Neurologic and Head and Neck Manifestations of Sickle Cell Disease

Steven, Andrew; Raghavan, Prashant; Rath, Tanya J.; Gandhi, Dheeraj

doi:10.1016/j.hoc.2016.03.004

Cited by 15 publications

(6 citation statements)

References 29 publications

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“…Following the embolic stroke, her subsequent RBCx had an HbS target of <30%, and she eventually switched to simple transfusion program due to continuous low HbS %. Another patient (Patient #2 in Table 1) was also transferred to the high‐risk group with a target HbS <30% following the identification of internal carotid artery stenosis on magnetic resonance angiogram ordered for evaluation of chronic headaches, a finding concerning for cerebral vasculopathy 14,21 . It is unclear whether the vasculopathy was preexisting and never reversed to normal or developed during the exchange program.…”

Section: Resultsmentioning

confidence: 99%

Hemoglobin S target of <50% as compared to 30% in chronic red cell exchange for secondary stroke prevention in sickle cell disease

Choi

Markantonis

Simone

et al. 2023

J of Clinical Apheresis

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BackgroundSickle cell disease (SCD) patients with a history of stroke are encouraged to receive chronic red blood cell exchange (RBCx) for stroke prevention. The American Society of Hematology guideline published in 2020 recommends an HbS target of <30%. However, this approach necessitates more frequent RBCx and more RBC units. UT Southwestern has devised a chronic exchange protocol that elevates the HbS target to <50% in patients with a low risk of stroke.Study DesignThis retrospective chart review study reviewed the medical records of patients receiving chronic RBCx with a target of HbS <50% over the past 10‐year period to assess the safety of maintaining higher HbS targets in SCD patients with a low risk of cerebrovascular accidents (CVA).ResultsAmong 49 SCD patients in the chronic RBCx program for secondary stroke prevention, 33 patients were maintained on an HbS target of <50% (average measured: 35.4%) for the duration of RBCx program enrollment (median 93.0 months, 95% CI, 83‐99). Stroke or transient ischemic attack (TIA) clearly attributable to changing target HbS had not been identified among the 33 study subjects. Seven patients experienced conversion between the HbS targets of <50% and <30% HbS target. Significant reductions were observed in the frequency of RBCx and usage of blood volume in four of them.ConclusionThe findings suggest that liberalizing the HbS target could confer clinical flexibility without increasing the risk of CVA in a selective population. Further studies to fully evaluate the potential benefits of this approach are indicated.

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Section: Resultsmentioning

confidence: 99%

Hemoglobin S target of <50% as compared to 30% in chronic red cell exchange for secondary stroke prevention in sickle cell disease

Choi

Markantonis

Simone

et al. 2023

J of Clinical Apheresis

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show abstract

“…Despite receiving secondary prophylaxis with transfusions, approximately 20% of SCD patients had stroke recurrence [87]. Bone marrow transplantation (BMT) in SCD patients below the age of 16 observed in the mean time of 7 years revealed that these patients were protected against stroke and even experienced vasculopathy stabilization [88].…”

Section: In Pediatric Patientsmentioning

confidence: 99%

Risk Factors for Recurrent Arterial Ischemic Stroke in Children and Young Adults

Sarecka-Hujar

Kopyta

2020

Brain Sciences

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Arterial ischemic stroke (AIS) experienced at a young age is undoubtedly a serious medical problem. AIS very rarely occurs at a developmental age, whereas in young adults, it occurs with a higher frequency. The etiologic mechanisms of AIS occurring in childhood and adulthood differ. However, for both age populations, neurological consequences of AIS, including post-stroke seizures, motor disability, and recurrence of the disease, are connected to many years of care, rehabilitation, and treatment. Recurrent stroke was observed to increase the risk of patients’ mortality. One of the confirmed risk factors for recurrent stroke in children is the presence of vasculopathies, especially Moyamoya disease and syndrome, and focal cerebral arteriopathy of childhood (FCA). FCA causes a 5-fold increase in the risk of recurrent stroke in comparison with idiopathic AIS. In turn, young adults with recurrent stroke were found to more often suffer from hypertension, diabetes mellitus, or peripheral artery disease than young patients with first-ever stroke. Some reports also indicate relationships between specific genetic polymorphisms and AIS recurrence in both age groups. The aim of the present literature review was to discuss available data regarding the risk factors for recurrent AIS in children and young adults.

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“…Se concluyó además que esta anomalía se asoció a niveles de hemoglobina más elevados y menor incidencia de úlceras en miembros inferiores [35]. La complicación neurológica más común en población pediátrica son los infartos cerebrales silentes y se asocian a una alta recurrencia, sin embargo, dicha recurrencia puede ser reducida significantemente mediante la realización de transfusiones sanguíneas de forma regular [36,37,38]. Además, la sobrevida a corto plazo (30 días) cuando se presentan accidentes cerebrovasculares en estos pacientes suele ser más pobre que en individuos sanos [39].…”

Section: Complicaciones Asociadasunclassified

Anemia de Células Falciformes: Correlación Clínico-Patológica

Saavedra¹

2019

archmed

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La anemia de células falciformes es la alteración hematológica heredada más común a nivel mundial. Esta dada por la presencia de hemoglobinas anormales (HbS y sus diferentes genotipos) a raíz de una mutación en las cadenas de globina. La tríada clínica consiste en eventos vaso-oclusivos, anemia hemolítica crónica y asplenia funcional. Hoy en día se ha documentado toda clase de complicaciones asociadas a la enfermedad, entre las cuales se encuentran: accidentes cerebrovasculares, infarto agudo de miocardio, hipertensión pulmonar, tromboembolismos venosos, infecciones, necrosis de la papila renal, enfermedad renal crónica, ulceraciones en miembros inferiores, enfermedades del aparato hepatobiliar y necrosis ósea avascular. Es de vital importancia la realización de un correcto enfoque clínico y terapéutico de los pacientes con anemia de células falciformes de modo que sea posible la prevención y tratamiento oportuno de las complicaciones asociadas a esta condición.

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Neurologic and Head and Neck Manifestations of Sickle Cell Disease

Cited by 15 publications

References 29 publications

Hemoglobin S target of <50% as compared to 30% in chronic red cell exchange for secondary stroke prevention in sickle cell disease

Hemoglobin S target of <50% as compared to 30% in chronic red cell exchange for secondary stroke prevention in sickle cell disease

Risk Factors for Recurrent Arterial Ischemic Stroke in Children and Young Adults

Anemia de Células Falciformes: Correlación Clínico-Patológica

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