Plasma Cholinesterase Abnormalities Flashcards Preview

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Flashcards in Plasma Cholinesterase Abnormalities Deck (23):
1

What is plasma cholinesterase (PC)

An enzyme found in PLASMA and most TISSUES, but NOT in rbcs.

2

What does PC degrade

acetylcholine
succinylcholine
Ester-linked LA
Cocaine
Mivacurium
Pilocarpine
Donepezil

3

How is PC transmitted

Autosomal recessive

4

What chromosome is PC associated with

chromosome 3q26

5

What type of variant is assoc. with PC

Atypical

6

What aa change is noted for PC

70 Aspartine replaced by Glycine

7

What is the name of the test used to determine if a patient has PC

Dibucaine Number

8

How does the Dibucaine test work

The aa Dibucaine will inhibit the NORMAL PC activity by 80% in disease-free patients

The aa Dibucaine will inhibit ABNORMAL PC by only 20%

9

True/False
The Dibucaine test measures activity parameters such as PC plasma concentration and enzyme efficiency

FALSE

10

What is the genotype for someone with PC deficiency

EaEa

11

Some genetic variations are inhibited by

Fluoride

12

What are the genotypes for fluoride deficiencies

Ef
Es

13

What isoenzyme variant of PC results in abnormally rapid hydrolysis of succs

C5 variant

14

What is the Porphyrias

The porphyrias are a group of rare inherited or acquired disorders of certain enzymes that normally participate in the production of porphyrins and heme

porphyrins are the main precursors of heme, an essential constituent of hemoglobin, myoglobin, catalase, peroxidase, respiratory and P450 liver cytochromes.

15

What are the two categories of porphyrias

hepatic
erthropoietic

16

Hepatic porphyrias are further classifed into which four conditions

**Acute intermittent prophyria
Porphyria cutanea tarda
Variegate porphria
Hereditary coproporphyria

17

Which hepatic porphyria is the most important and serious form

Acute intermittent porphyria

18

In AIP deficiency of uroporphyrinogen I synthetase results in

excess formation of porphobilinogen and simga-aminolevulinate which accumulates in the urine

19

The clinical manifestations of AIP include

SEVERE abdominal pain in young-middle aged females
*mistaken for acute cholecystitis, pancreatitis, appendicitis, or renal colic

Demyelination>>motor weakness, ANS dysfunction, emotional disturbances, psychosis, paralysis of resp. muscles

20

What events trigger attacks

starvation
dehydration
sepsis
female hormones
drugs

21

What drug classifications are known to precipitate porphyria

sedative/hypnotics-barb,ket,eto,benz
analgesics-lidocaine
anticonvulsants-phenytoin
antibiotics-sulfa drugs
steroids-Estrogen, Progesterones, corticost.
toxins-ethanol, lead, amphetamines

22

GIve ----- and ------ only for patients with porphyria

opioids and propofol

23

Treatment of acute attacks of AIP

Hydration
Glucose infusion
Hematin infusion
Analgesia