Sickle Cell Anemia (Subscribe)  

Background: Sickle cell disease (SCD) and its variants are genetic disorders of mutant hemoglobins (Hb). The most common form found in North America is homozygous Hb S disease, first described by Herrick in 1910. Morbidity, frequency of crisis, degree of anemia, and the organ systems involved vary considerably from individual...

Wheeless Textbook Discussion: - affects 1% of blacks - is more severe but less common than sickle cell trait (8% prevalence); - two main genotypes include Hb S and Hb C forms of disease; - crises usually begin at age 2-3 years...

Radiology of SCD

* 8-13% of blacks carry sickling factor
* symptoms: chronic ulcers, pain crises, many infections, priapism
xray findings:
* deossification due to marrow hyperplasia
  o decreased bone density in skull with widened diploic space
  o "H-shaped vertebrae" or "fish vertebrae"
  o rib notching
* thrombosis and infarction
  o AVN,...

Showing AVN of humeral heads and vertebral changes

Sickle cell disease is a haemoglobinopathy with autosomal recessive inheritance. Beta chain of HbA has valine substituted for glutamine at position 6 10% of black people have this condition in the UK In the homozygote, deoxygenated HbS becomes insoluble, leading to red cells becoming rigid and sickle shaped The onset of sickle...

Radiology case 38--651 Clinical presentation: There are two cases, each in a young woman of African ancestry. The condition is an inherited anaemia with recurrent episodes that can affect many body systems.
There is biconcave narrowing of the vertebral bodies. The depressions in the end-plates are squared. The bone density is...

Sickle Cell Disease and Related Hemoglobinopathies POSNA Core Curriculum
Objectives
1. Describe different combinations of normal and abnormal hemoglobins and their phenotypic expression
2. Describe the pathophysiology of vasooclusion secondary to hemoglobinopathies
3. Define and describe hand-foot syndrome
4. Describe features of osteomyelitis secondary...

Selected abstracts

We report the athletic, the clinical, and the pathological details of a case of fatal rhabdomyolysis during training in a college football player with sickle cell trait (SCT) who collapsed minutes after running 16 successive sprints of 100 yd each.
Counting this case, at least 15 college football players with SCT...

Nursing oriented list of references on caring for patients with Sickle Cell Disease Haemoglobinopathy Association of Counsellors

Covers the whole condition in detail. Chapter 21 has a good account of the musculoskeletal consequences of SCD and an excellent list of references