The spleen in the sickling disorders: an update

@article{Khatib2008TheSI,
  title={The spleen in the sickling disorders: an update},
  author={Rana Khatib and Raja Rabah and Sharada A. Sarnaik},
  journal={Pediatric Radiology},
  year={2008},
  volume={39},
  pages={17-22}
}
In early life, patients with sickle cell disease (SCD) can have acute, life-threatening emergencies related to splenic hypofunction (overwhelming bacterial sepsis), as well as anemic crises from acute splenic sequestration because of sudden pooling of blood in the spleen. The landmark penicillin prophylaxis study in 1985 showed a remarkable decrease in mortality from sepsis in young children with SCD who were treated with oral penicillin prophylaxis compared to placebo. Since that study… 
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References

SHOWING 1-10 OF 41 REFERENCES
[Splenic sequestration in patients with sickle cell disease. ].
TLDR
Parents of infants and toddlers with sickle cell disease need to learn how to palpate the spleen in order to detect splenomegaly as early as possible and take the child to the hospital.
First report of reversal of organ dysfunction in sickle cell anemia by the use of hydroxyurea: splenic regeneration.
TLDR
It is suggested that chronic HU therapy may reverse splenic dysfunction in certain patients and suggest that this drug may have efficacy beyond the elimination of pain in SCA.
Management of acute splenic sequestration crisis in sickle cell disease.
  • R. Grover, D. Wethers
  • Medicine
    Journal of the Association for Academic Minority Physicians : the official publication of the Association for Academic Minority Physicians
  • 1990
TLDR
Surgery after the first episode of ASSC in the child 5 years of age and older and the choice of a year or more of long-term transfusion therapy for the child under 3 are recommended.
Acute chest syndrome in the postoperative sickle cell patient.
Infections in sickle cell anemia: pathogenesis and control.
TLDR
Experience would suggest that pneumococcal vaccines may provide only modest protection in the child with SCD less than 5 years of age, and prophylactic antibiotic regimens have yet to be submitted to rigid scientific investigation.
Hyposplenism: A comprehensive review. Part I: Basic concepts and causes
TLDR
In splenectomized, and functionally hyposplenic subjects, the pneumococcal conjugate vaccine is more effective, because it utilizes a T cell dependent mechanism, and should be the preferred vaccine in these circumstances.
Bone marrow transplantation corrects the splenic reticuloendothelial dysfunction in sickle cell anemia.
TLDR
The data indicate that BMT can correct "permanent asplenia" in SCA patients, however, it remains to be determined if such treatment can also correct other SCA-related organ dysfunctions.
Complications of splenectomy.
  • C. Shatney
  • Medicine
    Acta anaesthesiologica Belgica
  • 1987
TLDR
Surgeons are changing their attitudes concerning splenectomy for trauma, both accidental and iatrogenic, and nonoperative management of hemodynamically stable patients with isolated splenic injury and splenorrhaphy in patients requiring laparotomy are now firmly entrenched in the surgical armamentarium.
Hydroxyurea in Children with Sickle Cell Disease: Impact on Splenic Function and Compliance with Therapy
TLDR
Hydroxyurea in children is associated with sustained excellent compliance and monitoring this compliance is uncomplicated, and splenic function in most hydroxyurea-treated children did not change over 1 year of therapy.
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