Exam Questions - Goueli Flashcards
If a patient is a post-menapausal female (or a male of any age), with Iron-Deficiency Anemia, what test will you order?
Colonoscopy for a GI Bleed
What is the mechanism of Anemia of Chronic Disease?
Increased inflammatory cytokines, such as IL-6 induces Hepcidin overload in which the iron stores are increased, but not bio-available.
What is the primary therapy for Hereditary Hemochromatosis?
Phlebotomy treatment started before cirrhosis or DM; maintain ferritin between 20-50 ng/mL
Iron-Deficiency Anemia Iron Indicides
Low serum iron, serum ferritin, transferrin iron saturation percentage, hemoglobin
Ferritin less than 10
High TIBC and Transferrin
Anemia of Chronic Disease Iron Indicies
Low serum iron, transferrin iron saturation percentage, TIBC, transferrin, hemoglobin
High Serum Ferritin
What mutation causes Hereditary Hemochromatosis?
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
Why do vegetarians develop iron diseases?
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.
Your patient has TB and is being treated with isoniazid. Recently, the patient has been experiencing peripheral neuropathy and other CNS effects. Why?
Vitamin B6 (pyridoxine) Deficiency
What type of anemia results when hemoglobin cannot be produced?
Microcytic anemia due to lead poisoning
What two enzymes in the heme metabolism pathway can be affected by lead toxicity?
Ferrocheletase and delta-ALA dehydratase
When are direct, conjugated bilirubin levels elevated?
Biliary obstruction, gallstone, bile not getting out of the liver
When are the indirect, unconjugated bilirubin levels elevated?
Hemolysis in the vasculature
Acute Intermittent Porphyria Symptoms
GI pain, vomiting, constipation, hyponatremia (low sodium), neuropathy, CV
Hereditary Coproporphyria
Acute Intermittent Porphyria symptoms with Photosensitivity
Porphyria Cutanea Tarda
Deficiency of hepatic uroporphyrinogen decarboxylase (URO-D) causing increased iron stores; 50% of patients with sporadic form are Hepatitis C positive
What is the treatment for Porphyria Cutanea Tarda?
Phlebotomy 500 mL (1 unit = 250 mg Fe) per week until remission
What are some promoters of Iron Absorption?
Ascorbic Acid Citric Acid Spices B-carotene Alcohol
What are some inhibitors of Iron Absorption?
Phytic Acid
Polyphenols
Tannins
Calcium
In Low Iron environments, what happens to Ferritin and Transferrin?
Decrease Ferritin production, but Increase Transferrin production.
In High Iron environments, what happens to Ferritin and Transferrin?
Increase Ferritin production, but decrease Transferrin production.
Causes of Iron-Deficiency Anemia
Celiac Disease Autoimmune Gastritis H. Pylori Crohn's Disease Gastric Surgery
How can one acquire sideroblastic anemia?
Multiple Blood Transfusions (20+ units of blood)
Hereditary Hemochromotosis is what type of disorder?
Autosomal inherited iron overload disorder affecting the hepcidin/ferroportin access
What is the classic triad of Hereditary Hemochromatosis?
Diabetes Mellitus, Hepatomegaly, Hyperpigmentation
