RESUMEN
BACKGROUND: Hereditary angioedema (HAE) is known for mortality when it is not treated properly. Many advances have occurred over the past decades that affected patients' lives. However, not all patient populations have access to the same diagnosis and treatment resources. OBJECTIVE: To evaluate mortality from HAE in a large cohort in a reference center in Brazil. Furthermore, the research intended to describe patients' life span, the asphyxia evolution, and factors related to the fatal outcome. METHODS: A cohort of 433 patients from 46 families was evaluated in this prospective and retrospective study. Families were organized in clusters and were given a verbal autopsy to arrange data collection for the deaths and analyze symptoms during life. Causes of death were classified as deaths from laryngeal edema (LE) or other causes. RESULTS: Of 433 patients evaluated, 254 were not given the diagnosis of HAE. A total of 75 fatal events were evaluated. Only 10 of 75 patients were given the diagnosis of HAE before death, and the HAE diagnosis was made after death in 65 of 75 patients using verbal autopsy. The final cause of death after the investigation was asphyxiation owing to LE in 39 of 75 (52%) and deaths owing to other causes in 36 of 75 (48%). Ten deaths had occurred in the past decade. Time from onset of symptoms to seeking medical assistance was a median of 4 hours, and the time to death was a median of 8 hours. Three patients received fresh-frozen plasma and none received medications specific to HAE attacks. Throat pain or discomfort was the most common symptom, experienced by 71.8% of patients. The most common mistaken diagnosis at the original death certificate was allergy or anaphylaxis. The life span of patients who died of LE was reduced by 20 years compared with those who died of other causes. CONCLUSIONS: Hereditary angioedema remains a threat to life in the studied population. The large number of patients who do not receive a diagnosis makes the situation even more severe and is responsible for most deaths. Death analyses add knowledge to an understanding of the diseases and their impact on patients' lives, improving the targeting of public health efforts.
Asunto(s)
Angioedemas Hereditarios , Edema Laríngeo , Angioedemas Hereditarios/complicaciones , Angioedemas Hereditarios/diagnóstico , Angioedemas Hereditarios/mortalidad , Asfixia , Brasil/epidemiología , Humanos , Edema Laríngeo/etiología , Edema Laríngeo/mortalidad , Estudios Prospectivos , Estudios RetrospectivosRESUMEN
This study aims to review the global mortality secondary to laryngeal edema in patients diagnosed with hereditary angioedema and their relatives over the years, as well as to describe epidemiological and clinical findings associated with this outcome. An extensive search of the literature was made in PubMed, Scopus, and Embase to identify mortality rates secondary to laryngeal edema in patients with hereditary angioedema. The search was carried out in September of 2020 and in April of 2021, and keywords based on the MeSH terms were searched in three databases. The filter of language was used for finding only articles in English, and there was no limit to the year of publication. A total of twenty-three articles fulfilled the inclusion criteria for review and data extraction. The analyzed studies included 3292 patients and 411 deaths from asphyxia due to laryngeal edema. One hundred and three deaths in close relatives were described as secondary to the same cause. The main findings were summarized in tables: year and place of publication, the number of patients and deaths from laryngeal edema, patients previously diagnosed, and death age. Death rates from laryngeal edema had an average of one death for every 20 patients. Eight studies reported deaths in relatives. For every 7.4 patients in these studies, one relative died. The percentage among deaths in general associated with laryngeal edema was evaluated in three studies (32.7%, 44.4%, and 56%). The high frequency of this outcome suggests that deaths still occur, and improvement of hereditary angioedema treatment still needs to be met.
Asunto(s)
Angioedemas Hereditarios , Edema Laríngeo , Angioedemas Hereditarios/tratamiento farmacológico , Angioedemas Hereditarios/epidemiología , Proteínas Inactivadoras del Complemento 1 , Proteína Inhibidora del Complemento C1/uso terapéutico , Humanos , Edema Laríngeo/tratamiento farmacológico , Edema Laríngeo/etiologíaRESUMEN
Introducción: el edema de Reinke es la acumulación de fluidos en la capa externa de la lámina propia de las cuerdas vocales. Produce disfonía y raras veces obstrucción respiratoria. Las etiologías más frecuentes son el tabaquismo, el reflujo gastroesofágico y el mal uso y abuso vocal. Objetivos: determinar, mediante tratamiento quirúrgico, la tasa de resolución de la disnea inspiratoria severa provocada por edema de Reinke bilateral de cuerdas vocales. Diseño: estudio descriptivo y retrospectivo. Material y métodos: revisión de las historias clínicas electrónicas de todos los pacientes que consultaron y fueron tratados por disnea inspiratoria severa provocada por edema de Reinke bilateral de las cuerdas vocales, en el servicio de Otorrinolaringología del Hospital Italiano de Buenos Aires, entre febrero de 2007 y abril de 2015. Resultados: fueron tratados 4 pacientes de sexo femenino que consultaron por disnea inspiratoria severa. Fumaban más de 30 cigarrillos por día. La técnica quirúrgica consistió en resecar todo el edema polipoideo en forma bilateral, preservando el borde libre de las cuerdas vocales. Conclusiones: el edema de Reinke obstructivo es una patología infrecuente. La tasa de resolución de la disnea inspiratoria severa en las cuatro enfermas tratadas fue del100%. La resección total del edema y de la mucosa excedente, preservando un pequeño sector para que recubra el borde libre de la cuerda vocal (cordectomía vs. cordotomía), fue la técnica quirúrgica preferida. (AU)
Introduction: the Reinke edema is an accumulation of fluid in the outer layer of the lamina propria of the vocal cords. Causes dysphonia and rarely produces respiratory obstruction. Objectives: to determine the rate of resolution of the severe inspiratory dyspnea caused by bilateral Reinke edema of vocal cords with surgical treatment. Design: descriptive and retrospective study. Material and methods: review of the electronic medical records of all patients who consulted and were treated for severe inspiratory dyspnea caused by bilateral Reinke edema of the vocal chords in the Hospital Italiano de Buenos Aires between February 2007 and April 2015. Results: four women were treated, consulted for severe inspiratory dyspnea. Smoked more than 30 cigarettes per day. The surgical technique consisted in to resect all the bilateral polypoid edema, while preserving the free edge of the vocal cords. Conclusions: the obstructive Reinke edema is an infrequent pathology. The rate of resolution of the severe inspiratory dyspnea in the four patients treated was 100%. The total resection of the edema and mucosa excess, preserving a small sector to cover the free edge of the vocal cord (cordectomy vs cordotomy) was the preferred surgical technique. (AU)
Asunto(s)
Humanos , Femenino , Anciano , Edema Laríngeo/cirugía , Edema Laríngeo/patología , Signos y Síntomas Respiratorios , Tabaquismo/complicaciones , Reflujo Gastroesofágico/complicaciones , Edema Laríngeo/etiología , Edema Laríngeo/fisiopatología , Edema Laríngeo/diagnóstico por imagen , Epidemiología Descriptiva , Corticoesteroides/uso terapéutico , Disnea/cirugía , Disnea/diagnóstico , Inhibidores de la Bomba de Protones/uso terapéutico , Disfonía , Disfunción de los Pliegues Vocales/complicaciones , Disfunción de los Pliegues Vocales/terapiaRESUMEN
OBJECTIVE: To assess the behavior of the immunoexpression of protein p53 in Reinke's edema and laryngeal squamous cell carcinoma. STUDY DESIGN: retrospective. METHODS: we recovered the histological paraffin blocks of patients who were subjected to Reinke's edema and laryngeal squamous cell carcinoma surgery in 2000-2011. The paraffin blocks were cut into 3-µm sections; the specimens were prepared in silanized slides (one slide for each paraffin block) and subjected to immunohistochemical reaction according to the Avidin Biotin Peroxidase method. Monoclonal primary anti-p53 antibodies were used at 1:50 dilution. Slides were examined under a light microscope at different magnitudes and results were interpreted based on the degree of brown staining in the nuclei of epithelial cells and in the extent of the fragment by using a semi-quantitative score from 0 to 3. RESULTS: 67 slides of Reinke's edema and 60 slides of laryngeal squamous cell carcinoma were included. Scores 2 and 3 for staining of the nuclei of epithelial cells were recorded for 46 slides of Reinke's edema (68.65%) and for 57 slides of laryngeal squamous cell carcinoma (95%). As to the extent of the fragment, scores 2 and 3 were recorded for 74% slides of Reinke's edema and for 95% slides of carcinomas. CONCLUSION: the positive immunoexpression for protein p53, positive in 95% carcinomas and 74% Reinke's edemas, makes us aware of the possible preneoplastic condition of the latter lesion. Further studies are needed to identify and reveal the genetic changes that lead to these results.
Asunto(s)
Carcinoma de Células Escamosas/metabolismo , Edema Laríngeo/metabolismo , Neoplasias Laríngeas/metabolismo , Lesiones Precancerosas/metabolismo , Fumar/metabolismo , Proteína p53 Supresora de Tumor/biosíntesis , Adulto , Anciano , Carcinoma de Células Escamosas/etiología , Carcinoma de Células Escamosas/patología , Femenino , Humanos , Inmunohistoquímica , Edema Laríngeo/etiología , Edema Laríngeo/patología , Mucosa Laríngea/metabolismo , Mucosa Laríngea/patología , Neoplasias Laríngeas/etiología , Neoplasias Laríngeas/patología , Masculino , Persona de Mediana Edad , Lesiones Precancerosas/etiología , Lesiones Precancerosas/patología , Estudios Retrospectivos , Fumar/efectos adversos , Fumar/patologíaRESUMEN
PURPOSE: This prospective randomized double-blind placebo-controlled study paired trial groups to investigate the effect of intravenous dexamethasone and nebulized L-epinephrine on the clinical development of postextubation laryngeal edema. MATERIAL AND METHODS: Sixty-four patients aged 1 day to 12 years who needed intubation from August/1997 to February/1999 in the Pediatric Intensive Care Unit (PICU) of Santa Casa de São Paulo School of Medicine were paired according to age, height and weight, anthropometric z-scores, duration of tracheal intubation and number of intubation attempts. Patients received intravenous dexamethasone (0.2mg/kg every 6h), with or without nebulized L-epinephrine (0.5mg/kg every 4h) in the study groups; in the control groups, they received nebulized or intravenous isotonic saline solution, or both. DEPENDENT VARIABLES: frequency and severity of the laryngeal edema (according to Westley Clinical Scoring System), as well as the time interval (min) between extubation and the first postextubation laryngeal edema scores. Postextubation respiratory rate and transcutaneous oxygen saturation were employed as indicators of the clinical conditions for extubation. RESULTS: Laryngeal edema frequencies did not change according to treatments, chi(2) (3, N=64)=0.58, p>0.05, and the highest scores were not reached in the 24h after extubation, chi(ranks)(2) (3, N=16)=1.03, p>0.05. CONCLUSIONS: Dexamethasone and l-epinephrine did not reduce the clinical progression of airway obstruction due to laryngeal edema in the early postextubation period.
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Obstrucción de las Vías Aéreas/etiología , Obstrucción de las Vías Aéreas/prevención & control , Dexametasona/administración & dosificación , Epinefrina/administración & dosificación , Intubación Intratraqueal/efectos adversos , Administración por Inhalación , Niño , Preescolar , Enfermedad Crítica/terapia , Relación Dosis-Respuesta a Droga , Método Doble Ciego , Esquema de Medicación , Quimioterapia Combinada , Femenino , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Inyecciones Intravenosas , Unidades de Cuidado Intensivo Pediátrico , Intubación Intratraqueal/métodos , Edema Laríngeo/etiología , Edema Laríngeo/prevención & control , Masculino , Probabilidad , Estudios Prospectivos , Valores de Referencia , Medición de Riesgo , Resultado del TratamientoRESUMEN
INTRODUCTION: mechanical ventilation is used in all Neonatal Intensive Care Unit (NICU). The patients with more than a week with mechanical ventilation can develop complications in the airway. The diagnosis is carrying out with a bronchoscope. OBJECTIVE: to determine the type of complications presented in a group of neonates who had received, one o more weeks, mechanical ventilation, through bronchoscopy. METHODS: we realized a retrospective reviewed of the charts of patients with mechanical ventilation in a NICU during a one-year period and whom a bronchoscopy was carried out for respiratory distress after extubation. We analyzed; the type of lesion, age of gestation and birth weight, gender, mechanical ventilation days, atelectasis, age and days of extra uterine life in the moment of the bronchoscopy. In the variables we achieved frequency, averages, media and standard deviations. RESULTS: we reviewed 55 charts. The mean gestational age was 34 weeks, birth weight 2075 g and 38 days with mechanical ventilation. Male 52.7 %. Atelectasis in 78.7 %, a third of the patients required mechanical ventilation after extubation. The airway lesions were (%); bronchial stenosis 25.4, laryngotracheobronchitis 18.1, laryngeal edema 10.9, laryngotracheitis 7.2, laryngomalacia 7.2 %, ulcer 7.2, cord paralysis 5.4, granulom 3.6 and normal 3.6. Three required tracheotomy. CONCLUSION: subglotic stenosis was the complications more frequent. Ideally all the neonates after mechanical ventilation shoud realized a bronchoscopy for preventing complications.
Asunto(s)
Enfermedades de la Laringe/etiología , Respiración Artificial/efectos adversos , Enfermedades de la Tráquea/etiología , Bronquitis/diagnóstico , Bronquitis/etiología , Broncoscopía , Femenino , Humanos , Lactante , Recién Nacido , Enfermedades de la Laringe/diagnóstico , Edema Laríngeo/diagnóstico , Edema Laríngeo/etiología , Laringoestenosis/diagnóstico , Laringoestenosis/etiología , Masculino , Estudios Retrospectivos , Enfermedades de la Tráquea/diagnósticoRESUMEN
The Allergo-vigilance Network including 200 French allergologists and 28 European ones, has three objectives: to index the cases of lethal or prelethal anaphylaxis, to evaluate the prevalence of food allergies, clinical pictures and allergens, and to implement the post-marketing surveillance of the allergic risk of novel foods. The first survey dealing with life-threatening food allergies leads to an estimation of 15,000 to 30,000 reactions per year in France. The AVN could be a helpful tool for public health organizations.
Asunto(s)
Anafilaxia/epidemiología , Bases de Datos Factuales , Hipersensibilidad a los Alimentos/epidemiología , Encuestas Epidemiológicas , Edema Laríngeo/epidemiología , Vigilancia de la Población , Adulto , África del Norte/epidemiología , Anafilaxia/etiología , Arachis/efectos adversos , Argentina/epidemiología , Asma/epidemiología , Asma/etiología , Niño , Reacciones Cruzadas , Proteínas en la Dieta/efectos adversos , Europa (Continente)/epidemiología , Francia/epidemiología , Frutas/efectos adversos , Humanos , Edema Laríngeo/etiología , Hipersensibilidad al Látex/epidemiología , Martinica/epidemiología , Nueces/efectos adversos , Prevalencia , Reunión/epidemiología , Estados Unidos/epidemiologíaRESUMEN
BACKGROUND: The purpose of our study was to ascertain the causes for early reintervention after thyroidectomy performed by a surgical team using a systematized surgical technique. MATERIAL/METHODS: We analyzed 1131 patients, 939 (83.1%) women and 192 (16.9%) men, average age 38.7 years (range 12 to 79). Of these patients, there were 675 hemithyroidectomies with isthmusectomy (59.74%), 189 subtotal thyroidectomies (16.71%), and 267 total thyroidectomies, alone or with regional lymphatic dissection at levels VI and VII (23.55%). Statistical analysis was performed by main tendency measures and chi square (chi-squared) for comparison of two independent samples; the dependent variable was the rate of early reintervention, while the independent variables included causes, time of presentation, hormonal functional state and extent of surgery. RESULTS: Early reintervention was necessary in 11 cases (0.97%). 9 were due to hematoma (0.79%) resolved with drainage and hemostasis, and two (0.18%) due to acute respiratory failure (ARF) caused by laryngeal edema, resolved by tracheostomy. Analysis based on diagnosis, extent of surgery and functional state failed to reveal statistically significant differences. The maximum time presentation of complications was 6 hours. CONCLUSIONS: The most intense postoperative monitoring is necessary during the first six hours. The low frequency of early reintervention and the appearance of complications in less than 8 hours enable thyroid surgery to be performed on a short-stay basis with adequate safety margins.
Asunto(s)
Drenaje/estadística & datos numéricos , Hematoma/cirugía , Edema Laríngeo/cirugía , Complicaciones Posoperatorias/cirugía , Insuficiencia Respiratoria/cirugía , Tiroidectomía , Traqueostomía/estadística & datos numéricos , Adolescente , Adulto , Anciano , Niño , Grupos Diagnósticos Relacionados , Femenino , Hematoma/etiología , Técnicas Hemostáticas , Humanos , Edema Laríngeo/etiología , Tiempo de Internación , Estudios Longitudinales , Masculino , Persona de Mediana Edad , Cuidados Posoperatorios , Estudios Prospectivos , Distribución Aleatoria , Traumatismos del Nervio Laríngeo Recurrente , Insuficiencia Respiratoria/etiología , Seguridad , Tiroidectomía/métodos , Tiroidectomía/estadística & datos numéricos , Factores de TiempoRESUMEN
OBJECTIVE: To report on the results of using a lateral thyrotomy approach on the paraglottic space to gain greater access for laryngocele resection under direct vision. STUDY DESIGN: A 26-year prospective and retrospective study. The study was conducted on 10 adult patients (5 men and 5 women) who had laryngocele of varying size on the paraglottic space. Six of the patients had internal laryngocele and four had exteriorized laryngocele. Five laryngoceles were left-sided, three were right-sided, and two were bilateral. METHODS: A V-shaped, full-thickness thyroid lamina resection with the triangle base at the superior border and the apex at a point midway of the thyroid lamina vertical extent was performed. RESULTS: A V-shaped lateral thyrotomy made exposure to the paraglottic space possible for direct submucosal laryngocele dissection. This approach has presented no complications to date. Postoperative minor edema or hematoma was found in the aryepiglottic and ventricular folds, but this disappeared within a few days. There was no recurrence; the minimum follow-up was 1 year. CONCLUSION: The triangular lateral thyrotomy approach provided access to the paraglottic space and superb visibility for resection of laryngocele of any size under direct vision, thus avoiding recurrence, morbidity, and complications.
Asunto(s)
Glotis/cirugía , Laringe/anomalías , Cartílago Tiroides/cirugía , Adolescente , Adulto , Anciano , Disección/efectos adversos , Epiglotis/patología , Femenino , Estudios de Seguimiento , Hematoma/etiología , Humanos , Enfermedades de la Laringe/etiología , Edema Laríngeo/etiología , Laringe/patología , Laringe/cirugía , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Recurrencia , Estudios RetrospectivosRESUMEN
Se presenta un caso de paciente se sexo masculino de 17 años, portador de Sarcoidosis localizada en laringe (supraglotis), con la metodología de estudio, diagnóstico y tratamiento empleado, mostrando una remisión lenta, pero estable. Además se hace una revisión de dicha patología en cuanto a sus características generales, diagnóstico, histopatología y alternativas terapéuticas
Asunto(s)
Humanos , Masculino , Adolescente , Enfermedades de la Laringe/diagnóstico , Sarcoidosis/diagnóstico , Corticoesteroides/uso terapéutico , Edema Laríngeo/diagnóstico , Edema Laríngeo/etiología , Edema Laríngeo/tratamiento farmacológico , Laringoscopía , Laringe/patología , Sarcoidosis/tratamiento farmacológicoRESUMEN
Se describen las complicaciones no infecciosas en niños tratados con asistencia mecánica de la ventilación. Desde noviembre de 1990 a junio de 1993 (32 meses) ingresaron 459 pacientes entre 12 días y 14 años de edad a la unidad de tratamiento intensivo pediátrica del Hospital Clínico de la Universidad Católica, 225 (49,01 por ciento) de los cuales fueron tratados con ventilación mecánica. El motivo del empleo de respirador mecánico fue cirugía cardíaca en 76 casos, bronconeumonía o enfermedad pulmonar difusa en 43, choque séptico 23, meningoencefalitis bacteriana aguda en 17, cirugía del sistema nervioso central en 16, estado convulsivo prolongado en 11, cirugía general en 7 y politraumatismo en 6 pacientes. Se registraron 57 complicaciones (25,33 por ciento) no infecciosas de la ventilación mecánica, edema subglótico postextubación en 18 casos, barotrauma en 14, entubación de un bronquio en 7, extubación accidental, oclusión de tubo y atelectasias en 5 cada una, fibrilación ventricular al cambiar tubo endotraqueal, hipercapnia por filtro grande y rotura del tubo endotraqueal, todas con un caso. Las complicaciones relacionadas con el uso de vía aérea artificial correspondían a 73,7 por ciento (42/57) y las secundarias al empleo de ventilación mecánica (barotrauma y atelectasias) a 26,3 por ciento (15/57). La forma de presentación más frecuente del barotrauma, que se registró en 14 casos fue el neumotórax (10 casos ). De los 14 pacientes con baro trauma 9 fallecen. En todos los pacientes que presentaron barotrauma, se usó presiones inspiratorias máximas superiores a 40 cm de H2O. Los promedios de presión inspiratoria máxima y presión positiva de final espiración (56,5 ñ 12,5 cm H2O y 11,7 ñ 4,2 cm de H2O respectivamente) de los pacientes con barotrauma fueron mayores que en sus iguales sin la complicación.La mortalidad general de la unidad de cuidados intensivos pediátrica en el período del estudio fue 9,3 por ciento, ante 18,2 por ciento en los pacientes con ventilación mecánica. Probablemente la presión inspiratoria máxima no debe sobrepasar 40 cm de H2O si se desea evitar el barotrauma