更新于：2024-11-01

Hydrocephalus, Endocardial Fibroelastosis, and Cataracts

脑积水，心内膜纤维弹性增生和白内障

更新于：2024-11-01

基本信息

别名

Communicating hydrocephalus, endocardial fibroelastosis (EFE) and congenital cataracts、HEC (hydrocephalus, endocardial fibroelastosis, cataract) syndrome、HEC SYNDROME

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简介

Syndrome that is characterised by communicating hydrocephalus, endocardial fibroelastosis and congenital cataracts. It has been described in two children, both of whom died a few months after birth (the first as a result of a respiratory infection and the second due to cardiac complications). The aetiology of the syndrome is unknown but a viral or genetic origin has been proposed.

关联

项与脑积水，心内膜纤维弹性增生和白内障相关的药物

Fosaprepitant Dimeglumine

福沙匹坦双葡甲胺

靶点

NK1R

作用机制

NK1R 拮抗剂

在研机构

原研机构

在研适应症

非在研适应症

最高研发阶段批准上市

首次获批国家/地区

欧盟 [+3]

首次获批日期2008-01-11

项与脑积水，心内膜纤维弹性增生和白内障相关的临床试验

CTR20171385 / Completed临床3期

在HEC患者中进行福沙匹坦与福沙匹坦（意美）预防CINV的有效性及安全性的随机、拟双盲、阳性对照研究

在高致吐化疗患者中比较注射用福沙匹坦双葡甲胺与注射用福沙匹坦双葡甲胺（意美®）预防化疗所致的恶心、呕吐（CINV）的有效性及安全性。

开始日期2018-03-30

申办/合作机构

杭州九源基因工程有限公司

CTR20150598 / Completed临床3期

对比福沙匹坦双葡甲胺与阿瑞匹坦在预防HEC所致恶心、呕吐中的有效性及安全性的随机、双盲、阳性对照研究

评价注射用福沙匹坦双葡甲胺预防高致吐性化疗引起的恶心和呕吐（CINV）的有效性和安全性。

开始日期2015-02-06

申办/合作机构

江苏豪森医药研究院有限公司

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100 项与脑积水，心内膜纤维弹性增生和白内障相关的临床结果

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100 项与脑积水，心内膜纤维弹性增生和白内障相关的转化医学

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0 项与脑积水，心内膜纤维弹性增生和白内障相关的专利（医药）

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项与脑积水，心内膜纤维弹性增生和白内障相关的文献（医药）

2024-10-01·Journal of NeuroInterventional Surgery

Safety and efficacy of tailored antiplatelet therapy using prasugrel or ticagrelor based on clopidogrel responsiveness in endovascular treatment for intracranial aneurysms: a meta-analysis

Article

作者: Cho, Young Dae ; Jang, Kyoung Min ; Choi, Hyunho ; Jang, Ju Sung

BackgroundClopidogrel (CPG)-based dual antiplatelet therapy (DAPT) in combination with aspirin has been widely used before endovascular procedures for intracranial aneurysms to prevent procedural thromboembolic complication (TEC). However, the main drawback of CPG is the high proportion of hyporesponders. This study sought to investigate the usefulness of tailored DAPT using novel P2Y12 inhibitors (prasugrel or ticagrelor, (PSG/TCG)) guided by a platelet reactivity test (PRT), compared with CPG-based conventional DAPT.MethodData were extracted from PubMed, Embase, and Cochrane Central Register of Controlled Trials by two independent reviewers. A random effects model was used to investigate the procedural TEC and hemorrhagic complications (HEC) of the tailored DAPT and conventional therapy by risk ratios (RR) and 95% confidence intervals (95% CI). Additionally, we performed subgroup analyses to directly compare prasugrel/ticagrelor with CPG.ResultsSix studies comprising 2557 patients were included in the analysis. Compared with conventional non-tailored therapy, PRT-guided tailored DAPT with PSG/TCG was associated with a lower risk of TEC (RR 0.40, 95% CI 0.22 to 0.74, P=0.004) without increasing HEC rates. The subgroup analysis showed that the switch to PSG/TCG in CPG hyporesponders was related to a lower incidence of TEC (RR 0.46, 95% CI 0.23 to 0.95, P=0.03) without a difference in HEC, compared with maintenance of CPG in CPG responders.ConclusionEvidence from this analysis supports PRT-guided tailored DAPT (using PSG/TCG) as a better choice for preparation towards endovascular procedures to treat aneurysms. Furthermore, it suggests that PSG/TCG is not limited to the role of a substitute for CPG but may be a first-line agent for DAPT.

2024-06-02·Expert Opinion on Drug Metabolism & Toxicology

Pharmacokinetics, pharmacodynamics, safety, and tolerability of dopamine-receptor antagonists for the prevention of chemotherapy-induced nausea and vomiting

Review

作者: Nguyen, Carolyn ; Orhan, Adile ; Herrstedt, Jørn ; Chan, Alexandre

INTRODUCTIONDopamine (D)_2,3-receptor antagonists (RAs) were the first antiemetics used in the prophylaxis of chemotherapy-induced nausea and vomiting (CINV).AREAS COVEREDEight D_2,3-RAs, amisulpride, domperidone, droperidol, haloperidol, metoclopramide, metopimazine, olanzapine and prochlorperazine are reviewed focusing on pharmacokinetics, pharmacodynamics, antiemetic effect and side effects.EXPERT OPINIONSince the introduction of D_2,3-RAs, antiemetics such as corticosteroids, 5-hydroxytryptamine (5-HT)₃-RAs and neurokinin (NK)₁-RAs have been developed. The classical D_2,3-RAs are recommended in the prophylaxis of CINV from low emetic risk chemotherapy, but not as a fixed component of an antiemetic regimen for moderately or highly (HEC) emetic risk chemotherapy. D_2,3-RAs are also used in patients with breakthrough nausea and vomiting. It should be emphasized, that most of these drugs are not selective for dopamine receptors.The multi-receptor targeting agent, olanzapine, is recommended in the prophylaxis of HEC-induced CINV as part of a four-drug antiemetic regimen, including a 5-HT₃-RA, dexamethasone and a NK₁-RA. Olanzapine is the most effective agent to prevent chemotherapy-induced nausea.Side effects differ among various D_2,3-RAs. Metopimazine and domperidone possess a low risk of extrapyramidal side effects. Domperidone and metoclopramide are prokinetics, whereas metopimazine delays gastric emptying and haloperidol does not influence gastric motility. Many D_2,3-RAs increase the risk of prolonged QTc interval; other side effects include sedation and orthostatic hypotension.

2023-06-01·Clinical Pediatrics

Antenatal Hyperechogenic Colon and Cystinuria

Article

作者: Şeker, Erdal ; Özçakar, Zeynep Birsin ; Yalçınkaya, Fatoş ; Mekik Akar, Ece ; Koç, Acar ; Çakar, Nilgün

A male newborn was investigated for history of antenatal hyperechogenic colon (HEC) detected at 32 weeks of gestation. In the first week of life, urinary ultrasonography showed nephrolithiasis. Urinary amino acid analysis expressed increased excretion of dibasic amino acids, and high urinary cystine levels were detected in both spot and 24-hour urine specimens. He was diagnosed as cystinuria, and genetic analysis of the patient revealed a heterozygous mutation in SLC7A9 gene. Antenatal presentation of cystinuria with HEC is rare and reported to be associated with a more severe disease course.