AAP Health Supervision for Children with Sickle Cell Disease

Question 1

What is the inheritance pattern for sickle cell disease?

Answer 1

Autosomal recessive

Question 2

What are the important acute manifestations of sickle cell disease?

Answer 2

• 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

Question 3

What are the important chronic clinical manifestations in sickle cell disease?

Answer 3

• 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