Important SEQs of Heme Metabolism with Answer keys for 1st year MBBS Students
Note : For answer key of below given short essay questions check the download link at the end of the post .
Q1 a) Enumerate the Heme containing proteins other than hemoglobin
b) Write down the biosynthesis steps of heme synthesis
Q2 a) Differentiate between the biochemical role of ALA synthase 1 & 2
b) Write down the regulation of heme synthesis
Q3 a ClassifyPorphyrias
b) Write down the enzyme deficient with associated clinical features of
Acute intermittent porphyria
Q4 a) Illustrate the process of degradation of Heme
b) Discuss the biochemical basis of jaundice in new born
Q5 a) what is hyperbilirubinemia? Differentiate between uncojugated & conjugated
b) Explain briefly about the concept of direct & indirect bilirubin
Q6 a) Define and classify the Jaundice, Differentiate b/w Hepatic & Post hepatic jaundice
b) Why excess bilirubin is deposited in eyes of jaundice patients
Q7 a) Enumerate normal Hemoglobin types & write down the significance of HbAIC
b).What is the biochemical cause of HbS & how would you diagnose the case?
Q8 a) Enlist causes of non hemolytic uncojugated hyperbilirubinemia
b) Differentiate between Criglar Najjar syndrome type 1 & type 2
Q9 a) Describe Briefly the importance of glycosylated Hb (HbAIC)
b) Why Brain is not damaged in adults due to unconjugated hyperbilirubinemia
Q10 A 6 month old infant presents to the paediatrician with complaints of painful hands and feet. On examination the chid is jaundiced and has an enlarged spleen. Complete Blood Count (CBC) shows a Hb of 5.2. Based on this clinical scenario, answer the following questions:
a) What is the most likely diagnosis?
b) How will the RBCs appear on peripheral blood smear exam?
c) What is the test of choice to confirm diagnosis?
d) What is the genetic basis of this disease?
e) How will you treat the child?
Q 11. a. Give an illustrative diagrammatic view of complete degradation pathway
of heme in to bilirubin.
Total serum bilirubin level of new born baby is highly increased up to 20mg that diffuses to the skin, eyes and other tissues; baby was immediately taken to the incubator for phototherapy
What are the possible biochemical causes of jaundice in new born baby
ii Elaborate the mechanism of Phototherapy to decrease highly increased bilirubin in tissues
Q 12 What are the hall marks of obstructive jaundice
Q 13. Classify porphyrias ?give an overview of enzyme deficiency, Major sign
& symptoms & laboratory findings of all types of Porphyria
Q14. Out line the committed step of heme synthesis along with coenzyme & inhibitor of committed step
Q15. Define Porphyrias with their mode of genetic inheritance & their treatment
Q16 Define with causes & types of Jaundice
Q17. Name the drugs which increases the activity of ALA Synthase 2 in the liver
Q18. What is enzyme inducer give example with function?