更新于：2024-05-01
Congenital Ichthyosiform Erythroderma
先天性鳞癣样红皮病
更新于：2024-05-01
基本信息
别名 Alligator skin、Congenital Ichthyosiform Erythroderma、Congenital Ichthyosiform Erythrodermas + [31] |
简介 Designation for several severe forms of ichthyosis, present at birth, that are characterized by hyperkeratotic scaling. Infants may be born encased in a collodion membrane which begins shedding within 24 hours. This is followed in about two weeks by persistent generalized scaling. The forms include bullous (HYPERKERATOSIS, EPIDERMOLYTIC), non-bullous (ICHTHYOSIS, LAMELLAR), wet type, and dry type. |
关联
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原研机构 |
在研适应症 |
非在研适应症 |
最高研发阶段 |
首次获批国家/地区 |
首次获批日期 |
靶点 |
作用机制 |
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 |
首次获批国家/地区 |
首次获批日期 |
靶点 |
作用机制 |
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 |
首次获批国家/地区 |
首次获批日期 |
Non-interventional Study of Patients With Netherton Syndrome to Characterise the Natural History of Disease
Phase 2 study evaluating the efficacy of injectable gentamicin in hereditary ichthyosis - GENTIC - RC31/22-0320
A Randomized, Double-Blind, Vehicle-Controlled, First-in-Human Safety, Tolerability and Proof-of-Concept Study of Topical ATR12-351 in Adults With Netherton Syndrome
100 项与 先天性鳞癣样红皮病 相关的临床结果
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100 项与 先天性鳞癣样红皮病 相关的转化医学
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2024-03-01Infection prevention in practice
Epidemiological differences, clinical aspects, and short-term prognosis of patients with healthcare-associated and community-acquired infective endocarditis.
Article
作者: Juliana Barros Becker ; Valdir Ambrósio Moisés ; María Dolores Guerra-Martín ; Dulce Aparecida Barbosa
Background:
The prevalence of healthcare-associated infective endocarditis in Brazil is poorly known.
Aim:
To analyze the epidemiological, clinical and microbiological characteristics, and the prognosis of healthcare-associated infective endocarditis (HAIE) compared with community-acquired infective endocarditis (CIE) and identify the associated factors with hospital mortality.
Method:
A historical cohort study was carried out, with a data collection period from January 2009 to December 2019 at the Federal University of São Paulo. Data were collected from medical records of patients with infective endocarditis (IE) hospitalized during the study period. Patients were classified into three groups: CIE, non-nosocomial HAIE (NN-HAIE) and nosocomial HAIE (NHAIE).
Results:
A total of 204 patients with IE were included; of these, 127 (62.3%) were cases of HAIE, of which 83 (40.7%) were NN-HAIE and 44 (21.6%) were NHAIE. Staphylococcus spp. Were the main causative agents, especially in HAIE groups (P<0.001). Streptococcus spp. were more prevalent in the CIE group (P<0.001). In-hospital mortality was 44.6%, with no differences between groups. Independent risk factors for in-hospital mortality were age ≥ 60 years (odds ratio (OR): 6.742), septic shock (OR 5.264), stroke (OR 3.576), heart failure (OR 7.296), and Intensive Care Unit admission (OR 7.768).
Conclusion:
HAIE accounted for most cases in this cohort, with a higher prevalence of non-nosocomial infections. Staphylococcus spp. were the main causative agents. Hospital mortality was high, 44.6%, with no difference between groups.
2024-02-22Journal of neural engineering
Customizing the human-avatar mapping based on EEG error related potentials during avatar-based interaction.
Article
作者: Fumiaki Iwane ; Thibault Porssut ; Olaf Blanke ; Ricardo Chavarriaga ; Jose Del R Millan ; Bruno Herbelin ; Ronan Boulic
OBJECTIVE:
A key challenge of virtual reality (VR) applications is to maintain a reliable human-avatar mapping. Users may lose the sense of controlling (sense of agency), owning (sense of body ownership), or being located (sense of self-location) inside the virtual body when they perceive erroneous interaction, i.e. Break-in-embodiment (BiE). However, the way to detect such an inadequate event is currently limited to questionnaires or spontaneous reports from users. The ability to implicitly detect BiE in real-time enables us to adjust human-avatar mapping without interruption.
Approach. We propose and empirically demonstrate a novel Brain Computer Interface (BCI) approach that monitors the occurrence of BiE based on the users' brain oscillatory activity in real-time to adjust the human-avatar mapping in VR. We collected EEG data of 37 participants while they performed reaching movements with their avatar with different magnitude of distortion. 
Main results. Our BCI approach seamlessly predicts occurrence of BiE in varying magnitude of erroneous interaction. The mapping has been customized by BCI-reinforcement learning (RL) closed-loop system to prevent BiE from occurring. Furthermore, a non-personalized BCI decoder generalizes to new users, enabling "Plug-and-Play" ErrP-based non-invasive BCI. The proposed VR system allows customization of human-avatar mapping without personalized BCI decoders or spontaneous reports. 
Significance. We anticipate that our newly developed VR-BCI can be useful to maintain an engaging avatar-based interaction and a compelling immersive experience while detecting when users notice a problem and seamlessly correcting it.
2024-02-21RMD open
Immunogenicity and influence on disease activity of recombinant zoster vaccine in patients with rheumatoid arthritis treated with DMARDs.
Article
作者: Shotaro Kojima ; Taro Iwamoto ; Yoshihisa Kobayashi ; Manami Kato ; Fumiyoshi Takizawa ; Tomoaki Ida ; Junya Suzuki ; Yosuke Toda ; Kazusa Miyachi ; Arifumi Iwata ; Shunsuke Furuta ; Kei Ikeda ; Hiroshi Nakajima
OBJECTIVES:
This study aimed to determine the immunogenicity and the influence on disease activity of an adjuvanted recombinant varicella-zoster virus (VZV) subunit vaccine (RZV) in patients with rheumatoid arthritis (RA) treated with disease-modifying antirheumatic drugs (DMARDs).
METHODS:
This prospective longitudinal study enrolled 53 patients with RA (aged ≥50 years) treated with DMARDs (conventional synthetic (cs)DMARDs 20, biological (b)DMARDs 23 and targeted synthetic (ts)DMARDs 10) and 10 control individuals. The participants received two intramuscular RZV 2 months apart. VZV-specific CD4+ T cell responses (cell-mediated immunity; CMI) and IgG antibody responses (humoral immunity; HI) were assessed at 0 and 3 months after the first RZV administration using flow cytometry and enzyme immunoassay, respectively. Disease activity (Disease Activity Score 28-C reactive protein and Clinical Disease Activity Index), flares and adverse events were monitored for 6 months after the first vaccination.
RESULTS:
VZV-specific CMI and HI significantly increased in the three DMARDs-treated patients with RA after RZV administration compared with the corresponding prevaccination values (p<0.001-0.014), and the magnitudes and fold-increases of those responses were not significantly different among the three DMARDs-treated patients with RA. Furthermore, the vaccine response rates of CMI and HI were not significantly different between csDMARDs-treated patients and b-DMARDs or ts-DMARDs-treated patients. Meanwhile, no significant increases in disease activity indices or adverse events were observed in these patients during the 6-month follow-up period after the first vaccination. RZV-induced RA flares occurred in two patients (3.8%) but were mild and controllable.
CONCLUSION:
RZV is robustly immunogenic and has a clinically acceptable safety profile in elderly patients with RA receiving DMARDs.
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