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1.
Arch. argent. pediatr ; 121(6): e202202850, dic. 2023. tab, fig
Artículo en Inglés, Español | LILACS, BINACIS | ID: biblio-1517878

RESUMEN

Introducción. La prueba de provocación oral (PPO) para el diagnóstico de alergia a las proteínas de la leche de la vaca (APLV) presenta riesgos y requiere de recursos. Nuestro objetivo fue evaluar condiciones y pruebas complementarias para identificar una alta probabilidad de APLV. Población y métodos. Análisis secundario sobre estudio de pacientes atendidos en una unidad de alergia entre 2015 y 2018. Se determinaron las probabilidades prepruebas asociadas a los síntomas y sus combinaciones, y las probabilidades pospruebas luego de realizadas pruebas cutáneas y determinación de inmunoglobulina E (IgE) sérica. Resultados. Se evaluó la información de 239 pacientes. Se observaron probabilidades mayores al 95 % en pacientes con angioedema y combinación de urticaria y vómitos. Usando puntos de corte propuestos por Calvani et al., la combinación de vómitos con rinitis, sin angioedema, también superó el 95 %. Conclusión. Se ofrece una metodología para identificar pacientes en los que puede diagnosticarse APLV sin realización de PPO.


Introduction. The oral food challenge (OFC) for the diagnosis of cow's milk protein allergy (CMPA) poses risks and requires resources. Our objective was to assess conditions and complementary tests used to identify a high probability of CMPA. Population and methods. Secondary analysis of a study of patients seen at a unit of allergy between 2015 and 2018. Pre-testing probabilities associated with symptoms and their combinations and post-testing probabilities after skin prick testing and serum immunoglobulin E (IgE) levels were determined. Results. The data from 239 patients were assessed. A probability greater than 95% was observed for angioedema and a combination of urticaria and vomiting. Based on the cut-off points proposed by Calvani et al., the combination of vomiting with rhinitis, without angioedema, also exceeded 95%. Conclusion. A methodology is provided to identify patients in whom CMPA may be diagnosed without an OFC.


Asunto(s)
Humanos , Animales , Lactante , Hipersensibilidad a la Leche/diagnóstico , Hipersensibilidad a la Leche/epidemiología , Angioedema/complicaciones , Vómitos , Bovinos , Pruebas Cutáneas/métodos , Proteínas de la Leche/efectos adversos
2.
Rev Alerg Mex ; 70(4): 194, 2023 Sep.
Artículo en Español | MEDLINE | ID: mdl-37933935

RESUMEN

Background: Hereditary angioedema type 1 (HAE1) is an autosomal dominant disorder, characterized by quantitative and qualitative deficiency of C1 inhibitor, excessive production of bradykinin and causing recurrent angioedema in varying degrees of severity that affects quality of life and life itself. from the patients. Lanadelumab is a human monoclonal antibody, a specific inhibitor of plasma kallikrein, approved for long-term prophylaxis of HAE1. Case report: A 59-year-old female patient, diagnosed with HAE 1 since November 1987, without therapeutic response to danazol, fresh frozen plasma, or C1 inhibitor derived from intravenous plasma, requiring 3 to 9 monthly vials of icatibant acetate due to angioedema. laryngeal, cutaneous and visceral with highly altered quality of life indices. Lanadelumab 300 mg subcutaneously every 14 days was started. At the start of treatment, the AECT1 score was 1 point; AE-Qol2: 57 points, AAS3: 32 points, being followed up at 5, 10 and 12 months. After one year of treatment, the records showed an AECT1 of 19 points; AE-Qol2: 36 points and AAS3: 5 points. The requirement for icatibant acetate has been no more than 3 vials per month. Conclusion: In accordance with the literature, lanadelumab offered a significant decrease in angioedema activity and a significantly positive impact on the pa- tient's quality of life, confirming that lanadelumab is an effective option for long-term HAE prophylaxis. .


Antecedentes: El angioedema hereditario tipo 1 (AEH1) es un trastorno autosómico dominante, caracterizado por la carencia cuantitativa y cualitativa del C1 inhibidor, producción excedida de bradicinina y causar angioedema recurrente en diversos grados de severidad que afecta la calidad de vida y la vida misma de los pacientes. Lanadelumab es un anticuerpo monoclonal humano, inhibidor específico de la calicreína plasmática, aprobado para profilaxis a largo plazo del AEH1. Reporte de caso: Paciente femenino de 59 años, con diagnóstico de AEH 1 desde noviembre de 1987, sin respuesta terapéutica a danazol, plasma fresco con- gelado ni C1 inhibidor derivado del plasma intravenoso, requerimiento de 3 a 9 ampolletas mensuales de acetato de icatibant por angioedema laríngeo, cutáneo y visceral con índices de calidad de vida sumamente alterada. Se inició lanadelumab 300 mg cada 14 días subcutánea. Al inicio del tratamiento el puntaje de AECT1 fue de 1 punto; AE-Qol2:57 puntos, AAS3: 32 puntos, realizándose un seguimiento a los 5, 10 y 12 meses. Al año de tratamiento, los registros mostraron un AECT1 de 19 puntos; AE-Qol2: 36 puntos y AAS3: 5 puntos. El requerimiento de acetato de icatibant ha sido a no más de 3 ampolletas por mes. Conclusión: En concordancia a la literatura, lanadelumab ofreció al caso, una importante disminución de la actividad del angioedema y un impacto significativa- mente positivo en la calidad de vida de la paciente, constatando que lanadelumab es una opción eficaz en la profilaxis a largo plazo del AEH.


Asunto(s)
Angioedema , Angioedemas Hereditarios , Femenino , Humanos , Persona de Mediana Edad , Calidad de Vida , Anticuerpos Monoclonales Humanizados/farmacología , Anticuerpos Monoclonales Humanizados/uso terapéutico , Angioedemas Hereditarios/tratamiento farmacológico
3.
Medicina (B Aires) ; 83(5): 772-792, 2023.
Artículo en Español | MEDLINE | ID: mdl-37870335

RESUMEN

Urticaria is a distinctive pattern of inflammatory response of the skin and/or mucous membranes characterized by the sudden appearance of vanishing wheals, angioedema, or both, associated with pruritus. Acute forms are frequent and limited to outbreaks of less than 6 weeks; while the chronic ones have a prevalence of less than 1%, longer duration and can be spontaneous or inducible. The etiopathogenic mechanisms involved in this disease include autoallergy, autoimmunity, and inflammation with cell activation, mainly of the mast cell, leading to its degranulation with the release of vasoactive mediators. Along its approach, diagnostic confirmation, search for indicators of its etiopathogenesis, detection of cofactors that can modulate its activity, recognition of comorbidities, evaluation of possible biomarkers and the assessment of disease activity, impact and control are essential. The pharmacological management aims to control the symptoms, until the urticaria, which is self-resolving, is gone. This is described in a stepwise fashion with increasing complexity.


La urticaria es un patrón distintivo de respuesta inflamatoria de piel y/o mucosas caracterizada por la aparición súbita de ronchas evanescentes, angioedema o ambos, asociados a prurito. Las formas agudas son frecuentes y se limitan a brotes de menos de 6 semanas; mientras que las crónicas tienen una prevalencia menor al 1%, mayor duración y pueden ser espontáneas o inducibles. Los mecanismos etiopatogénicos involucrados en esta enfermedad incluyen la autoalergia, la autoinmunidad y la inflamación con la activación celular, principalmente del mastocito, lo que lleva a su degranulación con liberación de mediadores vasoactivos. En su abordaje son fundamentales la confirmación diagnóstica; la búsqueda de indicadores de su etiopatogenia; la detección de cofactores que pueden modular su actividad; el reconocimiento de comorbilidades; la evaluación de posibles biomarcadores y, el impacto en la calidad de vida, el registro de la actividad y el control de la enfermedad. El manejo farmacológico tiene por objetivo controlar los síntomas, mientras la urticaria resuelve de forma espontánea. Este se describe de forma escalonada con una complejidad creciente.


Asunto(s)
Angioedema , Urticaria Crónica , Urticaria , Humanos , Urticaria/diagnóstico , Urticaria/tratamiento farmacológico , Urticaria/etiología , Urticaria Crónica/diagnóstico , Urticaria Crónica/tratamiento farmacológico , Enfermedad Crónica
4.
Digit J Ophthalmol ; 29(2): 40-44, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37727462

RESUMEN

A 59-year-old woman presented with a 24-hour history of severe, bilateral, painless vision loss starting 1 day after immediately sequential, bilateral, phacorefractive surgery with multifocal intraocular lens (IOL) implantation at another institution. Best-corrected visual acuity was counting fingers at 1 foot in the right eye and 20/100 in the left eye. Slit-lamp evaluation showed a dense fibrin membrane on the anterior surface of the IOL and significant anterior chamber inflammation in both eyes. B-scan ultrasound revealed bilateral vitreous haze, without membrane formation. The rapid onset, absence of sharp pain, ciliary injection, conjunctival chemosis, eyelid edema, and erythema raised suspicion for bilateral toxic anterior segment syndrome. Significant clinical improvement after high-dose prednisone and hourly prednisolone acetate eye drops supported the diagnosis. After 6 months of tapering anti-inflammatory therapy, optical coherence tomography of the macula showed no pathologic changes, and the patient's best-corrected visual acuity improved to 20/25 in both eyes.


Asunto(s)
Angioedema , Oftalmopatías , Femenino , Humanos , Persona de Mediana Edad , Ceguera , Cámara Anterior , Afecto
5.
Arch Argent Pediatr ; 121(6): e202202850, 2023 12 01.
Artículo en Inglés, Español | MEDLINE | ID: mdl-37342978

RESUMEN

Introduction. The oral food challenge (OFC) for the diagnosis of cow's milk protein allergy (CMPA) poses risks and requires resources. Our objective was to assess conditions and complementary tests used to identify a high probability of CMPA. Population and methods. Secondary analysis of a study of patients seen at a unit of allergy between 2015 and 2018. Pre-testing probabilities associated with symptoms and their combinations and post-testing probabilities after skin prick testing and serum immunoglobulin E (IgE) levels were determined. Results. The data from 239 patients were assessed. A probability greater than 95% was observed for angioedema and a combination of urticaria and vomiting. Based on the cut-off points proposed by Calvani et al., the combination of vomiting with rhinitis, without angioedema, also exceeded 95%. Conclusion. A methodology is provided to identify patients in whom CMPA may be diagnosed without an OFC.


Introducción. La prueba de provocación oral (PPO) para el diagnóstico de alergia a las proteínas de la leche de la vaca (APLV) presenta riesgos y requiere de recursos. Nuestro objetivo fue evaluar condiciones y pruebas complementarias para identificar una alta probabilidad de APLV. Población y métodos. Análisis secundario sobre estudio de pacientes atendidos en una unidad de alergia entre 2015 y 2018. Se determinaron las probabilidades prepruebas asociadas a los síntomas y sus combinaciones, y las probabilidades pospruebas luego de realizadas pruebas cutáneas y determinación de inmunoglobulina E (IgE) sérica. Resultados. Se evaluó la información de 239 pacientes. Se observaron probabilidades mayores al 95 % en pacientes con angioedema y combinación de urticaria y vómitos. Usando puntos de corte propuestos por Calvani et al., la combinación de vómitos con rinitis, sin angioedema, también superó el 95 %. Conclusión. Se ofrece una metodología para identificar pacientes en los que puede diagnosticarse APLV sin realización de PPO.


Asunto(s)
Angioedema , Hipersensibilidad a la Leche , Femenino , Animales , Bovinos , Humanos , Lactante , Hipersensibilidad a la Leche/diagnóstico , Hipersensibilidad a la Leche/epidemiología , Pruebas Cutáneas/métodos , Angioedema/complicaciones , Proteínas de la Leche/efectos adversos , Vómitos
6.
Braz. J. Anesth. (Impr.) ; 73(2): 223-226, March-Apr. 2023. graf
Artículo en Inglés | LILACS | ID: biblio-1439598

RESUMEN

Abstract The authors report the case of a 71-year-old woman presented to the Emergency Department with acute ischemic stroke. She was treated with rt-PA and interventional endovascular revascularization and developed rapidly progressing angioedema that led to emergency intubation. The standard treatment was not very effective and the swelling improved after infusion of fresh frozen plasma. Angioedema after rt-PA infusion could be a life-threatening emergency that requires quick airway management by skilled professionals. As this condition is triggered by several factors, such as unregulated histamine and bradykinin production, the traditional treatment recommended by the guidelines may not be sufficient and the use of FFP can be considered as a safe and valuable aid.


Asunto(s)
Humanos , Femenino , Persona de Mediana Edad , Anciano , Accidente Cerebrovascular Isquémico/complicaciones , Angioedema/inducido químicamente , Angioedema/terapia , Plasma , Histamina , Manejo de la Vía Aérea
7.
Braz J Anesthesiol ; 73(2): 223-226, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-33932385

RESUMEN

The authors report the case of a 71-year-old woman presented to the Emergency Department with acute ischemic stroke. She was treated with rt-PA and interventional endovascular revascularization and developed rapidly progressing angioedema that led to emergency intubation. The standard treatment was not very effective and the swelling improved after infusion of fresh frozen plasma. Angioedema after rt-PA infusion could be a life-threatening emergency that requires quick airway management by skilled professionals. As this condition is triggered by several factors, such as unregulated histamine and bradykinin production, the traditional treatment recommended by the guidelines may not be sufficient and the use of FFP can be considered as a safe and valuable aid.


Asunto(s)
Angioedema , Accidente Cerebrovascular Isquémico , Femenino , Humanos , Anciano , Accidente Cerebrovascular Isquémico/complicaciones , Angioedema/inducido químicamente , Angioedema/terapia , Manejo de la Vía Aérea , Histamina , Plasma
8.
Braz J Anesthesiol ; 73(4): 500-502, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-34848320

RESUMEN

Acquired angioedema with C1 inhibitor deficiency (AAE-C1INH) is a very rare condition of bradykinin-mediated angioedema. One of its major complications is potentially life-threatening, laryngeal edema. We report a 53-year-old woman with AAE-C1INH proposed for an elective broncofibroscopy. The direct stimulation caused by broncofibroscopy poses a high risk of angioedema, thus presenting an anesthetic challenge. Due to the risk of death, it is essential to adopt preventive measures. Short-term prophylaxis was performed, and the acute treatment was readily available. A well-structured multidisciplinary periprocedural plan makes it possible to safely approach the airway, in a remote area of the hospital.


Asunto(s)
Anestésicos , Angioedema , Angioedemas Hereditarios , Femenino , Humanos , Persona de Mediana Edad , Angioedemas Hereditarios/terapia
10.
Braz. J. Anesth. (Impr.) ; 73(4): 500-502, 2023. graf
Artículo en Inglés | LILACS | ID: biblio-1447618

RESUMEN

Abstract Acquired angioedema with C1 inhibitor deficiency (AAE-C1INH) is a very rare condition of bradykinin-mediated angioedema. One of its major complications is potentially life-threatening, laryngeal edema. We report a 53-year-old woman with AAE-C1INH proposed for an elective broncofibroscopy. The direct stimulation caused by broncofibroscopy poses a high risk of angioedema, thus presenting an anesthetic challenge. Due to the risk of death, it is essential to adopt preventive measures. Short-term prophylaxis was performed, and the acute treatment was readily available. A well-structured multidisciplinary periprocedural plan makes it possible to safely approach the airway, in a remote area of the hospital.


Asunto(s)
Humanos , Femenino , Adulto Joven , Angioedemas Hereditarios/terapia , Anestésicos , Angioedema
13.
An Bras Dermatol ; 97(5): 592-600, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35853771

RESUMEN

BACKGROUND: Chronic Spontaneous Urticaria (CSU) is characterized by recurrent wheals and/or angioedema for longer than 6-weeks. Guidelines recommend Omalizumab (Oma) as first-line and Cyclosporine-A (Cs-A) as second-line treatment in antihistamine resistant CSU. This step-wise algorithm might be time-consuming and costly. OBJECTIVE: To determine indicators of response to Oma or Cs-A in CSU patients. METHODS: We retrospectively analyzed data from seven centers in Turkey; the inclusion criteria for patients were to receive both Oma and Cs-A treatment (not concurrently) at some point in time during their follow-up. Clinical and laboratory features were compared between groups. RESULTS: Among 110 CSU patients; 47 (42.7%) were Oma-responders, 15 (13.6%) were Cs-A-responders, and 24 (21.8%) were both Oma and Cs-A responders and 24 (21.8%) were non-responders to either drug. High CRP levels were more frequent in Cs-A-responders (72.7% vs. 40.3%; p = 0.055). Oma-responders had higher baseline UCT (Urticaria Control Test) scores (6 vs. 4.5; p = 0.045). Responders to both drugs had less angioedema and higher baseline UCT scores compared to other groups (33.3% vs. 62.8%; p = 0.01 and 8 vs. 5; p = 0.017). Non-responders to both drugs had an increased frequency in the female gender and lower baseline UCT scores compared to other groups (87.5% vs. 61.6%; p = 0.017 and 5 vs. 7; p = 0.06). STUDY LIMITATIONS: Retrospective nature, limited number of patients, no control group, the lack of the basophil activation (BAT) or BHRA (basophil histamine release assay) tests. CONCLUSIONS: Baseline disease activity assessment, which considers the presence of angioedema and disease activity scores, gender, and CRP levels might be helpful to predict treatment outcomes in CSU patients and to choose the right treatment for each patient. Categorizing patients into particular endotypes could provide treatment optimization and increase treatment success.


Asunto(s)
Angioedema , Antialérgicos , Urticaria Crónica , Urticaria , Enfermedad Crónica , Ciclosporina , Femenino , Humanos , Omalizumab , Estudios Retrospectivos , Resultado del Tratamiento
14.
Arq. Asma, Alerg. Imunol ; 6(2): 197-213, abr.jun.2022. ilus
Artículo en Inglés, Portugués | LILACS | ID: biblio-1400201

RESUMEN

A urticária crônica é uma condição que afeta mais de um milhão de brasileiros, com grande impacto na qualidade de vida. Mesmo com diretrizes bem difundidas para o seu diagnóstico e tratamento, seu manejo pode ser desafiador em pacientes pediátricos, idosos e gestantes. Para auxiliar o médico especialista nestes casos, o Departamento Científico de Urticária da Associação Brasileira de Alergia e Imunologia elaborou esta revisão com as principais dúvidas e dificuldades referentes ao tema nestes grupos de pacientes.


Chronic urticaria is a condition that affects more than a million Brazilians with a significant impact on quality of life. Although there are well-established guidelines for diagnosis and treatment, the management of chronic urticaria may be challenging in pediatric, older, and pregnant patients. With the purpose of helping specialists manage these cases, the Urticaria Scientific Department of the Brazilian Association of Allergy and Immunology prepared this review with the most common doubts and difficulties about this topic in those patient groups.


Asunto(s)
Humanos , Embarazo , Lactante , Preescolar , Niño , Anciano , Anciano de 80 o más Años , Mujeres Embarazadas , Diagnóstico Diferencial , Omalizumab , Urticaria Crónica , Antagonistas de los Receptores Histamínicos H1 , Pacientes , Médicos , Calidad de Vida , Sociedades Médicas , Terapéutica , Urticaria , Lactancia , Diagnóstico , Alergia e Inmunología , Angioedema
15.
Arq. Asma, Alerg. Imunol ; 6(2): 214-224, abr.jun.2022. ilus
Artículo en Inglés, Portugués | LILACS | ID: biblio-1400202

RESUMEN

A urticária aguda é uma causa frequente de consulta com alergistas, caracterizada por urticas e/ou angioedema. Embora autolimitada e benigna, pode causar desconforto significativo e raramente representar uma doença sistêmica grave ou reação alérgica com risco de vida. Nesta revisão, elaborada pelo Departamento Científico de Urticária da Associação Brasileira de Alergia e Imunologia, foram abordadas as principais questões referentes ao tema para auxiliar o médico especialista e generalista.


Acute urticaria is a frequent cause of consultations with allergists, being characterized by wheals and/or angioedema. Although self-limited and benign, it may cause significant discomfort and uncommonly represent a serious systemic disease or life-threatening allergic reaction. In this review prepared by the Urticaria Scientific Department of the Brazilian Association of Allergy and Immunology, the main questions about this topic are addressed to help specialists and general practitioners.


Asunto(s)
Humanos , Urticaria , Epinefrina , Hipersensibilidad a la Leche , Hipersensibilidad al Huevo , Hipersensibilidad a las Drogas , Hipersensibilidad a los Mariscos , Hipersensibilidad a Nueces y Cacahuetes , Antagonistas de los Receptores Histamínicos H1 , Anafilaxia , Picaduras de Arañas , Médicos , Sociedades Médicas , Terapéutica , Antiinflamatorios no Esteroideos , Síndrome de Sweet , Dermatitis Alérgica por Contacto , Corticoesteroides , Síndrome Hipereosinofílico , Síndrome de Schnitzler , Mastocitosis Cutánea , Diagnóstico , Alergia e Inmunología , Eritema , Angioedemas Hereditarios , Hipersensibilidad a los Alimentos , Alergólogos , Hipersensibilidad , Angioedema
16.
Rev Soc Bras Med Trop ; 55: e00632022, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35613221

RESUMEN

Mass vaccination campaigns are essential to control the ongoing novel severe acute respiratory syndrome coronavirus 2 (SARS-Cov-2) pandemic. The Covishield vaccine consists of the replication-deficient simian adenovirus vector ChAdOx1, which contains the full-length structural spike protein of SARS-CoV-2. Occasionally, it can lead to cutaneous reactions that contribute to fear of vaccination, hesitancy, and incomplete vaccination schedules. We report a case of facial angioedema following the first dose of Covishield in a 63-year-old woman with no previous history of allergies or hypersensitivity to drugs or vaccines. No rebound of angioedema was recorded after the second homologous and third heterologous doses.


Asunto(s)
Angioedema , COVID-19 , ChAdOx1 nCoV-19 , Adenoviridae , Angioedema/inducido químicamente , Anticuerpos Antivirales , COVID-19/prevención & control , ChAdOx1 nCoV-19/efectos adversos , Femenino , Estudios de Seguimiento , Humanos , Persona de Mediana Edad , SARS-CoV-2 , Vacunación/efectos adversos
17.
Iran J Allergy Asthma Immunol ; 21(1): 98-100, 2022 Feb 06.
Artículo en Inglés | MEDLINE | ID: mdl-35524384

RESUMEN

Cold-induced urticaria is considered as a subtype of physical urticaria and also the second most common type of chronic inducible urticaria. Contact with cold surfaces or the environment may cause systemic reactions, especially during aquatic activities. A 22-year-old female patient with a history of sulfa drug allergy began her condition 2 years before the presence of generalized pruritic erythema with hives as well as 2 episodes that had been characterized by facial angioedema and syncope 3-5 minutes after being in contact with cold air or surfaces.  On both events, she had just been outdoors on a cold, winter day. She was suspected to have cold-induced urticaria; thereby she had a positive reaction to the ice cube test. Due to the previous episodes of anaphylaxis, the patient was trained to administer intramuscular epinephrine. After 4 weeks of starting the treatment with antihistamines, no new events or injuries had occurred. Cold-induced urticaria may cause life-threatening reactions. The rate of anaphylaxis in these patients is low however, this case is presented to inform the importance of identifying this type of systemic reaction and preventing strategies.


Asunto(s)
Anafilaxia , Angioedema , Urticaria Crónica , Urticaria , Adulto , Anafilaxia/diagnóstico , Anafilaxia/etiología , Angioedema/diagnóstico , Angioedema/tratamiento farmacológico , Angioedema/etiología , Cara , Femenino , Humanos , Urticaria/diagnóstico , Urticaria/etiología , Adulto Joven
18.
Int Arch Allergy Immunol ; 183(5): 572-577, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35325890

RESUMEN

BACKGROUND: Acquired deficiency of C1 inhibitor (AAE-C1-INH) is a very rare cause of recurrent angioedema, with few cases reported in the literature. We aimed to describe a series of patients with AAE-C1-INH who were diagnosed and received care at angioedema reference centers in Brazil, affiliated to the Brazilian Group of Studies on Hereditary Angioedema. METHODS: Fourteen patients from 8 Brazilian Angioedema Reference Centers, diagnosed with AAE-C1-INH, were included in this study. Clinical data collected included sex, date of birth, date of onset of symptoms, date of diagnosis, plasma levels of antigenic and/or functional C1-INH, levels of C4 and C1q, location and treatment of angioedema attacks, long-term prophylaxis, associated diseases, and definitive treatment. RESULTS: Fourteen patients were identified with AAE-C1-INH. Most patients (10/14; 71.4%) were female. The median age at onset of symptoms was 56.5 years (range, 14-74 years; interquartile range [IQR], 32-64 years), and median age at diagnosis was 58.0 years (range, 20-76 years; IQR, 38-65 years), with a median time until diagnosis of 2 years (range, 0-6 years; IQR, 1-3 years). The most common manifestations were cutaneous (face, eyelids, lips, trunk, hands, feet, and genitals). Most patient had low levels of C4 (13/14; 92.8%) and of antigenic C1-INH (8/14; 57.1%). Four had decreased functional activity of C1-INH (4/7; 57.1%) and C1q levels were low in 5 patients (5/12; 41.6%). Underlying diseases were identified in all 14 patients, with lymphoma of the splenic marginal zone and monoclonal gammopathy of undetermined significance being the most frequent. Nine patients (64.2%) needed long-term prophylactic treatment for recurrent angioedema and 5 patients (46.7%) required treatment for angioedema attacks. Most of them (12/14; 85.7%) had resolution of angioedema. CONCLUSION: Therapy of AAE-C1-INH aims to control symptoms; however, diagnosis and treatment of the underlying disease, when present, should be an important target and may lead to the resolution of angioedema in patients with AAE-C1-INH.


Asunto(s)
Angioedema , Angioedemas Hereditarios , Adolescente , Adulto , Anciano , Angioedema/diagnóstico , Angioedema/etiología , Angioedemas Hereditarios/terapia , Brasil/epidemiología , Proteína Inhibidora del Complemento C1/genética , Complemento C1q/uso terapéutico , Femenino , Humanos , Masculino , Persona de Mediana Edad , Adulto Joven
19.
Arq. Asma, Alerg. Imunol ; 6(1): 108-115, jan.mar.2022. ilus
Artículo en Inglés, Portugués | LILACS | ID: biblio-1400116

RESUMEN

Introdução: A urticária crônica é uma doença com prevalência em pelo menos 0,1% da população, definida pela presença de pápulas pruriginosas, angioedema ou ambos por período superior a seis semanas. Os pacientes com urticária crônica têm um severo prejuízo na qualidade de vida. Objetivo: Avaliar o impacto da urticária crônica na qualidade de vida dos portadores da doença dentro de um serviço especializado no estado de Sergipe. Métodos: Trata-se de um estudo descritivo observacional a partir de dados coletados de 40 pacientes atendidos, em 2021, no Serviço de Alergia e Imunologia do Ambulatório de Alergia e Imunologia do Decós Day Hospital, através de dois questionários específicos para a avaliação da qualidade de vida na urticária crônica: o Chronic Urticaria Quality of Life Questionnaire e o Urticaria Control Test. Resultados: Foi possível identificar uma correlação positiva, através do questionário Urticaria Control Test, entre a intensidade dos sintomas e a piora da qualidade de vida (r = 0,774; p < 0,001). Também foi possível identificar uma correlação positiva entre a intensidade dos sintomas e a piora da qualidade de vida, desta vez mensurada pela escala Chronic Urticaria Quality of Life Questionnaire (r = 0,768; p < 0,001). Noventa por cento dos pacientes afirmaram se sentir cansados durante o dia porque não dormiram bem, 87,5% sentem dificuldade para se concentrar, 90% sentem-se nervosos, 80% afirmaram sentirem-se para baixo, 75% disseram ter vergonha das lesões da urticária que aparecem no corpo, e 60% tem vergonha de frequentar lugares públicos. Conclusões: A urticária crônica compromete a qualidade de vida, medida pelos questionários Urticaria Control Test e Chronic Urticaria Quality of Life Questionnaire. O comprometimento da qualidade de vida dos doentes com urticária crônica ocorre principalmente nos aspectos psicológicos, nos relacionamentos sociais e na qualidade do sono.


Introduction: Chronic urticaria is a disease with a prevalence in at least 0.1% of the population, defined by the presence of pruritic papules, angioedema or both for a period longer than six weeks. Patients with chronic urticaria have a severe loss in quality of life. Objective: To assess the impact of chronic urticaria on the quality of life of patients with the disease within a specialized service in the state of Sergipe. Methods: This is a descriptive observational study based on data collected from 40 patients treated, in 2021, at the Allergy and Immunology Service of the Allergy and Immunology Outpatient Clinic of Decós Day Hospital, using two specific questionnaires for quality assessment of life in chronic urticaria: the Chronic Urticaria Quality of Life Questionnaire and the Urticaria Control Test. Results: It was possible to identify a positive correlation, through the Urticaria Control Test questionnaire, between the intensity of symptoms and the worsening of quality of life (r = 0.774, p < 0.001). It was also possible to identify a positive correlation between the intensity of symptoms and worsening quality of life, this time measured by the Chronic Urticaria Quality of Life Questionnaire scale (r = 0.768, p < 0.001). 90% said they felt tired during the day because they didnt sleep well, 87.5% found it difficult to concentrate, 90% felt nervous, 80% said they felt down, 75% said they were ashamed of the urticaria lesions that appear on the body and 60% are ashamed to go to public places. Conclusions: Chronic urticaria compromises quality of life, as measured by the Urticaria Control Test and the Chronic Urticaria Quality of Life Questionnaire. The impairment of the quality of life of patients with chronic urticaria occurs mainly in the psychological aspects, in social relationships and in the quality of sleep.


Asunto(s)
Humanos , Calidad de Vida , Urticaria Crónica , Pacientes , Vergüenza , Signos y Síntomas , Sueño , Estudios Transversales , Encuestas y Cuestionarios , Alergia e Inmunología , Calidad del Sueño , Angioedema
20.
Rev Esp Enferm Dig ; 114(6): 365-366, 2022 06.
Artículo en Inglés | MEDLINE | ID: mdl-35118870

RESUMEN

We present the case of a patient with small bowel angioedema induced by iodinated contrast media during computed tomography. It is important to know this entity and to differentiate it from other intestinal diseases in order to avoid inappropriate treatment.


Asunto(s)
Angioedema , Enfermedades Intestinales , Angioedema/inducido químicamente , Angioedema/diagnóstico por imagen , Medios de Contraste/efectos adversos , Humanos , Enfermedades Intestinales/inducido químicamente , Intestino Delgado , Tomografía Computarizada por Rayos X
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