Iron Disorders

Iron Disorders

Iron Disorders Part 1

What is the cause of African Iron Overload ?

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Iron found in the beer making barrels

Iron Disorders Part 1
Iron Disorders

What is the inheritance pattern of hyper-ferritinemia cataract syndrome?

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AD

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Iron Disorders

Which types of malignancies cause the majority of excessively high ferritin?

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Hematologic malignancies

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Iron Disorders

What chronic inflammatory diseases can cause iron overload?

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Metabolic syndrome and diabetes

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Iron Disorders

What are some examples of acute conditions in which the ferritin is elevated?

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Severe HIV and HLH both can cause hemophagocytosis

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Iron Disorders

How can phlebotomy be used to distinguish primary versus secondary iron overload in somebody with underlying liver disease?

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In somebody with underlying liver disease phlebotomy would not reduce ferritin levels while it would not somebody with primary hemochromatosis

Iron Disorders
Iron Disorders

Can phlebotomy replace other modalities to diagnose iron overload?

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Yes

Iron Disorders
Iron Disorders

How much iron is removed with a single unit of phlebotomy of 500 mL of blood ?

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250mg

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Iron Disorders

When would you consider a liver biopsy besides liver MRI for patients with iron overload?

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When there are elevated liver function enzymes as well as hyperferritinemia and it is unclear which was the precipitating factor

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Iron Disorders

What is the gold standard for determining liver iron overload?

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Liver biopsy

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Iron Disorders

What type of MRI scans can be used to detect iron overload?

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T2 and R2

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Iron Disorders

What TSAT cut-offs correlate well with iron overload in men and women respectively?

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45 and 40%

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Iron Disorders

What would iron overload with macrocytic anemia suggest?

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B12 deficiency

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Iron Disorders

What should the MCV be with hereditary hemochromatosis?

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Normal

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Iron Disorders

Is an MRI of the liver or heart required at initial screening of hemochromatosis?

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No it can be held until transferrin saturation levels are available

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Iron Disorders

Which endogenous enzyme can help mitigate the effects of excess iron?

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Glutathione S transferase

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Iron Disorders

Which molecule causes the tremendous amount of damage when iron overload occurs?

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Hydroxyl radical

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Iron Disorders

Which condition with ineffective erythropoesis leads to significant iron overload? Thalassemia

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Iron Disorders
Iron Disorders

Should children and families with hemochromatosis be screened?

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No can wait until adulthood

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Iron Disorders

What are the 2 concerning gene mutations for hemochromatosis?

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C82Y and H63D

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Iron Disorders

Mutations in which gene results in hemochromatosis?

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HFE

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Iron Disorders

Which 2 organs are the most concerning for iron overload?

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Liver and heart

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Iron Disorders

How much iron is a single unit of packed red blood cells?

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250 mg

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Iron Disorders

What are the 3 main causes of iron overload?

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Hemochromatosis as well as ineffective erythropoiesis from thalassemia and red blood cell transfusions

Iron Disorders
Iron Disorders

How is iron recycled in the body?

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Macrophages in the reticuloendothelial system in the liver spleen and bone marrow

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Iron Disorders

Is heme iron or nonheme iron better absorbed ?

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Heme iron

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Iron Disorders

Where is the majority of iron in the body found?

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Circulating red blood cells

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Iron Disorders

What is the normal iron content of the blood?

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3 to 4 g

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Iron Disorders

What percentage of Caucasians have homozygous C282Y ?

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1 out of 150 people

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Iron Disorders

How common is the HFE gene mutation in the general population of Caucasians?

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6%

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Iron Disorders

Which enzyme mutations have been implicated in the rare variants of hereditary hemochromatosis?

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Ferroportin Hemojuvilin Hepcidin and ceruloplasmin

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Iron Disorders

What should the target ferritin (serum) be when phlebotomy is initiated ?

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200 ug/L

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Iron Disorders

What is the most common hemochromatosis mutation ?

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HFE C282Y

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Iron Disorders

Which ethnic group has the highest penetrance of hereditary hemochromatosis?

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Caucasians

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Iron Disorders

Do men or women with homozygous C282Y mutations tend to have more normal ferritin levels?

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Women, 50% versus men, 20%

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Iron Disorders

What percent of men with hemochromatosis will develop a liver associated complication?

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5%

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Iron Disorders

How many mL blood should be phlebotomized at first diagnosis of hemochromatosis ?

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500mL

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Iron Disorders

Should serum ferritin or transferrin saturation be used as an end-point for phlebotomy ?

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Ferritin

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Iron Disorders

What procedure is available for hemochromatosis patients that cannot tolerate phlebotomy ?

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Erythrocytapheresis

Iron Disorders
Iron Disorders Part 2

How often do patient is using deferiprone require CBC counts?

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Weekly

Iron Disorders Part 2
Iron Disorders Part 2

What is a difference between Exjade and Jadenu ?

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Exjade can be administered as a liquid suspension

Iron Disorders Part 2
Iron Disorders Part 2

Is ophthalmology evaluation mandatory for patients requiring deferoxamine ?

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Yes

Iron Disorders Part 2
Iron Disorders Part 2

Is audiology monitoring mandatory for patients requiring deferoxamine ?

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Yes

Iron Disorders Part 2
Iron Disorders Part 2

What are some chronic complications with deferoxamine?

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Visual and auditory neurotoxicity

Iron Disorders Part 2
Iron Disorders Part 2

What are some acute complications with deferoxamine?

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Abdominal pain diarrhea nausea vomiting

Iron Disorders Part 2
Iron Disorders Part 2

How is deferoxamine administered?

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Subcutaneous infusion over 8 to 12 hours

Iron Disorders Part 2
Iron Disorders Part 2

What is the minimum number of nights per week that patients need to be treated with deferoxamine?

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4

Iron Disorders Part 2
Iron Disorders Part 2

What is the difference between a bidentate and tridentate chelator ?

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the number of molecules needed per atom of iron to chelate completely

Iron Disorders Part 2
Iron Disorders Part 2

How is iron free deferoxamine excreted?

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In the bile

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Iron Disorders Part 2

How is iron bound deferoxamine excreted ?

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In the urine

Iron Disorders Part 2
Iron Disorders Part 2

Which of the available iron chelation agents can be taken orally?

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Deferiprone and Deferasirox

Iron Disorders Part 2
Iron Disorders Part 2

Is there prospective randomized data showing improvement in overall survival with the use of iron chelation therapy after allogeneic stem cell transplant ?

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no

Iron Disorders Part 2
Iron Disorders Part 2

What is the correlation between pretransplant ferritin levels and allogenic stem cell transplant patients in 5-year overall survival?

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Worse

Iron Disorders Part 2
Iron Disorders Part 2

Which iron chelation combination is the least studied?

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Deferiprone and deferasirox

Iron Disorders Part 2
Iron Disorders Part 2

Can hypogonadism in women be reversed with iron chelation therapy?

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Yes

Iron Disorders Part 2
Iron Disorders Part 2

Can hyperglycemia be normalized with iron chelation therapy?

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Yes

Iron Disorders Part 2
Iron Disorders Part 2

Can the iron overload cardiomyopathy be reversed with chelation agents?

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Yes

Iron Disorders Part 2
Iron Disorders Part 2

What are the 3 iron chelating agents approved for use in Thalassemia ?

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Deferasirox Deferoxamine and Deferiprone

Iron Disorders Part 2
Iron Disorders Part 2

Should iron chelation be considered after bone marrow transplant?

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Yes especially if significant transfusions were used

Iron Disorders Part 2
Iron Disorders Part 2

Which inherited anemia syndrome should prompt consideration of iron chelation therapy?

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Thalassemia and sickle cell disease

Iron Disorders Part 2
Iron Disorders Part 2

Management of which hematologic condition should problems consideration of iron chelation therapy?

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Transfusion dependent myelodysplastic syndrome

Iron Disorders Part 2
Iron Disorders Part 2

Which porphyria is associated with hemochromatosis?

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Porphyria cutanea tarda

Iron Disorders Part 2
Iron Disorders Part 2

Which joints are commonly affected in the arthropathy associated with hereditary hemochromatosis?

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Second and third metacarpophalangeal joints

Iron Disorders Part 2
Iron Disorders Part 2

Would a combination of arthropathy and unexplained hyperferritinemia warrant a work-up for hereditary hemochromatosis?

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yes

Iron Disorders Part 2
Iron Disorders Part 2

Which cancers are patients with hereditary hemochromatosis susceptible to?

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HCC and potentially to breast and colon

Iron Disorders Part 2
Iron Disorders Part 2

Which 2 infections are patients with iron overload susceptible to?

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Yersinia enterocolitis and Vibrio vulnificus

Iron Disorders Part 2
Iron Disorders Part 2

What is the mechanism of iron overload induced diabetes?

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Iron deposition selectively in beta cells

Iron Disorders Part 2
Iron Disorders Part 2

What percentage of patients with H63D homozygous disease will develop iron overload?

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0%

Iron Disorders Part 2
Iron Disorders Part 2

At what age do clinical manifestations of hereditary hemochromatosis manifest in men?

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40

Iron Disorders Part 2
Iron Disorders Part 2

What percentage of patients with homozygous C282Y have clinically relevant iron overload?

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10%

Iron Disorders Part 2
Iron Disorders Part 2

What are very rare but potentially fatal complications of deferasirox ?

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Fatal gastrointestinal hemorrhage and acute renal & heptic failure

Iron Disorders Part 2
Iron Disorders Part 2

Should all first-degree relatives of patients with hereditary hemochromatosis tested?

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Yes even if they are asymptomatic

Iron Disorders Part 2
Iron Disorders Part 2

What transferrin saturation cutoff should prompt testing for hemochromatosis in women ?

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55%

Iron Disorders Part 2
Iron Disorders Part 2

Would positivity for hereditary hemochromatosis and a second-degree relative prompt genetic testing?

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Yes

Iron Disorders Part 2
Iron Disorders Part 2

How often have complete blood count to be checked for patients using deferasirox ?

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Monthly

Iron Disorders Part 2
Iron Disorders Part 2

Was the most common reason for using combination chelating agents?

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Single agent is not enough to achieve targets

Iron Disorders Part 2
Iron Disorders Part 2

Was the significant advantage of deferasirox over deferoxamine ?

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Oral vs IV dosing

Iron Disorders Part 2
Iron Disorders Part 2

Is there randomized phase 3 data to support the use of deferasirox over deferoxamine in thalassemia associated iron overload?

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Yes

Iron Disorders Part 2
Iron Disorders Part 2

What are some important targets for ferritin for patients that are starting iron chelation therapy?

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Ferritin less than 1000 mcg/L and MRI T2 signal > 20 msec

Iron Disorders Part 2
Iron Disorders Part 2

Which iron chelation agent is more commonly used in the United States and Europe?

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Deferasirox

Iron Disorders Part 2
Iron Disorders

What should the target ferritin (serum) be when phlebotomy is initiated ?

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200 ug/L

Iron Disorders
Iron Disorders

Which ethnic group has the highest penetrance of hereditary hemochromatosis?

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Caucasians

Iron Disorders
Iron Disorders Part 3

Iron Disorders Part 3

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Iron Disorders Part 3

Iron Disorders Part 3
Iron Disorders

Which ethnic group in the United States has the highest prevalence of iron deficiency?

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Hispanic Americans

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Iron Disorders

Can gastritis lead to clinically significant iron deficiency anemia ?

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Yes

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Iron Disorders

What are the 3 leading causes of occult gastrointestinal bleeding?

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Gastritis/malignancy/angiodysplasia

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Iron Disorders

Which 2 parasites can cause iron deficiency from GI blood loss?

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Hookworm and whipworm

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Iron Disorders

How much iron is usually lost during pregnancy?

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1000 mg

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Iron Disorders

How much iron is lost during hemodialysis over the course of 1 year?

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2000 mg

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Iron Disorders

How common is gastrointestinal blood loss as a cause of iron deficiency anemia in premenopausal women?

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Less than 0.2%

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Iron Disorders

What are the leading causes of diminished iron absorption?

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Celiac disease/bariatric surgery/H. pylori/autoimmune atrophic gastritis

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Iron Disorders

Are there non-meat sources of non-heme iron?

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Yes – cereals and grains

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Iron Disorders

Which food metabolites can diminish iron absorption?

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tannans / phosphates / phytates

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Iron Disorders

Which water-soluble vitamin is necessary for proper iron absorption?

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Vitamin C

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Iron Disorders

Which form of iron salt is nonabsorbable?

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Ferric oxide

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Iron Disorders

Does an acidic or alkaline gastrointestinal environment to promote iron absorption?

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Acidic

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Iron Disorders

What occurs to iron stores after initial treatment with EPO stimulating agents?

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Transient deficiency which is functional given the amplified iron requirements

Iron Disorders
Iron Disorders

What leads to iron deficiency in hemodialysis patients?

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Hemosiderin deposition in the urinary tract leading to iron accumulation

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Iron Disorders

Which life-threatening pulmonary complications can lead to iron deficiency?

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Pulmonary hemosiderosis

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Iron Disorders

Mutations in which 2 genes are known to cause inheritable iron deficiency?

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TMPRSS6 and SLC11A2

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Iron Disorders

Where is the majority of iron found in the body?

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Circulating red blood cells

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Iron Disorders

How much total body iron does a typical adult have ?

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3 to 4 g

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Iron Disorders

What percentage of non-hemoglobin bound iron is bound to iron-containing proteins?

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10%

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Iron Disorders

Where is the majority of iron found ?

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Liver/spleen/bone marrow

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Iron Disorders

What is pagophagia ?

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Ice craving

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Iron Disorders

Ingestion of which vegetable leads to red-colored urine in patients with iron deficiency?

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Beets

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Iron Disorders

What nail changes can be seen with iron deficiency anemia?

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Koilonychia

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Iron Disorders

What is the gold standard for documenting iron deficiency?

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Prussian blue staining of the bone marrow

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Iron Disorders

Which 2 cells need to be assessed for iron stores in the bone marrow to confirm iron deficiency anemia?

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Macrophages and erythroid precursors

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Iron Disorders

Which CBC index can help distinguish microcytic anemia from iron deficiency versus underlying thalassemia?

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Red blood cell index

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Iron Disorders

What is the sensitivity and specificity for a ferritin cutoff of 30 ng/mL for iron deficiency anemia?

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90 to 98% respectively

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Iron Disorders

Which soluble transferrin receptor index can be used screen for iron deficiency?

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Soluble transferrin receptor and (mg/L) divided by serum ferritin log ( mcg/L )

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Iron Disorders

Can elevated reticulocyte hemoglobin content measures rule out iron deficiency?

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Yes

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Iron Disorders

What is the limitation of bone marrow iron staining?

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Dietary iron can interfere with interpretation

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Iron Disorders

How long should one wait after taking oral iron pill the measure serum iron levels?

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24 hours

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Iron Disorders

How long after intravenous iron therapy should iron studies and a CBC be rechecked?

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4 weeks

Iron Disorders
Iron Disorders

What are the effects of tumor necrosis factor alpha inhibiting antibodies on iron studies?

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Reduction in ferritin levels

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Iron Disorders

What is the specificity of a ferritin level less than 16 ng/mL for iron deficiency?

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99%

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Iron Disorders

What is the sensitivity of a ferritin level less than 16 nanograms per mL for iron deficiency?

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59%

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Iron Disorders

What Transferrin saturation cutoff is diagnostic of iron deficiency independent of ferritin?

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18% and below

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Iron Disorders

Can iron deficiency be confirmed without laboratory studies?

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Empiric trial of iron therapy with appropriate correction of anemia

Iron Disorders
Iron Disorders

What is the difference between the iron elixir and oral solution?

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The elixir contains 44 mg of iron while the solution contains 75 mg per 5mL

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Iron Disorders

What is the limitation of enteric-coated iron capsules?

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Delivery of iron at the terminal portion of the small intestine hence poor absorption

Iron Disorders
Iron Disorders

Is 1 tablet/day of ferrous sulfate appropriate ?

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Yes

Iron Disorders
Iron Disorders

Is alternate day dosing of ferrous sulfate an appropriate strategy?

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Yes

Iron Disorders
Iron Disorders

How should oral iron be administered in the context of antacids?

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2 hours before and 4 hours after

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Iron Disorders

Has intravenous iron therapy been proven to reduce blood transfusion requirements?

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Yes

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Iron Disorders

What are the main variables to consider when selecting parenteral iron formulations?

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Cost/infusion time/number of administrations required

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Iron Disorders

What is the cheapest form of intravenous iron in the United States?

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Iron dextran

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Iron Disorders

What dose of intravenous iron therapy is appropriate for the vast majority of patients?

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1000 mg

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Iron Disorders

Are premedications required prior to intravenous iron therapy?

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no

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Iron Disorders

Is low molecular weight iron dextran safe in pregnancy?

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Yes

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Iron Disorders

Is a test dose required for ferric gluconate?

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Only if patients have a history of infusion reactions

Iron Disorders
Iron Disorders

Is a test dose required for iron sucrose?

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Yes – if patients have a history of drug allergies

Iron Disorders
Iron Disorders

What is one of the major limitations of ferrous gluconate for iron replacement?

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The need for numerous infusions

Iron Disorders
Iron Disorders

What is one of the major limitations of iron sucrose for iron replacement?

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The need for numerous infusions

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Iron Disorders

Can ferumoxytol be administered as a single infusion?

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Yes

Iron Disorders
Iron Disorders

What is the molecular structure of ferumoxytol?

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A super paramagnetic iron oxide nanoparticle coated with a low molecular weight semisynthetic carbohydrate

Iron Disorders
Iron Disorders

How long does it take to administer the total dose of ferumoxytol as a single infusion?

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30 minutes

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Iron Disorders

Is ferumoxytol safe in pregnancy?

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Yes

Iron Disorders
Iron Disorders

What is the main limitation of ferumoxytol in the context of imaging?

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Brighter signals can be seen on MRI for up to 3 months following administration

Iron Disorders
Iron Disorders

What is the structure of ferric carboxymaltose?

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Ferric carboxymaltose is a colloidal iron hydroxide complex with much tighter binding of elemental iron to the carbohydrate Polymer compared to other intravenous preparations

Iron Disorders
Iron Disorders

Can ferric carboxymaltose be administered as a single dose?

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Yes up to 1 g

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Iron Disorders

Which rare electrolyte disturbance has been reported with ferric carboxymaltose?

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Hypophosphatemia

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Iron Disorders

What is the mechanism of iron derisomaltose?

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A matrix with very tight iron binding resulting slow release of free iron

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Iron Disorders

What are examples of nonallergic infusion reactions that can be seen with intravenous iron therapy?

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Urticaria/dizziness/muscle spasms/palpitations

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Iron Disorders

What is the Fishbane reaction in the context of IV iron therapy?

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Facial flushing and myalgias of the chest and back

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Iron Disorders

Which rheumatologic condition can experience a flare during intravenous iron therapy?

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Rheumatoid arthritis

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Iron Disorders

How long can hemoglobin correction take after intravenous iron therapy?

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Up to 8 weeks

Iron Disorders
Iron Disorders

How long after intravenous iron therapy can the symptoms of restless leg syndrome subside?

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Generally within 3 days

Iron Disorders
Iron Disorders Part 3

Iron Disorders Part 3

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Iron Disorders Part 3

Iron Disorders Part 3
Iron Disorders Part 3

Iron Disorders Part 3

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Iron Disorders Part 3

Iron Disorders Part 3
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