Question

Daryl is a 32?year?old man who presents with a 6?month history of shortness of breath even during activities that require only minor exertion. He has noticed increasing limitation in his exercise capacity and denies wheezing, cough, or phlegm production. He has smoked for 15 years and continues to smoke. Daryl's father has been told he has early compensated cirrhosis but has never drank alcohol; his mother is in good health. Daryl’s serum protein electrophoretic results are shown below:     Adult reference Values        Patient Results Albumin 3.5?5.0   3.6 ?1 ? Globulins 0.1?0.4   0.0 ?2 ? Globulins 0.6?1.0   0.7 ? ? Globulins 0.7?1.1   0.8 ? – Globulins 0.8?1.6   1.0 Total Protein 6.0?8.0   6.1 Patient’s Electrophoretic Pattern                                    18US MLSC 4051: Clinical Chemistry 4 1. What protein fraction(s) is/are abnormal in the patient’s serum? (0.5 pt) 2. An abnormality in this/these fraction(s) is/are most often associated with changes in what protein? (1 pt) 3.   What is the function of this protein? (1 pt) 4.   Could this protein abnormality be related to both the patient’s symptoms and his father’s cirrhosis? Explain your answer. (1 pt) 5. What other test(s) may be done to confirm this abnormality? (1 pt) 6.   What charge is on the plasma proteins when they are dissolved in the typical pH 8.6 electrophoretic buffer? Explain (1 pt)    7.   Are there conditions that will cause an increase in this protein in the serum? If so, state at least one such condition and list at least three other specific proteins that would be abnormal in addition to this protein. (2.5 pts) 8.   Calculate the Total Globulin levels and the A/G ratio for this patient (1 pt)

Answer 1

1. Alpha1-Globulin's normal range for adults is 0.1-0.4. Darly is 32 year old and hence his serum concentration of Alpha1-Globulin should fall within this range. But his serum concentration for Alpha1-Globulin is 0.0 which is abnormal.

2. Alpha1-Globulin are serum globular proteins. These serum proteins inhibit activities of certain proteases. For example, ?₁-antitrypsin is an alpha1-Globulin protein and its activity is to inhibit the activity of trypsin which is a protease. As there is abnormality in the serum concentration of Alpha1-Globulin, there is increased activity of these proteases.

3. Function of these proteins is to control the activity of proteases. As mentioned above, proteases like trypsin, chymotrypsin are involved in braking down of protein and their activities need to be controlled otherwise they cleave all proteins which they come across. There comes the role of Alpha1-Globulins.

4. Compensated cirrhosis is a condition where liver is is heavily scarred but symptoms associated with cirrhosis are unnoticed. Usually lack of gamma-globulin is one cause for cirrhosis and its serum concentration is usually assessed to diagnose cirrhosis. Alcohol consumption is also main reason for scarring of liver tissue. Globulin/albumin ratio present in serum is presented to understand the disease status of patient.

As patient is lack of alpha1-globulin in patient serum, ratio of globulin/albumin ratio varies and hence gives clue of cirrhosis.