosis and Hypertension
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Thoracic aortic aneurism is associated with what disease?
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syphalis
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Most common preexisitng condition for aortic dissection?
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Cystic medial degeneration
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Type A Debakey
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Aortic Dissection
proximal lesions, affect ascending or both ascending and descending |
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Type B Debakey
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Aortic Dissection
distal lesions, beginning distal to subclavian |
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What are the two most common mechanisms
for vasculitis? |
Immune and Infectious
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What are common clinical findings for vasculitis?
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–Fever, myalgias, arthralgias, malaise
–Tissue ischemia |
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What are the three main mechanisms which initiate noninfectious vasculitis?
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Immune complex depostion
Antineutrophil cytoplasmic antibodies Anti‐endothelial cell antibodies |
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What is an ANCA?
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Antineutrophil Cytoplasmic antibodies
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What are the two types of ANCA?
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• Anti‐myeloperoxidase (MPO‐ANCA)
• Anti‐proteinase‐3 (PR3‐ANCA) |
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What is P-ANCA associated wtih?
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microscopic polyangiitis,
Churg‐Strauss syndrome |
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What is C-ANCA associated wtih?
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Wegners
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Anti‐Endothelial cell antibodies are associated with what vasculitides?
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Kawasaki Disease
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Giant cell (temporal) arteritis affects what size vessels? What is the major morphologic finding?
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large
Graulomatous Inflammation Sen in people over 50 |
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Giant Cell (Temporal) Arteritis is associated with what other disorder?
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polymyalgia rheumatica
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What are the significant clinical features with giant cell temporal arteritis? How do you get a dx?
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get sed rate and biopsy
One sized vision loss which can be perminant needs to be top concern. Treat with steroids |
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Takayasu arteritis affects what size vessel? What is the other name ?
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Large
also known as "aortic arch syndrome", "nonspecific aortoarteritis" and the "pulseless disease |
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What are the major morphologic findings of Takayasu?
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intimal fibrosis and vascular narrowing of the aorta and sometimes pulnonary arteries
Blockage due to granulomatous inflammation |
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What are the major sx of Takayasu?
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Occular disturbances and weakening of pulses in upper extremity
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Who is most commonly affected with Takayasus?
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Young asian women in their 20's
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PAN affects what size vessels?
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Medium
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PAN affects renal and visceral vessels, what does
it spare? |
Pulmonary
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What are the morphologic features of PAN?
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Segmental transmural necrotizing inflammation
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Do patients with PAN get glomerulonephritis?
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Renal artery often involved, major cause of death however small vessels not affected so no
glomerulonephritis |
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Common manifestations of Polyarterits Nordosa...
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Fever of unknown origin, weight loss,
hypertension, abdominal pain, melena, muscle aches and pains, peripheral neuritis |
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Treatment/Prognosis for PAN?
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Fatal if untreated, corticosteroids and
cyclophosphamide result in 90% remission/cure |
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What are the three features of Churg Strauss syndrome? What anca is associated wtih it?
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Asthma, allergic rhinitis
and eosinophillia P-ANCA in 50% OF Patients |
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What other vasculitis has the same
characteristic lesions as Churg Strauss? |
PAN- you can see granulomas
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What is the other name for Kawasaki disease?
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Mucocutantous Lymphnode syndrome
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What arteries does Kawasaki involve?
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Coronary
• Coronary arteritis can lead to aneurysm that rupture or thrombose = acute MI • Leading cause of acquired heart disease in children |
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how does Kawasaki appear?
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• Fever conjunctival and oral erythema and • Fever, conjunctival and oral erythema and
erosion, edema of hands and feet, erythema of palms and soles and later desquamation of palms and soles and later desquamation • Cervical lymphadenopathy |
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how do you treat Kawasakis?
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IV immunoglobulin and ASA
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What size vessels are affected in microscopic polyangiitis? How does it present? •
• Major clinical features? |
Also called hypersensitivity vasculitis pr leukocytoclastic vasculitis
Pressents with palpable pupura Clinically see hemoptysis, hematuria, proteinuria, arthralgias, bowel pain, muscle pain/weakness, hemorrhage |
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How can you make a dx for microscopic polyangiitis?
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P-ANCA and skin biopsy
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What is the triad that characterizes Wegener Granulomatosis?
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Acute necrotizing granulomas of Upper and Lower respiratory tracts
nec/gran vasculitis of small and medium vessles primarily in upper lung/airway Renal diesase |
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What is the clinical clue for Wegners? HOw do you diagnose? Whos is affected?
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Ulcerative lesions of nose, pharynx, or palate
Dx with C-ANCA M>F over 40 y |
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What is Thromboangiitis Obliterans?
Who get it? |
–Buerger Disease
–Segmental, thrombosing, acute and chronic inflammation of intermediate and small arteries. YOUNG MALES THAT ARE HEAVY SMOKERS |
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What disorder has cold sensitivity of the hands? What should the DDX also include
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(Raynaud)
Happens with younf women, but be sure to rule out SLE. Sclederma, Bergers, atherosclerosis |
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What is a disorder of the deep vein legs called that can cause clots to form?
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Thrombophlebitis
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What may be the first sign of a DVT?
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Thrombophlebitis
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The superior vena caval syndrome is cause by what? Clinical Features?
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Neoplasmsn that compress: Carcniomas or lymphomas
Features are: Dusky Cyanosis dialation of veins of head and arms: (Deoxygenated blood is trying to return) |
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The inferior vena caval syndrome is caused by what? Clinical features?
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Compression of trhe inverior vena cava or thrombus that propigates upward
Clinical: Leg edema, distention of superficial lower legs and Massive proteinuria since the renal veins are involved |
