Exam Questions - Goueli

Question 1

If a patient is a post-menapausal female (or a male of any age), with Iron-Deficiency Anemia, what test will you order?

Answer 1

Colonoscopy for a GI Bleed

Question 2

What is the mechanism of Anemia of Chronic Disease?

Answer 2

Increased inflammatory cytokines, such as IL-6 induces Hepcidin overload in which the iron stores are increased, but not bio-available.

Question 3

What is the primary therapy for Hereditary Hemochromatosis?

Answer 3

Phlebotomy treatment started before cirrhosis or DM; maintain ferritin between 20-50 ng/mL

Question 4

Iron-Deficiency Anemia Iron Indicides

Answer 4

Low serum iron, serum ferritin, transferrin iron saturation percentage, hemoglobin
Ferritin less than 10
High TIBC and Transferrin

Question 5

Anemia of Chronic Disease Iron Indicies

Answer 5

Low serum iron, transferrin iron saturation percentage, TIBC, transferrin, hemoglobin
High Serum Ferritin

Question 6

What mutation causes Hereditary Hemochromatosis?

Answer 6

Type 1 HH is classically caused by HFE gene mutation at C282Y that decreases hepcidin expression
Type 4 is the only one that’s autosomal dominant inheritance

Question 7

Why do vegetarians develop iron diseases?

Answer 7

Heme Iron (Fe2+/Ferrous) is best absorbed and found in beef, fish, and chicken. Non-heme iron (Fe3+/Ferric) is insoluble and not readily absorbed from vegetables and cereals.

Question 8

Your patient has TB and is being treated with isoniazid. Recently, the patient has been experiencing peripheral neuropathy and other CNS effects. Why?

Answer 8

Vitamin B6 (pyridoxine) Deficiency

Question 9

What type of anemia results when hemoglobin cannot be produced?

Answer 9

Microcytic anemia due to lead poisoning

Question 10

What two enzymes in the heme metabolism pathway can be affected by lead toxicity?

Answer 10

Ferrocheletase and delta-ALA dehydratase

Question 11

When are direct, conjugated bilirubin levels elevated?

Answer 11

Biliary obstruction, gallstone, bile not getting out of the liver

Question 12

When are the indirect, unconjugated bilirubin levels elevated?

Answer 12

Hemolysis in the vasculature

Question 13

Acute Intermittent Porphyria Symptoms

Answer 13

GI pain, vomiting, constipation, hyponatremia (low sodium), neuropathy, CV

Question 14

Hereditary Coproporphyria

Answer 14

Acute Intermittent Porphyria symptoms with Photosensitivity

Question 15

Porphyria Cutanea Tarda

Answer 15

Deficiency of hepatic uroporphyrinogen decarboxylase (URO-D) causing increased iron stores; 50% of patients with sporadic form are Hepatitis C positive

Question 16

What is the treatment for Porphyria Cutanea Tarda?

Answer 16

Phlebotomy 500 mL (1 unit = 250 mg Fe) per week until remission

Question 17

What are some promoters of Iron Absorption?

Answer 17

Ascorbic Acid
Citric Acid
Spices
B-carotene
Alcohol

Question 18

What are some inhibitors of Iron Absorption?

Answer 18

Phytic Acid
Polyphenols
Tannins
Calcium

Question 19

In Low Iron environments, what happens to Ferritin and Transferrin?

Answer 19

Decrease Ferritin production, but Increase Transferrin production.

Question 20

In High Iron environments, what happens to Ferritin and Transferrin?

Answer 20

Increase Ferritin production, but decrease Transferrin production.

Question 21

Causes of Iron-Deficiency Anemia

Answer 21

Celiac Disease
Autoimmune Gastritis
H. Pylori
Crohn's Disease
Gastric Surgery

Question 22

How can one acquire sideroblastic anemia?

Answer 22

Multiple Blood Transfusions (20+ units of blood)

Question 23

Hereditary Hemochromotosis is what type of disorder?

Answer 23

Autosomal inherited iron overload disorder affecting the hepcidin/ferroportin access

Question 24

What is the classic triad of Hereditary Hemochromatosis?

Answer 24

Diabetes Mellitus, Hepatomegaly, Hyperpigmentation

Question 25

Hemochromatosis Iron Indicies

Answer 25

High serum iron, serum ferritin, transferrin iron saturation percentage
Low TIBC and Transferrin
Normal Hemoglobin