Folic Acid Supplementation in Children With Sickle Cell Disease

Condition:   Anemia, Sickle Cell Interventions:   Dietary Supplement: Folic Acid Supplement;   Dietary Supplement: Placebo Sponsor:   University of British Columbia Not yet recruiting
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials

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Conclusions: Rarely, an emergency splenectomy is required in complex settings, mostly refractory hematological conditions, in a deteriorating patient when all other measurements have failed. A multidisciplinary team approach is mandatory in the treatment of these complex cases.What is known• Conservative treatment is advised for splenic injury.• Many hematological disorders are responsible of splenic pathology.What is new• Emergency splenectomy in children for reasons other than trauma is a treatment of last resort that should be performed in a multidisciplinary context.• The outcome of emergency splene...
Source: European Journal of Pediatrics - Category: Pediatrics Source Type: research
Abstract Sickle cell disease afflicts millions of people worldwide and approximately 100,000 Americans. Complications are myriad and arise as a result of complex pathological pathways 'downstream' to a point mutation in DNA, and include red blood cell membrane damage, inflammation, chronic hemolytic anemia with episodic vaso-occlusion, ischemia and pain, and ultimately risk of cumulative organ damage with reduced patient lifespan. The National Heart, Lung, and Blood Institute's 2014 evidence-based guideline for sickle cell disease -management states additional research is needed before investigational curative the...
Source: Haematologica - Category: Hematology Authors: Tags: Haematologica Source Type: research
Condition:   Sickle Cell Disease Intervention:   Drug: IMR-687 Sponsor:   Imara, Inc. Enrolling by invitation
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
Oxidative damage to red blood cells (RBCs) may contribute to pathogenesis of sickle cell anaemia. Reducing the deleterious effects of oxidants by exposing RBCs to a number of antioxidants has been shown to have protective effects against lipid and protein peroxidation. We hypothesise that antioxidants may also have beneficial effects on the abnormal membrane permeability of sickle cells. Increased cation permeability of these cells encourages HbS polymerisation by causing RBC dehydration and also leads to externalisation of the prothrombotic aminophospholipid phosphatidylserine (PS). Three antioxidants with different mecha...
Source: Frontiers in Physiology - Category: Physiology Source Type: research
We examined adhesion to LN in a microfluidic device, of RBCs from men with homozygous sickle cell anemia. Adhesion under hypoxic, but not ambient, conditions was greater in men with a history of priapism, with median adhesion of 529 RBCs per 32 mm2/unit area (range 5–5248) rising to 3268 RBCs per 32 mm2/unit area (range 49–18,368, P = 0.004), under ambient and hypoxic conditions, respectively (n = 14). This was not seen in RBCs from men without a history of priapism (median 402 (range 14–785) and 122 (range 31–4112) RBCs per 32 mm2/unit area), ambient and hypoxic conditions, respective...
Source: Blood Cells, Molecules, and Diseases - Category: Hematology Source Type: research
It’s only been seven years since scientists first learned how to precisely and reliably splice the human genome using a tool called CRISPR, making it possible to think about snipping out disease-causing mutations and actually cure, once and for all, genetic diseases ranging from sickle cell anemia to certain types of cancer and even blindness. Doctors are plunging ahead in search of ways to use the relatively new technology to start treating patients. In China last November, scientist Jiankui He stunned—and dismayed—the genetic community when he announced he had already used CRISPR, which many believe sti...
Source: TIME: Health - Category: Consumer Health News Authors: Tags: Uncategorized crispr Genetics Source Type: news
There is a question about what role race and wealth play in how much attention and funding the disease receives.
Source: NYT - Category: American Health Authors: Tags: Sickle Cell Anemia Preventive Medicine Blacks Cystic Fibrosis Discrimination Source Type: news
CONCLUSION: Transfusion of PIPA-treated RBCs further increases the metabolic benefits of RCE to patients with SCA, significantly reducing the levels of post-transfusion plasticizers. PMID: 31385330 [PubMed - as supplied by publisher]
Source: Transfusion - Category: Hematology Authors: Tags: Transfusion Source Type: research
We examined adhesion to LN in a microfluidic device, of RBCs from men with homozygous sickle cell anemia. Adhesion under hypoxic, but not ambient, conditions was greater in men with a history of priapism, with median adhesion of 529 RBCs per 32 mm2/unit area (range 5-5248) rising to 3268 RBCs per 32 mm2/unit area (range 49-18,368, P = 0.004), under ambient and hypoxic conditions, respectively (n = 14). This was not seen in RBCs from men without a history of priapism (median 402 (range 14-785) and 122 (range 31-4112) RBCs per 32 mm2/unit area, ambient and hypoxic conditions, respectively (P = N.S., N =...
Source: Blood Cells, Molecules and Diseases - Category: Hematology Authors: Tags: Blood Cells Mol Dis Source Type: research
AbstractSickle cell disease (SCD) is the most important hemoglobinopathy worldwide in terms of frequency and social impact, recently recognized as a global public health problem by the World Health Organization. It is a monogenic but multisystem disorder with high morbidity and mortality. Vaso-occlusion, hemolytic anemia and vasculopathy are the hallmarks of SCD pathophysiology. This review focuses both on “time-dependent” acute clinical manifestations of SCD and chronic complications commonly described in adults with SCD. The review covers a broad spectrum of topics concerning current management of SCD targete...
Source: Internal and Emergency Medicine - Category: Emergency Medicine Source Type: research
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