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ABNORMAL HEMOGLOBIN (Sores (Treatment (Dressing, Antibiotic, Antiseptic),…
ABNORMAL HEMOGLOBIN
Sores
Acute
Chronic
Persistent Infection
Long Duration
Treatment
Dressing
Antibiotic
Antiseptic
Differences beween edema and elephantiasis
Edema:Medical term for swelling,It is caused by capillary damage where blood vessels release fluid to the surrounding tissue
Elephantiasis : It is a chronic diseases which parasitic worms such as Brugia malayi obstruct the lymphatic system,causing swelling .It is transmitted by musquito
What Tests Should be Done?
Solubility
Electrophoresis
Full Blood Picture
Sickling Test
Anemia
Microcyctic Anemia
the size is smaller than normal RBC
Sickle Cell Anaemia
Valine to Glutamate
Shape
Function and difference
Why?
Vasocclusion
Impede blood flow
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Blood flow is blocked and oxygen supply interrupted.
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Damage to RBC
Increase Heme breakdown
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Anemia
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At low partial pressure of oxygen, HbS will release oxygen and polymerises into network of fibrous polymer that stiffens and distorts red blood cell.
Red blood cell has life span of 20 days
short life span doesn't allow parasites
Plasmodium falciparum
, parasite involved in Malaria, to complete the intracellular stage of its development.
Malaria is common in Africa
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Amount of bilirubin increases
Red blood cell is sickle-shaped and rigid.
Will produce a protrusion on β-globin chain that has a complemetary site on another β-globin chain
Hemoglobin is now known as HbS
Hb Polymerization
Symptoms
Infection
Dehydration
Acidosis
Dactylitis
Lower Leg Ulcer
Treatment
Blood Transfusion
Bone Marrow Transplantaion
Hydroxyurea
Macrocytic Anemia
The size is larger than the normal RBC
Normocytic Anemia
Same size but the number of RBC is lower than normal
Hb F:
consist of 2 alpha and 2 gamma polypeptide chain
Hb A :
Composed of 2 alpha and 2 beta polypetide chain
Hb S:
Composed of two alpha and two mutated beta polypeptide chains
How to advice patient with SCA
Regular checkup with expert hematologist
Prevent infection
Live a healthy lifestyle
Importance of 1st & 2nd degree relaionship in SCA
1st relationship (parents,siblings,offsprings) share 50% gene.
Have hiher possibility to inherit SCA
2nd degree relationship (grandparents, grandchildren, uncle, aunty) share about 25% gene
Have a little chance of inheriting SCA
How to do genetic counselling
Provide informations, advices & suggestions
Comparison between abnormal and normal hemoglobin
