This was prepared by Dr. Mlaluko to explain Acute chest syndrome

1. SICKLE CELL DISEASE
(SCD)
PRESENTER
Dr. Mlaluko Rajabu

2. Outlines
Definition
Pathophysiology
Clinical features
Type of crises
Management

3. Definition
Is a genetic disorder of the red blood cells
characterized by red blood cells that assume an
abnormal, rigid, sickle shape.

4. Cont…
• Sickle-cell disease is an autosomal partially
recessive disorder of haemoglobin chains in
red blood cells

5. Cont…

6. Pathophysiology
The primary pathology springs from production
of abnormal hemoglobin (Hbs) which has a valine
for glutamic acid substitution at the sixth position
of beta globin chain.
 Under low-oxygen conditions (e.g at high
altitude), the absence of a polar amino acid at
position six of the β-globin chain promotes the
aggregation of hemoglobin, which distorts red
blood cells into a sickle shape.

7. Cont….
Sickle RBCs are rigid to deform as a
consequence are unable to pass through a
narrow capillaries leading to vessel occlusion
and causes various pathologies

8. Clinical features
Clinical features vary with: Age and
Environmental factors
Early: Painful swelling of hand & foot (so-
called hand and foot syndrome/dactylitis)

9. Cont…
Others stigmata
 Pale
 Jaundice
 Hepatosplenomegally
 Weakness/ paralysis
 Bossing of head
 Poor growth

10. Type of Crises
 Vaso-occlusive crises
Aplastic crisis a/w Parvo virus
Splenic sequestration crisis
Acute fall in Hb , shock (compensated
Reticulocyte count)
 Hemolytic crisis
ACUTE CHEST SYNDROME

11. ACUTE CHEST SYNDROME
Acute illness x-tized by radiological finding
of new pulmonary infiltrates with fever and
respiratory symptoms. It is most fatal crisis.
Clinical features
Chest pain, tachypnea, respiratory distress,
fever, decreased oxygen saturation and chest
X-ray infiltrates

12. Cont…

13. Cont…
Etiology
Associated with; infections (pneumonia),
pulmonary fat embolism or infarction.
 Mostly organism identified are; M.
pneumoniae, RSV, S. aureus, S. pneumoniae
(decreased)
Parvo-B19 a/w bone marrow infarction/
necrosis.

14. Risk factors
Low HbF
Young age
Presence of asthma or other hyperactive lung
disorder
Smoking
Recent trauma or surgery

15. Management
The treatment for ACS includes broad-
spectrum antibiotics such as cephalosporin and
macrolide
Ampicillin, Gentamycin, Ceftriaxone
Supportive; Analgesics,oxygenation,hydration,
blood transfusion, bronchodilators(if asthmatic
or bronchospasm present).

16. QN
Is there any role of steroids
to these patients??

17. References
1. Standard treatment guidelines & National essential medicines list Tanzania
mainland, 2017
2. Mediscape