[MCQ] Hemochromatosis- Part 2

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Hemochromatosis- Part 2

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Question 1

Which of the following about hemochromatosis is false ?

A	Autosomal recessive
B	Symptomatic organ involvement begins in midlife
C	Hematologic anomalies are not usually seen
D	None of the above

Question 1 Explanation:

Although iron metabolism is abnormal, erythropoiesis is not jeopardized, and hematologic anomalies are not usually seen.

Question 2

HFE gene is located on which of the following chromosome ?

A	4
B	5
C	6
D	7

Question 2 Explanation:

HFE stands for High Iron Fe. HFE gene is located on short arm of chromosome 6 at location 6p21.3 and encodes human hemochromatosis protein also known as the HFE protein.

Question 3

Which of the following is the most common mutation in hemochromatosis ?

A	H63D
B	H95D
C	C282Y
D	C126Y

Question 3 Explanation:

A homozygous G to A mutation resulting in a cysteine to tyrosine substitution at position 282 (C282Y) is the most common mutation in HFE gene responsible for hemochromatosis. HFE mutation H63D results in a substitution of histidine to aspartic acid at codon 63.

Question 4

In hemochromatosis, progressive accumulation of iron increases which of the following ?

A	Plasma iron
B	Saturation of transferrin
C	Plasma ferritin
D	All of the above

Question 4 Explanation:

In hemochromatosis, mucosal absorption of iron is greater than body requirements and amounts to 4 mg/day or more. In hemochromatosis, progressive accumulation of iron increases plasma iron, saturation of transferrin, and results in a progressive increase of plasma ferritin.

Question 5

Crucial molecule in iron metabolism that links body stores with intestinal iron absorption is ?

A	Ferroportin (FPN)
B	Iron oxidase hephaestin (Heph)
C	Hepcidin
D	Duodenal cytochrome B (DcytB)

Question 5 Explanation:

Hepcidin is the crucial molecule in iron metabolism, linking body stores with intestinal iron absorption. Alcohol suppresses hepatic hepcidin secretion.

Question 6

Hepcidin responds to changes in body-iron requirements through which of the following ?

A	HFE & TfR2
B	Hemojuvelin (HJV)
C	Bone Morphogenetic Protein (BMP)/SMAD pathway
D	All of the above

Question 6 Explanation:

Hepcidin responds to changes in body-iron requirements by signals mediated by diferric transferrin through two mechanisms. One involves HFE & TfR2, while the other involves hemojuvelin (HJV) & Bone Morphogenetic Protein (BMP)/SMAD pathway.

Question 7

Dietary inorganic iron is reduced from ferric (Fe3+) iron to ferrous (Fe2+) state by ?

A	Ferroportin (FPN)
B	Iron oxidase hephaestin (Heph)
C	Duodenal cytochrome B (DcytB)
D	DMT1

Question 7 Explanation:

Dietary inorganic iron traverses the brush border membrane of duodenal enterocytes via DMT1 after reduction of ferric (Fe3+) iron to the ferrous (Fe2+) state by duodenal cytochrome B (DcytB).

Question 8

Hepcidin binds to which of the following ?

A	Ferroportin
B	Transferrin
C	Hemosidrin
D	All of the above

Question 8 Explanation:

Iron moves from enterocyte to the circulation via a process requiring basolateral iron exporter ferroportin (FPN) and iron oxidase hephaestin (Heph). In the circulation, iron binds to plasma transferring. Hepcidin is a liver-derived peptide that represses basolateral iron transport in intestine & iron release from macrophages and other cells by binding to ferroportin. Hepcidin, in turn, responds to signals in liver mediated by HFE, TfR2, and hemojuvelin. Mutations in genes encoding HFE, TfR2, hemojuvelin and hepcidin lead to decreased hepcidin release and increased iron absorption, resulting in hemochromatosis.

Question 9

Which of the following is false about HFE-related hemochromatosis ?

A	HFE protein forms a complex with 2-microglobulin & transferrin receptor 1 (TfR1)
B	Reduced TfR1-mediated iron uptake by intestinal crypt cell
C	Upregulation of divalent metal transporter (DMT1) increases intestinal iron absorption
D	None of the above

Question 10

Which is usually the first organ to be affected in hemochromatosis ?

A	Liver
B	Heart
C	Pancreas
D	Skin

Question 10 Explanation:

Liver is usually the first organ to be affected in hemochromatosis, and hepatomegaly is present in >95% of symptomatic patients. The association of hepatomegaly, skin pigmentation, diabetes mellitus, heart disease, arthritis, and hypogonadism should suggest hemochromatosis

Question 11

Bronzing or characteristic metallic or slate-gray skin pigmentation in hemochromatosis is due to ?

A	Increased iron in the epidermis
B	Decreased iron in the epidermis
C	Increased iron in the dermis
D	Decreased iron in the dermis

Question 11 Explanation:

In hemochromatosis, characteristic metallic or slate-gray skin pigmentation referred to as bronzing results from increased melanin and iron in the dermis. Pigmentation is diffuse but more pronounced on face, neck, extensor aspects of lower forearms, dorsa of the hands, lower legs, and genital regions, as well as in scars.

Question 12

Which of the following is the least common in hemochromatosis ?

A	Arthropathy
B	Hepatomegaly
C	Cardiac involvement
D	Diabetes mellitus

Question 13

Which of the following is usually the first joint involved in hemochromatosis ?

A	Wrist
B	First metatarsophalangeal joint
C	Second & third metacarpophalangeal joints
D	Sternoclavicular joints

Question 13 Explanation:

Joints of hands, especially second & third metacarpophalangeal joints, are usually the first joints involved in hemochromatosis. This feature helps to distinguish chondrocalcinosis associated with hemochromatosis from the idiopathic form.

Question 14

Which of the following is most common in hemochromatosis ?

A	Hypogonadism
B	Adrenal insufficiency
C	Hypothyroidism
D	Hypoparathyroidism

Question 14 Explanation:

In hemochromatosis, hypogonadism due to impairment of hypothalamic-pituitary function by iron deposition occurs in both sexes and may antedate other clinical features. Adrenal insufficiency, hypothyroidism, and hypoparathyroidism are rare manifestations.

Question 15

Ingestion of which of the following promotes iron absorption ?

A	Ascorbic acid
B	Flavinoids
C	Carotene
D	Calcium

Question 15 Explanation:

Ingestion of large doses of ascorbic acid promotes iron absorption

Question 16

1 mL blood contains approximately how much iron ?

A	0.2 mg
B	0.3 mg
C	0.4 mg
D	0.5 mg

Question 16 Explanation:

1 mL blood contains approximately 0.5 mg iron.

Question 17

An increase of 1 mg/L in serum ferritin level reflects an increase of about how much iron in body stores ?

A	2 mg
B	3 mg
C	4 mg
D	5 mg

Question 17 Explanation:

An increase of 1 mg/L in serum ferritin level reflects an increase of about 5 mg iron in body stores

Question 18

Which of the following is the strongest predictor of hemochromatosis expression in individuals homozygous for C282Y mutation ?

A	Plasma iron
B	Total iron-binding capacity
C	Serum ferritin
D	C-reactive protein

Question 18 Explanation:

Serum ferritin level >1000 mg/L is the strongest predictor of disease expression among individuals homozygous for the C282Y mutation

Question 19

Which of the following indicates risk of severe fibrosis in a C282Y homozygous subject ?

A	Serum ferritin level <1000 mg/L
B	Normal serum alanine amino transaminase values
C	No hepatomegaly
D	None of the above

Question 19 Explanation:

There is virtually no risk of severe fibrosis in a C282Y homozygous hemochromatosis subject with serum ferritin level <1000 mg/L, normal serum alanine amino transaminase values, no hepatomegaly, and no excess alcohol intake

Question 20

Normal hepatic iron index is ?

A	< 0.2
B	< 0.5
C	< 0.75
D	< 1.0

Question 20 Explanation:

Normal hepatic iron index is < 1. In symptomatic hemochromatosis it is > 2. In homozygotes with early, asymptomatic hemochromatosis it si between 1 and 2.

Question 21

One 500 mL unit of blood contains how much iron ?

A	20 - 25 mg
B	50 - 100 mg
C	100 - 200 mg
D	200 - 250 mg

Question 21 Explanation:

One 500 mL unit of blood contains 200 - 250 mg iron, and up to 25 g iron or more may have to be removed.

Question 22

HFE mutations are related to which of the following diseases ?

A	Porphyria cutanea tarda (PCT)
B	Nonalcoholic steatosis (NASH)
C	Chronic HCV infection
D	All of the above

Question 22 Explanation:

Also, subjects homozygous for C282Y are at increased risk of breast & colorectal cancer

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See all quizzes of Paget’s Disease and Other Dysplasias of Bone at here:

Hemochromatosis- Part 1| Hemochromatosis- Part 2

1 Which of the following about hemochromatosis is false ?
A. Autosomal recessive
B. Symptomatic organ involvement begins in midlife
C. Hematologic anomalies are not usually seen
D. None of the above

2 HFE gene is located on which of the following chromosome ?
A. 4
B. 5
C. 6
D. 7

3 Which of the following is the most common mutation in
hemochromatosis ?
A. H63D
B. H95D
C. C282Y
D. C126Y

4 In hemochromatosis, progressive accumulation of iron increases
which of the following ?
A. Plasma iron
B. Saturation of transferrin
C. Plasma ferritin
D. All of the above

5 Crucial molecule in iron metabolism that links body stores with
intestinal iron absorption is ?
A. Ferroportin (FPN)
B. Iron oxidase hephaestin (Heph)
C. Hepcidin
D. Duodenal cytochrome B (DcytB)

6 Hepcidin responds to changes in body-iron requirements through
which of the following ?
A. HFE & TfR2
B. Hemojuvelin (HJV)
C. Bone Morphogenetic Protein (BMP)/SMAD pathway
D. All of the above

7 Dietary inorganic iron is reduced from ferric (Fe3+) iron to ferrous
(Fe2+) state by ?
A. Ferroportin (FPN)
B. Iron oxidase hephaestin (Heph)
628 MCQ’s FOR MEDICAL PROFESSIONALS BY PROF. AJAY MATHUR Endocrinology
C. Duodenal cytochrome B (DcytB)
D. DMT1

8 Hepcidin binds to which of the following ?
A. Ferroportin
B. Transferrin
C. Hemosidrin
D. All of the above

9 Which of the following is false about HFE-related hemochromatosis
?
A. HFE protein forms a complex with 2-microglobulin &
transferrin receptor 1 (TfR1)
B. Reduced TfR1-mediated iron uptake by intestinal crypt cell
C. Upregulation of divalent metal transporter (DMT1)
increases intestinal iron absorption
D. None of the above
10 Which is usually the first organ to be affected in
hemochromatosis ?
A. Liver
B. Heart
C. Pancreas
D. Skin

11 Bronzing or characteristic metallic or slate-gray skin pigmentation
in hemochromatosis is due to ?
A. Increased iron in the epidermis
B. Decreased iron in the epidermis
C. Increased iron in the dermis
D. Decreased iron in the dermis

12 Which of the following is the least common in
hemochromatosis ?
A. Arthropathy
B. Hepatomegaly
C. Cardiac involvement
D. Diabetes mellitus

13 Which of the following is usually the first joint involved in
hemochromatosis ?
A. Wrist
B. First metatarsophalangeal joint
C. Second & third metacarpophalangeal joints
D. Sternoclavicular joints

14 Which of the following is most common in hemochromatosis ?
A. Hypogonadism
B. Adrenal insufficiency
C. Hypothyroidism
D. Hypoparathyroidism

15 Ingestion of which of the following promotes iron absorption ?
A. Ascorbic acid
B. Flavinoids
C. Carotene
D. Calcium

16 1 mL blood contains approximately how much iron ?
A. 0.2 mg
B. 0.3 mg
C. 0.4 mg
D. 0.5 mg

17 An increase of 1 mg/L in serum ferritin level reflects an increase
of about how much iron in body stores ?
A. 2 mg
B. 3 mg
C. 4 mg
D. 5 mg

18 Which of the following is the strongest predictor of
hemochromatosis expression in individuals homozygous for
C282Y mutation ?
A. Plasma iron
B. Total iron-binding capacity
C. Serum ferritin
D. C-reactive protein

19 Which of the following indicates risk of severe fibrosis in a C282Y
homozygous subject ?
A. Serum ferritin level <1000 mg/L
B. Normal serum alanine amino transaminase values
C. No hepatomegaly
D. None of the above

20 Normal hepatic iron index is ?
A. < 0.2
B. < 0.5
C. < 0.75
D. < 1.0

21 One 500 mL unit of blood contains how much iron ?
A. 20 – 25 mg
B. 50 – 100 mg
C. 100 – 200 mg
D. 200 – 250 mg

22 HFE mutations are related to which of the following diseases ?
A. Porphyria cutanea tarda (PCT)
B. Nonalcoholic steatosis (NASH)
C. Chronic HCV infection
D. All of the above

Hemochromatosis

[MCQ] Hemochromatosis- Part 2

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Hemochromatosis- Part 2

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[MCQ] Porphyrias-Part 1

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