Hemoglobin and Iron Metabolism — Flashcards

Flashcard 51: Which enzyme is defective in X-linked porphyria?_____

Answer: ALA Synthase-2

Flashcard 52: What is the byproduct of conversion of heme to biliverdin?_____

Answer: CO

Flashcard 53: Most of the functional iron in the body is found in _____

Answer: Hemoglobin

Flashcard 54: HbF is resistant to _____ elution, which is the principle of the Kleihauer-Betke test

Answer: acid

Extra: The **Kleihauer-Betke (KB) test** relies on the resistance of HbF to **acid elution**. It is used to quantify fetal-maternal hemorrhage (FMH). In contrast, HbF is also resistant to **alkali denaturation**, which is the basis of the **Apt test** and **Singer test**. Note: HbA (adult hemoglobin) is susceptible to both acid elution and alkali denaturation.

Flashcard 55: Which test is used to determine urinary and fecal urobilinogen?_____

Answer: Ehrlich test

Flashcard 56: At birth, HbF constitutes _____% of total Hb at birth

Answer: 70

Flashcard 57: Which 3 hemoglobins are also known as embryonic hemoglobins?_____

Answer: Gower-I, Gower-II, and Hb Portland

Flashcard 58: The greenish-black color of the first stool in the newborn is due to _____.

Answer: biliverdin

Flashcard 59: Chromosome 11, apart from the β-globin chain, also synthesizes the _____ chains of embryonic Hb.

Answer: ε (epsilon) and γ (gamma)

Flashcard 60: 1g of hemoglobin produces _____ mg of bilirubin.

Answer: 35