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Sickle Cell Disease - Wiley Online Library

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Last Updated: 12 June 2022

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Developmental disorders in children born to women with sickle cell disease: A report from the Boston Birth Cohort

"Children exposed to maternal sickle cell disease have numerous potential causes for developmental disorders, but no information is known about long-term effects for these childrens. " To compare developmental outcomes between children exposed to maternal SCD and matched, unexposed controls, we used the Boston Birth Cohort to compare developmental outcomes among children exposed to maternal SCD and matched, unexposed controls. ".

Source link: https://onlinelibrary.wiley.com/doi/10.1002/jha2.478


Retention of functional mitochondria in mature red blood cells from patients with sickle cell disease

"Sickle cell disease is an inherited blood disorder characterized by sickled red blood cells, which are more sensitive to haemolysis and can play a role in disease pathophysiology. " Patients with SCD can be treated with RBC transfusion, but SCD patients with SCD have elevated risks of alloimmunization. We hypothesized that RBCs from patients with SCD have functionally active mitochondria and can elicit a type 1 interferon reaction. In comparison to healthy blood donors, we examined blood samples from more than 100 patients with SCD and discovered elevated frequencies of mitochondria in reticulocytes and mature RBCs. Producing type 1 interferons, which have been shown to raise RBC alloimmunization rates, is a hallmark of mitochondrial transplantation. These results show that mitochondria stored in mature RBCs are effective and can provoke immune responses, indicating that improper mitochondria retention in RBCs could play under-appreciated role in SCD issues and be a RBC alloimmunization risk factor. ".

Source link: https://onlinelibrary.wiley.com/doi/10.1111/bjh.18287


Mental health assessment of youth with sickle cell disease and their primary caregivers during the COVID‐19 pandemic

"Compared to largely pre-u2010 pandemic findings, the prevalence of mental health problems in dyad residents seemed to have increased: fewer youth and more caregivers were affected during the pandemic," one group explained; many of both groups seemed to have lacked hope. ".

Source link: https://onlinelibrary.wiley.com/doi/10.1002/pbc.29797


Validation of Patient‐reported Vaso‐occlusive Crisis Day as an Endpoint in Sickle Cell Disease Studies

A SCD Diary was included in the Evaluation of Longitudinal Pain Study in Sickle Cell Disease, a longitudinal, six-month non-interventional study, with the aim of establishing a daily patientu2010reported outcome for patients with SCD to correctly identify their VOC status and history of a pain epidemic in order to validate a daily patientu2010reported outcome for patients with SCD in order to establish their VOC status and experience of a pain epidemic. " On the first or last VOC Day of a VOC Day of a VOC Event, mean tu2010test scores were compared between the three non-u2010VOC Days before and after the event, with the event's mean. VOC Days mean severity and function scores on all VOC Days in comparison to all non-u2010VOC Days were higher, with statistically significant mean differences between first and last VOC Days and respective third non-u2010VOC Days cited in the original/last VOC Days. Participants expressed relief from ordinary pain in that the pain crisis description accurately portrayed the VOC experience, and almost all agreed that the pain relief described the VOC experience accurately.

Source link: https://onlinelibrary.wiley.com/doi/10.1111/ejh.13790

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions