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Porphyrias-Part 2
See all quizzes of Porphyrias at here:
1 Urinary PBG is virtually always increased during acute attacks
of ?
A. AIP
B. HCP
C. VP
D. All of the above
2 Which of the following is false about acute intermittent porphyria
(AIP) ?
A. Autosomal recessive
B. Due to half-normal level of HMB-synthase activity
C. Common in Scandinavia & Great Britain
D. Almost always latent before puberty
3 Attacks of AIP can be provoked by ?
A. Infections
B. Surgery
C. Ethanol
D. All of the above
4 Which of the following is false about acute intermittent porphyria
(AIP) ?
A. Abdominal pain is the most common symptom
B. Urinary retention is characteristic
C. Peripheral neuropathy is due to axonal demyelinization
D. ALA & PBG levels increased in plasma & urine during acute
attacks
5 In AIP, excretion of ALA & PBG decrease dramatically after ?
A. Intravenous glucose
B. Intravenous hemin
C. Intravenous calcium
D. Intravenous amono acids
6 In AIP, treatment of seizures is preferred with the use of ?
A. Clonazepam
B. Phenytoin
C. Barbiturates
D. Any of the above
7 In AIP, heme is given in the form of ?
A. Lyophilized hematin
B. Heme albumin
C. Heme arginate
D. All of the above
8 Long-term risk of which of the following is increased in AIP ?
A. Hypertension
B. Chronic renal disease
C. Hepatocellular carcinoma
D. All of the above
9 Which of the following is deficient in all types of porphyria cutanea
tarda (PCT) ?
A.
B.
C. Hepatic URO-decarboxylase
D. COPRO-oxidase
10 Which of the following is not an autosomal dominant hepatic
porphyria ?
A. Hereditary Coproporphyria (HCP)
B. Variegate Porphyria (VP)
C. Hepatoerythropoietic porphyria (HEP)
D. Variegate Porphyria (VP)
11 Susceptibility factor in type 2 PCT is ?
A. Hepatitis C
B. HIV
C. Elevated iron levels
D. All of the above
12 In PCT, skin friability & small white papules on back of hands &
fingers is called ?
A. Milia
B. Trichia
C. Pilia
D. Cryslia
13 Which of the following statements about PCT is false ?
A. Neurologic features are absent
B. Hemochromatosis gene (HFE) mutations frequent
C. Chronic liver disease present
D. None of the above
14 URO-decarboxylase activity in liver, erythrocytes is very
significantly decreased in ?
A. Type 1 Porphyria cutanea tarda
B. Type 2 Porphyria cutanea tarda
C. Hepatoerythropoietic porphyria (HEP)
D.
15 Which of the following is useful in the treatment of porphyria
cutanea tarda ?
A. Pentamidine
B. Hydroxyurea
C. Chloroquine phosphate
D. Quinine
16 Diagnosis of Hereditary Coproporphyria (HCP) is confirmed by
which of the following ?
A. Urinary ALA
B. Urinary PBG
C. Plasma porphyrins
D. Increased fecal porphyrins
17 Variegate porphyria (VP) results from the deficient activity of ?
A.
B. URO-synthase
C. PROTO-oxidase
D. COPRO-oxidase
18 Variegate porphyria (VP) is particularly common in ?
A. China
B. Brazil
C. Australia
D. South Africa
19 Which of the following is increased in Variegate porphyria
(VP) ?
A. Fecal protoporphyrin
B. Plasma porphyrin
C. Urinary COPRO III
D. All of the above
20 Which of the following is called Günther disease ?
A. X-Linked Sideroblastic Anemia (XLSA)
B. Congenital Erythropoietic Porphyria (CEP)
C. Erythropoietic Protoporphyria (EPP)
D. Hepatoerythropoietic porphyria (HEP)
Congenital erythropoietic porphyria (CEP) is also called Günther disease.
21 Which of the following is the most common erythropoietic
porphyria in children ?
A. X-Linked Sideroblastic Anemia (XLSA)
B. Congenital Erythropoietic Porphyria (CEP)
C. Erythropoietic Protoporphyria (EPP)
D. Hepatoerythropoietic porphyria (HEP)
22 Which of the following is an autosomal recessive disorder ?
A. Hepatoerythropoietic porphyria (HEP)
B. Congenital Erythropoietic Porphyria (CEP)
C. Erythropoietic Protoporphyria (EPP)
D. All of the above
