Porphyrin and Bilirubin Flashcards
(21 cards)
What are two spontaneous oxidation products in heme synthesis?
Uroporphyrin and Coproporphyrin
What is the Porphyria described below?
<p>
Porphobilinogen Deaminase Deficiency
High ALA and PBG.
This porphyria is neurologic</p>
<p>
| Acute Intermittent Porphyria (AIP)</p>
Which Porphyria is described below?
<p style="text-align: center;"> Protoporphyrin Oxidase Deficiency</p> <p style="text-align: center;"> High ALA and PBG</p> <p style="text-align: center;"> High URO and COPRO</p> <p style="text-align: center;"> Neurologic and Cutaneous</p>
<p>
| Variegate Porphyria</p>
Which Porphyria is described below?
<p style="text-align: center;"> Coproporphyrin Oxidase Deficiency</p> <p style="text-align: center;"> Similar to Variegate Form</p> <p style="text-align: center;"> Neurologic and Cutaneous</p>
<p>
| Coproporphyria</p>
Which Porphyria is described below?
<p>
Uroporphyrinogen III cosynthetase deficiency</p>
<p>
High URO I</p>
<p>
Cutaneous only because it is only decreased in activity by a small amount</p>
<p>
| Congenital Erythropoietic Porphyria</p>
Which Porphyria is described below?
<p style="text-align: center;"> Ferrochelatase deficiency</p> <p style="text-align: center;"> High Protoporphyrin</p> <p style="text-align: center;"> Cutaneous</p>
<p>
| Protoporphyria</p>
Which Porphyria is described below?
<p style="text-align: center;"> Uroporphyrinogen decarboxylase deficiency</p> <p style="text-align: center;"> High URO</p> <p style="text-align: center;"> Cutaneous</p> <p style="text-align: center;"> </p>
<p>
| Porphyria Cutanea Tarda</p>
<p>
| When assaying for bilirubin; reaction in water detects what kind of bilirubin?</p>
<p>
| Direct or conjugated bilirubin</p>
<p>
| When assaying for bilirubin; reaction in alcohol detects what kind of bilirubin?</p>
<p>
| Direct and Indirect bilirubin</p>
<p>
| How do you assay for Indirect bilirubin?</p>
<p>
Do the reaction in water to get Direct Bilirubin.</p>
<p>
Do the reaction in alcohol to get Total Bilirubin.</p>
<p>
Indirect Bilirubin= Total - Direct</p>
<p>
| Prehepatic Jaundice is primarily caused by what?</p>
<p>
| Overproduction of bilirubin-> Hemolytic Anemia</p>
Which Jaundice is described below?
<p style="text-align: center;"> Caused by overproduction of bilirubin</p> <p style="text-align: center;"> High Indirect Bilirubin</p> <p style="text-align: center;"> High urobilinogen in stool, serum, and urine</p> <p style="text-align: center;"> NO bilirubin in urine</p>
<p>
| Prehepatic Jaundice</p>
Which Jaundice is described below?
<p style="text-align: center;"> High Direct and Indirect Serum Bilirubin</p> <p style="text-align: center;"> High Urinary bilirubin</p> <p style="text-align: center;"> High Urinary Urobilinogen</p>
<p>
| Hepatic Jaundice</p>
Which type of Jaundice is described below?
<p>
| Characterized by a transient glucuronyl transferase deficiency</p>
<p>
| Neonatal Jaundice</p>
<p>
| What is kernicterus?</p>
<p>
| Mental retardation due to the buildup of unconjugated bilirubin which can cross the blood brain barrier</p>
<p>
| Kernicterus can result from which type of jaundice?</p>
<p>
| Neonatal</p>
Which syndrome is described below?
<p>
Leads to mental retardation</p>
<p>
Conjugation defect in which the person is homozygotic for the gene that encodes glucuronyl transferase</p>
<p>
| Crigler-Najar Syndrome</p>
Which Disease is described below?
<p>
Heterozygote for the gene encoding glucuronyl transferase</p>
<p>
Persistant low level prehepatic jaundice</p>
<p>
| Gilbert's Disease</p>
Which Syndrome is described below?
<p style="text-align: center;"> Defect in secretion of bilirubin into the bile duct</p> <p style="text-align: center;"> Results in:</p> <p style="text-align: center;"> High Urinary Bilirubin</p> <p style="text-align: center;"> Low Urinary Urobilinogen</p> <p style="text-align: center;"> High Serum Conjugated bilirubin</p> <p style="text-align: center;"> Normal serum unconjugated bilirubin</p>
<p>
| Dubin-Johnson Syndrome</p>
Which type of Jaundice is described below?
<p style="text-align: center;"> High serum direct bilirubin</p> <p style="text-align: center;"> High Urinary direct bilirubin</p> <p style="text-align: center;"> Low urobilinogen in serum and stool-> light colored stool</p>
<p>
| Posthepatic Jaundice</p>
