Porphyrin and Bilirubin

Question 1

What are two spontaneous oxidation products in heme synthesis?

Answer 1

Uroporphyrin and Coproporphyrin

Question 2

What is the Porphyria described below?

<p>
Porphobilinogen Deaminase Deficiency
High ALA and PBG.
This porphyria is neurologic</p>

Answer 2

<p>

| Acute Intermittent Porphyria (AIP)</p>

Question 3

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>

Answer 3

<p>

| Variegate Porphyria</p>

Question 4

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>

Answer 4

<p>

| Coproporphyria</p>

Question 5

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>

Answer 5

<p>

| Congenital Erythropoietic Porphyria</p>

Question 6

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>

Answer 6

<p>

| Protoporphyria</p>

Question 7

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>

Answer 7

<p>

| Porphyria Cutanea Tarda</p>

Question 8

<p>

| When assaying for bilirubin; reaction in water detects what kind of bilirubin?</p>

Answer 8

<p>

| Direct or conjugated bilirubin</p>

Question 9

<p>

| When assaying for bilirubin; reaction in alcohol detects what kind of bilirubin?</p>

Answer 9

<p>

| Direct and Indirect bilirubin</p>

Question 10

<p>

| How do you assay for Indirect bilirubin?</p>

Answer 10

<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>

Question 11

<p>

| Prehepatic Jaundice is primarily caused by what?</p>

Answer 11

<p>

| Overproduction of bilirubin-> Hemolytic Anemia</p>

Question 12

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>

Answer 12

<p>

| Prehepatic Jaundice</p>

Question 13

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>

Answer 13

<p>

| Hepatic Jaundice</p>

Question 14

Which type of Jaundice is described below?

<p>

| Characterized by a transient glucuronyl transferase deficiency</p>

Answer 14

<p>

| Neonatal Jaundice</p>

Question 15

<p>

| What is kernicterus?</p>

Answer 15

<p>

| Mental retardation due to the buildup of unconjugated bilirubin which can cross the blood brain barrier</p>

Question 16

<p>

| Kernicterus can result from which type of jaundice?</p>

Answer 16

<p>

| Neonatal</p>

Question 17

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>

Answer 17

<p>

| Crigler-Najar Syndrome</p>

Question 18

Which Disease is described below?

<p>
Heterozygote for the gene encoding glucuronyl transferase</p>

<p>
Persistant low level prehepatic jaundice</p>

Answer 18

<p>

| Gilbert&#39;s Disease</p>

Question 19

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>

Answer 19

<p>

| Dubin-Johnson Syndrome</p>

Question 20

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>

Answer 20

<p>

| Posthepatic Jaundice</p>