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Sickle cell disease: the clinical manifestations
1Marmora Medical Commons, NJ 08223, USA.
The Journal of the American Osteopathic Association
|October 1, 1995
Summary
Sickle cell disease (SCD) causes widespread complications affecting multiple organ systems, including bone, eyes, kidneys, and lungs. Early diagnosis and management are crucial for patients with SCD due to high risks of stroke, infection, and other serious health issues.
Area of Science:
- Hematology
- Genetics
- Internal Medicine
Background:
- Sickle cell disease (SCD) disproportionately affects individuals of African descent in the U.S.
- The vaso-occlusive state in SCD leads to diverse multisystemic complications.
- SCD requires careful consideration of various diagnostic challenges across different organ systems.
Purpose of the Study:
- To outline the broad spectrum of diagnostic considerations in sickle cell disease.
- To highlight key complications in musculoskeletal, ocular, renal, and pulmonary systems.
- To emphasize the importance of early detection and management of SCD complications.
Main Methods:
- Review of common SCD complications across organ systems.
- Discussion of diagnostic challenges and differential diagnoses.
- Highlighting the role of imaging (CT, MRI) and pulmonary function tests.
Main Results:
- High incidence of avascular necrosis, leg ulcers, osteomyelitis, and retinopathy in SCD.
- Frequent renal failure, dehydration, pneumonia, and sickle cell chest syndrome.
- Significant risk of stroke (25%), cholelithiasis, and high mortality from Streptococcus pneumoniae infections due to asplenia.
Conclusions:
- Sickle cell disease presents complex diagnostic challenges due to its multisystemic nature.
- Prompt identification and management of SCD complications are vital for patient outcomes.
- Awareness of associated conditions like cholelithiasis and concurrent rheumatologic/immunologic diseases is essential.