Cerebral Hemodynamic Responses to Disease-Modifying and Curative Sickle Cell Disease Therapies.

Background And Objectives: Sickle cell disease (SCD) is a hemoglobinopathy resulting in hemoglobin-S production, hemolytic anemia, and elevated stroke risk. Treatments include oral hydroxyurea, blood transfusions, and hematopoietic stem cell transplantation (HSCT). Our objective was to evaluate the neurologic relevance of these therapies by characterizing how treatment-induced changes in hemoglobin (Hb) affect brain health biomarkers.

Iron deficiency and heart failure with preserved ejection fraction.

Objective: Aim: We aimed to assess the prevalence of ID in patients HFpEF and its relation to functional capacity and quality of life.

Patients And Methods: Materials and Methods: We included in the analysis 121 consecutive outpatients newly diagnosed of HFpEF and tested with iron-related parameters. Patients were subdivided in two groups according to the presence of ID (n = 76, mean age 65.

Sickle cell disease in India: the journey and hope for the future.

India, the most populous nation in the world, also has a high frequency of the sickle hemoglobin (HbS) allele globally. The Arab Indian HbS haplotype in India is characterized by a relatively high percentage of fetal Hb, with widely varying frequencies of α-thalassemia. Hence, sickle cell disease (SCD) in India was perceived to be mild.

Pharmacological blocking of neutrophil extracellular traps attenuates immunothrombosis and neuroinflammation in cerebral cavernous malformation.

Cerebral cavernous malformation (CCM) is a neurovascular disease with symptoms such as strokes, hemorrhages and neurological deficits. With surgery being the only treatment strategy, understanding the molecular mechanisms of CCM is crucial in finding alternative therapeutic options for CCM. Neutrophil extracellular traps (NETs) were recently reported in CCM, and NETs were shown to have positive or negative effects in different disease contexts.

STROKE AS A LIFE-THREATENING COMPLICATION IN CHILDREN WITH LINEAR SCLERODERMA OF FACE.

Objective: To investigate the spectrum of neurological disorders in children with juvenile localized scleroderma (JLS) on face and JLS without plaques on face and head.

Materials And Methods: 156 children with JLS were examined were with a neurological examination MRI, EEG, genetic thrombophilia markers detection.

Results: Neurological disorders (ND) were found in 56 from 114 (49%) of the patients with scleroderma of head and face (LSH)(group1) and in 30% (13 from 42) with JLS without plaques on face (Group 2).

The impact of iron deficiency on prognosis in stroke and non-stroke populations: A retrospective cohort study.

Background And Aims: Iron deficiency (ID) leads to a significant global health burden, but research on the impact of ID on the prognosis of stroke patients is rare. We aim to investigate the impact of ID on the all-cause mortality of both the stroke and non-stroke individuals.

Methods And Results: This retrospective cohort study used data from the National Health and Nutrition Examination Survey (NHANES) conducted from 1999 to 2002.

Effects of Preoperative Hemoglobin on Microsurgical Reconstruction and Perioperative Blood Transfusion Requirement: A Meta-Analysis and Systematic Review of the Literature.

Purpose: Although blood loss in microsurgical reconstruction is rarely large enough to be life-threatening, preoperative anemia can be a particular issue in terms of postoperative morbidity, impaired wound healing, and compromised tissue viability. We seek to review the effect of preoperative hemoglobin (Hgb) levels on perioperative blood transfusion (PBT) requirements and complications to guide management of patients with preexisting anemia undergoing reconstruction.

Methods: PubMed, Embase, and Scopus were queried for relevant articles.

Cerebral Oxygen Metabolic Stress in Children and Adults With Large Vessel Vasculopathy Due to Sickle Cell Disease.

Background And Objectives: Large vessel vasculopathy (LVV), or moyamoya syndrome, increases the risk of stroke in patients with sickle cell disease (SCD), yet effective treatments are lacking. In atherosclerotic carotid disease, previous studies demonstrated elevated oxygen extraction fraction (OEF) as a predictor of ipsilateral stroke. In a SCD cohort, we examined hemispheric hemodynamic and oxygen metabolic dysfunction as tissue-based biomarkers of cerebral ischemic risk in patients with LVV.

Outcomes of Emicizumab in Acquired Hemophilia Patients: A Systematic Review.

Background: Emicizumab, a bispecific factor VIII mimetic antibody, was approved in 2018 for bleeding prophylaxis in congenital hemophilia A with or without inhibitors. Since then, several case reports and case series have described the off-label use of emicizumab in acquired hemophilia A (AHA), and data from two clinical trials were recently published (AGEHA, GTH-AHA-EMI).

Objectives: To describe the reported data on the outcomes of emicizumab, highlighting its benefit/risk profile in treatment.

HEALTH-RELATED QUALITY OF LIFE OF CHILDREN WITH AND WITHOUT SICKLE CELL ANAEMIA AT EDWARD FRANCIS SMALL TEACHING HOSPITAL, THE GAMBIA.

Introduction/background: Children with sickle cell anaemia (SCA) experience recurrent vaso-occlusive crises and complications with possible impact on their health-related quality of life (HRQoL).

Objectives: This study determined HRQoL of 130 children aged 5-15 years with SCA and compared it to age- and sex-matched apparently healthy haemoglobin AA children in The Gambia. It also determined the impact of SCD severity, and sociodemographic and clinical data on HRQoL.

Association of White Matter Hyperintensities With Serum Lipid Profile and Hematologic Parameters in Migraine Patients.

Introduction Migraine is one of the most common neurological diseases. Comorbidities, especially stroke, may be observed. White matter hyperintensities are common in migraine patients.

Longitudinal assessment of transcranial Doppler imaging in children with sickle cell disease without neurological symptoms.

Introduction: Stroke is one of the most devastating complications of sickle cell disease (SCD). Transcranial Doppler Imaging (TCDI) is the least invasive screening method to predict patients at risk for developing stroke in the disease. After a 10-year follow-up, we longitudinally assessed the TCDI in children with SCD without neurological symptoms.

Association of human leucocyte antigen loci with vaccine-induced immune thrombotic thrombocytopenia: Potential role of the interaction between platelet factor 4-derived peptides and MHC-II.

No risk factors have been identified for vaccine-induced immune thrombotic thrombocytopenia (VITT) so far. The aim of this study was to identify human leucocyte antigen (HLA) alleles potentially associated with VITT susceptibility. Specific HLA class II alleles were detected with significantly higher frequency in VITT patients compared with Italian controls: DPB1*17:01, DQA1*05:01, and DRB1*11:04.

The effect of tranexamic acid on postpartum bleeding in women with moderate and severe anaemia (WOMAN-2): an international, randomised, double-blind, placebo-controlled trial.

Background: Tranexamic acid, given within 3 h of birth, reduces bleeding deaths in women with postpartum haemorrhage. We examined whether giving tranexamic acid shortly after birth can prevent postpartum haemorrhage in women with moderate or severe anaemia.

Methods: This international, randomised, double-blind, placebo-controlled trial was done in 34 hospitals across four countries (Nigeria, Pakistan, Tanzania, and Zambia).