Unit 5 Flashcards
What is heme composed of?
Protoporphyrin ring + Fe
What is hemoglobin composed of?
Heme + 4 globin chains
What is the storage form of iron?
Fe3+
Ferric
What is the functional form of iron?
Fe2+
Ferrous
What are porphyrins?
Any class of pigments whose molecules contain a flat ring of four linked heterocyclic groups
What is Porphyrias
Diseases caused by enzyme deficiencies in the Heme production pathway.
*People will generally look purple.
What is Hemin
A black inorganic compound which forms from heme when RBCs are lysed and exposed to air.
What is the most common Primary Porphyrias?
Porphyria Cutanea Tarda (PCT)
What is Porphyria Cutanea Tarda deficient in?
Uroporphyrinogen Decarboxylase
What test is ordered to confirm PCT
Serum porphyrin
What is PCT defined by?
Blistering
What is Acute Intermittent Porphyria deficient in
PBG Deaminase
What is AIP defined by
Blistering
Nerve Impairments
What test is ordered to confirm AIP
Urine porphobilinogen
What is Erythropoietic Porphyria deficient in
Ferrochelatase
What will Erythropoietic Porphyria show an increase in
Total erythrocyte protoporphyrin
What is Erythropoietic Porphyria defined by
Blistering
What is the rate limiting step in porphyrin synthesis
ALA-Dehydratase
How do we test for ALA-Dehydratase Deficiency
Delta-aminolevulinic
What is the most common presenting symptom of ALA-Dehydratase deficiency?
Abdominal pain
What does ALA-Dehydratase deficiency cause
Accumulation of d-aminolevulinic acid
What does lead poisoning do?
Inhibition of most of the enzymes in the synthetic pathway
What secondary porphyria has an elevated level of succinylacetone
Hereditary tyrosinemia
What method do we use for Porphobilinogen
Watson-Schwartz
What Porphobilinogen method do we not use but is tested on
Hoesch
What is a must for all samples being tested for Porphyrins
MUST BE LIGHT PROTECTED
How are porphyrins evaluated
HPLC
What is a screening test for lead poisoning
Ferrochelatase
What form of Bili is lipophilic
Indirect/Unconjucated
What form of Bili is hydrophilic
Direct/Conjugated
Syndromes from an increase in unconjugated bilirubin
Crigler-Najjar
Gilbert
Syndromes when conjugated Bilirubin with biliary obstruction
Dubin-Johnson
Rotor
What is delta bilirubin
Conjugated bilirubin that is covalently bound to Albumin
How is hem mostly produced
Breakdown of RBCs in the spleen
What does Heme Oxygenase in macrophages do
Oxidize heme to biliverdin
What does biliverdin reductase in macrophages do
Reduces biliverdin to bilirubin
What is bilirubin bound to while being transported
Albumin
What conjugates bilirubin
UGT1A1
Uridine Diphosphate Glucuronyltranferase
What are the serious autosomal dominant Hyperbilirubinemia disorders
Dubin-Johnson (conjugated)
Crigler-Najjar (Unconjugated)
What are the weak sauce autosomal recessive Hyperbilirubinemia disorders
Rotor (conjugated)
Gilbert (unconjugated)
What is bilirubin turned into in the gut by bacteria
Stercobilin
What is stercobilin turned into after it is reabsorbed
Urobilin
What is the main cause of Prehepatic Jaundice
Hemolysis
In prehepatic jaundice what are the levels of Unconjugated and conjugated bilirubin
Unconjugated will be drastically increased
Conjugated will be normal or mildly increased
What can cause an increased amount of unconjugated bilirun be caused by
decreased bilirubin uptake by liver cells
Increased bilirubin burden
What is posthepatic jaundice is from?
Failure of liver to excrete conjugated bilirubin
In posthepatic jaundice what are the levels of Unconjugated and conjugated bilirubin
Both will be increased
Why is there jaundice when there is a blocked duct or biliary tract problems?
The body is reabsorbing bilirubin due to bile sludging and bc it can’t get out of the body.
What are the tests that measure bilirubin
Evelyn-Malloy
Jendrassik-Grof
What does Evelyn-Malloy use as an accelerator
Methanol
What does Jendrassik-Grof use as an accelerator
Cafferine-benzoate-acetate
What is the pH for Evelyn-Malloy method?
1.2
What is the absorbance for the Evelyn-Malloy method?
red-purple chromagen at 560nm
What is the absorbance for the Jendrassik-Grof method?
Blue chromagen at 600 nm
What is needed to reduce alcohol interference in a sample?
To be fasting
How does lipemia affected bilirubin
Falsely increases
What does hemolysis do to the bilirubin method
decrease the reaction with diazo reagent
How much bilirubin will be lost in an hour if exposed to light?
50%
How long can bilirubin be stored?
Off cells:
2 days RT
1 week fridge
forever if frozen
What is Hgb electrophoresis first performed on?
Cellulose acetate strip
What is used with cellulose acetate for Hgb electrophoresis?
alkaline buffer (pH 8)
To distinguish Hgb what is the electrophoresis performed on?
Citrate Agar Gel with Acid buffer (pH 6)
What is the order of Hgb from slowest to fastest?
C
S
F
A
What hemoglobins are considered fast Hgb?
Hgb H
Barts
What is Hgb H made out of?
4 beta chains (Beta globin tetramer)
What is Barts Hgb made out of?
4 gamma chains
Standard alkaline and acid electrophoresis can’t distinguish what 2 Hgbs?
Hgb D and G
How is Hgb E made?
Mutated Beta chain
What is HgbA1C?
Glycated hemoglobin
What is carboxyhemoglobin
carbon monoxide binds to hemoglobin and displaces oxygen
What is methemoglobin
Alkaline media or oxidizing agents transform normal Fe2+ into Fe3+, conferring a inability to reversibly bind oxygen
What is Sulfhemoglobin
Sulfation of hemoglobin casues an inability to bind oxygen
Steps in Iron Spectrophotometry Method?
- Release Iron from transferrin with addition of acid
- Reduction of Fe3+ to Fe2+ with ascorbic acid
- React Fe2+ with a chromogen
How much of a carrier protein is bound to Iron
66%
What is the quantitative defect of Hgb
alpha thalassemia
Alpha thalassemia trait has how many genes missing?
2 genes
Hgb H Ds has how many genes
1
3 missing
What Alpha Thalassemia results in utero death?
Hgb Barts/Hydorps fetalis
-All genes are missing
How do we get Hgb H disease?
Inadequate alpha globin production so body produces beta hemoglobin that form together
What is Beta+
reduced beta hgb production
What is B0?
complete lack of beta hgb production
paracentesis fluid is from where?
peritoneal cavity
thoracentesis fluid is from where?
pleural cavity
Athrocentesis fluid is from where?
joint
What is ascites
Pathological accumulation of fluid in the peritoneal cavity
What is effusion
Pathological accumulation of fluid in most other body cavities
what is transudate
an effusion which is a filtrate of the plasma
what is exudate
an effusion which is protein-rich and usually caused by an infection or cancer
*likely has inflammatory cells and occassionally pus
What is Xanthrochromia
a yellowish tinge to CSF
*indicates prior bleeding in the CSF
What is pseudochylous
an effusion which has high levels of cholesterol
What can be found in refridgerated joint aspirations and urine samples
crystals
What can increase due to anaerobic metabolism of cells
Lactate
What is a spinal tap used to diagnosis?
Multiple Sclerosis
Hemorrhage
Meningitis
Other infections of CNS
Where does a spinal tap occur?
Epidural space between L4/5
What is the CSF tube order
1st- Storage/Cytology
2nd- Chemistry/Serology
3rd-Micro
4th- Heme
Milky consistency in Pleural Effusion indicates?
Chylothorax (lymphatic obstruction)
What does a putrid odor in Pleural Effusion indicate?
Anaerobic Empyema
What is glucose directly correlated with in Pleural Effusions?
pH bc the products of glycolysis are acidic
What does Black Pleural fluid suggest
-Malignant melanoma
-non-small cell lung carcinoma
-ruptured pancreatic pseudocyst
-charcoal-containing empyema
Peritoneal fluid is usually taken from patients with?
Ascites
What is ascites?
Abnormal collection of fluid in the peritoneal cavity due to either portal hypertension or low serum osmolality
What is transudates Specific Gravity
<1.010
What is Exudates Specific Gravity
> 1.020
Why would the rare pericardial fluid test be ordered?
- Therapeutic pericardiocentesis for cardiac temponade
- Clinical suspicion of purulent, tuberculosis, or neoplastic pericarditis
- Large pericardial effusions of unknown etiology
What are the 4 categories of Synovial Effusions
- Inflammatory
- Noninflammatory
- Hemorrhagic
- Septic
What are the routine Synovial testing
Gross examination
leukocyte count
gram stain and bacterial cx
crystal examination
uric acid
When is amniotic fluid traditionally taken?
15-18 weeks
Why would we collect amniotic fluid
Assess for Genetic disorders
How can we distinguish between urine and amniotic fluid
Urea
Glucose
Creatinine
Protein
**Urine has higher value of Urea and Creatinine
Preeclampsia signs and sypmtoms
Hemolysis- Elevated AST, ACP, LD
Elevated Liver Enzymes- AST, ALT, ALP
Low Platelets- profound thrombocytopenia
What is the only reason to evaluate seminal fluid
Infertility
What are the ways we assess seminal fluid
Ejaculate Volume
Total Sperm Count
Concentration
Shape of the head
Movement of the Sperm
What is traumatic tap
The first tube is quite bloody and the blood lessens in each subsequent tube
What is subarachnoid hemorrhage
The first tube is quite bloody and the last tube is just as bloody