porphyria Flashcards
1
Q
- Urine ALA, PBG
* None in rbc and stool
A
– AIP
2
Q
– CEP versus HEP
A
- Uro in RBCs of CEP
* Proto in RBCs of HEP
3
Q
– VP versus PCT
A
• Copro > or = Uro in urine in VP
4
Q
• Copro in Urine, rbcs and stool
A
– HCP
5
Q
- Uro in Urine
* Proto in rbc and Copro in stool
A
– HEP
6
Q
- None in urine
- Proto in rbc and stool
- Rbcs transiently fluoresce
A
– EPP
7
Q
- Urine– URO
- Uro rbc and Copro stool
- Rbcs and teeth fluoresce
A
– CEP
8
Q
• Urine
– Uro < or = Copro
– None in rbc and Proto in stool
– Plasma fluoresces at 626 nm diagnostic
A
– VP
9
Q
• Urine fluoresces
– Uro > Corpo (3:1 or greater)
• None in rbc and Isocopro in stool
A
– PCT
10
Q
- Gunther’s
- Red urine soon after birth
- Red teeth
- Hypertrichosis (werewolf appearance)
- Extreme photosensitivity
A
– CEP
11
Q
only oxidized porphyrin
A
• Protoporphyrin IX
12
Q
- Porphyrins deposited in liver
* Beta carotene may help
A
– EPP
13
Q
– EPP
• Immediate burning on sun exposure
?? sunlight can trigger
A
• Protoporphyrin IX absorbs at 400-410 nm and 500-600 nm
14
Q
– ALA dehydrase deficiency
– Acute intermittent porphyria (AIP)
– Hepatic coproporphyria (HC)
– Variegate Porphyria (VP)
A
Porphyrias
• Acute
15
Q
1 type Porphyrias
A
– PCT
