AAP Health Supervision for Children with Sickle Cell Disease Flashcards
1
Q
What is the inheritance pattern for sickle cell disease?
A
Autosomal recessive
2
Q
What are the important acute manifestations of sickle cell disease?
A
- bacterial sepsis or meningitis
- recurrent vaso-occlusive pain (dactylitis, MSK or abdominal pain)
- splenic sequestration
- aplastic crises
- acute chest syndrome
- stroke
- priapism
- hematuria incld papillary necrosis
3
Q
What are the important chronic clinical manifestations in sickle cell disease?
A
- anemia
- jaundice
- splenomegaly
- functional asplenia
- cardiomegaly and functional murmurs
- hyposthenuria and enuresis
- proteinuria
- cholelithiasis
- delayed growth and sexual maturation
- restrictive lung disease
- pulmonary hypertension
- avascular necrosis
- proliferation retinopathy
- leg ulcers
- transfusional hemosiderosis
