CMN 577: UNIT 1 - GENETIC DISORDERS
Questions with Correct Answers | Updated
(100% Correct Answers)
What age most frequently has AIP? Answer: 18-40 years
T/F: Women have greater incidence of AIP than men Answer: TRUE
What are the s/sx of AIP? Answer: 1. intermittent abdominal pain
2. complete resolution of sx between attacks
3. neurological sx -- neuropathy (peripheral or autonomic)
4. nausea, constipation, urinary retention, muscle weakness, and
dark (reddish) urine
What are the neuropathy characteristics and sx of AIP? Answer:
asymmetric or bilateral
mild to severe
may cause respiratory dysfunction, quadriplegia, seizures, psychosis,
and AMS
What labs (+/-) reflect AIP? Answer: 1. significant hyponatremia
2. no fever and absence of leukocytosis
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, 2
3. increased quantities of porphobilinogen found in the urine during
an ACUTE attack
4. Normal findings on US, XR, CAT scan, and MRI
What tests would you order on pt with AIP? Answer: 24h urine
collection or random urine porphobilinogen level
What is the normal value for a typical 24h porphobilinogen level?
Answer: </= 2.5 mg
What is the normal value for a typical random urine
porphobilinogen level? Answer: </= 2.0 mg/L
T/F: Fresh urine may be clear and normal in color but will darken
upon standing for a brief period of time. Answer: TRUE
What are some precipitating factors or exacerbating factors for AIP?
Answer: Smoking, ETOH use, stress, exposure to sunlight,
dieting/fasting
Certain meds -- sulfa, barbiturates, hormones
(estrogen/progesterone), e-mycin, and spironolactone
What is the tx for AIP? Answer: GLUCOSE
High carb diet - at least 300 grams of carbs/day during acute
attacks.
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