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监测波兰家族性高胆固醇血症儿童降脂治疗的效果。

Monitoring the Effects of Hypolipidemic Treatment in Children with Familial Hypercholesterolemia in Poland.

作者信息

Hennig Matylda, Brandt-Varma Agnieszka, Wołoszyn-Durkiewicz Anna, Bautembach-Minkowska Joanna, Buraczewska Marta, Świętoń Dominik, Mickiewicz Agnieszka, Rynkiewicz Andrzej, Gruchała Marcin, Limon Janusz, Wasąg Bartosz, Chmara Magdalena, Walczak Mieczysław, Myśliwiec Małgorzata

机构信息

The Department of Paediatrics, Diabetology and Endocrinology, Faculty of Medicine, Medical University of Gdansk, 80-210 Gdansk, Poland.

Surrey and Sussex Healthcare NHS Trust, Canada Avenue, Redhill RH15RH, UK.

出版信息

Life (Basel). 2020 Nov 4;10(11):270. doi: 10.3390/life10110270.

DOI:10.3390/life10110270
PMID:33158089
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7694222/
Abstract

Familial hypercholesterolemia (FH) is the most common monogenic autosomal dominant disorder. FH results in an increased cardiovascular mortality rate. However, cardiovascular risk control factors enable the avoidance of approximately 80% of strokes and cardiovascular diseases. Therefore, early detection and implementation of lipid-lowering treatment is essential. In the present study, 57 pediatric patients aged 9.57 ± 3.26 years with FH were enrolled in the study. Researchers checked the lipid profile and performed the ultrasound imaging including intima-media thickness (IMT) measurement and echo (e)-tracking in the study group. Patients were treated with a low-cholesterol diet solely or along with pharmacological treatment with statins. Subsequently, patients were monitored for 12 months. The positive results of dietary treatment were observed in 40 patients. The efficacy of 12 months of nutritional therapy along with pharmacological treatment was reported in 27 patients. We observed a significant decrease in the carotid beta index stiffness and an insignificant decrease in the IMT in the group of patients treated with statins. The obtained data show that statin therapy in children with FH allow for the reduction of the degree of atherosclerotic vessel changes.

摘要

家族性高胆固醇血症(FH)是最常见的单基因常染色体显性疾病。FH会导致心血管死亡率增加。然而,心血管风险控制因素能够避免约80%的中风和心血管疾病。因此,早期检测和实施降脂治疗至关重要。在本研究中,纳入了57名年龄为9.57±3.26岁的FH儿科患者。研究人员检查了研究组的血脂谱,并进行了超声成像,包括内膜中层厚度(IMT)测量和回声(e)跟踪。患者单独接受低胆固醇饮食治疗或与他汀类药物的药物治疗联合使用。随后,对患者进行了12个月的监测。40名患者观察到饮食治疗的积极效果。27名患者报告了12个月营养治疗与药物治疗联合使用的疗效。我们观察到,在接受他汀类药物治疗的患者组中,颈动脉β指数硬度显著降低,IMT略有降低。获得的数据表明,FH儿童使用他汀类药物治疗可降低动脉粥样硬化血管病变的程度。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/903e/7694222/fe04b9e9ce4d/life-10-00270-g008.jpg
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Curr Atheroscler Rep. 2020 Sep 1;22(11):64. doi: 10.1007/s11883-020-00884-2.
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Evolocumab in Pediatric Heterozygous Familial Hypercholesterolemia.依洛尤单抗在儿科杂合子家族性高胆固醇血症中的应用。
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Arterial stiffness improvement after adding on PCSK9 inhibitors or ezetimibe to high-intensity statins in patients with familial hypercholesterolemia: A Two-Lipid Center Real-World Experience.
家族性高胆固醇血症患者在高强度他汀类药物基础上加用 PCSK9 抑制剂或依折麦布对动脉僵硬度的改善作用:双脂质中心真实世界经验。
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