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[Patient with severe congenital neutropenia associated with ELANE gene mutation: c.684C>G, p.Tyr228Ter]. [j].严重先天性中性粒细胞减少症与ELANE基因突变的相关性:c.684C . bbb . 0 G . p. tyr28。
Pub Date : 2025-09-30 DOI: 10.29262/ram.v72i3.1501
Sabrina Dinorah Sotelo-de Jesús, Alonso Gutiérrez-Hernández

Introduction: Severe congenital neutropenia type 1 (SCN1) is a rare inherited disorder caused by arrested granulocyte maturation, frequently associated with mutations in the ELANE gene, which encodes neutrophil elastase.

Case report: A 3-year-5-month-old female patient with a history of recurrent perianal infections and necrotizing fasciitis secondary to appendicitis complicated by septic shock. She required laparotomy, appendectomy, ileostomy, debridement, and vasoactive amine support. During her hospitalization, persistent severe neutropenia, lymphopenia, and eosinophilia were identified. Bone marrow aspirate showed arrested myeloid leukemia with an absence of neutrophils. Among the infectious isolates, the following were isolated: P. aeruginosa and Stenotrophomonas maltophilia, as well as rhinovirus/enterovirus. The neutrophil count remained persistently low (100-530/μL), responding to G-CSF (5 mcg/kg/dose). Genetic sequencing revealed a heterozygous missense mutation in ELANE (c.684C>G, p.Tyr228Ter).

Conclusion: The diagnosis of NCG1 was clinically supported by severe infections, persistent neutropenia, absence of mature granulocytes in the bone marrow, and genetic confirmation. This mutation generates a premature stop codon. Other relevant variants include GFI1, HAX1, VPS45, JAGN1, CSF3R, and WAS. NCG1 should be suspected in pediatric patients with recurrent severe infections and persistent neutropenia. Early identification and the use of G-CSF can improve clinical outcome and reduce infectious complications.

重度先天性1型中性粒细胞减少症(SCN1)是一种罕见的遗传性疾病,由粒细胞成熟受阻引起,通常与编码中性粒细胞弹性酶的ELANE基因突变有关。病例报告:一名3岁至5个月大的女性患者,有复发性肛周感染和继发于阑尾炎的坏死性筋膜炎并脓毒性休克的病史。她需要开腹手术、阑尾切除术、回肠造口术、清创和血管活性胺支持。在住院期间,发现持续严重的中性粒细胞减少症、淋巴细胞减少症和嗜酸性粒细胞增多症。骨髓抽吸显示骨髓性白血病停止,中性粒细胞缺失。其中分离出铜绿假单胞菌和嗜麦芽窄养单胞菌,以及鼻病毒/肠道病毒。中性粒细胞计数持续低(100-530/μL),对G-CSF (5 mcg/kg/剂量)有反应。基因测序结果显示ELANE存在杂合错义突变(c.684C >g, p.Tyr228Ter)。结论:NCG1的诊断有严重感染、持续性中性粒细胞减少、骨髓中缺乏成熟粒细胞、基因证实等临床支持。这种突变产生一个过早终止密码子。其他相关变异包括GFI1、HAX1、VPS45、JAGN1、CSF3R和WAS。在反复严重感染和持续性中性粒细胞减少的儿童患者中应怀疑NCG1。早期识别和使用G-CSF可以改善临床结果并减少感染并发症。
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引用次数: 0
[Desensitization to Acetylsalicylic Acid in patients with coronary artery disease]. [冠心病患者对乙酰水杨酸脱敏]。
Pub Date : 2025-09-30 DOI: 10.29262/ram.v72i3.1519
Brenda Mariel Valerdi-Zepeda, Daniela Rivero-Yeverino, Aida Inés López-García, Chrystopherson Gengyny Caballero-López, Juan Jesus Ríos-López

Case report: A 77-year-old male patient with a history of facial angioedema 20 years prior after ingesting 500 mg of acetylsalicylic acid (ASA). In 2024, he underwent angioplasty with coronary stenting without ASA. He was admitted to the emergency department with chest pain. Coronary angiography confirmed acute myocardial infarction requiring stenting and repositioning. The catheterization department recommended triple therapy with clopidogrel, anticoagulant, and ASA. Due to the history of adverse reactions, a consultation was requested to assess tolerance to ASA. An oral challenge/desensitization was performed. Prior to the procedure, he presented with a peak expiratory flow (PEF) of 450 L/min, normal vital signs (VS), and a 5-point clinical score. ASA was administered every 15 minutes in progressive doses of 10 mg, 32 mg, 85 mg, and 174 mg, until reaching a cumulative dose of 301 mg. PEF, SV, and EC were monitored every 15 minutes for up to 4 hours after the start of treatment, with no changes. The following day, a loading dose of 300 mg of ASA was administered prior to coronary catheterization, followed by a maintenance dose of 100 mg/d.

Conclusion: Various desensitization regimens exist. Khan proposes a regimen with 90-minute intervals using doses of 40.5 mg, 81 mg, 162 mg, and 325 mg. In our case, reducing the intervals to 15 minutes facilitated desensitization in a shorter time, which is useful in cardiac emergencies. Desensitization allowed tolerance to the loading dose prior to cardiac intervention and the maintenance dose without adverse reactions.

病例报告:一位77岁男性患者,20年前服用500mg乙酰水杨酸(ASA)后出现面部血管性水肿。2024年,他在没有ASA的情况下接受了冠状动脉支架成形术。他因胸痛被送进急诊室。冠状动脉造影证实急性心肌梗死需要支架植入和重新定位。导管科推荐氯吡格雷、抗凝剂和ASA三联治疗。由于不良反应史,要求会诊评估对ASA的耐受性。进行口腔刺激/脱敏。手术前,患者呼气峰流量(PEF)为450 L/min,生命体征(VS)正常,临床评分为5分。ASA每15分钟分10 mg、32 mg、85 mg和174 mg的渐进剂量给药,直到达到301 mg的累积剂量。PEF、SV和EC在治疗开始后4小时内每15分钟监测一次,无变化。第二天,冠状动脉插管前给予300 mg ASA负荷剂量,随后维持剂量为100 mg/d。结论:存在多种脱敏方案。Khan提出了一个90分钟间隔的治疗方案,剂量分别为40.5毫克、81毫克、162毫克和325毫克。在我们的病例中,将间隔时间减少到15分钟有助于在更短的时间内脱敏,这在心脏紧急情况下是有用的。脱敏使心脏干预前的负荷剂量和维持剂量耐受无不良反应。
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引用次数: 0
[Clinical efficacy and safety of sublingual whole bacterial extract as an adjuvant to allergen-specific immunotherapy in allergic rhinitis]. 舌下全菌提取物辅助过敏原特异性免疫治疗变应性鼻炎的临床疗效和安全性。
Pub Date : 2025-09-30 DOI: 10.29262/ram.v72i3.1516
Jahnisi Riley-Pérez, Juan Jesús Ríos-López, Natalia Elizabeth Valdés-González, Aída Inés López-García, Daniela Rivero-Yeverino, Chrystopherson Gengyny Caballero-López, Jose Sergio Papaqui Tapia

Background: In allergic rhinitis (AR), allergen-specific immunotherapy (SIT) is the only disease-modifying treatment. It may include adjuvants to improve tolerance and safety.

Objective: To evaluate the efficacy, safety, quality of life, medication reduction, and number of infections with sublingual (SL) administration of whole bacterial extract (WBE) as an adjuvant to SIT in AR.

Methods: An experimental, randomized, double-blind, controlled study was conducted with 40 patients aged 5 to 60 years with moderate or severe AR due to SSTN. Patients were randomly assigned to an experimental group (SLIT with a standardized extract for Dermatophagoides pteronyssinus and/or Dermatophagoides farinae and SBE) or a control group (SLIT with a standardized extract for Dermatophagoides pteronyssinus and/or Dermatophagoides farinae). TNSS, RQLQ, medication, infections, and adverse effects were assessed at 30, 60, and 90 days.

Results: Both groups showed clinical improvement; however, there was only a significant change in the TNSS in the experimental group (p=0.049). Respiratory infections decreased in both groups, and the reduction in medication use was more evident in the experimental group from the first month. Two patients in the control group reported mild local adverse effects.

Conclusion: Compared with other studies that used different adjuvants and routes of administration, similarities were observed in the reduction of symptoms and medication use. No previous studies were found comparing the reduction of infections or improvement in quality of life with SIT and bacterial lysates. Bacterial suspension as an adjuvant to SLIT with standardized extracts in AR for mites is effective and safe.

背景:在变应性鼻炎(AR)中,过敏原特异性免疫治疗(SIT)是唯一的疾病改善治疗方法。它可能包括佐剂,以提高耐受性和安全性。目的:评价舌下(SL)给予全菌提取物(WBE)辅助治疗AR的疗效、安全性、生活质量、减量及感染发生率。方法:对40例5 ~ 60岁中重度SSTN所致AR患者进行随机、双盲、对照实验研究。患者被随机分配到实验组(使用标准提取物的割开组)或对照组(使用标准提取物的割开组和/或粉蚧)。在30、60和90天分别评估TNSS、RQLQ、用药、感染和不良反应。结果:两组临床表现均有改善;而实验组只有TNSS有显著变化(p=0.049)。两组患者的呼吸道感染均有所减少,且从第一个月开始,实验组的用药减少更为明显。对照组2例患者报告轻度局部不良反应。结论:与其他使用不同佐剂和给药途径的研究相比,在减轻症状和用药方面有相似之处。以前没有研究发现将SIT和细菌裂解物在减少感染或改善生活质量方面进行比较。细菌悬浮液作为SLIT的佐剂与标准提取物在AR治疗螨虫是有效和安全的。
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引用次数: 0
[Case report. Job syndrome as an unusual finding in a pediatric patient]. [病例报告。工作综合症在儿科患者中是一种不寻常的发现。
Pub Date : 2025-09-30 DOI: 10.29262/ram.v72i3.1494
Alan Orlando Morales-Reyes, Vanessa Alondra Juárez-Mendoza, Anette Michelle Ávila-Silva, Christian Jair Ramos-Gómez, Felipe de Jesús Sánchez-Jaime

Background: Job Syndrome is a primary immunodeficiency disorder with an incidence of 1 per million people. It is characterized by elevated IgE levels, eosinophilia, recurrent infections (bacterial and fungal), eczematous dermatitis, musculoskeletal and vascular anomalies. Diagnosis is based on IgE > 1000 IU/ml and eosinophilia. Treatment is multidisciplinary, including bone marrow transplantation, stem cell therapy, and human immunoglobulin.

Case report: Evolution: We present a 13-year-old male with a history of asthma and atopic dermatitis treated with subcutaneous immunotherapy since 2016. He developed furunculous lesions that progressed to the frontal area, nose, forearms, abdomen, legs, ankles, and feet. Despite treatment with hydroxyzine, mupirocin, tacrolimus, clobetasol, and deflazacort, he showed no improvement. In 2021, he was admitted to the pediatric department, where the diagnosis was confirmed based on Grimbacher criteria. Treatment with human immunoglobulin was initiated, with adequate response. Currently, the patient is in good general condition with persistence of some skin lesions.

Conclusions: The therapeutic strategy focuses on the prevention and management of infections and symptoms. It is important to identify complications in the early stages of the disease to treat them effectively. This syndrome, although rare, should be considered a serious condition; early identification of this condition is crucial to improve outcomes in patients with similar presentations.

背景:工作综合症是一种原发性免疫缺陷疾病,发病率为百万分之一。其特征是IgE水平升高,嗜酸性粒细胞增多,反复感染(细菌和真菌),湿疹性皮炎,肌肉骨骼和血管异常。诊断依据是IgE水平1000 IU/ml和嗜酸性粒细胞增多。治疗是多学科的,包括骨髓移植、干细胞治疗和人类免疫球蛋白。病例报告:演变:我们报告一名13岁男性,自2016年以来接受皮下免疫治疗,有哮喘和特应性皮炎病史。他出现疖状病变,并进展到额部、鼻子、前臂、腹部、腿、脚踝和脚。尽管给予羟嗪、莫匹罗星、他克莫司、氯倍他索和地沙柯治疗,他仍无好转。2021年,他被送入儿科,根据格里姆巴赫标准确诊。开始使用人免疫球蛋白治疗,反应良好。目前,患者总体情况良好,但持续存在一些皮肤病变。结论:治疗策略侧重于感染和症状的预防和管理。重要的是在疾病的早期阶段确定并发症以有效治疗。这种综合征虽然罕见,但应被视为一种严重的疾病;早期识别这种情况对于改善具有类似表现的患者的预后至关重要。
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引用次数: 0
[Drug addiction and hereditary angioedema type 1, a dangerous combination]. [药物成瘾和遗传性血管性水肿1型,危险的组合]。
Pub Date : 2025-09-30 DOI: 10.29262/ram.v72i3.1514
Alejandra Rodríguez-Carbajal, Nathalia Quintanar Félix, María Isabel Rodríguez Dumas, Jaime Armando Soto Domínguez, Marya Lizzeth Rosaldo Solis, Vanessa Daniela Castillo León, Margarita Ortega Cisneros, Ileana María Madrigal-Beas

Introduction: Hereditary angioedema (HAE) is a rare disease characterized by episodes of cutaneous and submucosal inflammation. Its global prevalence ranges from 1:50,000 to 1:100,000 individuals and is often underdiagnosed. It is classified into three main types based on C1 inhibitor (C1-INH) levels and functionality. Type 1 is the most common (85% of cases). It is characterized by low plasma concentrations of functional C1-INH as a result of mutations in the SERPING1 gene.

Case report: We present the case of a 20-year-old male resident of Guadalajara with HAE type 1 diagnosed at age 12 and a history of drug use. He was admitted with edema in the left posterior thoracic region, which progressed to the neck, causing respiratory distress. During ambulance transport, the patient suffered cardiorespiratory arrest, requiring cardiopulmonary resuscitation. He was administered a bradykinin type 2 receptor antagonist and systemic steroid without success, and was admitted to the emergency department under advanced airway management. Laboratory tests and a CT scan revealed no significant findings. Given the persistence of facial edema, a human C1 esterase inhibitor was administered, and the patient was admitted to the intensive care unit for monitoring.

Conclusion: Based on laboratory and CT results, substance abuse was suspected as a triggering factor. The literature reports that individuals with cocaine dependence present a persistent proinflammatory state characterized by reduced baseline levels of the anti-inflammatory interleukin IL-10 and a significant increase in the proinflammatory cytokine TNF-α after exposure to stressful stimuli or use. Although the evidence in the medical literature is limited, the use of psychoactive substances could act as a triggering factor for seizures in patients with hereditary angioedema type 1. This case underscores the importance of identifying potential less-studied aggravating factors and suggests the need for a targeted history of psychoactive substance use in patients with HAE. This could be explained by their effects on vasodilation, the release of inflammatory mediators, and increased vascular permeability, mechanisms that promote bradykinin accumulation. Given the potential risk, it is essential to consider drug use in the comprehensive evaluation of precipitating factors in these patients.

遗传性血管性水肿(HAE)是一种罕见的疾病,以皮肤和粘膜下炎症发作为特征。其全球流行率为1:50 000至1:10 000人,而且往往未得到充分诊断。它根据C1抑制剂(C1- inh)水平和功能分为三种主要类型。1型最常见(85%的病例)。其特点是由于SERPING1基因突变导致血浆中功能性C1-INH浓度低。病例报告:我们报告一名20岁的瓜达拉哈拉男性居民,12岁时诊断为1型HAE,并有吸毒史。患者入院时左胸后区水肿,并进展至颈部,引起呼吸窘迫。在救护车运送过程中,患者出现了心肺骤停,需要心肺复苏。他被给予缓激肽2型受体拮抗剂和全身类固醇治疗,但没有成功,并在先进的气道管理下被送入急诊科。实验室检查和CT扫描没有明显的发现。鉴于面部水肿持续存在,给予人C1酯酶抑制剂,并将患者送入重症监护病房进行监测。结论:基于实验室和CT结果,怀疑药物滥用是诱发因素。文献报道,可卡因依赖个体在暴露于应激刺激或使用后呈现持续的促炎状态,其特征是抗炎白细胞介素IL-10基线水平降低,促炎细胞因子TNF-α显著升高。尽管医学文献中的证据有限,但精神活性物质的使用可能是遗传性血管性水肿1型患者癫痫发作的触发因素。该病例强调了识别潜在的较少研究的加重因素的重要性,并提示有必要对HAE患者的精神活性物质使用史进行针对性研究。这可以解释为它们对血管舒张的影响,炎症介质的释放,血管通透性的增加,促进缓激素积累的机制。考虑到潜在的风险,在对这些患者的诱发因素进行综合评价时,必须考虑药物的使用情况。
{"title":"[Drug addiction and hereditary angioedema type 1, a dangerous combination].","authors":"Alejandra Rodríguez-Carbajal, Nathalia Quintanar Félix, María Isabel Rodríguez Dumas, Jaime Armando Soto Domínguez, Marya Lizzeth Rosaldo Solis, Vanessa Daniela Castillo León, Margarita Ortega Cisneros, Ileana María Madrigal-Beas","doi":"10.29262/ram.v72i3.1514","DOIUrl":"https://doi.org/10.29262/ram.v72i3.1514","url":null,"abstract":"<p><strong>Introduction: </strong>Hereditary angioedema (HAE) is a rare disease characterized by episodes of cutaneous and submucosal inflammation. Its global prevalence ranges from 1:50,000 to 1:100,000 individuals and is often underdiagnosed. It is classified into three main types based on C1 inhibitor (C1-INH) levels and functionality. Type 1 is the most common (85% of cases). It is characterized by low plasma concentrations of functional C1-INH as a result of mutations in the SERPING1 gene.</p><p><strong>Case report: </strong>We present the case of a 20-year-old male resident of Guadalajara with HAE type 1 diagnosed at age 12 and a history of drug use. He was admitted with edema in the left posterior thoracic region, which progressed to the neck, causing respiratory distress. During ambulance transport, the patient suffered cardiorespiratory arrest, requiring cardiopulmonary resuscitation. He was administered a bradykinin type 2 receptor antagonist and systemic steroid without success, and was admitted to the emergency department under advanced airway management. Laboratory tests and a CT scan revealed no significant findings. Given the persistence of facial edema, a human C1 esterase inhibitor was administered, and the patient was admitted to the intensive care unit for monitoring.</p><p><strong>Conclusion: </strong>Based on laboratory and CT results, substance abuse was suspected as a triggering factor. The literature reports that individuals with cocaine dependence present a persistent proinflammatory state characterized by reduced baseline levels of the anti-inflammatory interleukin IL-10 and a significant increase in the proinflammatory cytokine TNF-α after exposure to stressful stimuli or use. Although the evidence in the medical literature is limited, the use of psychoactive substances could act as a triggering factor for seizures in patients with hereditary angioedema type 1. This case underscores the importance of identifying potential less-studied aggravating factors and suggests the need for a targeted history of psychoactive substance use in patients with HAE. This could be explained by their effects on vasodilation, the release of inflammatory mediators, and increased vascular permeability, mechanisms that promote bradykinin accumulation. Given the potential risk, it is essential to consider drug use in the comprehensive evaluation of precipitating factors in these patients.</p>","PeriodicalId":101421,"journal":{"name":"Revista alergia Mexico (Tecamachalco, Puebla, Mexico : 1993)","volume":"72 3","pages":"77"},"PeriodicalIF":0.0,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145214807","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
[Drug hypersensitivity: When systemic symptoms and pustules converge]. [药物过敏:全身性症状和脓疱汇合]。
Pub Date : 2025-09-30 DOI: 10.29262/ram.v72i3.1538
Ariadna Palafox-Olvera, Edgardo Adrian Puerto-Díaz, Jaime Mellado-Ábrego, Leticia Hernández-Nieto, Gloria Castillo-Narvaéz, Carol Vivian Moncayo-Coello

Background: Overlap (SCARs) severe cutaneous adverse drug reactions, are defined as cases that fulfill diagnostic criterio for at least two of these drug-associated reactions, according to scoring systems. We present a case of an overlapping SCAR.

Case report: A 53-year-old female was diagnosed with diabetes and was being treated with metformin and linagliptin, and with high blood pressure with nifedipine. Secondary to an isolated seizure, she was treated with phenytoin, 5 weeks later, she presented with erythema in the chest region accompanied by pruritus that spread to the abdomen. Treatment was initiated with antihistamines, with poor improvement, progressing to generalized erythema and fever. Upon admission, she presented with generalized polymorphic skin lesions of a maculopapular rash and bullous lesions on the forearms, as well as pustular lesions on the face. Laboratory findings: Leukocytosis with neutrophilia (67%), eosinophilia =3,880/mm3, and acute kidney injury. Biopsy: Chronic interface dermatitis, superficial perivasculitis, and eosinophilia. According to RegiSCAR scoring system with 4 points and the EuroSCAR score with 6 points, both considered probable The patient began steroid therapy with methylprednisolone at 1.5 mg/kg for 3 days, followed by reduced doses of prednisone.

Conclusions: The patient presented a severe cutaneous adverse reaction 5 weeks after starting phenytoin, which showed overlap according to the scales. Secondary to the ambiguities among SCARs, confirmed cases of overlap are rare. In the acute stage of the disease, early identification of SCARs can be difficult due to overlapping features.

背景:根据评分系统,重叠(scar)严重皮肤药物不良反应被定义为满足至少两种药物相关反应诊断标准的病例。我们提出一个重叠疤痕的病例。病例报告:一名53岁的女性被诊断为糖尿病,正在用二甲双胍和利格列汀治疗,高血压用硝苯地平治疗。继发于孤立性癫痫发作,患者接受苯妥英治疗,5周后,患者出现胸部红斑并伴有瘙痒,并扩散至腹部。治疗开始使用抗组胺药,但改善不佳,进展为全身红斑和发热。入院时,患者表现为全身性多形态皮肤病变,包括前臂黄斑丘疹和大疱性病变,以及面部脓疱性病变。实验室结果:白细胞增多伴中性粒细胞增多(67%),嗜酸性粒细胞增多= 3880 /mm3,急性肾损伤。活检:慢性界面皮炎,浅表血管周围炎,嗜酸性粒细胞增多。RegiSCAR评分系统为4分,EuroSCAR评分为6分,均认为可能。患者开始以1.5 mg/kg剂量甲基强的松类固醇治疗3天,随后减少泼尼松剂量。结论:患者在使用苯妥英5周后出现严重的皮肤不良反应,鳞片上有重叠。除了疤痕不明确之外,确认的重叠病例很少。在疾病的急性阶段,由于重叠的特征,早期识别疤痕可能是困难的。
{"title":"[Drug hypersensitivity: When systemic symptoms and pustules converge].","authors":"Ariadna Palafox-Olvera, Edgardo Adrian Puerto-Díaz, Jaime Mellado-Ábrego, Leticia Hernández-Nieto, Gloria Castillo-Narvaéz, Carol Vivian Moncayo-Coello","doi":"10.29262/ram.v72i3.1538","DOIUrl":"https://doi.org/10.29262/ram.v72i3.1538","url":null,"abstract":"<p><strong>Background: </strong>Overlap (SCARs) severe cutaneous adverse drug reactions, are defined as cases that fulfill diagnostic criterio for at least two of these drug-associated reactions, according to scoring systems. We present a case of an overlapping SCAR.</p><p><strong>Case report: </strong>A 53-year-old female was diagnosed with diabetes and was being treated with metformin and linagliptin, and with high blood pressure with nifedipine. Secondary to an isolated seizure, she was treated with phenytoin, 5 weeks later, she presented with erythema in the chest region accompanied by pruritus that spread to the abdomen. Treatment was initiated with antihistamines, with poor improvement, progressing to generalized erythema and fever. Upon admission, she presented with generalized polymorphic skin lesions of a maculopapular rash and bullous lesions on the forearms, as well as pustular lesions on the face. Laboratory findings: Leukocytosis with neutrophilia (67%), eosinophilia =3,880/mm3, and acute kidney injury. <i>Biopsy</i>: Chronic interface dermatitis, superficial perivasculitis, and eosinophilia. According to RegiSCAR scoring system with 4 points and the EuroSCAR score with 6 points, both considered probable The patient began steroid therapy with methylprednisolone at 1.5 mg/kg for 3 days, followed by reduced doses of prednisone.</p><p><strong>Conclusions: </strong>The patient presented a severe cutaneous adverse reaction 5 weeks after starting phenytoin, which showed overlap according to the scales. Secondary to the ambiguities among SCARs, confirmed cases of overlap are rare. In the acute stage of the disease, early identification of SCARs can be difficult due to overlapping features.</p>","PeriodicalId":101421,"journal":{"name":"Revista alergia Mexico (Tecamachalco, Puebla, Mexico : 1993)","volume":"72 3","pages":"99"},"PeriodicalIF":0.0,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145214875","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
[The challenge of delabeling antibiotic allergy in Colombian children: do parents trust the negative result of the drug provocation test with the implicated antibiotic?] 哥伦比亚儿童抗生素过敏标签去除的挑战:家长是否相信药物激发试验的阴性结果与相关抗生素?]
Pub Date : 2025-09-30 DOI: 10.29262/ram.v72i3.1465
Ana María Rincón-González, Diana Carolina Galeano-Tamayo, Libia Susana Díez Zuluaga

Objective: To explore the factors associated with the use of the antibiotic implicated in an adverse reaction following a negative challenge test in pediatric patients in a clinical allergology service of a health institution in Medellin.

Methods: Observational cross-sectional study with analytical intent, in pediatric patients with a history of adverse reaction to an antibiotic, who underwent a provocation test and obtained a negative result. Sociodemographic and clinical data were obtained from medical records and parents or caregivers of patients who met the inclusion criteria were surveyed.

Results: Between January 2016 and December 2021, 571 antibiotic provocations were performed. Only 19.2% of patients received the implicated antibiotic or antibiotics of the same class after a negative challenge test, without experiencing adverse reactions related to the new use. However, 53% of those who did not receive the antibiotic, despite needing it, mainly because their parents or caregivers continued to report the allergy label. Additionally, 26.9% of parents or caregivers did not recall the negative test result, and 44.2% would refuse future administration of the antibiotic to their children, identifying failure to recall the negative challenge test result as a factor associated with this behavior.

Conclusions: Local strategies (providing individualized forms for parents and healthcare providers) should be designed to explain the meaning of a negative test result, as well as follow-up visits to verify adherence to recommendations, thus increasing the effectiveness of antibiotic allergy delabeling.

目的:探讨麦德林一家卫生机构临床变态反应科服务儿科患者阴性激发试验后不良反应中抗生素使用的相关因素。方法:具有分析目的的观察性横断面研究,对有抗生素不良反应史的儿科患者进行激发试验,结果为阴性。从医疗记录中获得社会人口学和临床数据,并对符合纳入标准的患者的父母或照顾者进行调查。结果:2016年1月至2021年12月,共进行571次抗生素挑衅。只有19.2%的患者在阴性激发试验后接受了相关抗生素或同类抗生素,没有出现与新用途相关的不良反应。然而,53%的人没有接受抗生素治疗,尽管他们需要抗生素,主要是因为他们的父母或照顾者继续报告过敏标签。此外,26.9%的父母或看护人不记得阴性测试结果,44.2%的父母或看护人拒绝未来给孩子使用抗生素,这表明未能回忆起阴性挑战测试结果是与这种行为相关的一个因素。结论:应设计当地策略(为家长和医疗保健提供者提供个性化表格)来解释阴性检测结果的含义,并进行随访以验证是否遵守建议,从而提高抗生素过敏去除标签的有效性。
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引用次数: 0
[Relationship between FeNO levels and asthma severity: a cross-sectional study at the University Hospital of Puebla]. [FeNO水平与哮喘严重程度的关系:普埃布拉大学医院的一项横断面研究]。
Pub Date : 2025-09-30 DOI: 10.29262/ram.v72i3.1517
Jorge Andres Naranjo-Vallejo, Juan Jesús Ríos-López, Aida Inés López-García, Daniela Rivero-Yeverino, Chrystopherson Gengyny Caballero-López, José Sergio Papaqui-Tapia

Introduction: The evaluation of patients with asthma uses clinical tools such as the Asthma Control Test (ACT), pulmonary function tests, and biomarkers. Among these, an elevated fractional exhaled nitric oxide (FeNO) level can predict a higher risk of exacerbations and response to corticosteroids and/or biological therapies, although it does not always reflect poor clinical control. Our objective was to determine the relationship between asthma severity and FeNO levels in patients attending our service.

Methods: A cross-sectional study was conducted in patients over 12 years of age with controlled asthma assessed by ACT. FeNO was measured using the NIOX-VERO® device. Descriptive analysis and Spearmans correlation coefficient were applied.

Results: Thirty-six patients (66.7% women) with a mean age of 36.3 years (SD ± 14.4) were included. According to GINA, 63.9% had mild asthma and 36.1% had moderate asthma. 22.2% had low FeNO levels (<25 ppb) and 52.8% had high levels (>50 ppb). The mean FeNO level in mild asthma was 62.7 ppb (SD ± 62.7) and in moderate asthma, 54.8 ppb (SD ± 36.2). No significant correlation was found between asthma severity and FeNO levels (rs = 0.150, p = 0.306).

Conclusions: Although more than half of the patients had elevated FeNO levels, no significant association was observed with asthma severity. This finding is consistent with previous studies and suggests that FeNO, although useful as a biomarker of type 2 inflammation, should not be used in isolation to assess clinical severity.

哮喘患者的评估使用临床工具,如哮喘控制试验(ACT)、肺功能试验和生物标志物。其中,分数呼气一氧化氮(FeNO)水平升高可以预测更高的恶化风险和对皮质类固醇和/或生物治疗的反应,尽管它并不总是反映临床控制不良。我们的目的是确定就诊患者哮喘严重程度与FeNO水平之间的关系。方法:对12岁以上的哮喘患者进行横断面研究,通过ACT进行评估。使用NIOX-VERO®设备测量FeNO。采用描述性分析和spearman相关系数。结果:纳入36例患者(女性占66.7%),平均年龄36.3岁(SD±14.4)。根据GINA的数据,63.9%患有轻度哮喘,36.1%患有中度哮喘。22.2%的人FeNO水平低(50 ppb)。轻度哮喘患者的平均FeNO水平为62.7 ppb (SD±62.7),中度哮喘患者为54.8 ppb (SD±36.2)。哮喘严重程度与FeNO水平无显著相关性(rs = 0.150, p = 0.306)。结论:尽管超过一半的患者FeNO水平升高,但与哮喘严重程度无显著相关性。这一发现与先前的研究一致,并表明尽管FeNO作为2型炎症的生物标志物有用,但不应单独用于评估临床严重程度。
{"title":"[Relationship between FeNO levels and asthma severity: a cross-sectional study at the University Hospital of Puebla].","authors":"Jorge Andres Naranjo-Vallejo, Juan Jesús Ríos-López, Aida Inés López-García, Daniela Rivero-Yeverino, Chrystopherson Gengyny Caballero-López, José Sergio Papaqui-Tapia","doi":"10.29262/ram.v72i3.1517","DOIUrl":"https://doi.org/10.29262/ram.v72i3.1517","url":null,"abstract":"<p><strong>Introduction: </strong>The evaluation of patients with asthma uses clinical tools such as the Asthma Control Test (ACT), pulmonary function tests, and biomarkers. Among these, an elevated fractional exhaled nitric oxide (FeNO) level can predict a higher risk of exacerbations and response to corticosteroids and/or biological therapies, although it does not always reflect poor clinical control. Our objective was to determine the relationship between asthma severity and FeNO levels in patients attending our service.</p><p><strong>Methods: </strong>A cross-sectional study was conducted in patients over 12 years of age with controlled asthma assessed by ACT. FeNO was measured using the NIOX-VERO<sup>®</sup> device. Descriptive analysis and Spearmans correlation coefficient were applied.</p><p><strong>Results: </strong>Thirty-six patients (66.7% women) with a mean age of 36.3 years (SD ± 14.4) were included. According to GINA, 63.9% had mild asthma and 36.1% had moderate asthma. 22.2% had low FeNO levels (<25 ppb) and 52.8% had high levels (>50 ppb). The mean FeNO level in mild asthma was 62.7 ppb (SD ± 62.7) and in moderate asthma, 54.8 ppb (SD ± 36.2). No significant correlation was found between asthma severity and FeNO levels (rs = 0.150, p = 0.306).</p><p><strong>Conclusions: </strong>Although more than half of the patients had elevated FeNO levels, no significant association was observed with asthma severity. This finding is consistent with previous studies and suggests that FeNO, although useful as a biomarker of type 2 inflammation, should not be used in isolation to assess clinical severity.</p>","PeriodicalId":101421,"journal":{"name":"Revista alergia Mexico (Tecamachalco, Puebla, Mexico : 1993)","volume":"72 3","pages":"79"},"PeriodicalIF":0.0,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145215091","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
[Omalizumab Withdrawal Protocol for Chronic Spontaneous Urticaria: Clinical Case]. 慢性自发性荨麻疹的Omalizumab停药方案:临床病例。
Pub Date : 2025-09-30 DOI: 10.29262/ram.v72i3.1520
Daniela Ramírez-Vázquez, Chrystopherson Gengyny Caballero-López, Aida Inés López-García, Daniela Rivero-Yeverino D, Juan Jesus Ríos-López, José Sergio Papaqui-Tapia, Jahnisi Riley-Pérez

Background: Chronic spontaneous urticaria is characterized by the daily appearance of hives and/or angioedema (Fig. 1) without a specific trigger, lasting more than 6 weeks. Treatment is based on second-generation H1 antihistamines, and in patients with an insufficient response, therapy with omalizumab has been effective; however, it is a costly and long-term treatment. The WAO (World Allergy Organization) has proposed a tapering regimen upon achieving complete remission.

Case report: A 66-year-old female was referred for evaluation due to generalized hives of 6 years duration under treatment with fexofenadine 180 mg, with a partial response. Triple doses of H1 antihistamines were started, with no improvement, maintaining the UCT (Urticaria Control Test) at 5 points, and omalizumab 300 mg was added every 4 weeks, with an adequate clinical response. After complete remission, omalizumab tapering began in November 2023 according to the WAO protocol (Fig. 2). The final dose was administered in March 2025.

Conclusion: We present the case of a patient with chronic spontaneous urticaria. After achieving complete remission with omalizumab, the tapering protocol proposed by the WAO was implemented. During this period, our patient remained asymptomatic, with UCT scores greater than 13, allowing for progressive reduction of antihistamine use. The omalizumab tapering protocol for the treatment of chronic spontaneous urticaria is safe and effective, reduces the risk of disease reactivation, and prevents prolonged treatment.

背景:慢性自发性荨麻疹的特征是每天出现荨麻疹和/或血管性水肿(图1),无特定诱因,持续6周以上。治疗基于第二代H1抗组胺药,对于反应不足的患者,使用omalizumab治疗是有效的;然而,这是一个昂贵且长期的治疗方法。WAO(世界过敏组织)提出了一种完全缓解后逐渐减少的方案。病例报告:一名66岁女性因接受非索非那定180mg治疗后出现持续6年的广泛性荨麻疹而被转介评估,部分缓解。开始使用三剂量的H1抗组胺药,没有改善,将UCT(荨麻疹控制试验)维持在5点,每4周添加300 mg的omalizumab,具有足够的临床反应。完全缓解后,根据WAO方案,omalizumab于2023年11月开始逐渐减少(图2)。最后一剂是在2025年3月注射的。结论:我们报告了一例慢性自发性荨麻疹患者。在使用omalizumab达到完全缓解后,实施了WAO提出的逐渐减少方案。在此期间,我们的患者仍然无症状,UCT评分大于13,允许逐步减少抗组胺药的使用。用于治疗慢性自发性荨麻疹的omalizumab减量方案是安全有效的,降低了疾病再激活的风险,并防止延长治疗。
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引用次数: 0
[Non-allergic immediate hypersensitivity to Brentuximab vedotin in pediatrics: usefulness of skin testing to guide provocation testing]. [儿科对布伦妥昔单抗的非过敏性立即超敏反应:皮肤试验指导激发试验的有效性]。
Pub Date : 2025-09-30 DOI: 10.29262/ram.v72i3.1534
Karla Daniela González-Silva, Pablo Perea-Valle, José Alonso Gutiérrez-Hernández

Background: Hypersensitivity reactions to monoclonal antibodies represent a clinical challenge, especially when there are no equivalent therapeutic alternatives. Brentuximab vedotin (BV), an anti-CD30 monoclonal antibody indicated for relapsed Hodgkin lymphoma, has been associated with immediate hypersensitivity in 1.2% of cases.

Case report: An 11-year-old patient with relapsed Hodgkin lymphoma presented with grade 3 anaphylaxis (Brown scale) with hypotension, dyspnea, cough, wheezing, bipalpebral edema, conjunctival injection, nausea, and altered consciousness during the fifth cycle of chemotherapy with AVD (doxorubicin, vinblastine, and dacarbazine) + BV. Intramuscular epinephrine and fluid therapy were administered, with resolution of the condition. Laboratory Studies: BV skin tests performed 2 weeks after the event were negative. Outcome: Given the immediacy and severity of the event, and the need to continue treatment, a successful pharmacological challenge with AVD agents was performed. Subsequently, two cycles of BV desensitization were performed (cycles 6 and 7), using different protocols, without adverse reactions.

Conclusion: Although skin tests were negative, the clinical presentation was consistent with immediate non-allergic hypersensitivity. Desensitization allowed treatment to continue with adequate tolerance and without recurrence. Repeat skin tests are currently being considered 4 to 6 weeks after the index event; if negative, a BV challenge test is considered. Skin tests support safety prior to a controlled challenge. Desensitization is an effective strategy for continuing essential treatments in pediatric oncology.

背景:对单克隆抗体的超敏反应是一个临床挑战,特别是当没有等效的治疗方案时。Brentuximab vedotin (BV)是一种用于复发性霍奇金淋巴瘤的抗cd30单克隆抗体,在1.2%的病例中与立即过敏相关。病例报告:一名11岁的复发霍奇金淋巴瘤患者,在AVD(阿霉素、vinblastine和达卡巴嗪)+ BV化疗的第5个周期中,出现3级过敏反应(布朗评分),伴有低血压、呼吸困难、咳嗽、喘息、双睑水肿、结膜注射、恶心和意识改变。肌肉注射肾上腺素和液体治疗,病情得到缓解。实验室研究:事件发生2周后进行BV皮肤试验呈阴性。结果:考虑到事件的即时性和严重性,以及继续治疗的需要,我们成功地使用AVD药物进行了药理学挑战。随后,采用不同的方案进行了两个BV脱敏周期(第6和第7周期),未发生不良反应。结论:虽然皮肤试验阴性,但临床表现与立即非过敏性超敏反应一致。脱敏使治疗继续,有足够的耐受性和无复发。目前正在考虑在指数事件发生后4至6周进行重复皮肤测试;如果阴性,则考虑进行细菌性细菌感染试验。在控制挑战之前,皮肤测试支持安全性。脱敏是儿童肿瘤持续基本治疗的有效策略。
{"title":"[Non-allergic immediate hypersensitivity to Brentuximab vedotin in pediatrics: usefulness of skin testing to guide provocation testing].","authors":"Karla Daniela González-Silva, Pablo Perea-Valle, José Alonso Gutiérrez-Hernández","doi":"10.29262/ram.v72i3.1534","DOIUrl":"10.29262/ram.v72i3.1534","url":null,"abstract":"<p><strong>Background: </strong>Hypersensitivity reactions to monoclonal antibodies represent a clinical challenge, especially when there are no equivalent therapeutic alternatives. Brentuximab vedotin (BV), an anti-CD30 monoclonal antibody indicated for relapsed Hodgkin lymphoma, has been associated with immediate hypersensitivity in 1.2% of cases.</p><p><strong>Case report: </strong>An 11-year-old patient with relapsed Hodgkin lymphoma presented with grade 3 anaphylaxis (Brown scale) with hypotension, dyspnea, cough, wheezing, bipalpebral edema, conjunctival injection, nausea, and altered consciousness during the fifth cycle of chemotherapy with AVD (doxorubicin, vinblastine, and dacarbazine) + BV. Intramuscular epinephrine and fluid therapy were administered, with resolution of the condition. Laboratory Studies: BV skin tests performed 2 weeks after the event were negative. Outcome: Given the immediacy and severity of the event, and the need to continue treatment, a successful pharmacological challenge with AVD agents was performed. Subsequently, two cycles of BV desensitization were performed (cycles 6 and 7), using different protocols, without adverse reactions.</p><p><strong>Conclusion: </strong>Although skin tests were negative, the clinical presentation was consistent with immediate non-allergic hypersensitivity. Desensitization allowed treatment to continue with adequate tolerance and without recurrence. Repeat skin tests are currently being considered 4 to 6 weeks after the index event; if negative, a BV challenge test is considered. Skin tests support safety prior to a controlled challenge. Desensitization is an effective strategy for continuing essential treatments in pediatric oncology.</p>","PeriodicalId":101421,"journal":{"name":"Revista alergia Mexico (Tecamachalco, Puebla, Mexico : 1993)","volume":"72 3","pages":"101"},"PeriodicalIF":0.0,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145215144","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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Revista alergia Mexico (Tecamachalco, Puebla, Mexico : 1993)
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