The spleen in the sickling disorders: an update

@article{Khatib2008TheSI,
  title={The spleen in the sickling disorders: an update},
  author={Rana Khatib and Raja M 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… Expand
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Prevalence and predictive factors of splenic sequestration crisis among 423 pediatric patients with sickle cell disease in Tunisia.
TLDR
The high prevalence of SSC in SCD and the high frequency of recurrence after a first episode were confirmed and were intimately linked to the presence of splenomegaly, chronic pallor revealing the disease as well as reticulocytosis. Expand
Spleen histology in children with sickle cell disease and hereditary spherocytosis: hints on the disease pathophysiology.
TLDR
SCD spleens display greater histologic effacement than HS, and SCD-related changes suggest impaired function due to vascular damage, which may contribute to guide the clinical management of patients. Expand
Splenic morphological changes are accompanied by altered baseline immunity in a mouse model of sickle-cell disease.
TLDR
Altered SCD splenic morphological characteristics result in an impaired systemic immune response, and this model suggests that functional asplenia from infarctions may be a major contributor to increased infectious mortality in sickle-cell disease. Expand
Carriage of encapsulated bacteria in Gabonese children with sickle cell anaemia.
  • F. Schaumburg, B. Biallas, +8 authors A. Adegnika
  • Biology, Medicine
  • Clinical microbiology and infection : the official publication of the European Society of Clinical Microbiology and Infectious Diseases
  • 2013
TLDR
Carriage and resistance rates are similar in children with and without SCA, and data provide the basis to guide empiric therapy of invasive diseases caused by S. pneumoniae, Staph. Expand
Non-traumatic spleen disorders in children. Assessment by imaging.
TLDR
The aim was to show the behaviour of the spleen in children using the different imaging techniques: its normal anatomy, the principal anatomical variants and the most common spleen disorder correlating with clinical symptoms, serology and histology. Expand
Non-traumatic spleen disorders in children. Assessment by imaging
TLDR
The behaviour of the spleen in children is shown using the different imaging techniques: its normal anatomy, the principal anatomical variants and the most common spleen disorder correlating with clinical symptoms, serology and histology. Expand
Current sickle cell disease management practices in Nigeria.
TLDR
The care available for SCD in Nigeria is still suboptimal and there is an urgent need for concerted effort to tackle the problem, but to make a significant impact on the burden of the disease would require more focus at the primary care level. Expand
WEB-BASED MONITORING OF PAIN MANAGEMENT IN ADOLESCENT AND YOUNG ADULT SICKLE CELL PATIENTS THROUGH DAILY SELF-ASSESSMENT
TLDR
The implementation of a web-based application to monitor daily pain levels is relevant to public health because it is likely to provide medical teams with the tools necessary to track patient pain levels more precisely and to enable healthcare providers to be more proactive with regard to pain management in patients with SCD. Expand
Incidence of Sickle Cell Anaemia among Children Attending Maryam Abacha Women and Children Hospital, Sokoto
TLDR
Improved knowledge regarding Sickle cell anaemia disease and its comprehensive care among Nigerian physicians will enhance quality of care for affected childrens and policy for regular genotype test by government and other stakeholders before marriage among Nigerians will help to prevent the disease. Expand
Abdominal ultrasound with scintigraphic and clinical correlates in infants with sickle cell anemia: baseline data from the BABY HUG trial.
TLDR
In infants with sickle cell anemia, sonographic spleen volume does not reflect function, but increased renal volume correlates with GFR and is consistent with hyperfiltration. Expand
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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. Expand
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. Expand
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. Expand
Postsplenectomy course in homozygous sickle cell disease.
TLDR
Splenectomy does not increase the risk of death or bacteremic illness in patients with SS disease and, if otherwise indicated, should not be deferred for these reasons. Expand
Splenic complications of the sickling syndromes and the role of splenectomy.
TLDR
With careful perioperative management, splenectomy is both safe and beneficial in a select group of patients with SCD and S-beta-Thal. Expand
Milzsequestrationen bei Patienten mit Sichelzellerkrankungen
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. Expand
Acute splenic sequestration in homozygous sickle cell disease: natural history and management.
TLDR
Of a cohort of 308 children with homozygous sickle cell disease diagnosed at birth, 89 experienced 132 clinically significant attacks of acute splenic sequestration (ASS) over a 10-year period, and the improvement in outcome is likely to have resulted from improvement in medical management and earlier detection of ASS. Expand
Acute chest syndrome in the postoperative sickle cell patient.
TLDR
Interestingly, five of six cases were after laparoscopic procedures suggesting that the advantages of laparoscopy, such as reduced postoperative pain, do not extrapolate to decreased incidence of ACS. Expand
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. Expand
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. Expand
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