双脑综合征:临床特征,病理生理学和长期结果
Antonio Ruggiero1,2, Palma Maurizi1,2, Alberto Romano1
1Pediatric Oncology Unit, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, 00168 Rome, Italy.
Children (Basel, Switzerland)
|February 27, 2026
概括
脑膜综合征 (DS) 是一种罕见的婴儿疾病,导致婴儿发育不良. 早期诊断和相关质瘤的化疗改善了生存率,但长期影响仍然存在.
科学领域:
- 儿科内分泌学 儿科内分泌学
- 儿科瘤学 儿科瘤学
- 神经瘤学神经瘤学
背景情况:
- 脑膜综合征 (DS) 是一种罕见的儿科疾病,其特点是尽管摄入了足够的热量,但无法壮成长.
- 它主要影响12个月以下的婴儿,在诊断上具有挑战性.
- 拉塞尔首先描述了这种情况,通常与下丘脑 - 脑质结质瘤有关.
研究的目的:
- 综合审查临床表现,病理生理学,诊断和Diencephalic综合征的结果.
- 突出早期识别和多学科管理的重要性.
主要方法:
- 综合文献综述. 这是一个全面的文献综述.
- 检查临床表现,病理生理学,相关病理学,诊断方法和长期结果.
主要成果:
- 在婴儿期,DS会出现白血病,保持身高,过度活跃以及视觉通路的干扰.
- 低度的下丘脑 - 脑质质瘤,特别是飞细胞性星瘤是主要原因.
- 五年后的生存率超过90%,但像视力障碍和内分泌缺陷这样的显著的长期疾病是常见的.
结论:
- 早期识别二脑综合征对于预防不可逆转的并发症至关重要,特别是视力丧失.
- 化疗是潜在质瘤的首选一线治疗方法,改善了生存率并减少了辐射毒性.
- 幸存者需要终身监测和干预后果,如下丘脑肥胖症.
相关概念视频
Cardiomyopathy II: Dilated Cardiomyopathy
667
Dilated cardiomyopathy, or DCM, is a progressive myocardial disorder characterized by ventricular chamber dilation and contractile dysfunction.EtiologyVarious factors can cause DCM, including hypertension and heavy alcohol intake, which contribute to the weakening and enlargement of the heart muscle. Viral infections, such as Coxsackievirus B, adenoviruses, and influenza, can lead to DCM by causing inflammation and damage to heart tissue. Certain chemotherapeutic agents, including daunorubicin,...
667
COPD: Pathogenesis and Clinical Features
2.0K
Chronic obstructive pulmonary disease (COPD) is a group of lung conditions that progressively worsen over time, including chronic bronchitis and emphysema. This cluster of diseases collectively leads to a gradual and irreversible decline in lung function over time.
The primary cause for the onset of COPD is cigarette smoking and exposure to air pollution. These hazardous factors initiate a chain reaction within the lungs, resulting in chronic inflammation, damage to the airways, and a...
The primary cause for the onset of COPD is cigarette smoking and exposure to air pollution. These hazardous factors initiate a chain reaction within the lungs, resulting in chronic inflammation, damage to the airways, and a...
2.0K
Gastroesophageal Reflux Disease II: Clinical Features and Management
1.0K
Gastroesophageal reflux disease, or GERD, is a persistent medical condition that affects many individuals worldwide. Its clinical manifestations can vary greatly, making diagnosis and management challenging for healthcare professionals. The following is a comprehensive overview of the clinical manifestations, assessment, and management strategies for GERD.
Clinical Manifestations
GERD presents itself in a multitude of ways, with symptoms varying from person to person. The hallmark symptoms are...
Clinical Manifestations
GERD presents itself in a multitude of ways, with symptoms varying from person to person. The hallmark symptoms are...
1.0K
Pathophysiology of Heart Failure
4.2K
Heart failure (HF) is a progressive syndrome involving ventricles that leads to inadequate cardiac output. It can be classified based on location and output or ejection fraction. Ejection fraction (EF) is an essential measurement in the diagnosis and surveillance of HF. Reduced EF corresponds to systolic heart failure (HFrEF). However, HF with preserved ejection fraction (HFpEF) is becoming increasingly prevalent. Also known as diastolic HF, this form of HF is related to aging. The...
4.2K
Heart Failure III: Clinical Manifestations
719
Heart failure (HF) manifests primarily as dyspnea, fatigue, and fluid retention, resulting in peripheral and pulmonary edema. Symptoms may vary depending on which ventricle is more affected, left or right.Left-Sided Heart FailureAlso known as left ventricular failure, this condition results from the left ventricle's inability to fill or eject sufficient blood into the systemic circulation. It leads to pulmonary congestion, which occurs when the left ventricle fails to eject blood effectively...
719
Acute Coronary Syndrome II: Pathophysiology and Clinical Manifestations
535
The pathophysiology of Acute Coronary Syndrome [ACD] involves several key processes:The main underlying cause of ACD is atherosclerosis, a chronic inflammatory disease characterized by the buildup of lipid-laden plaques within the coronary arteries.As the atherosclerotic plaque grows in the coronary artery, it may become unstable due to the formation of a lipid-rich core and a thin fibrous cap. Inflammatory cells within the plaque, such as macrophages, secrete enzymes that degrade the...
535


