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[Prophylaxis in hereditary angioedema with normal C1 inhibitor]. 正常C1抑制剂对遗传性血管性水肿的预防作用。
Q3 Medicine Pub Date : 2021-10-01 DOI: 10.29262/ram.v68i4.850
Dina Glocer, Ariela González, Mercedes Elizabeth Parrales-Villacreses, Pablo Martínez, Claudio Alberto Salvador Parisi

Introduction: Hereditary angioedema is an infrequent genetic disorder; which mainly manifests with cutaneous and mucosal swelling. Minor trauma may trigger potentially life-threatening events. In type I and II hereditary angioedema, plasma-derived C1-inhibitor concentrate can be used as short-term prophylaxis. For hereditary angioedema, prophylaxis is not yet standardized, but normal C1 inhibitor could be beneficial.

Case report: A 69-year-old woman, with a genetic diagnosis of hereditary angioedema with normal C1 inhibitor, who needed multiple dental extractions. The surgical procedure was performed under general anesthesia, using 1000 U of plasma-derived C1-inhibitor concentrate as prophylaxis an hour before. The patient was admitted in the ICU for postsurgical care and the outcome was good.

Conclusion: We highlight the possibility of successfully using plasma-derived C1-inhibitor concentrate as prophylaxis in patients with hereditary angioedema with normal C1 inhibitor.

简介:遗传性血管性水肿是一种罕见的遗传性疾病;主要表现为皮肤和粘膜肿胀。轻微的创伤可能引发潜在的危及生命的事件。对于I型和II型遗传性血管性水肿,血浆源性c1抑制剂浓缩物可作为短期预防用药。对于遗传性血管性水肿,预防尚未标准化,但正常的C1抑制剂可能是有益的。病例报告:一名69岁女性,遗传诊断为遗传性血管性水肿,C1抑制剂正常,需要多次拔牙。手术在全身麻醉下进行,术前1小时使用1000u血浆源性c1抑制剂浓缩物作为预防用药。患者在ICU接受术后护理,预后良好。结论:我们强调血浆来源的C1抑制剂浓缩物可以成功预防患有正常C1抑制剂的遗传性血管性水肿患者。
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引用次数: 0
[Clinical profile of patients with chronic urticaria in a tertiary care hospital of the social security system in Costa Ricas]. [哥斯达黎加社会保障系统三级医院慢性荨麻疹患者的临床概况]。
Q3 Medicine Pub Date : 2021-10-01 DOI: 10.29262/ram.v68i4.881
Giovanni Sedó-Mejía, Stephanie Lotz-Esquivel, Olga Patricia Monge-Ortega, Caridad Pino-García

Objective: To describe the medical and laboratory profile of the patients who have been diagnosed with chronic urticaria in the outpatient clinic of a tertiary care hospital of the social security system in Costa Rica.

Methods: All patients over 13 years of age who had been diagnosed with chronic urticaria between January 1st, 2014, and December 31st, 2018 were included. Variables of medical and laboratory data were statistically analyzed, and the treatment of the patients between their first and last medical consultation was compared.

Results: This was about 160 patients who had been diagnosed with chronic urticaria; 129 of them were women, 45.7 % between the ages of 30-49 years; in 17.5 % of them (28 patients), inducible urticaria was associated; 54.8 % (46/84) had positive antithyroperoxidase antibodies; 16.9 % (27 patients) maintained a single dose of second-generation anti-H1 antihistamines in the last consultation; 16.3 % (26 patients) no longer required antihistamine treatment in the last consultation. Cyclosporine was used in 8.8 % (14 patients), and omalizumab was used in 2.5 % (four patients).

Conclusion: Since this is a real-life study in a geographic region with limited publications on this pathology, we trust that the provided information will contribute to the scientific community in order to improve the quality of life of patients with chronic urticaria through a timely diagnosis and an effective and efficient treatment.

目的:描述哥斯达黎加社会保障系统三级医院门诊诊断为慢性荨麻疹患者的医学和实验室资料。方法:纳入2014年1月1日至2018年12月31日期间诊断为慢性荨麻疹的所有13岁以上患者。对医学变量和实验室数据进行统计分析,并比较患者首次和最后一次就诊的治疗情况。结果:约160名被诊断为慢性荨麻疹的患者;其中女性129例,年龄在30 ~ 49岁之间占45.7%;其中17.5%(28例)伴有诱导性荨麻疹;54.8%(46/84)抗甲状腺过氧化物酶抗体阳性;16.9%(27例)的患者在最后一次咨询时维持单剂量的第二代抗h1抗组胺药;16.3%(26例)患者在最后一次咨询时不再需要抗组胺药治疗。8.8%(14例)使用环孢素,2.5%(4例)使用奥玛单抗。结论:由于这是一项真实的研究,在一个地理区域,关于这种病理的出版物有限,我们相信所提供的信息将有助于科学界通过及时诊断和有效治疗来提高慢性荨麻疹患者的生活质量。
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引用次数: 0
[Rheumatic diseases and allergies, the duality of the immune system]. [风湿病和过敏,免疫系统的二元性]。
Q3 Medicine Pub Date : 2021-10-01 DOI: 10.29262/ram.v68i4.984
Gabriela Athziri Sánchez-Zuno, Edith Oregón-Romero, Jorge Hernández-Bello, Guillermo González-Estevez, Rodrigo Vargas-Rosales, José Francisco Muñoz-Valle

Autoimmune rheumatic diseases are multisystemic disorders that mainly affect joints and muscles; some examples of these conditions are: rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and Sjögren's syndrome (SS). In general, autoimmune rheumatic diseases have a high prevalence worldwide and are highly disabling for those who have them. At present, one of the main limitations for the management of these diseases is that their triggering factor continues to be unknown in most cases and the knowledge of the factors associated with their exacerbation is limited. In this review, the possible link of allergies as a possible trigger for autoimmune rheumatic diseases will be explored. We will also analyze the basic and general aspects of both diseases and the development of allergic processes and hypersensitivity reactions to drugs used in the treatment of rheumatic diseases.

自身免疫性风湿病是主要影响关节和肌肉的多系统疾病;这些情况的一些例子是:类风湿性关节炎(RA),系统性红斑狼疮(SLE)和Sjögren综合征(SS)。总的来说,自身免疫性风湿病在世界范围内具有很高的患病率,并且对那些患有这些疾病的人来说是高度致残的。目前,这些疾病管理的主要限制之一是,在大多数情况下,它们的触发因素仍然未知,并且对与其恶化相关的因素的了解有限。在这篇综述中,过敏作为自身免疫性风湿病的可能触发因素的可能联系将被探讨。我们还将分析这两种疾病的基本和一般方面以及用于治疗风湿性疾病的药物的过敏过程和超敏反应的发展。
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引用次数: 1
[Food protein-induced enterocolitis syndrome in a patient with pseudo-Bartter syndrome associated with cystic fibrosis. A case report]. 伪bartter综合征伴囊性纤维化患者的食物蛋白诱导小肠结肠炎综合征。[病例报告]。
Q3 Medicine Pub Date : 2021-10-01 DOI: 10.29262/ram.v68i4.974
Bethy Camargo-Vargas, María Varela, Laura Wilches, Paula Andrea Martínez-Quiñones, Ranniery Acuña-Cordero

Introduction: Food protein-induced enterocolitis is a non-immunoglobulin E-mediated food allergy with acute manifestations like recurrent vomiting, dehydration, and shock. It is a rare pathology that requires a high index of suspicion. Pseudo-Bartter syndrome (metabolic alkalosis, hypokalemia and hypochloremia in the absence of tubulopathy) is an infrequent complication of cystic fibrosis.

Case report: A 5-month-old boy with recurrent vomiting, dehydration, and shock; who had been breastfed and had consumed baby formula three hours prior to the onset of symptoms. Laboratory tests confirmed hyponatremia, hypochloremic metabolic alkalosis, and hypokalemia in absence of tubulopathy; two iontophoresis showed altered results, stool elastase was decreased, and genetic sequencing confirmed the diagnosis of cystic fibrosis. The provocation test confirmed food protein-induced enterocolitis syndrome.

Conclusion: Recurrent vomiting and dehydration after the intake of milk formula must lead to suspicion of food protein-induced enterocolitis syndrome. If pseudo-Bartter syndrome is found, cystic fibrosis must be ruled out.

简介:食物蛋白性小肠结肠炎是一种非免疫球蛋白e介导的食物过敏,急性表现为反复呕吐、脱水和休克。这是一种罕见的病理,需要高度的怀疑。伪巴特综合征(代谢性碱中毒、低钾血症和低氯血症,无小管病变)是囊性纤维化的罕见并发症。病例报告:1例5个月大男孩反复呕吐、脱水、休克;他们在出现症状前三小时接受母乳喂养并食用婴儿配方奶粉。实验室检查证实低钠血症、低氯血症代谢性碱中毒和无小管病的低钾血症;两次离子电泳显示结果改变,粪便弹性酶降低,基因测序证实了囊性纤维化的诊断。激发试验证实为食物蛋白性小肠结肠炎综合征。结论:食用配方奶粉后反复呕吐、脱水,应怀疑为食物蛋白性小肠结肠炎综合征。如果发现伪巴特综合征,必须排除囊性纤维化。
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引用次数: 0
[Prosopis spp. in allergic diseases]. [变态反应性疾病中的虫属]。
Q3 Medicine Pub Date : 2021-10-01 DOI: 10.29262/ram.v68i4.842
Guillermo Velázquez-Sámano, María Lourdes Mendoza-Gertrudis, Andrea Aída Velasco-Medina, Alejandro Rosas-Alvarado

Objectives: To demonstrate cutaneous reactivity and the participation of Prosopis pollen in patients with allergic diseases who attend the Allergy and Clinical Immunology Service of the General Hospital of Mexico, in Mexico City.

Methods: A retrospective and cross-sectional study was carried out by analyzing clinical records that had a complete medical history of patients between the ages of 3 and 79 years, with diagnoses of allergic rhinitis, asthma, and rhinoconjunctivitis.

Results: 1285 patients were studied; the frequency of positive skin reactivity for Prosopis was 27.78 %; 82.97 % had a diagnosis of allergic rhinitis, 4.39 % had a diagnosis of asthma, and 12.64 % had a diagnosis of rhinoconjunctivitis.

Conclusions: Prosopis pollen plays an important role in diseases of allergic etiology. It is important to consider the antigenic combination with other families such as Chenopodiaceae.

目的:在墨西哥城墨西哥总医院变态反应和临床免疫学服务部就诊的变态反应性疾病患者中,证明棘豆花粉的皮肤反应性和参与作用。方法:回顾性和横断面研究通过分析具有完整病史的患者的临床记录,年龄在3 ~ 79岁之间,诊断为过敏性鼻炎、哮喘和鼻结膜炎。结果:共纳入1285例患者;皮肤反应阳性检出率为27.78%;诊断为过敏性鼻炎的占82.97%,诊断为哮喘的占4.39%,诊断为鼻结膜炎的占12.64%。结论:棘豆花粉在变应性病因疾病中起重要作用。重要的是要考虑与其他科如藜科的抗原性组合。
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引用次数: 2
[Neuropsychiatric aspects of anti-NMDA receptor encephalitis]. [抗nmda受体脑炎的神经精神方面]。
Q3 Medicine Pub Date : 2021-10-01 DOI: 10.29262/ram.v68i4.953
Miguel Restrepo-Martínez, Mariana Espinola-Nadurille, Juan Carlos López-Hernández, Victoria Martínez, José Alberto Téllez-Martínez, Paula Andrea Bustamante-Gómez, Paola Bautista-Gómez, Leo Bayliss, Jesús Ramírez-Bermúdez

Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a potentially lethal clinical entity that belongs to the group of antibody-mediated encephalitis against synaptic proteins. It shows IgG antibodies against the NR1 subunit of the NMDA receptor (NMDA-R), which have been associated with psychiatric and neurological symptoms that develop in stages in the course of the disease. The predominance of neuropsychiatric symptoms in the early stages of the disease results in an increased number of patients that search for psychiatric evaluation as their first contact with the health system. For this reason, it is vital for physicians to recognize this entity as an important differential diagnosis in their clinical practice because, despite the severity of this condition, more than 75 % of patients achieve a substantial recovery with appropriate and timely treatment. We present a review of the literature on this disease, with special emphasis on the neuropsychiatric aspects.

抗n -甲基- d -天冬氨酸(NMDA)受体脑炎是一种具有潜在致命性的临床实体,属于针对突触蛋白的抗体介导的脑炎。它显示针对NMDA受体NR1亚基(NMDA- r)的IgG抗体,这与疾病过程中分阶段发展的精神和神经症状有关。在疾病的早期阶段,神经精神症状的优势导致越来越多的患者将寻求精神病学评估作为他们与卫生系统的第一次接触。由于这个原因,医生在临床实践中认识到这个实体是一个重要的鉴别诊断是至关重要的,因为尽管这种情况很严重,超过75%的患者通过适当和及时的治疗获得了实质性的恢复。我们提出的文献回顾对这种疾病,特别强调在神经精神方面。
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引用次数: 0
[Main biases in clinical research]. 【临床研究中的主要偏倚】。
Q3 Medicine Pub Date : 2021-10-01 DOI: 10.29262/ram.v68i4.1003
Jessie Nallely Zurita-Cruz, Miguel Ángel Villasís-Keever

In developing a research protocol, authors must consider the possible errors that may occur throughout the study. In clinical research, two types of biases are recognized: random errors and systematic errors; the latter are called biases. To date, dozens of biases have been described, which is why the purpose of this article is to describe the main biases that can occur in clinical research studies, as well as strategies to avoid them or to minimize their effects. Since there are several classifications, in order to provide a more practical overview in this review, the biases are grouped into three types: selection biases, information (or performance) biases, and confounding biases. In addition, to make it even more specific, we describe the biases considering the purpose of the research: prognosis, therapeutics, causality, and diagnostic test studies.

在制定研究方案时,作者必须考虑在整个研究过程中可能出现的错误。在临床研究中,公认的偏差有两种:随机误差和系统误差;后者被称为偏见。迄今为止,已有数十种偏倚被描述,这就是为什么本文的目的是描述临床研究中可能出现的主要偏倚,以及避免偏倚或将其影响降至最低的策略。由于存在几种分类,为了在本综述中提供更实用的概述,偏差分为三种类型:选择偏差,信息(或性能)偏差和混杂偏差。此外,为了使其更加具体,我们描述了考虑到研究目的的偏差:预后,治疗,因果关系和诊断测试研究。
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引用次数: 0
[Autoinmune psychosis]. [自身免疫性精神病]。
Q3 Medicine Pub Date : 2021-10-01 DOI: 10.29262/ram.v68i4.981
Miguel Restrepo-Martínez, Juan Carlos López-Hernández, Mariana Espinola-Nadurille, Leo Bayliss, Raúl Medina-Rioja, Victoria Martínez-Ángeles, Javier Galnares-Olalde, José Alberto Téllez-Martínez, Jesús Ramírez-Bermúdez

With the advent of the description of autoimmune encephalitis by different neuronal cell-surface antibodies (anti-NMDAr, among others) and that psychosis may be the only manifestation without neurological symptoms (epilepsy, movement disorders, autonomic dysfunction, altered state of consciousness) in 6.5 % of patients, the term "autoimmune psychosis" has become remarkably interesting among researchers. In 2020, an international consensus for the description and diagnostic approach of autoimmune psychosis was created. Through this consensus, by taking different criteria into account, the definition of autoimmune psychosis was proposed at different degrees of certainty (possible, probable, and defined). The purpose of these criteria is to underpin the autoimmune origin in patients who present psychosis with atypical characteristics, thus justifying the realization of laboratory studies and complementary clinical tests (lumbar puncture, electroencephalogram, and magnetic resonance imaging of the brain); in addition, these criteria are applied in patients with psychosis without neurological symptoms that do not fully meet the criteria of autoimmune encephalitis. As in autoimmune encephalitis, the early initiation of immunotherapy has a direct impact on the functional prognosis of patients, so an early initiation of treatment must be considered in clinical scenarios of probable or definite autoimmune psychosis.

随着不同的神经元细胞表面抗体(抗nmdar等)对自身免疫性脑炎的描述的出现,以及精神病可能是6.5%患者中唯一没有神经症状(癫痫、运动障碍、自主神经功能障碍、意识状态改变)的表现,“自身免疫性精神病”一词在研究人员中变得非常有趣。2020年,对自身免疫性精神病的描述和诊断方法达成了国际共识。通过这一共识,考虑到不同的标准,自身免疫性精神病的定义被提出了不同程度的确定性(可能的、可能的和定义的)。这些标准的目的是支持具有非典型特征的精神病患者的自身免疫起源,从而证明实验室研究和补充临床检查(腰椎穿刺、脑电图和脑磁共振成像)的实现是合理的;此外,这些标准适用于没有神经系统症状但不完全符合自身免疫性脑炎标准的精神病患者。与自身免疫性脑炎一样,早期开始免疫治疗直接影响患者的功能预后,因此在可能或明确的自身免疫性精神病的临床情况下,必须考虑早期开始治疗。
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引用次数: 1
[Diagnosis of hereditary angioedema after thirty years of clinical manifestations]. 【遗传性血管性水肿诊断三十年后临床表现】。
Q3 Medicine Pub Date : 2021-07-01 DOI: 10.29262/ram.v68i3.874
Luiz Fernando Bacarini, Ana Luisa Vieira, Mariana Camargo, Tainá Mosca, Wilma Carvalho Neves-Forte

Introduction: Diagnosis and treatment of hereditary angioedema (HAE) are necessary to improve the quality of life and even the survival of patients.

Case report: A 52-year-old woman with angioedema for 30 years, which affects the face, tongue, and hands. It is asymmetric, with neither pruritus nor urticaria, without response to antihistamines or corticosteroids, with spontaneous resolution in 48 hours to 72 hours; with a family history of angioedema. Normal physical examination between exacerbations. Autoimmune and lymphoproliferative diseases were ruled out. Values of C1q, C4, C1-INH were normal. The diagnosis of HAE type C1-INH normal subtype Unknown was established. The total resolution of the crises was achieved after two months with androgen therapy. Outpatient follow-up has been given for four years and no angioedema crisis has been reported, which is associated with a radical change in the quality of life.

Conclusion: The patient was diagnosed with HAE after 30 years of clinical manifestations, after acquired angioedema was ruled out.

摘要:遗传性血管性水肿(遗传性血管性水肿,遗传性血管性水肿)的诊断和治疗对于提高患者的生活质量甚至生存是必要的。病例报告:52岁女性血管性水肿30年,影响面部、舌头和手部。它是不对称的,既没有瘙痒也没有荨麻疹,对抗组胺药或皮质类固醇没有反应,在48小时至72小时内自发消退;有血管性水肿家族史。两次发作之间的正常体格检查。排除了自身免疫性和淋巴细胞增生性疾病。C1q、C4、C1-INH值正常。确定HAE C1-INH正常亚型Unknown的诊断。经过两个月的雄激素治疗,危机得到了彻底解决。门诊随访4年,没有血管性水肿危象的报道,这与生活质量的根本改变有关。结论:在排除后天性血管性水肿后,患者在30年的临床表现后被诊断为HAE。
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引用次数: 0
[Food sensitization and factors associated with uncontrolled asthma in the Yaqui Valley, Mexico]. [墨西哥雅基山谷与未控制哮喘相关的食物致敏和因素]。
Q3 Medicine Pub Date : 2021-07-01 DOI: 10.29262/ram.v68i3.887
Diego Hazael Ramírez-Leyva, Leticia Díaz-Sánchez, María Citlaly Ochoa
OBJECTIVETo determine the frequency of food allergy sensitization and the factors associated with uncontrolled asthma in patients from the Yaqui Valley.METHODSA cross-sectional analytical study in a second-level hospital in Ciudad Obregon, Mexico. Asthma control was classified according to the Global Initiative for Asthma, the nutritional status with body mass index, food sensitization with skin prick tests, and the rest of variables through direct questioning or medical records. The frequencies of sensitization and the factors associated with uncontrolled asthma were determined through descriptive and inferential statistics; a value of p < 0.05 was considered statistically significant.RESULTS230 patients were included. The frequency of sensitization to at least one food allergen was of 79%. The most frequent sensitizing allergens were eggs, corn, beans, shrimp, and cow's milk.CONCLUSIONOur study describes the pattern of food sensitization in patients with asthma and the factors associated with the uncontrolled disease. It is the first investigation of its kind that has been carried out in this region of Mexico.
目的:了解雅桂河谷地区哮喘患者食物过敏致敏频率及控制哮喘的相关因素。方法:对墨西哥奥布雷贡市某二级医院进行横断面分析研究。通过直接询问或医疗记录,根据全球哮喘倡议、体重指数的营养状况、皮肤点刺试验的食物致敏性以及其他变量对哮喘控制进行分类。通过描述性和推断性统计确定致敏频率和与未控制哮喘相关的因素;p < 0.05为差异有统计学意义。结果:纳入230例患者。对至少一种食物过敏原致敏的频率为79%。最常见的致敏过敏原是鸡蛋、玉米、豆类、虾和牛奶。结论:我们的研究描述了哮喘患者食物致敏的模式以及与疾病失控相关的因素。这是在墨西哥这一地区进行的首次此类调查。
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引用次数: 0
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Revista alergia Mexico
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