更新于：2024-11-01

Hemiconvulsion-Hemiplegia-Epilepsy Syndrome

偏侧惊厥偏瘫癫痫综合征

更新于：2024-11-01

基本信息

别名

H-H-E syndrome、HHE syndrome、Hemiconvulsion-hemiplegia-epilepsy syndrome

+ [10]

简介

A rare acute encephalopathy with inflammation-mediated status epilepticus characterized by infancy-onset of refractory unilateral, mainly clonic status epilepticus during or shortly after a febrile episode without evidence of central nervous system infection, followed by permanent or transient hemiplegia with a minimum duration of one week. The majority of children develop pharmaco-resistant epilepsy a few months later. Brain imaging shows edematous swelling of the affected hemisphere at the time of the initial status, followed by hemiatrophy that does not correlate with any vascular territory.

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100 项与偏侧惊厥偏瘫癫痫综合征相关的临床结果

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100 项与偏侧惊厥偏瘫癫痫综合征相关的转化医学

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0 项与偏侧惊厥偏瘫癫痫综合征相关的专利（医药）

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129

项与偏侧惊厥偏瘫癫痫综合征相关的文献（医药）

2024-07-01·Journal of Dairy Science

Association between a genetic index for digital dermatitis resistance and the presence of digital dermatitis, heel horn erosion, and interdigital hyperplasia in Holstein cows

Article

作者: Banos, G ; Bedford, C ; Li, B ; Oikonomou, G ; Psifidi, A ; Griffiths, B E ; Anagnostopoulos, A ; Winters, M ; Barden, M ; Coffey, M

Digital dermatitis (DD) is a polybacterial disease endemic to most UK dairy farms. It poses a major financial and welfare threat and is characterized by high incidence and recurrence rates. We aimed to investigate the association between the UK EBV for resistance to digital dermatitis, the digital dermatitis index (DDI), and the frequency of DD, heel horn erosion (HHE), and interdigital hyperplasia (IH) in a population of Holstein dairy cows. We enrolled and genotyped 2,352 cows from 4 farms in a prospective cohort study. Foot lesion records were recorded by veterinary surgeons for each animal at 4 time points during a production cycle, starting at approximately 2 mo before calving and ending in late lactation. Importantly, these records were not used in the calculation of the DDI. Lesion records were matched to the animal's own DDI (n = 2,101) and their sire's DDI (n = 1,812). Digital dermatitis index values in our study population ranged from -1.41 to +1.2 and were transformed to represent distance from the mean expressed in SD. The relationship between the DDI and the presence of DD was investigated using a logistic regression model, with farm, parity, and a farm-parity interaction fitted as covariates. A multivariable logistic regression model was fitted to evaluate the relationship between HHE and DDI with farm fitted as a covariate. Finally, a univariable logistic regression model with DDI as explanatory variable was used to investigate the relationship between IH and DDI. The odds ratio of an animal being affected by DD was 0.69 for 1 SD increase in the animal's DDI (95% CI = 0.63-0.76). The odds of HHE and IH were 0.69 (95% CI = 0.62-0.76) and 0.58 (95% CI = 0.49-0.68) respectively for 1 SD increase in DDI. The adjusted probability of DD was 32% (95% CI = 27-36%) for cows with mean DDI value of 0, while it was 24% (95% CI = 20-29%) in cows with a DDI value of +1. Sire DDI breeding values were standardized in the same way and then binned into terciles creating an ordinal variable representing bulls of high, medium, and low genetic merit for DD resistance. The daughters of low genetic merit bulls were at 2.05 (95% CI = 1.60-2.64), 1.96 (95% CI = 1.53-2.50), and 2.85 (95% CI = 1.64-5.16) times greater odds of being affected by DD, HHE, and IH, respectively, compared with the daughters of high genetic merit bulls. The results of this study highlight the potential of digital dermatitis genetic indexes to aid herd management of DD, and suggest that breeding for resistance to DD, alongside environmental and management control practices, could reduce the prevalence of the disease.

2024-04-01·Contact Dermatitis

Immunohistochemical differentiation of keratins and involucrin between palmar psoriasis, chronic hand eczema and hyperkeratotic hand eczema

Article

作者: Seung, Na Reu ; Park, Eun Joo ; Kim, Kwang Ho ; Jang, Ye Ji ; Jung, Da Yeon ; Baek, Eun Joo

AbstractBackgroundCommon hyperkeratotic palmar skin lesions include chronic hand eczema (CHE), hyperkeratotic hand eczema (HHE), palmar psoriasis (PP). However, clinically differentiating these disorders is often challenging.ObjectivesTo compare the expressions of keratin (K) 5, K9, K14 and involucrin in palmar hyperkeratotic lesions (HHE, CHE and PP).Materials and MethodsImmunohistochemical staining was performed on skin biopsy specimens obtained from the palms of patients clinically diagnosed with CHE, HHE and PP (n = 21, 24 and 18, respectively).ResultsK5 and K14 expression levels were higher in the spinous and granular layers of PP and HHE compared to CHE. Involucrin was expressed in the basal layer of PP and HHE but not in CHE. K9 expression was decreased in PP and HHE compared to CHE.ConclusionKeratin and involucrin expression in the epidermis are markers of keratinocyte differentiation. Expression levels of keratin and involucrin were similar between the HHE and PP groups, suggesting that HHE shares pathogenesis with PP rather than CHE.

2023-05-18·Nanomaterials (Basel, Switzerland)

Efficient [Fe-Imidazole@SiO₂] Nanohybrids for Catalytic H₂ Production from Formic Acid.

Article

作者: Gkatziouras, Christos ; Louloudi, Maria ; Solakidou, Maria

Three imidazole-based hybrid materials, coded as IGOPS, IPS and impyridine@SiO₂ nanohybrids, were prepared via the covalent immobilization of N-ligands onto a mesoporous nano-SiO₂ matrix for H₂ generation from formic acid (FA). BET and HRTEM demonstrated that the immobilization of the imidazole derivative onto SiO₂ has a significant effect on the SSA, average pore volume, and particle size distribution. In the context of FA dehydrogenation, their catalytic activity (TONs, TOFs), stability, and reusability were assessed. Additionally, the homologous homogeneous counterparts were evaluated for comparison purposes. Mapping the redox potential of solution E_h vs. SHE revealed that poly-phosphine PP₃ plays an essential role in FA dehydrogenation. On the basis of performance and stability, [Fe²⁺/IGOPS/PP₃] demonstrated superior activity compared to other heterogeneous catalysts, producing 9.82 L of gases (VH₂ + CO₂) with TONs = 31,778, albeit with low recyclability. In contrast, [Fe²⁺/IPS/PP₃] showed the highest stability, retaining considerable performance after three consecutive uses. With VH₂ + CO₂ = 7.8 L, [Fe²⁺/impyridine@SiO₂/PP₃] activity decreased, and it was no longer recyclable. However, the homogeneous equivalent of [Fe²⁺/impyridine/PP₃] was completely inactive. Raman, FT/IR, and UV/Vis spectroscopy demonstrated that the reduced recyclability of [Fe²⁺/IGOPS/PP₃] and [Fe²⁺/impyridine@SiO₂/PP₃] nanohybrids is due to the reductive cleavage of their C-O-C bonds during catalysis. An alternative grafting procedure is proposed, applying here to the grafting of IPS, resulting in its higher stability. The accumulation of water derived from substrate's feeding causes the inhibition of catalysis. In the case of [Fe²⁺-imidazole@SiO₂] nanohybrids, simple washing and drying result in their re-activation, overcoming the water inhibition. Thus, the low-cost imidazole-based nanohybrids IGOPS and IPS are capable of forming [Fe²⁺/IGOPS/PP₃] and [Fe²⁺/IPS/PP₃] heterogeneous catalytic systems with high stability and performance for FA dehydrogenation.