Sequestro esplênico agudo em crianças com anemia falciforme

Araujo, Andréa Nogueira

doi:10.1590/s0021-75572009000400018

Cited by 2 publications

(1 citation statement)

References 2 publications

(1 reference statement)

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“…The rapidly increasing spleen size may lead to abdominal distension, sudden weakness, increased thirst, tachycardia and tachypnoea. Splenic sequestration crisis is an emergency because if left untreated, it can lead to death in 1-2 h due to circulatory failure [49].…”

Section: Splenic Sequestration Crisismentioning

confidence: 99%

Sickle Cell Disease—Genetics, Pathophysiology, Clinical Presentation and Treatment

Inusa

Hsu

Kohli

et al. 2019

IJNS

115

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Sickle cell disease (SCD) is a monogenetic disorder due to a single base-pair point mutation in the β-globin gene resulting in the substitution of the amino acid valine for glutamic acid in the β-globin chain. Phenotypic variation in the clinical presentation and disease outcome is a characteristic feature of the disorder. Understanding the pathogenesis and pathophysiology of the disorder is central to the choice of therapeutic development and intervention. In this special edition for newborn screening for haemoglobin disorders, it is pertinent to describe the genetic, pathologic and clinical presentation of sickle cell disease as a prelude to the justification for screening. Through a systematic review of the literature using search terms relating to SCD up till 2019, we identified relevant descriptive publications for inclusion. The scope of this review is mainly an overview of the clinical features of pain, the cardinal symptom in SCD, which present following the drop in foetal haemoglobin as young as five to six months after birth. The relative impact of haemolysis and small-vessel occlusive pathology remains controversial, a combination of features probably contribute to the different pathologies. We also provide an overview of emerging therapies in SCD.

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Section: Splenic Sequestration Crisismentioning

confidence: 99%

Sickle Cell Disease—Genetics, Pathophysiology, Clinical Presentation and Treatment

Inusa

Hsu

Kohli

et al. 2019

IJNS

115

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Efficacy of homologous inositol hexaphosphate‐loaded red blood cells in sickle transgenic mice

et al. 2012

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SummaryPatients with sickle cell disease (SCD) can present several severe symptoms during their lifetime, including painful events due to vascular occlusion (VOC). Even though multiple factors are involved in VOC, hypoxia is the most important triggering factor. Inositol hexaphosphate (IHP) reduces the oxygen-haemoglobin affinity thus improving the oxygen release in the blood stream and in the tissues. Thus, IHP-loaded homologous red blood cells (IHP-RBCs) could be able to reduce disorders in SCD. The effectiveness of treatment was assessed in two types of SCD transgenic mice (BERK and SAD). The administration of four repeated injections of IHP-RBCs in BERK mice resulted in an improved survival rate and brain development, prevention of severe anaemia and a greatly lowered risk of VOC. After one injection of IHP-RBCs, SAD mice were subjected to acute hypoxic stress. Analysis of the lungs revealed significantly decreased mRNA levels of molecules involved in intravascular disorders. Our results showed that transfusion of homologous IHP-RBCs, by increasing the oxygen delivery, reduces SCD disorders in sickle transgenic mice.

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Sequestro esplênico agudo em crianças com anemia falciforme

Cited by 2 publications

References 2 publications

Sickle Cell Disease—Genetics, Pathophysiology, Clinical Presentation and Treatment

Sickle Cell Disease—Genetics, Pathophysiology, Clinical Presentation and Treatment

Efficacy of homologous inositol hexaphosphate‐loaded red blood cells in sickle transgenic mice

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