Manifestations of Sickle Cell Disorder at Abdominal and Pelvic                     Imaging

Solomon, Nadia; Segaran, Nicole; Badawy, Mohamed; Elsayes, Khaled M.; Pellerito, John S.; Katz, Douglas S.; Moshiri, Mariam; Revzin, Margarita V.

doi:10.1148/rg.210154

Cited by 5 publications

(2 citation statements)

References 68 publications

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“…SCD is far from being the sole hemolytic anemia; the prothrombotic state induced by SCD is responsible for multi-organ dysfunction. Neurologic sequelae, leg ulcers, and renal dysfunction are part of the wide spectrum of multiorgan system involvement observed in SCD [ 11 ].…”

Section: Discussionmentioning

confidence: 99%

Endothelial dysfunction linked to ventricular dysfunction in children with sickle cell disease, a 3D speckle tracking study.

AbdelMassih,

Haroun,

AbdelAziz Afifi

et al. 2024

Journal of the Saudi Heart Association

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Background Sickle Cell Disease (SCD) is not a hematologic disease that occurs in isolation; it results in multi-organ complications. There is growing evidence of vascular stiffness as its underlying cause. This study aimed to investigate the relationship between endothelial stiffness and LV dysfunction in SCD patients and to explore its pathophysiology, particularly regarding the depletion of vasodilators such as Nitric Oxide (NO). Methodology 32 patients with established criteria for SCD and 40 healthy control subjects were selected for this case-control study. Comprehensive clinical assessment and assessment of endothelial function using Brachial Flow-mediated dilation (FMD) were performed, along with serum NO measurement, which was followed by diagnosis and echocardiographic assessment using 3D speckle tracking echocardiography (STE) and tissue Doppler imaging (TDI). Results Collected SCD cases showed echocardiographic features of Systo-diastolic dysfunction with reduced FMD compared to controls, denoting endothelial dysfunction in those patients. LDH showed a marked elevation, while serum NO showed a significant reduction in cases compared with controls. We also noted a positive correlation between FMD on the one hand and measures of ventricular dysfunction and level of serum NO on the other hand, the latter proving that reduction of NO is responsible for reduced endothelial function. Conclusion We present the first report to date to outline the role of vascular stiffness as measured by brachial FMD in the induction of left ventricular dysfunction in SCD. We recommend that more research be conducted regarding possible strategies to replenish serum NO stores to delay microvascular injury and, in turn, ventricular dysfunction in SCD.

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Section: Discussionmentioning

confidence: 99%

Endothelial dysfunction linked to ventricular dysfunction in children with sickle cell disease, a 3D speckle tracking study.

AbdelMassih,

Haroun,

AbdelAziz Afifi

et al. 2024

Journal of the Saudi Heart Association

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“…Microscopic alterations in kidney function frequently go unnoticed, contributing to delayed diagnoses. Furthermore, multiple recent studies underscores the importance of incorporating advanced ultrasound imaging techniques in SCN diagnosis, providing valuable insights into subtle renal changes that conventional methods might overlook (14,15). This additional study accentuates the need for a comprehensive diagnostic approach in addressing the challenges posed by SCN.…”

Section: Challenges In Diagnosismentioning

confidence: 99%

Sickle cell nephropathy; exploring epidemiology, diagnostic challenges, and precision medicine advances

Rastegar-Kashkouli,

Yousefi,

Jafari

et al. 2024

J Prev Epidemiol

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Sickle cell nephropathy (SCN) poses an important challenge in the realm of sickle cell disease (SCD) and sickle cell trait (SCT), especially impacting the pediatric population and progressing to chronic kidney disease (CKD) in adulthood. This review navigates through the epidemiology of SCN, emphasizing its variable clinical course and the age-related dynamics of onset. The intricate challenges in diagnosis are explored, highlighting the evolving landscape of early detection and treatment strategies. The prevalence of SCN is intricately linked to the global distribution of SCD and SCT, with higher incidences in regions where these conditions prevail. Diagnosing SCN presents significant challenges due to its variable clinical course, necessitating advanced imaging techniques and novel biomarkers. Promising biomarkers such as KIM-1 and MCP-1 show potential for early detection and intervention, addressing the limitations of conventional diagnostic methods. Precision medicine, focusing on genetic profiling, emerges as a crucial approach for risk stratification and personalized treatment plans. The multifaceted clinical manifestations of SCN, including hematuria, proteinuria, and hypertension, underscore the importance of timely intervention. Treatment strategies involve the administration of angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, hydroxyurea for preventing chronic organ damage, and kidney transplantation for end-stage renal disease. Early diagnosis and intervention are critical to mitigate the progression of SCN and its associated complications, emphasizing the need for collaborative efforts in research and precision medicine to improve outcomes for individuals with SCN.

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Pictorial review: challenges in distinguishing bilateral metaphyseal marrow abnormalities on magnetic resonance imaging

Booth,

Kapadia,

Tang

et al. 2024

Pediatr Radiol

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Manifestations of Sickle Cell Disorder at Abdominal and Pelvic Imaging

Cited by 5 publications

References 68 publications

Endothelial dysfunction linked to ventricular dysfunction in children with sickle cell disease, a 3D speckle tracking study.

Endothelial dysfunction linked to ventricular dysfunction in children with sickle cell disease, a 3D speckle tracking study.

Sickle cell nephropathy; exploring epidemiology, diagnostic challenges, and precision medicine advances

Pictorial review: challenges in distinguishing bilateral metaphyseal marrow abnormalities on magnetic resonance imaging

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