Abstract:
INTRODUCTION:Joint hypermobility (JH) is the hallmark of many hereditary soft connective tissue disorders, including Ehlers-Danlos syndromes and related disorders, disorders of the TGFβ-pathway, lateral meningocele syndrome, arterial tortuosity syndrome, and cutis laxa syndromes. Contemporary practice separates individuals with isolated, non-syndromic JH from patients with Mendelian syndromes and those with hypermobility spectrum disorders. The latter is a new nosologic entity grouping together individuals with JH and related musculoskeletal manifestations, but lacking inclusion criteria for well-defined and/or single-gene disorders. Area covered: Nomenclature of JH and JH-related disorders are summarized on a practically oriented perspective. Critical areas of clinical management comprise pain; cardiovascular and respiratory issues; fatigue and dysautonomia; bone fragility; and capillary, skin and soft tissue fragility. Medical management stands on low-evidence data. Ongoing preclinical and clinical studies are aimed to reach a more personalized pharmacological approach to the management of the cardiovascular risk, musculoskeletal pain, and reduced bone mass. Expert commentary: Correct classification of patients with JH-related disorders needs a systematic approach, in which a wide array of molecular tests should be intermingled with strong clinical competences in highly specialized settings. A multispecialty, hierarchical approach should be encouraged for optimal coordination of care in systemic phenotypes.
journal_name
Expert Rev Clin Pharmacoljournal_title
Expert review of clinical pharmacologyauthors
Baban A,Castori Mdoi
10.1080/17512433.2018.1497973subject
Has Abstractpub_date
2018-07-01 00:00:00pages
689-703issue
7eissn
1751-2433issn
1751-2441journal_volume
11pub_type
杂志文章,评审abstract::In acute myocardial ischemia (AMI) the optimal treatment is rapid revascularization by angioplasty or pharmacological thrombolysis. While this is essential to resuscitate the ischemic myocardium, it results in further reperfusion injury and extension of the infarction. The main hypothesis for the mechanism of reperfus...
journal_title:Expert review of clinical pharmacology
pub_type: 杂志文章
doi:10.1586/ecp.09.41
更新日期:2009-11-01 00:00:00
abstract:INTRODUCTION:Osteoarthritis is the most common cause of arthritis and one of the main causes of chronic pain. Although opioids are frequently employed for chronic pain treatment, their usage for osteoarthritis pain remains controversial due to the associated adverse effects. Most guidelines reserve their use for refrac...
journal_title:Expert review of clinical pharmacology
pub_type: 杂志文章,评审
doi:10.1080/17512433.2018.1381556
更新日期:2018-01-01 00:00:00
abstract::The aim of this article was to assess the efficacy and safety of apremilast in treatment of psoriatic arthritis (PsA) with meta-analysis method. We included four randomized clinical trials identified from MEDLINE, EMBASE, Cochrane Library, "ISRCTN Register" and "ClinicalTrials.gov" which compared apremilast with place...
journal_title:Expert review of clinical pharmacology
pub_type: 杂志文章,meta分析,评审
doi:10.1586/17512433.2016.1159130
更新日期:2016-06-01 00:00:00
abstract:INTRODUCTION:Dapsone is a sulfone drug used to treat infectious conditions and also numerous dermatologic diseases. Fixed drug eruption is a distinctive adverse cutaneous reaction associated with the initial administration and subsequent delivery of a specific agent. Areas covered: The authors preformed a literature se...
journal_title:Expert review of clinical pharmacology
pub_type: 杂志文章,评审
doi:10.1080/17512433.2017.1322508
更新日期:2017-07-01 00:00:00
abstract::Schizophrenia is a major mental illness with a lifetime prevalence of about 1%. Antipsychotic drugs, with a primary mechanism of action that involves dopamine receptor blockade, are the mainstay in the treatment of the disorder. However, despite optimum antipsychotic treatment, few patients return to pre-morbid levels...
journal_title:Expert review of clinical pharmacology
pub_type: 社论
doi:10.1586/17512433.2016.1095086
更新日期:2016-01-01 00:00:00
abstract:INTRODUCTION:In pediatric pharmacotherapy, many drugs are still used off-label, and their efficacy and safety is not well characterized. Different efficacy and safety profiles in children of varying ages may be anticipated, due to developmental changes occurring across pediatric life. AREAS COVERED:Beside pharmacokine...
journal_title:Expert review of clinical pharmacology
pub_type: 杂志文章,评审
doi:10.1080/17512433.2016.1198256
更新日期:2016-09-01 00:00:00
abstract:INTRODUCTION:Acute bacterial skin and skin structure infections (ABSSSI) are among the most frequent infectious diseases. Recently, several new antibiotics with activity against MRSA have been approved. Tedizolid, a second-generation oxazolidinone approved for ABSSSI offers theoretical advantages over first-generation ...
journal_title:Expert review of clinical pharmacology
pub_type: 杂志文章,评审
doi:10.1080/17512433.2020.1774362
更新日期:2020-06-01 00:00:00
abstract::Methotrexate is an antifolate agent used in the treatment of various cancers and some autoimmune diseases. In oncology, methotrexate is frequently administered at a high dose (>1 g/m(2)) and comes with various procedures to reduce the occurrence of toxicity and particularly to ensure optimal renal elimination. Drug-dr...
journal_title:Expert review of clinical pharmacology
pub_type: 杂志文章,评审
doi:10.1586/ecp.11.57
更新日期:2011-11-01 00:00:00
abstract::RNAi is a powerful gene silencing process that holds great promise in cancer therapy by the use of siRNA. The aim of this review is to give an outline on different approaches to deliver siRNA and to describe the advantages and disadvantages of these systems. The prospects for siRNA are to be substantially better than ...
journal_title:Expert review of clinical pharmacology
pub_type: 杂志文章,评审
doi:10.1586/ecp.12.33
更新日期:2012-07-01 00:00:00
abstract:INTRODUCTION:The incidence of kidney dysfunction increases with age and is highly prevalent among patients with hypertension. Since many therapeutic compounds are primarily eliminated through the kidneys, impaired renal function can have negative consequences on drug disposition, efficacy and safety. Therefore, regulat...
journal_title:Expert review of clinical pharmacology
pub_type: 杂志文章,评审
doi:10.1080/17512433.2017.1274651
更新日期:2017-03-01 00:00:00
abstract::Novel pharmaceutical advances in postoperative pain management include both non-opioid adjuvants as well as opioid analgesics. Optimizing postoperative analgesics includes improving onset of action, matching duration of analgesia to the setting of use, and minimizing adverse events. To improve on the current standard ...
journal_title:Expert review of clinical pharmacology
pub_type: 社论
doi:10.1586/17512433.2015.1072465
更新日期:2015-01-01 00:00:00
abstract:INTRODUCTION:Diabetes mellitus is associated with increased cardiovascular disease (CVD) risk. Areas covered: Main goal of hypolipidemic treatment in diabetic patients is low-density lipoprotein cholesterol (LDL-C) lowering with the use of statins. Addition of ezetimibe is useful in diabetic patients who cannot achieve...
journal_title:Expert review of clinical pharmacology
pub_type: 杂志文章,评审
doi:10.1080/17512433.2017.1263565
更新日期:2017-02-01 00:00:00
abstract::The QT interval from the ECG cannot be measured precisely. The relationship of the QT interval to the RR interval within individuals across time and different RR values, and across individuals eludes complete understanding. Intrinsic beat-to-beat variability in QT interval corrected for heart rate (QTc interval) is no...
journal_title:Expert review of clinical pharmacology
pub_type: 杂志文章
doi:10.1586/17512433.1.6.815
更新日期:2008-11-01 00:00:00
abstract::After the introduction of anti-tumor necrosis factor (anti-TNF) agents, the clinical outcome of patients with Inflammatory Bowel Disease (IBD) has improved significantly. However, use of anti-TNF therapy is complicated by loss of response. In order to maintain remission, adequate serum levels are required. Hence, ther...
journal_title:Expert review of clinical pharmacology
pub_type: 杂志文章,评审
doi:10.1586/17512433.2016.1133288
更新日期:2016-01-01 00:00:00
abstract::Zanamivir is currently not recommended by the WHO for the first-line treatment of human influenza A (H5N1) infections. Zanamivir pharmacokinetics and antiviral efficacy were reviewed to assess the basis for this recommendation. Following inhalation of 10 mg zanamivir in healthy humans, the drug is widely distributed t...
journal_title:Expert review of clinical pharmacology
pub_type: 杂志文章
doi:10.1586/ecp.09.60
更新日期:2010-01-01 00:00:00
abstract:INTRODUCTION:The broad use of immunotherapy is revolutionizing the treatment paradigms of many solid tumors. Although chemotherapy remains the treatment backbone for advanced gastric cancer, improvements in its molecular characterization and progresses in understanding its underpinning biology have supported clinical d...
journal_title:Expert review of clinical pharmacology
pub_type: 杂志文章,评审
doi:10.1080/17512433.2017.1313113
更新日期:2017-06-01 00:00:00
abstract::Development of the patch clamp technique by the Nobel Prize winners Bert Sakmann and Erwin Neher led to huge advances in ion channel research. Their work laid the foundations and revolutionized electrophysiological studies of cells and ion channels. These ion channels underlie many basic cellular physiological process...
journal_title:Expert review of clinical pharmacology
pub_type: 杂志文章
doi:10.1586/ecp.10.18
更新日期:2010-05-01 00:00:00
abstract::The last decades were characterized by enormous technological advances resulting in a better understanding of disease pathologies and improvement of treatment strategies. The development of targeted drugs, whose beginning can be traced back to Paul Ehrlich's theory of the 'magic bullet' approximately 100 years ago, is...
journal_title:Expert review of clinical pharmacology
pub_type: 历史文章,杂志文章
doi:10.1586/ecp.12.71
更新日期:2013-01-01 00:00:00
abstract::Introduction: An important gap within modern medicine is the lack of enough comparative effectiveness research of marketed medicines. Low-risk pragmatic randomized controlled trials (pRCTs) are those conducted resembling usual clinical practice that poses no or minimal incremental risk compared with normal clinical pr...
journal_title:Expert review of clinical pharmacology
pub_type: 杂志文章,评审
doi:10.1080/17512433.2020.1732816
更新日期:2020-03-01 00:00:00
abstract:OBJECTIVES:To estimate the prevalence of polypharmacy (≥5 drugs) among adults and to analyze related factors. METHODS:Cross-sectional study with 1,159 interviewees distributed across 104 cities and 253 primary healthcare services delivered through the Brazilian Unified Health System. Polypharmacy-related factors were ...
journal_title:Expert review of clinical pharmacology
pub_type: 杂志文章
doi:10.1080/17512433.2020.1836958
更新日期:2020-12-01 00:00:00
abstract::Second-generation antipsychotics (SGA) have been associated with risk of stroke in elderly patients, but the molecular and genetic background under this association has been poorly investigated. The aim of the present study was to prioritize a list of genes with an SGA altered expression in order to characterize the g...
journal_title:Expert review of clinical pharmacology
pub_type: 杂志文章,评审
doi:10.1586/17512433.2014.865515
更新日期:2014-01-01 00:00:00
abstract::Valproic acid is approved for treatment of seizures and manic episodes of bipolar disorder, and continues to be one of the most commonly prescribed antiepileptic drugs in the world. Hepatotoxicity is a rare but serious side effect resulting from its use, particularly in young patients. This adverse effect does not dis...
journal_title:Expert review of clinical pharmacology
pub_type: 杂志文章,评审
doi:10.1586/17512433.2014.871202
更新日期:2014-03-01 00:00:00
abstract:INTRODUCTION:Long-acting injectable antipsychotics (LAIAs) were introduced to improve treatment adherence and tolerability of oral formulations. After risperidone was introduced as the first long-acting injectable second-generation antipsychotic, during the last five years olanzapine pamoate, once-monthly paliperidone ...
journal_title:Expert review of clinical pharmacology
pub_type: 杂志文章,评审
doi:10.1080/17512433.2016.1191945
更新日期:2016-07-01 00:00:00
abstract::Sugammadex, a modified γ-cyclodextrin, is the first selective relaxant binding agent. Sugammadex forms very tight complexes in a 1:1 ratio with steroidal neuromuscular blocking agents (rocuronium > vecuronium > pancuronium). This guest-host complex, which exists in equilibrium, is stable because of its very high assoc...
journal_title:Expert review of clinical pharmacology
pub_type: 杂志文章
doi:10.1586/17512433.2.1.37
更新日期:2009-01-01 00:00:00
abstract:INTRODUCTION:Gaucher disease (GD) is an autosomal recessive disorder resulting from the deficiency of the lysosomal enzyme glucocerebrosidase (b-glucosidase), associated with varying degrees of visceral, bone and central nervous system pathology, leading to wide phenotypic diversity. Response to therapy and clinical ou...
journal_title:Expert review of clinical pharmacology
pub_type: 杂志文章,评审
doi:10.1080/17512433.2018.1549486
更新日期:2018-12-01 00:00:00
abstract:INTRODUCTION:Induction chemotherapy based on anthracyclines and cytarabine (Ara-C) combination remains the standard of care for acute myeloid leukemia (AML) patients who are considered candidate for intensive and curative approaches. However, the toxicity of this regimen is high, with disappointing clinical outcomes am...
journal_title:Expert review of clinical pharmacology
pub_type: 杂志文章
doi:10.1080/17512433.2019.1573668
更新日期:2019-03-01 00:00:00
abstract::Knee osteoarthritis is the most common form of arthritis. Given the aging of the world's population, the burden of this disease is expected to increase significantly over the next few decades. As a pharmacological agent, glucosamine has been investigated for the treatment of symptoms and progression of knee osteoarthr...
journal_title:Expert review of clinical pharmacology
pub_type: 杂志文章
doi:10.1586/ecp.09.17
更新日期:2009-07-01 00:00:00
abstract::Sodium-glucose co-transporter-2 inhibitors (SGLT-2I) are recently approved class of anti-hyperglycaemic agents for the treatment of type 2 diabetes mellitus (T2DM). SGLT-2I inhibits renal glucose reabsorption, thereby ensuing urinary glucose excretion in a dose-dependent manner. This caloric loss and osmotic diuresis,...
journal_title:Expert review of clinical pharmacology
pub_type: 杂志文章,评审
doi:10.1586/17512433.2016.1131121
更新日期:2016-01-01 00:00:00
abstract::Neuroblastoma (NB) is the most common extra cranial solid tumor of childhood, with 60% of patients presenting with high risk (HR) NB by means of clinical, pathological and biological features. The 5-year survival rate for HR-NB remains below 40%, with the majority of patients suffering relapse from chemorefractory tum...
journal_title:Expert review of clinical pharmacology
pub_type: 杂志文章,评审
doi:10.1586/17512433.2016.1160775
更新日期:2016-01-01 00:00:00
abstract::Drug-induced liver injury (DILI) is a common form of adverse drug reaction seen within the clinic. Sensitive, specific and non-invasive biomarkers of liver toxicity are required to help diagnose hepatotoxicity and also to identify safety liabilities during drug development. Limitations exist in the current gold standa...
journal_title:Expert review of clinical pharmacology
pub_type: 杂志文章,评审
doi:10.1586/17512433.2014.904201
更新日期:2014-05-01 00:00:00