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Sickle cell disease (SCD) is a highly complex inherited disorder of hemoglobin structure. Although the molecular lesion is a single-point mutation, the sickle gene is pleiotropic in nature causing multiple phenotypic expressions that constitute the various complications of the disease. Its manifestations could be acute, chronic, nociceptive, neuropathic that could occur singly or in various combinations. Pain continues to be the major factor of SCD phenotypic complications and the most common cause of admissions to the Emergency Department and/or the hospital. Although progress has been made in understanding the pathophysiology of SCD as well as in developing curative therapies such as hematopoietic stem cell transplantation and gene therapy, effective pain management continues to lag behind. Palliative therapies continue to be the major approach to the management of SCD and its complications. The advent of hydroxyurea made partial success in preventing the frequency of vaso-occlusive crises and l-glutamine awaits post-trial confirmation of benefits. The search for additional pharmacotherapeutic agents that could be used singly or in combination with hydroxyurea and/or l-glutamine awaits their dawn hopefully in the near future. The purpose of this review is to describe the various manifestations of SCD, their pathophysiology and their current management. Recent impressive advances in understanding the pathophysiology of pain promise the determination of agents that could replace or minimize the use of opioids. 相似文献
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《Neuropsychopharmacology》2019,85(12):1021-1035
BackgroundPain affects both sensory and emotional aversive responses, often provoking anxiety-related diseases when chronic. However, the neural mechanisms underlying the interactions between anxiety and chronic pain remain unclear.MethodsWe characterized the sensory, emotional, and cognitive consequences of neuropathic pain (chronic constriction injury) in a rat model. Moreover, we determined the role of the locus coeruleus (LC) neurons that project to the basolateral amygdala (BLA) using a DREADD (designer receptor exclusively activated by designer drugs).ResultsChronic constriction injury led to sensorial hypersensitivity in both the short term and long term. Otherwise, long-term pain led to an anxiety-like profile (in the elevated zero maze and open field tests), as well as increased responses to learn aversive situations (in the passive avoidance and fear conditioning tests) and an impairment of nonemotional cognitive tasks (in the novel object recognition and object pattern of separation tests). Chemogenetic blockade of the LC-BLA pathway and intra-BLA or systemic antagonism of beta-adrenergic receptors abolished both long-term pain-induced anxiety and enhanced fear learning. By contrast, chemogenetic activation of this pathway induced anxiety-like behaviors and enhanced the aversive learning and memory index in sham animals, although it had little effect on short- and long-term chronic constriction injury animals. Interestingly, modulation of LC-BLA activity did not modify sensorial perception or episodic memory.ConclusionsOur results indicate that dimensions associated with pain are processed by independent pathways and that there is an overactivation of the LC-BLA pathway when anxiety and chronic pain are comorbid, which involves the activity of beta-adrenergic receptors. 相似文献
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Amanda Leal Rocha Alessandra Figueiredo De Souza Laiz Fernandes Mendes Nunes Nayara Dias de Souza Cunha Clia Regina Moreira Lanza Denise Vieira Travassos Tarcília Aparecida da Silva 《Pediatric dermatology》2019,36(1):e27-e30
Drug‐induced reactions are complications associated with high mortality and significant morbidity. Stevens–Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) are examples of these conditions, which are characterized by skin and mucous lesions. Here, we report a case of a 9‐year‐old girl who presented with blisters associated with an extensive vesicular rash and multiple ulcerations on the lips and oral cavity. A drug‐induced hypersensitivity reaction to antibiotics was suspected, and a diagnosis of TEN was made. The patient was managed with withdrawal of the suspected causative agent, and the oral lesions were treated with low‐level laser therapy (LLLT) and oral hygiene. This case highlights that TEN requires interdisciplinary intervention with dental assistance and follow‐up to improve symptoms, nutrition, systemic condition, and quality of life. 相似文献
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目的调查呼吸专科护士慢性呼吸疾病管理现状,为提高慢性呼吸疾病的管理水平提供参考。方法依据《中国成人慢性呼吸疾病患者护理管理指南》设计调查问卷,对重庆、四川、贵州、陕西、河北、湖北、海南7省市的205名呼吸专科护士进行问卷调查。结果呼吸专科护士慢性呼吸疾病管理总分为(140.72±23.90)分。慢性呼吸疾病管理得分最低的3个条目有评估工具应用、个性化管理计划的实施、慢阻肺随访的次数及指导哮喘患者使用峰流速仪;呼吸专科护士所在单位慢性呼吸疾病管理平台建设均低于50%。结论呼吸专科护士慢性呼吸疾病管理处于中等偏下水平,评估是慢性呼吸疾病管理的薄弱点。需加强呼吸专科护士专业能力培养,规范慢性呼吸疾病全程管理,强化医院平台建设等,提高慢性呼吸疾病的管理水平。 相似文献
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