评估,诊断和治疗赛德纳姆胆病:共识指导方针
Terrence Thomas1, Michael Eyre2,3, Emanuela Ferrarin4
1Department of Paediatrics, Neurology Service, KK Women's and Children's Hospital, Singapore.
Pediatrics
|November 20, 2025
概括
国际专家为评估和管理患有西登汉氏病 (SC) 的儿童制定了共识指南. 这些指南涵盖了诊断,抗生素治疗和免疫治疗,以及为患者和家人提供支持,以改善结果.
科学领域:
- 儿科神经学 儿科神经学
- 运动障碍 运动障碍
- 类风湿病学 类风湿病学
背景情况:
- 赛德纳姆胆发病 (SC) 是急性类风湿性发烧 (ARF) 的一个显著的神经表现.
- 有效的管理需要评估,诊断和治疗的标准指南.
- 当前的做法可能会有所不同,影响患者的治疗结果和家庭的支持.
研究的目的:
- 建立国际共识的指导方针,以全面管理儿科赛德纳姆胆病.
- 为评估,诊断和治疗策略提供基于证据的建议.
- 改善SC儿童的生活质量和学术/社会功能.
主要方法:
- 一个德尔菲过程,涉及27名国际专家 (神经病学家,精神病学家,家长代表).
- 根据最佳证据和专家意见制定88项共识建议.
- 专注于临床症状,相关损伤,病因评估和治疗方式.
主要成果:
- 应该确定SC的关键症状 (胆病,低血压) 和相关的障碍 (行为,运动,认知).
- 建议在第一次呈现时接受抗生素治疗和长期预防.
- 免疫疗法 (皮质类固醇) 适度至严重的SC,用IVIg或血交换进行不充分的恢复.
结论:
- 共识指南标准化了SC管理的方法.
- 建议涉及病因评估,急性和长期治疗以及支持性护理.
- 未来的研究应该专注于改善患者和家人的生活经验和结果.
相关概念视频
Parkinson's Disease: Treatment
953
Neurodegenerative disorders, such as Parkinson's Disease (PD), involve the gradual and irreversible destruction of neurons in particular brain areas. These disorders exhibit standard features like proteinopathies, selective vulnerability of some neurons, and an interaction of intrinsic properties, genetics, and environmental influences in neural injury.
Parkinson's Disease is primarily a result of the loss of dopaminergic neurons in the substantia nigra pars compacta. The cornerstone of...
Parkinson's Disease is primarily a result of the loss of dopaminergic neurons in the substantia nigra pars compacta. The cornerstone of...
953
Parkinson's Disease: Overview
1.7K
Neurodegenerative disorders are progressive diseases that cause irreversible damage and loss to neurons in specific brain areas. Examples of these disorders include Parkinson's disease, Alzheimer's disease, Multiple Sclerosis (MS), and Amyotrophic Lateral Sclerosis (ALS). These disorders share characteristics such as proteinopathies, selective neuronal vulnerability, and a complex interplay between genetic and environmental factors. The primary therapeutic goal for these conditions is...
1.7K
Peripheral Arterial Disease II: Clinical Manifestations and Diagnostic Evaluation
330
Clinical manifestationsPeripheral Arterial Disease (PAD) manifests through a range of symptoms, from the characteristic intermittent claudication to atypical presentations and severe complications in advanced stages. Intermittent claudication, a hallmark symptom of PAD, presents as exercise-induced muscle pain that typically resolves within minutes of rest. This pain is reproducible and stems from inadequate blood flow, leading to the accumulation of lactic acid produced during anaerobic...
330
Myasthenia Gravis: Overview and Treatment
2.7K
Myasthenia gravis is a neuromuscular transmission disorder characterized by weakness and increased fatigability of skeletal muscles. It is an autoimmune disease affecting approximately one in 2000 people, where antibodies against the α1 subunit of nicotinic acetylcholine receptors are produced.
These antibodies interfere with the function of the nicotinic receptors in three ways: by binding to the receptor and disrupting acetylcholine binding; by causing cross-linking of receptors which...
These antibodies interfere with the function of the nicotinic receptors in three ways: by binding to the receptor and disrupting acetylcholine binding; by causing cross-linking of receptors which...
2.7K
Alzheimer's Disease: Treatment
772
Alzheimer's Disease (AD), a neurodegenerative disorder, is pathologically identified by amyloid plaques and neurofibrillary tangles composed of tau protein. AD pharmacotherapy aims to manage cognitive symptoms, delay disease progression, and treat behavioral symptoms. The treatment is primarily symptomatic and palliative, with no definitive disease-modifying therapy available. Cholinesterase inhibitors, including donepezil (Aricept), rivastigmine (Exelon), and galantamine (Razadyne), are...
772
Dysrhythmias V: Evaluating Dysrhythmias
315
Dysrhythmias, also known as arrhythmias, are disturbances in the heart's rhythm that range from benign to life-threatening. A thorough evaluation is crucial for appropriate management and involves a comprehensive medical history, physical examination, and various diagnostic tests.Medical HistorySymptoms: Collect detailed information on palpitations, dizziness, syncope, chest pain, and fatigue. Note their onset, frequency, and triggers.Previous Cardiac Issues: Document any history of heart...
315


