Exam 1: Porphyrias Flashcards
1
Q
Porphyrins are:
A
Primary precursors of heme
2
Q
Porphyria is:
A
Inborn deficiency of any enzyme in the heme pathway, leading to accumulation of precursors during times of increased heme production, which are toxic to tissues at high concentrations
3
Q
Inheritance pattern:
A
Autosomal dominant
Chromosome II
Non-sex linked
Variable expression
4
Q
Attacks most frequent in:
A
20 and 30-something females
5
Q
Triggers:
A
Hormonal fluctuations Menstruation Fasting/NPO/hypovolemia Dehydration Stress Infection
6
Q
S/S of attacks:
A
Abdominal pain w/ N/V ANS instability: tachycardia, HTN Electrolyte disturbances Skeletal muscle weakness Resp. failure Seizures Neuropsychiatric disturbances
