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Porphyria is a group of at least eight diseases caused by abnormalities in the chemical steps that lead to haeme production. The different types of porphyria show different signs and symptoms and can be strongly influenced by environmental factors. Mutations of the uroporphyrinogen decarboxylase (UROD) gene have been shown to be causative for porphyria cutanea tarda (PCT) in humans. Porphyria is a rare disorder in livestock. Although disorders of haeme biosynthesis have been described in cattle, pigs, sheep and cats, PCT has only been reported in pigs. We observed typical signs of porphyria (photosensitivity and porphyrinuria) in a flock of German Blackface sheep and postulated that the porphyria could be caused by a mutation in the UROD gene. To investigate this, we cloned and sequenced the ovine UROD gene. We identified a single point mutation (C --> T) in UROD which leads to an amino acid substitution at Leu 131 Pro, which is located within the active cleft site of the UROD protein. 相似文献
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《Cytokine & growth factor reviews》2014,25(4):443-451
This review focuses on treatment targets for the most common inflammatory skin diseases, eczema and psoriasis with an emphasis on cytokines expressed in the uppermost layer of the skin which is easily accessible for diagnostic and therapeutic approaches. Recently, a significant body of research has highlighted the influence of the skin barrier and the patients’ microbiome on skin inflammatory responses and we will comment on their impact on mediator regulation. Itch is a prominent dermatology symptom which is influenced by cytokines and can via itch–scratch cycle impact on the skin barrier and mediator expression associated with damage. Taking the contribution of pruritus and superficial skin damage into account, we address cytokines as targets for stratified treatment approaches in subgroups of eczema and psoriasis. 相似文献
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目的:探究尿毒症皮肤瘙瘁患者采取腹膜透析与血液透析治疗的临床效果,并为该病的临床治疗提供经验积累。方法:选取我院肾内科于2005年1月-2012年12月收治的52例尿毒症皮肤瘙痒患者,利用随机数字表法进行分组,分别设为研究组和对照组。其中对照组实施血液透析治疗,而研究组开展腹膜透析治疗。记录两组在治疗前和治疗后第8周末血生化客观指标及皮肤瘙痒程度,并做好对比。结果:两组在治疗前的皮肤瘙痒评分、β2-MG、PTH、p3,BUN、SCr值差异无统计学意义(P〉0.05);治疗后,研究组皮肤瘙瘁评分为(3.4±0.8)分,对照组为(5.8±0.9)分,差异有统计学意义(P〈0.05)。治疗后,研究组β2-MG、PTH及Ps.均低于对照组,差异有统计学意义(P〈0.05)。结论:尿毒症皮肤瘙痒患者的主要致敏因子为大中分子,采取腹膜透析能够有效清除大分子物质,进而达到瘙痒程度的有效改善。 相似文献
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Mariann Dinya E. Szkely K. Szentmihlyi Gy. Tasndi A. Blzovics 《Journal of trace elements in medicine and biology》2005,19(2-3):217-220
Porphyria cutanea tarda (PCT) is a disorder of hem biosynthesis resulting from a decreased activity of the uroporphyrinogen decarboxylase enzyme. Hem precursors are accumulated in the blood, liver and skin. Inherited and acquired factors also contribute to the pathogenesis of PCT. Hem precursors and porphyrins are excreted with urine and faeces. Whole blood of 8 PCT patients and 6 volunteers of Caucasian origin were analysed. In addition to routine laboratory measurements, 19 elements (Al, B, Ba, Ca, Cd, Co, Cu, Fe, K, Li, Mg, Mn, Mo, Na, Ni, P, S, V, Zn) were determined by means of inductively coupled plasma optical emission spectrometry (ICP-OES). Mg, P and S concentrations in whole blood were decreased significantly (p<0.05), whereas Ba was increased in PCT patients compared to controls. Metabolic alterations are reflected in the correlation of parameters. Positive correlations were found between the element pairs of Zn-Al, Zn-Mg, Zn-Mn, B-S, Fe-Mg, K-P, Mg-Mn for PCT patients, whereas in the control group Al-Mn, Ca-Cu, Ca-Na, Cu-Mg, Fe-K, Mg-Na, Zn-P showed positive correlations. 相似文献
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目的:研究盐酸非索非那定片联合胸腺肽对慢性荨麻疹患者风团及瘙痒情况的影响分析。方法:选择2012年7月至2013年7月在我院接受治疗的慢性荨麻疹患者108例作为研究对象。以数字法随机分成观察组(54例)和对照组(54例)。对照组患者单纯服用盐酸非索非那定片,观察组在此基础上加用胸腺肽。对比两组疗效,风团和瘙痒症状总积分情况及不良反应。结果:观察组的总有效率为72.22%(39/54),显著高于对照组的53.70%(29/54),差异有统计学意义(P0.05)。两组治疗后的症状总积分均显著下降,但观察组治疗2,4周后的症状总积分均显著小于对照组,差异均有统计学意义(P0.05)。观察组的总不良反应率为9.26%(5/54),与对照组的22.22%(12/54)相比,差异无统计学意义(P0.05)。结论:盐酸非索非那定与胸腺肽联合治疗慢性荨麻疹疗效较好,值得推荐。 相似文献
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Ken-ichi Kusakabe Yasuyoshi Iso Yukio Tada Masahiro Sakagami Yasuhide Morioka Nobuo Chomei Satomi Shinonome Keiko Kawamoto Hideyuki Takenaka Kiyoshi Yasui Hiroshi Hamana Kohji Hanasaki 《Bioorganic & medicinal chemistry》2013,21(11):3154-3163
The CB2 receptor has emerged as a potential target for the treatment of pruritus as well as pain without CB1-mediated side effects. We previously identified 2-pyridone derivatives 1 and 2 as potent CB2 agonists; however, this series of compounds was found to have unacceptable pharmacokinetic profiles with no significant effect in vivo. To improve these profiles, we performed further structural optimization of 1 and 2, which led to the discovery of bicyclic 2-pyridone 18e with improved CB2 affinity and selectivity over CB1. In a mouse pruritus model, 18e inhibited compound 48/80 induced scratching behavior at a dose of 100 mg/kg. In addition, the docking model of 18e with an active-state CB2 homology model indicated the structural basis of its high affinity and selectivity over CB1. 相似文献
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Peter G. Atanassoff Sorin J. Brull Junming Zhang Kenneth Greenquist David G. Silverman Robert H. Lamotte 《Somatosensory & motor research》2013,30(4):291-298
Pain reduces itch - a commonly known effect of scratching the skin. Experimentally produced itch from histamine is sometimes accompanied by secondary sensations of pain. The present study investigated the effects of eliminating this pain, by means of a local anesthetic, on the itch and the enhanced mechanically evoked itch and pain that occur after an intradermal injection of histamine. In ten human subjects, the volar forearm was injected with either 20 mul of 2% chloroprocaine (experimental arm), or 20 mul of saline (control arm). Histamine 10 mul was injected into each bleb, and the resulting magnitude of itch estimated. The borders of three cutaneous areas were mapped within which mechanical stimulation of the skin surrounding the bleb elicited abnormal sensations (dysesthesiae): alloknesis, defined as itch evoked by innocuous stroking, and hyperalgesia and hyperknesis, characterized, respectively, by enhanced pain and enhanced itch evoked by pricking the skin with a fine tipped filament. The magnitude and duration of itch were significantly greater and the areas of dysesthesia significantly larger for the experimental than for the control arm. It is hypothesized that there exist two classes of histamine-sensitive primary afferent neurons. One class is 'pruritic', and mediates itch whereas the other is 'antipruritic', and evokes a centrally mediated reduction in histamine-evoked itch and dysesthesiae. It is further suggested that the anesthetic blocked the discharges of the antipruritic afferents, preventing the central inhibition from occurring and thereby unmasking the effects of the pruritic afferents. 相似文献