Radiological findings of Posterior Reversible Encephalopathy Syndrome in transplanted children previous affected by hemoglobinopathy: A neuroimaging retrospective analysis

Publication date: 2019Source: European Journal of Radiology Open, Volume 6Author(s): Eliseo Picchi, Francesca Di Giuliano, Simone Marziali, Silvia Minosse, Valentina Ferrazzoli, Valerio Da Ros, Javid Gaziev, Chiara Adriana Pistolese, Roberto Floris, Francesco GaraciAbstractTo evaluate, by Magnetic Resonance Imaging, if there is a typical pattern or severity of PRES in transplanted children for hemoglobinopathy. Secondary point was to investigate the pattern and severity of PRES in children with thalassemia-THAL and sickle-cell disease-SCD after autologous hematopoietic stem cell transplantation (aHSCT). Finally, we evaluate the presence of atypical PRES presentation and the involved area of central nervous system. Two neuroradiologists analyzed retrospectively MRI of 21 transplanted children for THAL or SCD treated with CI, with neurological symptoms and signs of PRES. The Bartynski and Boardman classification has been used for PRES pattern while McKinney scale for PRES severity. Fisher Exact Probability test or Chi-square test were used to compare the categorical data. In the 21 transplanted children the PRES severity was typically mild (85.7%) without preferring radiological pattern at MRI. The analysis didn't show significant association between PRES pattern or PRES severity and previous hemoglobinopathy (THAL or SCD). No atypical PRES presentation has been found. PRES severity in transplanted children for hemoglobinopathy is typically mild. Notwithstanding children affect...
Source: European Journal of Radiology Open - Category: Radiology Source Type: research

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Sickle cell disease (SCD) is an autosomal recessive genetic condition that alters the shape and function of the hemoglobin molecule in red blood cells. While the overall survival rate among children with SCD has improved in recent years, pediatric rates of hospitalization, ED use, and mortality from complications of SCD remain high. Among patients ages 18 and older, hospital admission and ED usage are even greater—and the median age at death of people with SCD is considerably lower than that of the general population. Nurses who care for patients with SCD have an opportunity to improve health outcomes and quality of ...
Source: AJN - Category: Nursing Tags: Feature Articles Source Type: research
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Source: AJN - Category: Nursing Tags: Feature Articles Source Type: research
ConclusionExamples include introducing precise genomic modifications into primary T cells and hematopoietic stem cells via transfection of mRNA and RNPs encoding zinc finger nucleases and CRISPR/Cas9 components as well as single nucleotide editing for correction of disease causing genetic mutations including X-linked chronic granulomatous disease (X-CGD) and sickle cell disease (SCD). We also include data on redirecting the specificity of T cells and NK cells by expressing recombinant receptors. Finally, we provide examples of cellular engineering with induced pluripotent stem cells (iPSCs) and other types of progenitor cells.
Source: Cytotherapy - Category: Cytology Source Type: research
The objectives of this study were to identify cases of hypereosinophilia in a tertiary pediatric medical center, determine clinical characteristics and disease associations, and estimate the incidence of hypereosinophilia in the hospital and geographic populations.MethodsA retrospective chart review included patients
Source: The Journal of Allergy and Clinical Immunology: In Practice - Category: Allergy & Immunology Source Type: research
Sickle cell disease (SCD) results from a unique mutation in the β-globin gene and is the most common genetic hemoglobinopathy. It is characterized by hemolytic anemia, painful vaso-occlusive crisis, and progressive organ failure, including nephropathy. Red blood cells (RBCs) in patients with SCD are subjected to constant endogenous and exogenous oxidative stres s, which in turn increase hemolysis and contribute to vasculopathies. A gradient of hemolysis among SCD patients influences the SCD phenotype.
Source: Kidney International - Category: Urology & Nephrology Authors: Tags: Nephrology Digest Source Type: research
Abstract Background and Objectives: Altered copper and zinc homeostasis may influence the antioxidant defense system and consequently lead to oxidative stress and associated complications in sickle cell disease (SCD) patients. Iron levels have been reported to increase in sickle cell patients due to frequent blood transfusion, chronic intravenous haemolysis and increased absorption of iron from the gastrointestinal tract. These elevated levels of iron may also lead to extensive oxidative damage. The current study evaluated serum levels of iron, copper and zinc in SCD patients and "healthy" controls. Mate...
Source: Medicina (Kaunas) - Category: Universities & Medical Training Authors: Tags: Medicina (Kaunas) Source Type: research
Conclusion: There was no difference between VTE recurrence and choice of anticoagulation in SCD patients with VTE. Bleeding events were lower for DOACs compared to VKA or LMWH.Acta Haematol
Source: Acta Haematologica - Category: Hematology Source Type: research
Abstract BACKGROUND: Venous thromboembolism (VTE) is a recognized complication of sickle cell disease (SCD), yet the optimal pharmacologic anticoagulant is unknown. METHODS: A retrospective single-institution cohort study of patients with SCD complicated by first VTE from January 2009 through July 2017 was performed using ICD 9/10 codes. Data collected included the anticoagulant used, VTE recurrence, and incidence of bleeding. RESULTS: 109 patients with VTE were identified. SCD genotypes included HbSS in 92 (84%), HbSC in 13 (12%), and HbS-β+ thalassemia in 4 (4%). After the initial VTE event, 32 pa...
Source: Acta Haematologica - Category: Hematology Authors: Tags: Acta Haematol Source Type: research
CONCLUSION: Using NAAT for the detection of respiratory pathogens in adults presenting with SCD slightly improved the microbiological diagnostic of febrile ACS, although respiratory infections are not the main etiological factor. PMID: 31088757 [PubMed - as supplied by publisher]
Source: Medecine et Maladies Infectieuses - Category: Infectious Diseases Tags: Med Mal Infect Source Type: research
[Monitor] The Kabaka of Buganda Ronald Muwenda Mutebi II has injected shs494m in Sickle cell prevention and treatment campaign.
Source: AllAfrica News: Health and Medicine - Category: African Health Source Type: news
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