Abstract:
:Considering the progress made in the management of sickle cell disease during the past 30 years, along with the excellent results obtained with hematopoietic stem cell transplantation (SCT), it is important to reexamine why, who, when and how to recommend allogeneic SCT in children with sickle cell disease. While sickle cell disease has a low risk of death in children and a high risk for morbidity during aging, SCT carries an early risk of death, graft-vs-host disease and infertility. Nevertheless, SCT offers at least 95% chance of cure with low risk of chronic graft-vs-host disease when a matched-sibling donor is available and the risks of infertility can be reduced by ovarian, sperm or testis cryopreservation. Thus, all available therapies such as hydroxyurea, transfusions and SCT should be presented to the parents, providers, and affected children and discussed with them from infancy. Furthermore, the use of these therapies should be adjusted to the severity of the disease and to local availabilities in order to choose the treatment offering the best benefit/risk ratio.
journal_name
J Clin Medjournal_title
Journal of clinical medicineauthors
Bernaudin Fdoi
10.3390/jcm8101523subject
Has Abstractpub_date
2019-09-22 00:00:00issue
10issn
2077-0383pii
jcm8101523journal_volume
8pub_type
杂志文章,评审abstract::The recent advances in machine perfusion (MP) technology involve settings ranging between hypothermic, subnormothermic, and normothermic temperatures. Tissue level adenosine triphosphate (ATP) is a long-established marker of viability and functionality and is universal for all organs. In the midst of a growing number ...
journal_title:Journal of clinical medicine
pub_type: 杂志文章,评审
doi:10.3390/jcm8091421
更新日期:2019-09-09 00:00:00
abstract::The non-consensual sharing of an intimate image is a serious breach of a person's right to privacy and can lead to severe psychosocial consequences. However, little research has been conducted on the reasons for consuming intimate pictures that have been shared non-consensually. This study aims to investigate how the ...
journal_title:Journal of clinical medicine
pub_type: 杂志文章
doi:10.3390/jcm8050706
更新日期:2019-05-17 00:00:00
abstract::Current therapy of Clostridium difficile diarrhea (CDD) is problematic. Optimal treatment for CDD remains oral vancomycin, but there is little data on oral vancomycin dosing regimens. The objective of this C. difficile diarrhea study was to compare the efficacy of "high dose" vancomycin, 500 mg (PO) q6h, as sole treat...
journal_title:Journal of clinical medicine
pub_type: 杂志文章
doi:10.3390/jcm7040075
更新日期:2018-04-10 00:00:00
abstract::An elevation of serum low-density lipoprotein cholesterol (LDL-C) levels has been associated with endothelial dysfunction in statin naïve subjects. However, there is no information on endothelial function in subjects with extremely low levels of LDL-C. The purpose of the present study was to determine the relationship...
journal_title:Journal of clinical medicine
pub_type: 杂志文章
doi:10.3390/jcm9123796
更新日期:2020-11-24 00:00:00
abstract:BACKGROUND:The Dyskinesia Impairment Scale (DIS) is a new assessment scale for dystonia and choreoathetosis in children and youth with dyskinetic cerebral palsy. Today, the Burke-Fahn-Marsden Dystonia Rating Scale (BFM) is mostly used to assess dystonia in children with inherited dystonia. The aim of this study was to ...
journal_title:Journal of clinical medicine
pub_type: 杂志文章
doi:10.3390/jcm9082597
更新日期:2020-08-11 00:00:00
abstract::The main risk factor for primary open-angle glaucoma (POAG) is increased intraocular pressure (IOP). It is of interest that about half of the patients have an IOP within the normal range (normal-tension glaucoma, NTG). Additionally, there is a group of patients with a high IOP but no glaucomatous neurodegeneration (oc...
journal_title:Journal of clinical medicine
pub_type: 杂志文章
doi:10.3390/jcm9092979
更新日期:2020-09-15 00:00:00
abstract::Primary Sjögren's syndrome (pSS) is a chronic systemic autoimmune rheumatic disease characterized by lymphoplasmacytic infiltration of the salivary and lacrimal glands, whereby sicca syndrome and/or systemic manifestations are the clinical hallmarks, associated with a particular autoantibody profile. pSS is the most f...
journal_title:Journal of clinical medicine
pub_type: 杂志文章,评审
doi:10.3390/jcm9072299
更新日期:2020-07-20 00:00:00
abstract::Chronic pain is one of the major causes of disability in the general population. Even though there are effective treatment options available for reducing symptoms, these treatments often do not have consistent lasting effects. As the usage of mobile devices has increased enormously during the last few years, mobile ap...
journal_title:Journal of clinical medicine
pub_type: 杂志文章,评审
doi:10.3390/jcm9113557
更新日期:2020-11-05 00:00:00
abstract::Topical application of vascular endothelial growth factor A (VEGFA) inhibitors including Bevacizumab is used for antiangiogenic therapy at the ocular surface. While clinical studies have suggested that this approach is well-tolerated, the effect of the drug on limbal epithelial stem cells has not been studied. In this...
journal_title:Journal of clinical medicine
pub_type: 杂志文章
doi:10.3390/jcm8111925
更新日期:2019-11-09 00:00:00
abstract::(1) Background: Acute respiratory distress syndrome (ARDS) following cardiac arrest is common and associated with in-hospital mortality. We aim to investigate whether lung compliance during targeted temperature management is associated with neurological outcome in patients with ARDS after out-of-hospital cardiac arres...
journal_title:Journal of clinical medicine
pub_type: 杂志文章
doi:10.3390/jcm9020527
更新日期:2020-02-14 00:00:00
abstract:BACKGROUND:Due to its unknown etiology, the objective diagnosis and therapeutics of chronic fatigue syndrome/myalgic encephalomyelitis (CFS/ME) are still challenging. Generally, the patient-reported outcome (PRO) is the major strategy driving treatment response because the patient is the most important judge of whether...
journal_title:Journal of clinical medicine
pub_type: 杂志文章,评审
doi:10.3390/jcm9113463
更新日期:2020-10-28 00:00:00
abstract::(1) Background: L-arginine is a complex modulator of immune functions, and its levels are known to decrease under septic conditions. L-arginine may suppress leukocyte recruitment in vivo; however, little is known about the gestational age-specific effects of L-arginine on leukocyte recruitment in preterm infants. We n...
journal_title:Journal of clinical medicine
pub_type: 杂志文章
doi:10.3390/jcm9092772
更新日期:2020-08-27 00:00:00
abstract::There is a large body of research showing that psychological treatment can be effectively delivered via the internet, and Digital Mental Health Services (DMHS) are now delivering those interventions in routine care. However, not all attempts to translate these research outcomes into routine care have been successful. ...
journal_title:Journal of clinical medicine
pub_type: 杂志文章
doi:10.3390/jcm8081239
更新日期:2019-08-17 00:00:00
abstract:BACKGROUND:Sarcopenia is a prevalent muscle abnormality characterized by progressive and generalized loss of skeletal muscle mass and strength, common among patients with decompensated advanced chronic liver disease (dACLD). Irisin is a recently identified myokine, which is mainly expressed and secreted by skeletal mus...
journal_title:Journal of clinical medicine
pub_type: 杂志文章
doi:10.3390/jcm9103158
更新日期:2020-09-29 00:00:00
abstract:BACKGROUND:Hybrid minimally invasive esophagectomy (HMIE) has been proven to be superior when compared with open esophagectomy, with a significant reduction of postoperative morbidity. In HMIE, the laparotomy is replaced by a minimally invasive laparoscopic approach. The radical mediastinal resection plus reconstructio...
journal_title:Journal of clinical medicine
pub_type: 杂志文章
doi:10.3390/jcm8070978
更新日期:2019-07-05 00:00:00
abstract::This study sought to determine whether the implementation of regular and structured follow-up of patients with chronic heart failure (CHF), combined with therapeutic education and remote monitoring solution, leads to better management. This was a single-center retrospective study conducted in a cohort of patients with...
journal_title:Journal of clinical medicine
pub_type: 杂志文章
doi:10.3390/jcm9103106
更新日期:2020-09-25 00:00:00
abstract::The relationship between obesity and asthma exacerbations is still under debate. The aim of our work is to analyse the relationship between obesity and hospital re-admissions in asthmatics. A review was retrospectively performed on all hospital admissions of adult patients due to asthma exacerbation occurring in our h...
journal_title:Journal of clinical medicine
pub_type: 杂志文章
doi:10.3390/jcm9010221
更新日期:2020-01-14 00:00:00
abstract::Most human papillomavirus (HPV) antiviral strategies have focused upon inhibiting viral DNA replication, but it is increasingly apparent that viral DNA levels can be chemically controlled by approaches that promote its instability. HPVs and other DNA viruses have a tenuous relationship with their hosts. They must repl...
journal_title:Journal of clinical medicine
pub_type: 杂志文章
doi:10.3390/jcm4020204
更新日期:2015-01-01 00:00:00
abstract:BACKGROUND:Ghrelin is a 28-amino acid peptide with an orexigenic property, which is predominantly produced by the stomach. Acylated ghrelin is the active form of this hormone. Obestatin is a 23-amino acid peptide which is produced by post-translational modification of a protein precursor that also produces ghrelin. Obe...
journal_title:Journal of clinical medicine
pub_type: 杂志文章
doi:10.3390/jcm5070061
更新日期:2016-06-23 00:00:00
abstract::Androgen/androgen receptor (AR) signaling is a significant driver of prostate cancer progression, therefore androgen-deprivation therapy (ADT) is often used as a standard form of treatment for advanced and metastatic prostate cancer patients. However, after several years of ADT, prostate cancer progresses to castratio...
journal_title:Journal of clinical medicine
pub_type: 杂志文章,评审
doi:10.3390/jcm8030354
更新日期:2019-03-13 00:00:00
abstract::This study was conducted to determine whether overcrowding in the emergency department (ED) affects the occurrence of a return visit (RV) within 72 hours. The crowding indicator of index visit was the average number of total patients, patients under observation, and boarding patients during the first 1 and 4 hours fro...
journal_title:Journal of clinical medicine
pub_type: 杂志文章
doi:10.3390/jcm9051406
更新日期:2020-05-09 00:00:00
abstract::Effective behavior change approaches are necessary to help individuals avoid or mitigate risk factors, engage in behavioral patterns that lead to better health, and consequently, prevent several chronic diseases. Physical inactivity is considered one of the most harmful risk factors for chronic medical conditions, and...
journal_title:Journal of clinical medicine
pub_type: 杂志文章
doi:10.3390/jcm8030295
更新日期:2019-03-01 00:00:00
abstract::Aim. To evaluate by meta-analysis of interventional studies the effect of statin therapy on arterial wall inflammation. Background. Arterial exposure to low-density lipoprotein (LDL) cholesterol levels is responsible for initiation and progression of atherosclerosis and arterial wall inflammation. 18F-fluorodeoxygluco...
journal_title:Journal of clinical medicine
pub_type: 杂志文章,评审
doi:10.3390/jcm8010118
更新日期:2019-01-18 00:00:00
abstract::The aim of this article is to investigate pathological subjects from a population through different physical factors. To achieve this, particle swarm optimization (PSO) and K-means (KM) clustering algorithms have been combined (PSO-KM). Datasets provided by the literature were divided into three clusters based on age ...
journal_title:Journal of clinical medicine
pub_type: 杂志文章
doi:10.3390/jcm7040065
更新日期:2018-03-28 00:00:00
abstract::Background. Accumulating evidence suggests that inflammation plays a key role in acute kidney injury (AKI) pathogenesis. We explored the relationship between high-sensitivity C-reactive protein (hs-CRP) and AKI in acute myocardial infarction (AMI). Methods. We prospectively included 2,063 AMI patients in whom hs-CRP w...
journal_title:Journal of clinical medicine
pub_type: 杂志文章
doi:10.3390/jcm8122192
更新日期:2019-12-12 00:00:00
abstract:BACKGROUND:No dural substitute has proven to be complication-free in a large clinical trial, even suggesting some benefit during watertight closure. However, primary dural closure is not always possible due to dural shrinkage from electrocautery for dural bleeding. OBJECTIVE:This study is performed to analyze the clin...
journal_title:Journal of clinical medicine
pub_type: 杂志文章
doi:10.3390/jcm9040902
更新日期:2020-03-25 00:00:00
abstract::Electrical enabling motor control (eEmc) through transcutaneous spinal cord stimulation is a non-invasive method that can modify the functional state of the sensory-motor system. We hypothesize that eEmc delivery, together with hand training, improves hand function in healthy subjects more than either intervention alo...
journal_title:Journal of clinical medicine
pub_type: 杂志文章
doi:10.3390/jcm10020195
更新日期:2021-01-07 00:00:00
abstract::Stem cells have been the focus of intense research opening up new possibilities for the treatment of various diseases. Mesenchymal stromal cells (MSCs) are multipotent cells with relevant immunomodulatory properties and are thus considered as a promising new strategy for immune disease management. To enhance their eff...
journal_title:Journal of clinical medicine
pub_type: 社论
doi:10.3390/jcm8050626
更新日期:2019-05-08 00:00:00
abstract:INTRODUCTION:Cardiovascular risk factors, pre-existing comorbidities, molecular factors, and the direct effects of second- and third-generation BCR-ABL1 tyrosine kinase inhibitors on the vascular endothelium contribute to the progression of cardiovascular (CV) events, especially atherothrombotic conditions. The study o...
journal_title:Journal of clinical medicine
pub_type: 杂志文章
doi:10.3390/jcm9103269
更新日期:2020-10-12 00:00:00
abstract::Recurrent high-grade glioma (HGG) remains incurable with inevitable evolution of resistance and high inter-patient heterogeneity in time to progression (TTP). Here, we evaluate if early tumor volume response dynamics can calibrate a mathematical model to predict patient-specific resistance to develop opportunities for...
journal_title:Journal of clinical medicine
pub_type: 杂志文章
doi:10.3390/jcm9072019
更新日期:2020-06-27 00:00:00