USMLE RX SELF-ASSESSMENT ANALYST EXAM
QUESTIONS & CORRECT ANSWERS ACED RESULTS
Patients with Chronic Granulomatous Disease (CGD) have an increased susceptibility to what
organisms? - CORRECT ANSWER >>>-Catalase positive organisms
-CATs Need PLACESS to Belch their Hairballs
-CAT +
-Nocardia
-Pseudomonas
-Listeria
-Aspergillus
-Candida
-E. coli
-Staphylococcus
-Serratia
-Burkholderia cepacia
-H. pylori
What enzyme is deficient in patients with CGD? - CORRECT ANSWER >>>NADPH oxidase
Chronic Granulomatous Disease (CGD) - CORRECT ANSWER >>>defect in NADPH oxidase →
↑ susceptibility to infections with catalase + organisms
Impaired respiratory burst in neutrophils
1|Page
,Abnormal flow cytometry test (decreased green fluorescence)
Nitroblue tetrazolium dye reaction test fails to turn blue
What two tests can be used to diagnose CGD? - CORRECT ANSWER >>>1. Dihydrorhodaomine
(flow cytometry) test will show reduced fluorescence
2. Nitroblue tetrazolium dye reduction test; will fail to turn blue
What is the most common inheritance pattern for CGD? - CORRECT ANSWER >>>X-linked
recessive
More common in boys
vEDS is caused by pathogenic mutations in the ________________ gene - CORRECT ANSWER
>>>COL3A1 gene
encodes collagen type III alpha chain
________________ collagen is a fibril-forming collagen essential for mechanical strength and
elasticity of expansible tissues. - CORRECT ANSWER >>>Type III
The treatment of choice for Pneumocytis pneumonia is - CORRECT ANSWER
>>>trimethoprim-sulfamethoxazole
Terbinafine inhibits the fungal enzyme _______________________ and is used to treat
dermatophytoses - CORRECT ANSWER >>>squalene epoxidase
2|Page
,Pneumocystis pneumonia - CORRECT ANSWER >>>-caused by Pneumocytis jirovecii
-diffuse interstitial pneumonia
-Yeast-like fungus
-Disc-shaped yeast on methenamine silver stain
-Immunosuppression predisposes to disease
-Diffuse bilateral ground-glass opacities on chest imagine, with pneumatoceles
-Treatment/ Prophylaxis: TMP-SMX, pentamidine, dapsone, atovaquone
Pneumocytis pneumonia prophylaxis with TMP-SMX should be started in patients with HIV
infection with CD4 counts < _____________ - CORRECT ANSWER >>>< 200 cells/mm3
McArdle Disease - CORRECT ANSWER >>>-deficiency in myophosphorylase
-glycogen storage disease type V
-muscle cramps with exercise, red-colored urine due to myoglobinuria
-no increase in lactate
-Elevated CK
3|Page
, Myophosphorylase deficiency results from pathogenic mutations in the ___________ gene -
CORRECT ANSWER >>>PYGM gene —> encodes myophosphorylase
Myophosphorylase - CORRECT ANSWER >>>-Deficient in McArdle Disease (Type V)
-Liberates glucose-1-phosphate units from glycogen
alpha-1,6-glucosidase is one of the two catalytic activities of the debraching enzyme, which
liberates glucose from glycogen branch-points. Deficiency in this enzyme causes
____________________________ - CORRECT ANSWER >>>Cori Disease
-Hypoglycemia
-Increased liver glycogen
-Hepatomegaly
-Normal lactate
-No muscle cramping
PYGM gene mutation - CORRECT ANSWER >>>-Results in McArdle Disease (Type V)
-Myophosphorylase deficiency
Tinea versicolor, also called pityriasis versicolor, is caused by _____________________ infection
and most often occurs on the upper back or chest. - CORRECT ANSWER >>>Malassezia furfur
4|Page
QUESTIONS & CORRECT ANSWERS ACED RESULTS
Patients with Chronic Granulomatous Disease (CGD) have an increased susceptibility to what
organisms? - CORRECT ANSWER >>>-Catalase positive organisms
-CATs Need PLACESS to Belch their Hairballs
-CAT +
-Nocardia
-Pseudomonas
-Listeria
-Aspergillus
-Candida
-E. coli
-Staphylococcus
-Serratia
-Burkholderia cepacia
-H. pylori
What enzyme is deficient in patients with CGD? - CORRECT ANSWER >>>NADPH oxidase
Chronic Granulomatous Disease (CGD) - CORRECT ANSWER >>>defect in NADPH oxidase →
↑ susceptibility to infections with catalase + organisms
Impaired respiratory burst in neutrophils
1|Page
,Abnormal flow cytometry test (decreased green fluorescence)
Nitroblue tetrazolium dye reaction test fails to turn blue
What two tests can be used to diagnose CGD? - CORRECT ANSWER >>>1. Dihydrorhodaomine
(flow cytometry) test will show reduced fluorescence
2. Nitroblue tetrazolium dye reduction test; will fail to turn blue
What is the most common inheritance pattern for CGD? - CORRECT ANSWER >>>X-linked
recessive
More common in boys
vEDS is caused by pathogenic mutations in the ________________ gene - CORRECT ANSWER
>>>COL3A1 gene
encodes collagen type III alpha chain
________________ collagen is a fibril-forming collagen essential for mechanical strength and
elasticity of expansible tissues. - CORRECT ANSWER >>>Type III
The treatment of choice for Pneumocytis pneumonia is - CORRECT ANSWER
>>>trimethoprim-sulfamethoxazole
Terbinafine inhibits the fungal enzyme _______________________ and is used to treat
dermatophytoses - CORRECT ANSWER >>>squalene epoxidase
2|Page
,Pneumocystis pneumonia - CORRECT ANSWER >>>-caused by Pneumocytis jirovecii
-diffuse interstitial pneumonia
-Yeast-like fungus
-Disc-shaped yeast on methenamine silver stain
-Immunosuppression predisposes to disease
-Diffuse bilateral ground-glass opacities on chest imagine, with pneumatoceles
-Treatment/ Prophylaxis: TMP-SMX, pentamidine, dapsone, atovaquone
Pneumocytis pneumonia prophylaxis with TMP-SMX should be started in patients with HIV
infection with CD4 counts < _____________ - CORRECT ANSWER >>>< 200 cells/mm3
McArdle Disease - CORRECT ANSWER >>>-deficiency in myophosphorylase
-glycogen storage disease type V
-muscle cramps with exercise, red-colored urine due to myoglobinuria
-no increase in lactate
-Elevated CK
3|Page
, Myophosphorylase deficiency results from pathogenic mutations in the ___________ gene -
CORRECT ANSWER >>>PYGM gene —> encodes myophosphorylase
Myophosphorylase - CORRECT ANSWER >>>-Deficient in McArdle Disease (Type V)
-Liberates glucose-1-phosphate units from glycogen
alpha-1,6-glucosidase is one of the two catalytic activities of the debraching enzyme, which
liberates glucose from glycogen branch-points. Deficiency in this enzyme causes
____________________________ - CORRECT ANSWER >>>Cori Disease
-Hypoglycemia
-Increased liver glycogen
-Hepatomegaly
-Normal lactate
-No muscle cramping
PYGM gene mutation - CORRECT ANSWER >>>-Results in McArdle Disease (Type V)
-Myophosphorylase deficiency
Tinea versicolor, also called pityriasis versicolor, is caused by _____________________ infection
and most often occurs on the upper back or chest. - CORRECT ANSWER >>>Malassezia furfur
4|Page
