Question

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Questions

1. The clinical scenario is most consistent with which type of anemia? You may simply list your answer below using a bullet point format. This does not have to be in a complete sentence.
2. What data in the clinical scenario supports your diagnosis? You may simply list your answers below using a bullet point format. This does not have to be in a complete sentence.
3. What risk factor(s) caused the exacerbation of the anemia you identified in question 1? You may simply list your answer below using a bullet point format. This does not have to be in a complete sentence.
4. Describe the key pathophysiologic concepts. To answer this question completely, you must answer all of the sub-questions below using complete sentences. Each sub-question may be answered in 1-6 sentences.
   1. Describe the genetic alteration which results in sickle cell anemia.
   2. What is the pathological process which is responsible for causing this patient’s joint pain?
   3. Why did this patient have a stroke 16 years ago?
   4. Why did this patient have a splenic infarct?
   5. Why does this patient have icteric sclera and an elevated bilirubin?
   6. What is Acute Chest Syndrome? Does she or does she not have this?  

5. For what actual or potential complications related to the diagnosis in question 1 does she need to be monitored? You may simply list your answer(s) below using a bullet point format. This does not have to be in a complete sentence.

Answer 1

Genetic alteration and sickle cell Anemia:

- sickle cell disease is a group of haemoglobin disorder, caused by an inherited genetic alterations ( mutations)in the beta- globin gene..

- it is an autosomal recessive condition..it means afftected person has two altered beta-globin gene..

- Both parents have one altered copy of the gene that is called carriers.they will have mild Anemia..

- so child they have the chance 25% inheriting both gene alteration, they may get complications later.

- many different alteration in the beta- globin cause other sickle cell disease like beta- thalassemia.

Joint pain in sickle cell Anemia:

- when patient affected with sickle cell Anemia their red blood cells will become rigid and sticky and their shape will be sickle or half moon shape..

- due to this irregular shaped cell it get struct in small blood vessels and it can slow or block the blood flow and o2 for other parts..

- when their is obstructed blood flow to small blood vessels to joints pain will be there in joints..

- most of the people will have pain it will last for few weeks..

- due to severe joint pain it can damage their bone..

- there will joint swelling and severe pain..

Stroke:

- stroke is a complication of sickle cell Anemia disease among general population..

- because pathophysiology of SCD it is not only affect the RBCs but also the vascular endothelium, coagulopathy and inflammation..

- hemoglobin is mutated and gene encoding the B- globulin..

- they have chronic inflammation with more interleukin, chemokines and cytokine..

- Blockage occurs in large arteries in heart..Hypoxia and acidosis and more sickling occurs .

- this cause acute stroke and chronic cerebral Ischemia and other sickness .

Splenic infarcts in sickle cell Anemia:

- in sickle cell Anemia there will be abdominal painful crisis..it results multi-system organ failure..

- SCA affect any part of the body, spleen is one of the common organ affected with SCA..

- first spleen will be enlarged due to inadequate blood and O2 and atlast atrophy occurs due to repeated attack of vasooclusion and infarction..

- splenic complications lead to mortality.

- acute splenic sequestration crisis, hypersplenism, massive infarction and abscess are the complications..

- it made ultimately autosplenectomy..it is mostly affect the sickle cell trait..

Answer 2

Genetic alteration and sickle cell Anemia:

- sickle cell disease is a group of haemoglobin disorder, caused by an inherited genetic alterations ( mutations)in the beta- globin gene..

- it is an autosomal recessive condition..it means afftected person has two altered beta-globin gene..

- Both parents have one altered copy of the gene that is called carriers.they will have mild Anemia..

- so child they have the chance 25% inheriting both gene alteration, they may get complications later.

- many different alteration in the beta- globin cause other sickle cell disease like beta- thalassemia.

Joint pain in sickle cell Anemia:

- when patient affected with sickle cell Anemia their red blood cells will become rigid and sticky and their shape will be sickle or half moon shape..

- due to this irregular shaped cell it get struct in small blood vessels and it can slow or block the blood flow and o2 for other parts..

- when their is obstructed blood flow to small blood vessels to joints pain will be there in joints..

- most of the people will have pain it will last for few weeks..

- due to severe joint pain it can damage their bone..

- there will joint swelling and severe pain..

Stroke:

- stroke is a complication of sickle cell Anemia disease among general population..

- because pathophysiology of SCD it is not only affect the RBCs but also the vascular endothelium, coagulopathy and inflammation..

- hemoglobin is mutated and gene encoding the B- globulin..

- they have chronic inflammation with more interleukin, chemokines and cytokine..

- Blockage occurs in large arteries in heart..Hypoxia and acidosis and more sickling occurs .

- this cause acute stroke and chronic cerebral Ischemia and other sickness .

Splenic infarcts in sickle cell Anemia:

- in sickle cell Anemia there will be abdominal painful crisis..it results multi-system organ failure..

- SCA affect any part of the body, spleen is one of the common organ affected with SCA..

- first spleen will be enlarged due to inadequate blood and O2 and atlast atrophy occurs due to repeated attack of vasooclusion and infarction..

- splenic complications lead to mortality.

- acute splenic sequestration crisis, hypersplenism, massive infarction and abscess are the complications..

- it made ultimately autosplenectomy..it is mostly affect the sickle cell trait..