USMLE Step 1

USMLE Step 1 QUESTION of the DAY!

A 3 year-old has always been tired but now has been having convulsions and noticeably painful joints. After a CBC and other lab tests, a CT scan demonstrates hepatomegaly and renomegaly. It is determined that the CT results were due to accumulations of glycogen. This patients’ condition is due to a defect of what enzyme?

A) alpha-glucosidase
B) glycogen phosphorylase
C) aldolase B
D) iduronate sulfatase
E) glucose-6-phosphatase

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Answer: E) glucose-6-phosphatase. This patient has the glycogen storage disease- Type I Von Gierke. This disease if untreated will lead to failure to thrive, stunted growth, hepatomegaly, and renomegaly. A fasting hypoglycemia that leads to convulsions is also seen. Some patients even develop gout and skin xanthomas. A defect in glucose-6-phosphatase leads to accumulations of glycogen in the liver and kidneys.


USMLE Step 1 D.O.C. of the DAY!

Drug of choice for

Helicobacter pylori causing gastric ulcers

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Metronidazole + tetracycline


USMLE Step 1 Fact of the Day!

Sjogren Syndrome
-autoimmune disease- anti-Ro (ss-a) and anti-La (ss-b) anti-nuclear antibodies
-triad of xerostomia (dry mouth), keratoconjunctivitis sicca (dry eyes with corneal abrasion), and rheumatoid arthritis.
-may cause bilateral enlargement of the parotids
-occurs in middle aged women
-75% have rheumatoid factor, 70% have anti-nuclear antibodies above