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Multiple Allele Traits01:49

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Characterization of Sickling During Controlled Automated Deoxygenation with Oxygen Gradient Ektacytometry
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Published on: November 5, 2019

Osteoarticular involvement in sickle cell disease.

Geraldo Bezerra da Silva Junior1, Elizabeth De Francesco Daher, Francisco Airton Castro da Rocha

  • 1Department of Internal Medicine, Faculdade de Medicina, Universidade Federal do Ceará - UFC, Fortaleza, CE, Brazil ; School of Medicine, Centro de Ciências da Saúde, Universidade de Fortaleza - UNIFOR, Fortaleza, CE, Brazil.

Revista Brasileira De Hematologia E Hemoterapia
|October 11, 2012
PubMed
Summary

Sickle cell disease frequently causes bone complications like osteonecrosis and osteomyelitis due to blood vessel blockages. Further research is needed for better treatments for these painful bone issues.

Keywords:
Anemia, sickle cell/complicationsArthritisBone diseases/etiologyHemoglobin SC DiseaseOsteomyelitisOsteonecrosis

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Area of Science:

  • Hematology
  • Orthopedics
  • Rheumatology

Background:

  • Osteoarticular involvement in sickle cell disease (SCD) is understudied, primarily manifesting as osteonecrosis, osteomyelitis, and arthritis.
  • Painful vaso-occlusive crises and osteomyelitis are the most common and hospital-requiring complications in SCD patients.
  • Bone microcirculation is susceptible to sickling of hemoglobin S, leading to thrombosis, infarcts, and necrosis.

Purpose of the Study:

  • To review the osteoarticular manifestations of sickle cell disease.
  • To highlight the pathophysiology and clinical presentation of bone complications in SCD.
  • To emphasize the need for further research into specific therapies.

Main Methods:

  • Literature review of osteoarticular complications in sickle cell disease.
  • Analysis of the pathophysiology of vaso-occlusion in bone.
  • Summary of clinical features and current treatment approaches.

Main Results:

  • Osteonecrosis, particularly of the hip and shoulder, is a common, debilitating complication.
  • Dactylitis (hand-foot syndrome) is an acute vaso-occlusive complication.
  • Osteomyelitis is the most frequent joint infection in SCD; connective tissue diseases are rare.

Conclusions:

  • Sickle cell disease leads to significant and painful osteoarticular complications.
  • Current treatments are primarily symptomatic, underscoring the need for improved therapeutic strategies.
  • Understanding the complex pathophysiology is crucial for developing targeted treatments for SCD bone disease.