儿科肝细胞腺瘤:从对相关文献的系统审查中得出的教训
Isabelle Scheers1, Roberto Tambucci2, Catherine De Magnee2
1Pediatric Gastroenterology and Hepatology, Cliniques universitaires Saint-Luc. Laboratoire de Pédiatrie, Institut de Recherche Expérimentale et Clinique, Université catholique de Louvain, Brussels, Belgium.
JHEP reports : innovation in hepatology
|May 3, 2024
概括
儿科肝细胞腺瘤 (HCAs) 经常与诸如糖原储存疾病之类的易感性疾病发生. 这些良性肝脏瘤可以出血或转变,需要根据潜在的疾病和病变特征进行治疗.
科学领域:
- 肝病学 肝病学是一种肝病学.
- 儿科瘤学 儿科瘤学
- 胃肠病学 胃肠病学
背景情况:
- 肝细胞腺瘤 (HCAs) 是一种罕见的良性肝脏瘤,与成人相比,在儿童中具有明显的特征.
- 了解易感因素和并发症率对于有效管理儿科HCA至关重要.
研究的目的:
- 系统地描述儿科HCAs,包括其过程,并发症和管理策略.
- 调查易感性疾病和遗传因素对儿童HCA发育和结果的影响.
主要方法:
- 对儿科HCAs的病史,临床症状,成像,组织病理学和遗传学的系统文献综述.
- 包括316名被诊断患有HCAs的儿童.
主要成果:
- 主要在11.5岁左右的女孩中 (59.3%) 诊断出HCAs.
- 83.6%的病例与先导性疾病有关,最常见的是糖原储存疾病,移植系统突变和MODY3.3.
- 并发症包括出血 (24.7%) 和转变 (14.8%),转变与移植系统突变和β-catenin突变有关,以及因激素暴露而引起的出血和较大的病变.
结论:
- 儿科HCA通常与潜在的疾病有关,影响其行为和管理.
- 管理策略包括解决易受影响的因素,手术切除复杂的病变,以及为腺瘤移植肝脏.
- 了解遗传贡献的进展是改善HCA病原体洞察力,患者监测和治疗的关键.
相关概念视频
01:23Pharmacokinetics in Pediatric Patients: Overview and Drug Absorption
982
Understanding the physiological differences in the pediatric population is crucial for effective pharmacotherapy. Neonates, infants, and children exhibit significant variations in gastric pH, gastric emptying time, intestinal transit time, and biliary function. These variations profoundly affect oral drug absorption, necessitating a nuanced approach to pediatric dosing.Neonates present with a unique physiological profile, having a gastric pH greater than 4 and faster and more irregular gastric...
982
01:24Pharmacokinetics in Pediatric Patients: Drug Metabolism
417
In pediatric care, understanding the nuances of hepatic drug metabolism is crucial, as it significantly differs from that of adults. This divergence is primarily due to the developmental stage of drug-metabolizing enzymes, which affects how medications are processed in the body. In neonates, for instance, the activity of Phase I enzymes—critical for the initial breakdown of drugs—is markedly reduced, functioning at just 20–40% of the levels seen in adults. This reduction poses...
417
01:22Effect of Hepatic Disease on Pharmacokinetics: Pathophysiologic Assessment and Liver Function Test
275
In clinical practice, the direct measurement of hepatic blood flow to evaluate liver function presents significant challenges due to the intricate and specialized nature of the necessary techniques. Consequently, healthcare professionals often rely on empirical estimates derived from thorough patient examinations and liver function tests to gauge liver health. Among the tools at their disposal, the Child–Pugh and MELD scoring systems stand out for their ability to categorize and assess...
275