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Published on: March 14, 2017
Sickle cell disease: a review for the internist
Valeria Maria Pinto1, Manuela Balocco1, Sabrina Quintino1
1Centro della Microcitemia E Delle Anemie Congenite Ente Ospedaliero Ospedali Galliera, Via Volta 6, 16128, Genoa, Italy.
Insights
Sickle cell disease (SCD), a global health issue, causes severe complications. This review details acute and chronic manifestations and management for internists and emergency specialists.
Area of Science:
- Hematology
- Genetics
- Public Health
Background:
- Sickle cell disease (SCD) is a significant global hemoglobinopathy.
- It is a monogenic, multisystem disorder with substantial morbidity and mortality.
- Key pathophysiological features include vaso-occlusion, hemolytic anemia, and vasculopathy.
Purpose of the Study:
- To review the acute and chronic clinical manifestations of SCD.
- To discuss the current management strategies for SCD.
- To provide guidance for internists and emergency specialists managing SCD patients.
Main Methods:
- This is a review article.
- It synthesizes current knowledge on SCD pathophysiology and clinical care.
- Focuses on time-dependent acute and chronic complications.
Main Results:
- SCD presents with diverse acute and chronic complications.
- Effective management requires understanding of pathophysiology.
- Internists and emergency specialists play a crucial role in patient care.
Conclusions:
- SCD is a complex global health challenge requiring comprehensive management.
- Awareness of acute and chronic issues is vital for clinicians.
- This review supports healthcare professionals in managing SCD patients effectively.
Abstract:
Sickle cell disease (SCD) is the most important hemoglobinopathy worldwide in terms of frequency and social impact, recently recognized as a global public health problem by the World Health Organization. It is a monogenic but multisystem disorder with high morbidity and mortality. Vaso-occlusion, hemolytic anemia and vasculopathy are the hallmarks of SCD pathophysiology. This review focuses both on "time-dependent" acute clinical manifestations of SCD and chronic complications commonly described in adults with SCD. The review covers a broad spectrum of topics concerning current management of SCD targeted at the internists and emergency specialists who are increasingly involved in the care of acute and chronic complications of SCD patients.
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