儿童急性荨麻疹的心电图观察

目的 了解儿童急性荨麻疹对心脏的影响。方法 对急性荨麻疹患儿593例行常规心电图检查。结果 33例(5．6％)患儿出现心电图异常改变，主要表现为I度房室传导阻滞、室性早搏、T波低平。皮损消失后心电图亦恢复正常。结论 急性荨麻疹患儿可伴有心脏的损害，临床上应予以重视。     

Plötzlicher Tod bei Urticaria pigmentosa

Zusammenfassung Es wird über den plötzlichen Tod eines 25jährigen berichtet, bei dem seit 6 Jahren eine Urticaria pigmentosa bekannt war. Er wurde an einem kalten Novembermorgen auf dem taunassen Rasen tot aufgefunden. Histologisch fanden sich keine Anhaltspunkte für eine systemische Mastozytose. Die biochemischen Untersuchungen (stark erhöhter Histamin-Wert im Urin) erklärten zusammen mit der BAK von 0,72 und der Auffindesituation (Kältereiz) den todlichen Pathomechanismus, eine generalisierte anaphylaktoide Reaktion. Sonderdruckanfragen an: J. Bolt     

CAP系统变应原筛选试验在慢性荨麻疹病因诊断中的应用

目的:探讨变应原筛选试验在慢性荨麻疹病因诊断中的临床应用价值。方法:用Pharmacia CAP System技术,对49例慢性荨麻疹患者和20例正常人作吸入物变应原筛选试验(Phadiatop)以及食物变应原混合物特异性IgE(fx5E)检测。结果:49例患者血清中,Phadiatop阳性21例,阳性检出率42.8％,fx5E阳性3例,阳性检出率6.1％,20例正常人两项检测均为阴性。结论:慢性荨麻疹患者以吸入过敏多见,用CAP系统进行变应原筛选试验检测,是临床上有助于慢性荨麻疹病因诊断的重要检测手段。     

Monogenic lupus due to DNASE1L3 deficiency in a pediatric patient with urticarial rash,hypocomplementemia, pulmonary hemorrhage,and immune-complex glomerulonephritis

Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease and usually involves the skin, musculoskeletal system, and kidneys. More than 30 genes have been to monogenic lupus, so far. Monogenic lupus is often characterized by an early-onset, similar family history, and syndromic appearance. Herein we present a pediatric patient with DNASE1L3 deficiency, suffering from both urticarial skin lesions, recurrent hemoptysis, and renal involvement, eventually diagnosed as this rare monogenic lupus.The patient suffered from recurrent urticarial rash and hemoptysis since the age of 15 months of age. He had microscopic hematuria, mild proteinuria, hypocomplementemia, and positive antinuclear antibody, anti-dsDNA, and antineutrophil cytoplasmic antibodies. Renal biopsy yielded immunocomplex glomerulonephritis. Due to early-onset, similar sibling history and consanguineous parents, we suspected monogenic lupus and performed whole-exome sequencing, which further revealed a homozygous T97Ifs*2 mutation (NM_004944.4: c.290_291delCA/p.Thr97Ilefs*2) in DNASE1L3 gene.In conclusion, DNASE1L3 deficiency should be thought when juvenile SLE occurs with early disease-onset, pulmonary hemorrhage, glomerulonephritis, and recurrent urticarial rash along with ANCA positivity.     

Urticaria and its subtypes: the role of second-generation antihistamines

Urticaria is a heterogeneous group of debilitating skin disorders characterized by wheals, pruritus, and frequently angioedema. The various forms of urticaria are often chronic and can exact a toll on quality of life. New diagnostic criteria and management guidelines are available to assist primary care physicians in the identification and proper treatment of different subtypes of urticaria. Second-generation antihistamines are recommended as first-line therapy because of their high degree of efficacy and safety. It is important to note, however, that European indications for most agents in this class are limited to specific forms of urticaria. The exception is desloratadine, the only second-generation antihistamine approved for the treatment of all urticaria subtypes in the European Union. Guidelines and best practice suggest that doses of antihistamines up to 4 times higher than those normally recommended for urticaria may benefit patients who do not respond to standard doses of antihistamines. Adjunctive therapy with leukotriene receptor antagonists may be advantageous in certain subgroups of patients who have suboptimal responses to antihistamine monotherapy. In all cases, physicians should work closely with patients to ensure proper adherence to prescribed regimens—a component that is often lacking but holds the key to successful outcomes.     

禁食轮替疗法在慢性荨麻疹中的临床研究

目的：探讨禁食轮替疗法联合药物治疗由食物不耐受引起的慢性荨麻疹的近、远期疗效。方法：选取慢性荨麻疹食物特异性IgG抗体阳性病例60例,随机分为实验组30例与对照组30例,两组均采用扑尔敏4mg每天3次,西替利嗪1片每晚1次。实验组除与对照组相同的治疗外,加用禁食、轮替的方法,3个月后观察疗法疗效及不良反应,6个月后观察复发情况。结果：3个月后实验组痊愈率53.33%,高于对照组26.67%,两组比较差异有显著性（P＜0.05）。6个月后复发率实验组16.67%,对照组43.33%,两组比较差异有显著性（P＜0.05）,实验组患者的血清食物特异性IgG水平在治疗前后下降明显（P＜0.05）。结论：禁食轮替疗法可以有效减轻由食物过敏原特异性IgG引起的慢性荨麻疹患者的症状并降低不耐受食物的阳性率。     

Efficacy and safety of rupatadine in Japanese adult and adolescent patients with chronic spontaneous urticaria: A double-blind,randomized, multicenter,placebo-controlled clinical trial

Background

Rupatadine, a novel nonsedating second-generation H1-antihistamine with antiplatelet-activating factor activity, has been used in the treatment of allergic rhinitis and urticaria in European countries since 2003. However, its efficacy and safety in Japanese patients with chronic spontaneous urticaria (CSU) are unknown.

我院荨麻疹患儿体外过敏原分析

目的：对本院荨麻疹患儿过敏原情况进行分析探讨。方法：采用免疫球蛋白E测定试剂盒（化学发光法）检测血清总IgE （TIgE）和Phadia公司全自动体外变应原检测系统ImmunoCAP250荧光酶联免疫法检测血清吸入物过敏原过筛查试验（Phadiatop）、食物过敏原过筛查试验（Fx5E）和特异性IgE（SIgE）。结果：193例荨麻疹患儿中,TIgE阳性率为99.3%。Phadiatop、Fx5E总计阳性率为59.8%;Phadiatop、Fx5E阳性率分别为32.1%和49.7%。SIgE显示吸入物过敏原以屋尘、粉尘螨、屋尘螨为主（47.6%）,食物过敏原以牛奶、鸡蛋为主（56.3%,52.6%）。0-3岁年龄段患儿Fx5E阳性率为45.2%,明显高于Phadiatop的阳性率26.2%（P〈0.05）;〉3岁患儿中两者无明显差异（P〈0.05）。结论：荨麻疹患儿多属特应性体质,约60%与第I型变态反应有关;吸入物过敏原主要为屋尘、粉尘螨、屋尘螨,食物过敏原以牛奶、鸡蛋为主;0-3岁患儿食物过敏是其主要因素,而〉3岁患儿食物与吸入物过敏几乎均等存在。     

Interleukin-6 and tumor necrosis factor-alpha gene polymorphisms in chronic idiopathic urticaria

BackgroundThis study was performed to evaluate association of gene polymorphisms among proinflammatory cytokines and susceptibility to chronic idiopathic urticaria (CIU).MethodsNinety patients with prolonged urticaria more than 6 weeks were included as case group. Single nucleotide polymorphisms (SNPs) of IL-6 (G/C −174, G/A nt565) and TNF-α (G/A −308, G/A −238) were evaluated, using polymerase chain reaction (PCR); and the results were compared to the control group.ResultsG allele was significantly higher in the patients at locus of −238 of promoter of TNF-α gene (p < 0.001). Frequency of following genotypes were significantly lower in patients with CIU, compared to controls: AG at −308 and GA at −238 of TNF-α gene (p < 0.05 and p < 0.001, respectively), CG at −174 and GG at +565 of IL-6 gene (p < 0.05). Additionally, following genotypes were more common among patients with CIU: GG at −308 and −238 of TNF-α gene (p < 0.05 and p < 0.001, respectively), GG at −174 and GA at +565 of IL-6 gene (p < 0.05).ConclusionsPro-inflammatory cytokine gene polymorphisms can affect susceptibility to CIU. TNF-α promoter polymorphisms as well as IL-6 gene polymorphisms are associated with CIU.