Sickle Cell Disease Flashcards
What is SCD
A hereditary blood disorder affecting Hemoglobin cause it to sickle or form a crescent shape
What’s the prevalence of SCD in Nigeria
1-3%
How many subunits does Hemoglobin have?
4 subunits
2 alpha 2 beta
What are the protein components of Hb
Globin
What are the Non-protein components
Heme
Ferrous
Protoporphyrin
What type of mutation happens in SCD
Single Point Mutation
At what point does the mutation occur
Chromosome 11 point 6
Sickle Cell RBC Half Life
17days
Normal RBC half life
120 days
What are the Acute complications SCD
VOC
Infections
Severe Anaemia
What are the types of VOC
Acute VOC
Acute Chest Syndrome
Priapism
Myocardial Infarction
Renal Infarction
VTE
Stroke
What are the stressful conditions which exacerbate sickling
Hypoxia
Infection
Acidosis
Dehydration
Fever
Trauma
Illness
What are Chronic Complications of SCD
Chronic Compensated Hemolytic Anaemia
Pain
Neurological deficit
Pulmonary Conditions
Renal Hypertension
Osteoporosis
Delayed puberty
Hepatotoxicity
Chronic Leg ulcer
Cardiomyopathy
Delayed Growth
The Hemoglobinopathy seen in SCD is of a Systolic dysfunction T/F
False. Diastolic
What Investigations used for Diagnosis
Prenatal Screening
Neonatal Screening
HPLC
HB Electrophoresis
