scholarly journals Major Artery Occlusion: a Rare Complication of Sickle Cell Disease

1970 ◽  
Vol 2 (1) ◽  
pp. e2010007
Author(s):  
Adnan Agha
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Internal Carotid ◽  
Rare Complication ◽  
Artery Occlusion ◽  
Left Internal Carotid Artery ◽  
Cell Disease ◽  
Vessel Occlusion ◽  
Major Vessel ◽  
Haemolytic Anemia

Abstract: Sickle cell disease is hereditary hemoglobinopathy which causes haemolytic anemia, vaso-occlusive crisis, ischemic injuries and many other morbidities like cerebral infarction.  In this report, we describe a case of a young patient with sickle cell disease presenting with right-sided weakness and slurring of speech with examination confirming right-sided hemiparesis with motor aphasia. On further investigation, she was found to have frontotemporal infarction.  On magnetic resonance imaging with angiography, she was found to have absent circulation in left internal carotid artery probably secondary to sickle cell disease.  Major vessel occlusion is rare complication of sickle cell disease that one must bear in mind.

scholarly journals Feasibility of an mHealth self-management intervention for children and adolescents with sickle cell disease and their families

2021 ◽  
Author(s):  
Shannon Phillips ◽  
Julie Kanter ◽  
Martina Mueller ◽  
Amy Gulledge ◽  
Kenneth Ruggiero ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Rating Scale ◽  
Management Strategies ◽  
Self Management ◽  
Cell Disease ◽  
Vessel Occlusion ◽  
Feasibility Assessment ◽  
Management Intervention ◽  
Scale Scores

Abstract Sickle cell disease (SCD) is an inherited hemoglobinopathy that leads to blood vessel occlusion and multiorgan complications, including pain, that may be experienced daily. Symptom management often begins at home, and tools are needed to support self-management strategies that can be implemented by children with SCD and families. The purpose of this study was to assess the feasibility of the mHealth self-management intervention (application) Voice Crisis Alert V2 for children with SCD and families. Feasibility assessment was guided by the Reach, Efficacy, Adoption, Implementation, and Maintenance framework. Data were collected with 60 dyads (children with SCD/caregivers) at four time points. Self-management data were collected via application use, and postintervention interviews were conducted. Analyses included descriptive statistics and constant comparison with directed content analysis. Recruitment was completed in 28 weeks, with 82% retention at end-of-intervention. Mobile Application Rating Scale scores and interview data indicated high satisfaction. From baseline to mid-intervention, 94% of dyads used the application (75% of total use); 45% used the application from mid-intervention to the end-of-intervention. Dyads made 2,384 actions in the application; the most commonly used features were recording health history and recording and tracking symptoms. Few reported issues with the application; most issues occurred early in the study and were corrected. After the intervention period was completed, 37% continued to use the application. Feasibility was confirmed by meeting recruitment and retention goals, high adoption of the application, and high reported satisfaction with the application. Challenges with sustained use were encountered, and areas for improvement were identified.

scholarly journals Sickle Cell Disease: Role of Oxidative Stress and Antioxidant Therapy

Antioxidants ◽  
2021 ◽  
Vol 10 (2) ◽  
pp. 296
Author(s):  
Rosa Vona ◽  
Nadia Maria Sposi ◽  
Lorenza Mattia ◽  
Lucrezia Gambardella ◽  
Elisabetta Straface ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Globin Gene ◽  
Organ Damage ◽  
Cell Disease ◽  
Vessel Occlusion ◽  
Antioxidant Agents ◽  
Oxidant Status

Sickle cell disease (SCD) is the most common hereditary disorder of hemoglobin (Hb), which affects approximately a million people worldwide. It is characterized by a single nucleotide substitution in the β-globin gene, leading to the production of abnormal sickle hemoglobin (HbS) with multi-system consequences. HbS polymerization is the primary event in SCD. Repeated polymerization and depolymerization of Hb causes oxidative stress that plays a key role in the pathophysiology of hemolysis, vessel occlusion and the following organ damage in sickle cell patients. For this reason, reactive oxidizing species and the (end)-products of their oxidative reactions have been proposed as markers of both tissue pro-oxidant status and disease severity. Although more studies are needed to clarify their role, antioxidant agents have been shown to be effective in reducing pathological consequences of the disease by preventing oxidative damage in SCD, i.e., by decreasing the oxidant formation or repairing the induced damage. An improved understanding of oxidative stress will lead to targeted antioxidant therapies that should prevent or delay the development of organ complications in this patient population.

scholarly journals Concurrent Bilateral Central Retinal Artery Occlusion Secondary to Sickle Cell Crisis

2021 ◽  
Vol 9 ◽  
pp. 232470962110283
Author(s):  
Gowri Renganathan ◽  
Piruthiviraj Natarajan ◽  
Lela Ruck ◽  
Roberto Prieto ◽  
Bharat Ved Prakash ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Vision Loss ◽  
Central Retinal Artery Occlusion ◽  
Retinal Artery Occlusion ◽  
Artery Occlusion ◽  
Cell Disease ◽  
Sickle Cell Crisis ◽  
History Of ◽  
Retinal Artery

Vascular occlusive crisis with a concurrent vision loss on both eyes is one of the most devastating disability for sickle cell disease patients. Reportedly occlusive crisis in the eyes is usually temporary whereas if not appropriately managed can result in permanent vision loss. A carefully managed sickle cell crisis could prevent multiple disabilities including blindness and stroke. We report a case of a 24-year-old female with a history of sickle cell disease who had acute bilateral vision loss during a sickle crisis and recovered significantly with a timely emergent erythrocytapheresis.

scholarly journals Internal Carotid Artery Septa in Sickle Cell Disease: Risk Factor for Stroke?

2008 ◽  
Vol 35 (3) ◽  
pp. 378-380
Author(s):  
Pranshu Sharma ◽  
Mayank Goyal ◽  
Ali M. Al-Khathaami ◽  
Andrew Demchuk
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Blood Cells ◽  
Internal Carotid ◽  
Disease Risk ◽  
Beta Globin ◽  
Cell Disease ◽  
Cerebral Infarcts ◽  
Disease Risk Factor ◽  
Hemoglobin Molecule

Sickle cell disease is a hemoglobinopathy occurring due to replacement of valine for glutamic acid at the sixth position of the beta globin chain. The altered hemoglobin structure makes it prone for polymerization during hypoxic and infective stress. Polymerization of the hemoglobin molecule leads to sickling of the red blood cells in the vessels causing thrombosisvasoocclusive crises. Although abdomen and extremities are more often involved, silent cerebral infarcts and stroke can occur in up to 25-29% of patients and is the major cause of morbidity and mortality.

Pulmonary Artery Occlusion Pressure May Overdiagnose Pulmonary Artery Hypertension in Sickle Cell Disease

CHEST Journal ◽  
2011 ◽  
Vol 140 (4) ◽  
pp. 881A
Author(s):  
Sunil Sharma ◽  
Renuka Kadali ◽  
Ramesh Daggubati ◽  
Jimmy Efird ◽  
Hadi Chohan ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Pulmonary Artery ◽  
Sickle Cell ◽  
Pulmonary Artery Occlusion Pressure ◽  
Artery Occlusion ◽  
Pulmonary Artery Hypertension ◽  
Occlusion Pressure ◽  
Cell Disease

Sudden onset blindness in sickle cell disease due to retinal artery occlusion

2008 ◽  
Vol 50 (3) ◽  
pp. 624-627 ◽  
Author(s):  
Robert I. Liem ◽  
Diane M. Calamaras ◽  
Manpreet S. Chhabra ◽  
Beatrice Files ◽  
Caterina P. Minniti ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Retinal Artery Occlusion ◽  
Artery Occlusion ◽  
Sudden Onset ◽  
Cell Disease ◽  
Retinal Artery
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