Diretrizes brasileiras do angioedema hereditário 2022 ­ Parte 1: definição, classificação e diagnóstico / 2022 Brazilian guidelines for hereditary angioedema ­ Part 1: definition, classification, and diagnosis

O angioedema hereditário é uma doença autossômica dominante caracterizada por crises recorrentes de edema que acometem o tecido subcutâneo e o submucoso, com envolvimento de diversos órgãos. Os principais locais afetados são face, membros superiores e inferiores, as alças intestinais e as vias respiratórias superiores. Em decorrência da falta de conhecimento dessa condição por profissionais de saúde, ocorre atraso importante no seu diagnóstico, comprometendo a qualidade de vida dos indivíduos afetados. Além disso, o retardo no diagnóstico pode resultar em aumento da mortalidade por asfixia devido ao edema de laringe. A natureza errática das crises com variação do quadro clínico e gravidade dos sintomas entre diferentes pacientes, e no mesmo paciente ao longo da vida, se constitui em desafio no cuidado dos doentes que têm angioedema hereditário. O principal tipo de angioedema hereditário é resultante de mais de 700 variantes patogênicas do gene SERPING1 com deficiência funcional ou quantitativa da proteína inibidor de C1, porém nos últimos anos outras mutações foram descritas em seis outros genes. Ocorreram avanços importantes na fisiopatologia da doença e novas drogas para o tratamento do angioedema hereditário foram desenvolvidas. Nesse contexto, o Grupo de Estudos Brasileiro em Angioedema Hereditário (GEBRAEH) em conjunto com a Associação Brasileira de Alergia e Imunologia (ASBAI) atualizou as diretrizes brasileiras do angioedema hereditário. O maior conhecimento dos diversos aspectos resultou na divisão das diretrizes em duas partes, sendo nessa primeira parte abordados a definição, a classificação e o diagnóstico.

Hereditary angioedema is an autosomal dominant disease characterized by recurrent attacks of edema that affect the subcutaneous tissue and the submucosa, involving several organs. The main affected sites are the face, upper and lower limbs, gastrointestinal tract, and upper airways. Because health professionals lack knowledge about this condition, there is a significant delay in diagnosis, compromising the quality of life of affected individuals. Furthermore, delayed diagnosis may result in increased mortality from asphyxia due to laryngeal edema. The erratic nature of the attacks with variations in clinical course and severity of symptoms among different patients and in one patient throughout life constitutes a challenge in the care of patients with hereditary angioedema. The main type of hereditary angioedema results from more than 700 pathogenic variants of the SERPING1 gene with functional or quantitative deficiency of the C1 inhibitor protein, but in recent years other mutations have been described in six other genes. Important advances have been made in the pathophysiology of the disease, and new drugs for the treatment of hereditary angioedema have been developed. In this context, the Brazilian Study Group on Hereditary Angioedema (GEBRAEH) in conjunction with the Brazilian Association of Allergy and Immunology (ASBAI) updated the Brazilian guidelines on hereditary angioedema. Greater knowledge of different aspects resulted in the division of the guidelines into two parts, with definition, classification, and diagnosis being addressed in this first part.

First administration of ecallantide in Peru: a case report of a Peruvian patient with hereditary angioedema with normal C1-inhibitor / Primeira administração de ecalantida no Peru: relato de caso de paciente peruano com angioedema hereditário com inibidor de C1 normal

Ecallantide is a specific treatment currently indicated for acute crisis of hereditary angioedema (HAE) due to C1-inhibitor deficiency. Our objective is to report the first administration of ecallantide (Kalbitor®) in Peru, where the treatment was used in an HAE patient with normal C1-inhibitor and no F12 gene alteration. We report the case of a 32-year-old postpartum patient with HAE with normal C1-inhibitor who belongs to the Peruvian Association of Patients with Hereditary Angioedema. During pregnancy, she had increased frequency and intensity of abdominal pain and facial edema crisis and received maintenance treatment with tranexamic acid and spasmolytics, with moderate response. One month postpartum, the patient showed respiratory symptoms and tested positive for coronavirus disease (COVID-19) in a polymerase chain reaction (PCR) test, without any HAE crisis during the infectious process. Three months postpartum, she had an acute laryngeal edema crisis with difficulty breathing and speaking, nausea, and vomiting, triggered by nonsteroidal anti-inflammatory drugs (NSAIDs). The patient then received treatment with antihistamines, corticosteroids, and adrenaline, without improvement; for that reason, the allergist administered ecallantide (Kalbitor®) with good response within the first 15 minutes of administration. Some Peruvian HAE patients have developed mild-to-moderate facial and peripheral edema crisis after NSAID intake, without improvement after administration of allergy treatment. In our patient, HAE crisis was not triggered by COVID-19. The patient showed worsening HAE crisis during pregnancy. The first administration of ecallantide (Kalbitor®) in Peru had good response and tolerance to the treatment as shown in this report.

Ecallantide é um tratamento específico totalmente indicado na crise aguda de deficiência de inibidor de C1 HAE. Nosso objetivo é relatar a primeira administração de Ecallantide (Kalbitor®) no Peru, um caso de paciente peruano com EH com inibidor C1 normal sem alteração genética F12. Relatamos o caso de uma paciente de 32 anos, pós-parto, com HAE inibidor de C1 normal, pertencente à Associação Peruana de Angioedema Hereditário de Pacientes. Durante a gravidez, a paciente apresentou aumento na frequência e intensidade das crises de edema abdominal e facial e recebeu tratamento de manutenção com ácido tranexâmico e espasmolítico, com resposta moderada. Um mês após o parto, a paciente apresentou quadro respiratório e teste de PCR molecular positivo para Doença do Coronavírus (COVID-19), sem crise de AEH durante o processo infeccioso. Três meses após o parto, a paciente apresentou crise de edema agudo de laringe com dificuldade para respirar e falar, náuseas e vômitos, desencadeado por AINH. A paciente recebeu tratamento com anti-histamínicos, corticosteroides e adrenalina sem melhora, por isso o alergista administrou Ecallantide (Kalbitor®) com boa resposta nos primeiros 15 minutos após o início da administração. Alguns pacientes peruanos com AEH desenvolveram crises de edema facial e periférico leve a moderado após a ingestão de AINEs, sem melhora após a administração de tratamento para alergia. Em nossa paciente, a crise de AEH não foi desencadeada por infecção aguda por COVID-19. A paciente apresentou agravamento da crise de AEH durante a gravidez. Apresentamos a primeira administração de Ecallantide (Kalbitor®) no Peru, com boa resposta e tolerância ao tratamento.

Profile of Laryngeal Microsurgeries in Patients Over 60 Years Old

Abstract Introduction The increase in life expectancy is an incentive to the development of researches with the elderly population aiming at actions that may ensure healthy and active aging. Objective To analyze the profile of laryngeal microsurgery performed in patients > 60 years old. Methods A retrospective observational study, with a cross-sectional design. A retrospective analysis of the medical records of elderly patients submitted to laryngeal microsurgery was performed at a private hospital in Curitiba, state of Paraná, Brazil, between January 2004 and December 2016. Were included all of the patients > 60 years old that underwent laryngeal microsurgery during this period. Results During the studied period, 213 laryngeal microsurgeries were performed in 181 patients > 60 years old. There was a preponderance of male patients. The mean age was 67.6 years old. Squamous cell carcinoma (SCC) was the most prevalent disorder (26%), followed by Reinke edema (20%), papillomatosis (14%), polyps (11%), leukoplakia (8%), minor structural alterations (8%), associated lesions (9%), and others (4%). Men presented a higher probability of SCC diagnosis, regardless of the age group, while Reinke edema was more frequently observed in women. A directly proportional relation between the frequency of laryngeal cancer and age increase was also observed. No significant differences were observed in professional voice users. Conclusion Further researches are required to properly comprehend the factors associ- ated with laryngeal lesions and determine prevention and treatment approaches.

Relação entre avaliação vocal, acústica e qualidade de vida em voz de mulheres com diferentes graus de Edema de Reinke / Relationship between vocal, acoustic and quality of life evaluation in women with the different grade of Reinke's edema / Relación entre evaluación vocal, acústica y calidad de vida en voz de mujeres con diferentes grados de Edema de Reinke

Introdução: O Edema de Reinke caracteriza-se por um processo inflamatório crônico que acomete a camada superficial da lâmina própria da prega vocal. Atualmente, sua etiologia é atribuída ao tabagismo associado ao abuso vocal. Objetivo: Relacionar os dados das avaliações vocal, acústica e de qualidade de vida em voz com o grau do Edema de Reinke em mulheres. Método: Estudo observacional, analítico, transversal e prospectivo. Participaram 22 mulheres, com idades entre 45 e 78 anos (média 58,3), que foram submetidas à avaliação laringológica para observação das variáveis referentes ao grau do edema e a associação com outras lesões e/ou alterações laríngeas; avaliação perceptivo-auditiva da voz; análise acústica da voz; e auto avaliação vocal por meio do protocolo Qualidade de Vida em Voz (QVV). Os exames laringoscópicos e as amostras vocais foram analisados por juízes especialistas. Os dados obtidos foram analisados estatisticamente pelo teste de Mann-Whitney. Os sujeitos foram agrupados em dois grupos: Grupo 1 (G1) (edema grau 1) e Grupo 2 (G2) (edemas graus 2 e 3). Resultados: O G2 apresentou piores resultados do que o G1 quanto ao maior número de sintomas vocais; maior grau de desvio vocal na avaliação perceptivo-auditiva; resultados mais alterados nas medidas acústicas jitter, shimmer e proporção GNE; valores mais baixos em todos os domínios do QVV, indicando pior qualidade de vida. Conclusões: As características laringológicas referentes à progressão do Edema de Reinke estão diretamente relacionadas à piora dos parâmetros perceptivo-auditivos e acústicos da voz e a um maior impacto negativo da disfonia na qualidade de vida.

Introduction: Reinke's edema is characterized by a chronic inflammatory process that affects the superficial layer of the lamina propria of the vocal fold. Currently, its etiology is attributed to smoking associated with vocal abuse. Objective: To relate data of vocal, acoustic and quality of life evaluation in women with the different grade of Reinke's edema. Method: It is an observational, analytical and crosssectional study. Participants were 22 women, aged between 45 and 78 years old (mean 58.3 years), who passed by laryngology evaluation to observe the variables regarding of edema´s degree and the association with other laryngeal disorders; auditory-perceptual evaluation of voice; acoustic voice data analysis; and vocal self-assessment by Voice-Related Quality of Life protocol (VRQOL). Laryngological data and vocal samples were analyzed by expert judges. Data were statistically analyzed. The subjects were grouped into two groups: Group 1 (G1) (grade 1 of edema) and Group 2 (G2) (grades 2 and 3 of edema). Results: G2 had worse results than G1 in all associations: more vocal symptoms; higher degree of vocal deviation in auditoryperceptual evaluation; more abnormal results in acoustic measurements (jitter, shimmer and glottal to noise excitation ratio; lower values in all domains of VRQOL, indicating worse quality of life. Conclusions: The laryngeal data related to the progression of Reinke's edema are directly related to worsening of auditoryperceptual and acoustic data of voice and a greater negative impact of dysphonia in quality of life.

Introducción: El Edema de Reinke se caracteriza por un proceso inflamatorio crónico que acomete la capa superficial de la lámina propia del pliegue vocal. Actualmente, su etiología se atribuye al tabaquismo asociado al abuso vocal. Objetivo: Relacionar los datos de las evaluaciones vocal, acústica y de calidad de vida en voz con el grado del Edema de Reinke en mujeres. Método: Estudio observacional, analítico, transversal y prospectivo. En la mayoría de los casos, se observó un aumento de la mortalidad por rotavirus en el momento de la vacunación. evaluación perceptiva-auditiva de la voz; análisis acústico de la voz; y autoevaluación vocal a través del protocolo Calidad de Vida en Voz (QVV). Los exámenes laringoscópicos y las muestras vocales fueron analizados por jueces especialistas. Los datos obtenidos fueron analizados estadísticamente por la prueba de Mann-Whitney. Los sujetos fueron agrupados en dos grupos: Grupo 1 (G1) (edema grado 1) y Grupo 2 (G2) (edemas grados 2 y 3). Resultados: El G2 presentó peores resultados que el G1 en cuanto al mayor número de síntomas vocales; mayor grado de desvío vocal en la evaluación perceptivo-auditiva; resultados más alterados en las medidas acústicas jitter, shimmer y proporción GNE; valores más bajos en todos los ámbitos del QVV, indicando peor calidad de vida. Conclusión: Las características laringológicas referentes a la progresión del Edema de Reinke están directamente relacionadas con el empeoramiento de los parámetros perceptivo-auditivos y acústicos de la voz y un mayor impacto negativo de la disfonía en la calidad de vida.

Post-radiation airway management - An anesthesiologist's challenge

No abstract available.

Percepção auditiva de juízes leigos quanto ao gênero de mulheres com edema de Reinke / Auditory perception of lay judges about gender identification of women with Reinke's edema

RESUMO Objetivo Investigar a percepção auditiva de juízes leigos quanto ao gênero de mulheres com edema de Reinke, relacionada com o grau do edema e a frequência fundamental da voz. Método Estudo observacional, analítico e transversal. Participaram 46 juízes leigos que analisaram 48 vozes disfônicas - 24 vozes de mulheres com Edema de Reinke (Grupo Edema de Reinke - GER) e 24 vozes de mulheres e homens com outros tipos de disfonias (Grupo Controle - GC). Os juízes analisaram a contagem de números de 1 a 10 e julgaram as vozes como pertencentes a homem ou mulher, além de descreverem também se tinham certeza ou dúvida quanto à resposta. Os resultados do GER foram associados ao Grau do Edema (1, 2 ou 3) e à frequência fundamental média (F0), analisada por meio da vogal /Ɛ/. Resultados Observou-se que a porcentagem de erros em relação ao gênero foi maior no GER quando comparada à do GC, o percentual de certezas quanto ao gênero foi maior no GC. No GER, edemas de grau 1 ocasionaram menos erros quando comparados aos graus 2 e 3. A média da F0 das mulheres cujas vozes foram julgadas como masculinas (141 Hz) foi menor do que as identificadas corretamente (149 Hz). Conclusão Por meio da avaliação de juízes leigos, as vozes das mulheres com edema de Reinke são identificadas como vozes masculinas. O aumento do grau do edema e a redução da F0 estão relacionados à maior quantidade de erros e/ou dúvidas em relação à identificação do gênero de mulheres com edema de Reinke .

ABSTRACT Purpose To investigate the auditory perception of lay judges of the voice gender identification of women with Reinke's edema and to associate it with its severity and fundamental frequency (F0). Methods This is an observational, analytical and cross-sectional study. A total of 46 lay judges analyzed 48 samples (counting numbers 1 to 10) of 24 women with Reinke's edema - the Reinke's Edema Group (REG) and 24 individuals, men and women, with other types of voice disorders - Control Group (CG). The judges had to classify the voices as being of a man or a woman. Additionally, they needed to indicate their certainty or not about their choice. Results were associated with the severity of the Reinke's edema (Type 1, 2 or 3) and the F0 (extracted from the vowel /Ɛ/). Results Misidentification of gender was higher in the REG and certainty about the choice was higher in the CG. Type 1 cases caused fewer misidentifications compared to type 2 and 3. The women's voices that were identified as male voices had a lower F0 (141Hz) when compared to voices that were correctly identified (149Hz). Conclusion Women with Reinke's edema are frequently identified as men. Lower F0 was related with more misidentification and less certainty when assessing the speaker's gender.

Edema de Reinke obstructivo / Obstructive Reinke edema

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)

Demographic profile and risk factors of patients with benign vocal fold lesions diagnosed through laryngeal videoendoscopy and stroboscopy

OBJECTIVE: To determine the prevalence of benign vocal cord lesions among Filipino patients in a tertiary institution and identify the demographic characteristics and possible risk factors found among these patients.METHODS:Design: Retrospective Case SeriesSetting: Private Tertiary HospitalParticipants: Records of 2,375 patients who underwent laryngeal video endoscopy and stroboscopy from 2012-2014 were reviewed.RESULTS: There were 632 records of patients with benign vocal fold lesions, of which nodules were most common (211, 33.38%) followed by Reinke's edema (165, 26.10%), cysts (122, 19.30%) and polyps (74, 11.70%) with hoarseness as the most common symptom (542, 85.76%). More than half (336, 53.16%) were aged 21-40 years and almost two-thirds (469, 74.21%) were female. The most common associated factors were caffeine intake (445, 70.41%) and inadequate water intake (370, 58.54%), followed by alcohol (253, 40.03%). Smoking was only present in 146 (23.19%).CONCLUSIONS: Baseline evidence on the prevalence of benign vocal fold lesions in this institution as well as baseline data on the common characteristics and associated factors seen in the sample population may assist us in current practices and guide future studies directed toward treatment and prevention.

Demographic profile and risk factors of patients with benign vocal fold lesions diagnosed through laryngeal videoendoscopy and stroboscopy

@#<p style="text-align: justify;"><strong>OBJECTIVE:</strong> To determine the prevalence of benign vocal cord lesions among Filipino patients in a tertiary institution and identify the demographic characteristics and possible risk factors found among these patients.<br /><strong>METHODS:</strong><br /><strong>Design:</strong> Retrospective Case Series<br /><strong>Setting:</strong> Private Tertiary Hospital<br /><strong>Participants:</strong> Records of 2,375 patients who underwent laryngeal video endoscopy and stroboscopy from 2012-2014 were reviewed.<br /><strong>RESULTS:</strong> There were 632 records of patients with benign vocal fold lesions, of which nodules were most common (211, 33.38%) followed by Reinke's edema (165, 26.10%), cysts (122, 19.30%) and polyps (74, 11.70%) with hoarseness as the most common symptom (542, 85.76%). More than half (336, 53.16%) were aged 21-40 years and almost two-thirds (469, 74.21%) were female. The most common associated factors were caffeine intake (445, 70.41%) and inadequate water intake (370, 58.54%), followed by alcohol (253, 40.03%). Smoking was only present in 146 (23.19%).<br /><strong>CONCLUSIONS:</strong> Baseline evidence on the prevalence of benign vocal fold lesions in this institution as well as baseline data on the common characteristics and associated factors seen in the sample population may assist us in current practices and guide future studies directed toward treatment and prevention.</p>

Manejo del fracaso de la extubación en la unidad de terapia intensiva pediátrica / Management of extubation failure in the pediatric intensive care unit / Gestão de falha de extubação na Unidade de Terapia Intensiva Pediátrica

El fracaso de la extubación es la incapacidad de tolerar el retiro del tubo endotraqueal con necesidad de reintubación. Puede ser causada por la obstrucción de la vía aérea superior y por la aspiración o la incapacidad de manejar las secreciones, factores que se evidencian al retirar el tubo. La reintubación por fracaso respiratorio post-extubación debe ser evitada en lo posible, ya que aumenta el riesgo de neumonía asociada al respirador, la estancia hospitalaria y en terapia intensiva, y la morbimortalidad. Describimos el manejo del fracaso de la extubación de causa laríngea en la Unidad de Terapia Intensiva de un hospital pediátrico.

Extubation failure is the inability to tolerate removal of the endotracheal tube with subsequent reintubation. It can be caused by obstruction of the upper airway and aspiration or inadequate clearance of airway secretion, factors that become evident when removing the tube. Reintubation due to postextubation respiratory failure should be avoided if possible because it increases the risk of ventilator associated pneumonia, hospital and intensive care unit stay, and morbidity and mortality. We describe the management of failed extubation due to laryngeal cause in the Intensive Care Unit of a pediatric hospital.

Falha de extubação é a incapacidade de tolerar a remoção do tubo endotraqueal na necessidade de reintubação. Ela pode ser causada por obstrução das vias aéreas superiores e aspiração ou incapacidade para lidar com secreções, factores que são evidentes para remover o tubo. Reintubação devido a insuficiência respiratória pós-extubação deve ser evitada, se possível, porque aumenta o risco de pneumonia associada à ventilação mecânica, permanência hospitalar e terapia, morbidade e mortalidade. Nós descrevemos a gestão de extubação falhou devido à causa da laringe na Unidade de Terapia Intensiva de um hospital pediátrico.

Angioedema hereditario / Hereditary angioedema

El angioedema hereditario es una enfermedad poco frecuente, con herencia autosómica dominante que se caracteriza por presentar edemas en piel y en la mucosa de diferentes órganos, fundamentalmente el tubo digestivo y el aparato respiratorio. Las manifestaciones clínicas pueden ser ligeras o graves, en dependencia de su intensidad y localización. Las formas más graves son el edema de la glotis y del tubo digestivo, que llegan a ocasionar síntomas como deshidratación intensa y dolor abdominal, el que puede confundirse con un abdomen agudo y llevar a una intervención quirúrgica innecesaria. El edema se caracteriza por no ser pruriginoso, no presentar aumento de la temperatura, no dejar godet al presionarlo y generalmente existen antecedentes familiares. No tiene predilección por sexo, ni por el color de la piel. Para su diagnóstico es necesario hacer un interrogatorio minucioso y exámenes complementarios del sistema complemento. Se han descrito dos formas clásicas denominadas angioedema hereditario tipo I y tipo II, el primero es el más frecuente. Recientemente se ha descrito el tipo III que se presenta solo en mujeres, sin alteración cuantitativa o cualitativa de C1-inhibidor y se asocia con el consumo de medicamentos o anticonceptivos orales que contienen estrógenos. El tratamiento se basa fundamentalmente en el uso de andrógenos atenuados o de los antifibrinolíticos, así como evitar los factores de riesgo en caso de que estos se conozcan. En los casos que presenten cuadros agudos se puede utilizar el plasma fresco congelado y un concentrado purificado de C1- inhibidor (Berinert-500) de uso endovenoso y de respuesta rápida; aunque sin ser muy efectiva se puede usar la epinefrina subcutánea. Los esteroides y los antihistamínicos no tienen ninguna efectividad en el tratamiento de estos pacientes(AU)

Hereditary angioedema is a rare disease with autosomal dominant inheritance that is characterized by edema in skin and mucosa of various organs, mainly gastrointestinal tract and the respiratory system. Clinical manifestations may be mild or severe, depending on their location and intensity. The most severe forms are edema of the glottis, and the edema of gastrointestinal tract which can cause severe dehydration and abdominal pain that can be confused with an acute abdomen and unnecessary surgery. The edema is characterized by not being itchy, no temperature rise, non-marking when pressed and usually have a family history. No predilection for sex, or skin color. Its diagnosis is necessary to make a thorough examination and additional tests of the complement system. They described two classic forms called HAE type I and type II, type I is the most common. Recently it described the type III that occurs only in females, without quantitative or qualitative alteration of C1 inhibitor and is associated with the consumption of drugs or oral contraceptives containing estrogen. The treatment is based primarily on the use of attenuated androgens or antifibrinolytic and avoiding risk factors if they are known. In cases of acute conditions present you can use fresh frozen plasma and purified C1 inhibitor concentrate (Berinert-500) for intravenous use and rapid response; but the use of subcutaneous epinephrine may not be very effective. Steroids and antihistamines have no effectiveness when used in these patients(AU)

Relapsing polychondritis initially presenting with hoarseness and difficulty breathing in a 21-year-old male

@#<p style="text-align: justify;"><strong>OBJECTIVE:</strong> To present a case of relapsing polychondritis initially presenting with hoarseness and difficulty breathing and to discuss the diagnostic criteria and typical CT scan findings of relapsing polychondritis. <br /><strong>METHODS:</strong> <br /><strong>Design:</strong> Case Report<br /><strong>Setting:</strong> Tertiary Private Hospital in Metro Manila<br /><strong>Patient:</strong> One<br /><strong>RESULT:</strong> A 21-year-old man who was initially managed as a case of bronchial asthma for persistent hoarseness and recurrent difficulty breathing was found to have severe laryngeal edema on endoscopy, and soft tissue expansion of the cricoid cartilage with calcifications and irregular first tracheal ring on CT scan. He also had recurrent eye redness and developed bilateral aural inflammation, and was subsequently diagnosed to have relapsing polychondritis.<br /><strong>CONCLUSION:</strong> Relapsing polychondritis is a rare autoimmune disease characterized by recurrent inflammation and eventual destruction of cartilage throughout the body. Typical manifestations may not always be present, causing a delay in diagnosis. It should be considered in patients with intractable respiratory symptoms not responsive to treatment for upper respiratory tract infections or asthma. A CT scan may reveal signs of cartilage destruction and help in diagnosis.                  </p>

Analysis of clinical characteristics of food-dependent exercise-induced anaphylaxis at a single tertiary hospital

PURPOSE: Food-dependent exercise-induced anaphylaxis (FDEIA) is a rare subtype of food allergy in which both sensitization to food allergen and exercise as a trigger contribute to its development. However, its pathogenesis is still under investigation. This study compared clinical features, the causative foods, and the degree of sensitization to food between FDEIA and food anaphylaxis to characterize FDEIA more clearly. METHODS: We retrospectively reviewed the medical records of patients who were diagnosed with FDEIA (n=89) or food anaphylaxis (n=115) between 2003 and 2015 at Seoul National University Hospital. RESULTS: Subjects with FDEIA more frequently had urticaria than those with food anaphylaxis (88.8% vs. 76.5%, P=0.024). Whereas patients with FDEIA had less laryngeal edema than those with food anaphylaxis (12.4% vs. 30.4%, P=0.02). Wheat (67.4%) was the most common causative food allergen in FDEIA, whereas seafood (40.9%) was the most common culprit food allergen in food anaphylaxis. Also, subjects with FDEIA showed a lower atopic index score than those with food anaphylaxis (0.55±1.07 vs. 1.21±1.82, P=0.006). CONCLUSION: There were significant differences in clinical manifestation, causative food allergens and the degree of sensitization to food between FDEIA and food anaphylaxis.

Voice assessment on patients with Reinke's edema of vocal fold by application of micro-suture techniques / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To evaluate the vocal quality of patients with Reinke's edema before and after the endolaryngeal phonosurgery combined with micro-suture techniques.@*METHOD@#Twenty male patients with bilateral Reinke's edema were submitted to surgery. All patients received the endolaryngeal phonosurgery combined with micro-suture techniques on vocal fold and completed voice evaluation preoperative and postoperative 2 weeks, 3 months and 6 months. Acoustic voice parameters were compared with the control group without voice pathology.@*RESULT@#The result showed that at postoperative 2 weeks, compared with the preoperative evaluation the patients' subjective sound quality improved obviously. Acoustic voice parameters improved significantly (P0.05). At postoperative 6 months, voice quality still remain stable, all of the analyzed parameters had no obvious difference.@*CONCLUSION@#The phonosurgery combined with micro-suture techniques treat on the Reinke's edema, vocal cord postoperative recovery time is short, the sound quality receive basic recovery after 3 months.

Analysis of respiratory complications in 922 severely burned patients / 中华烧伤杂志

<p><b>OBJECTIVE</b>To discuss the distribution of the respiratory complications in severely burned patients and the prevention and treatment experience against them.</p><p><b>METHODS</b>Medical records of 922 adult patients with severe or extremely severe burn hospitalized in our burn ICU from January 2005 to December 2012 were screened and retrospectively analyzed, including patients transferred from other hospitals, patients with total burn area above 50% TBSA, the distribution and treatment of respiratory complications, and the mortality. Data were processed with chi-square test.</p><p><b>RESULTS</b>The constituent ratio of patients transferred to our hospital was 71.1% in 2007 and 40.2% in 2010, while it remained about 50.0% in the other years. The ratios of patients with total burn area larger than 50% TBSA and that of patients with respiratory complications (χ(2) = 2.637, P > 0.05) showed no significant changes each year. Among these 922 burn patients, 523 patients suffered respiratory complications, among which laryngeal edema (50.9%, 266 cases), pulmonary infection (21.6%, 113 cases), and ARDS (11.9%, 62 cases) were the main components, with no significant change each year (with χ(2) values respectively 6.132, 6.319, 0.016, P values above 0.05). Among the patients with respiratory complications, except for 36 were not treated actively, 487 were treated by ventilator among which 228 had undergone tracheostomy, and the constituent ratios in the 8 years were close. Fifteen patients died, with 2 died of laryngeal edema, 3 of ARDS, and 10 of sepsis or MODS as a result of sepsis.</p><p><b>CONCLUSIONS</b>Patients with severe burns were at high risk of respiratory complications, among which laryngeal edema was common, followed by pulmonary infection and ARDS. Prophylactic tracheostomy, mechanical ventilation, wound therapy, and anti-infection were all effective measures of prevention and treatment against these complications.</p>

Isolated central venous pressure elevation caused by hematoma formation compressing the superior vena cava following a Bentall operation: a case report / 대한마취과학회지

We present a case of a patient exhibiting isolated elevation of the central venous pressure with minimal hemodynamic deterioration in an immediate postoperative period after Bentall operation requiring re-exploration. Isolated elevation of the central venous pressure usually alerts physicians of a volume overload or right ventricular dysfunction. However, even in the absence of significant hemodynamic deterioration, the development of loculated hematoma that compresses the superior vena cava should be ruled out, as it can be life-threatening through the formation of cerebral and laryngeal edema, similar to superior vena cava syndrome. This case emphasizes the importance of a prompt differential diagnosis of the isolated central venous pressure elevation after cardiac surgery with transesophageal echocardiography for the administration of appropriate treatment.

One case of hereditary angioneurotic laryngeal edema / 临床耳鼻咽喉头颈外科杂志

Hereditary angioneurotic laryngeal edema (HALE) is an autosomal dominant hereditary disease in which there is a decrease or defect in the C1 inhibitor (C1-INH). The pathophysiology of HALE is characterized by recurrent spontaneous episodes of transient edema of the laryngeal mucose and submucosal tissue with remission at irregular. Patients may die because of a life-threatening acute upper airway obstruction caused by laryngeal edema. HALE was diagnosed on the clinical symptoms, family history, and markedly decreased serum C1-INH activity and C1-INH protein.

Persistent Dysphonia after Laryngomicrosurgery for Benign Vocal Fold Disease

OBJECTIVES: Laryngomicrosurgery (LMS) is used to manage most vocal fold lesions. However, the functional voice outcome of the LMS might be diverse due to the influence of various factors. We intend to evaluate the incidence and etiologic factors of persistent dysphonia after LMS for benign vocal fold disease (BVFD). METHODS: We performed a retrospective review of 755 patients who underwent LMS for BVFD. We analyzed the clinical characteristics, preoperative and postoperative two onths voice studies. Postsurgical dysphonia was defined as grade 1 or above in GRBAS (grade, roughness, breathiness, asthenia, and strain) scale. Thirty nine patients (5.2%; 25 males and 14 females; average, 42.9 years; range, 21 to 70 years) were diagnosed with postsurgical dysphonia. RESULTS: There was no correlation between the diagnosis, coexistence with laryngopharyngeal reflux disease, habit of smoking, or occupational voice abuse and voice outcome. The patients with a worse preoperative acoustic parameter had aworse voice outcome. Stroboscopic findings showed excessive scarring or bowing in 21 cases, presence of lesion remnant in eight cases, prolonged laryngeal edema in five and no abnormal findings in three. CONCLUSION: Great care should be taken in patients with worse preoperative jitter. With a few exceptions, postoperative dysphonia can be avoided by the use of an ppropriate surgical technique.

MRI Reveals Edema in Larynx (But Not in Brain) During Anaphylactic Hypotension in Anesthetized Rats

PURPOSE: Anaphylactic shock is sometimes accompanied by local interstitial edema due to increased vascular permeability. We performed magnetic resonance imaging (MRI) to compare edema in the larynx and brain of anesthetized rats during anaphylactic hypotension versus vasodilator-induced hypotension. METHODS: Male Sprague Dawley rats were subjected to hypotension induced by the ovalbumin antigen (n=7) or a vasodilator sodium nitroprusside (SNP; n=7). Apparent diffusion coefficient (ADC) and T2-relaxation time (T2RT) were quantified on MRI performed repeatedly for up to 68 min after the injection of either agent. The presence of laryngeal edema was also examined by histological examination. Separately, the occurrence of brain edema was assessed by measuring brain water content using the wet/dry method in rats with anaphylaxis (n=5) or SNP (n=5) and the non-hypotensive control rats (n=5). Mast cells in hypothalamus were morphologically examined. RESULTS: Mean arterial blood pressure similarly decreased to 35 mmHg after an injection of the antigen or SNP. Hyperintensity on T2-weighted images (as reflected by elevated T2RT) was found in the larynx as early as 13 min after an injection of the antigen, but not SNP. A postmortem histological examination revealed epiglottic edema in the rats with anaphylaxis, but not SNP. In contrast, no significant changes in T2RT or ADC were detectable in the brains of any rats studied. In separate experiments, the quantified brain water content did not increase in either anaphylaxis or SNP rats, as compared with the non-hypotensive control rats. The numbers of mast cells with metachromatic granules in the hypothalamus were not different between rats with anaphylaxis and SNP, suggesting the absence of anaphylactic reaction in hypothalamus. CONCLUSION: Edema was detected using the MRI technique in the larynx during rat anaphylaxis, but not in the brain.

MRI Reveals Edema in Larynx (But Not in Brain) During Anaphylactic Hypotension in Anesthetized Rats

PURPOSE: Anaphylactic shock is sometimes accompanied by local interstitial edema due to increased vascular permeability. We performed magnetic resonance imaging (MRI) to compare edema in the larynx and brain of anesthetized rats during anaphylactic hypotension versus vasodilator-induced hypotension. METHODS: Male Sprague Dawley rats were subjected to hypotension induced by the ovalbumin antigen (n=7) or a vasodilator sodium nitroprusside (SNP; n=7). Apparent diffusion coefficient (ADC) and T2-relaxation time (T2RT) were quantified on MRI performed repeatedly for up to 68 min after the injection of either agent. The presence of laryngeal edema was also examined by histological examination. Separately, the occurrence of brain edema was assessed by measuring brain water content using the wet/dry method in rats with anaphylaxis (n=5) or SNP (n=5) and the non-hypotensive control rats (n=5). Mast cells in hypothalamus were morphologically examined. RESULTS: Mean arterial blood pressure similarly decreased to 35 mmHg after an injection of the antigen or SNP. Hyperintensity on T2-weighted images (as reflected by elevated T2RT) was found in the larynx as early as 13 min after an injection of the antigen, but not SNP. A postmortem histological examination revealed epiglottic edema in the rats with anaphylaxis, but not SNP. In contrast, no significant changes in T2RT or ADC were detectable in the brains of any rats studied. In separate experiments, the quantified brain water content did not increase in either anaphylaxis or SNP rats, as compared with the non-hypotensive control rats. The numbers of mast cells with metachromatic granules in the hypothalamus were not different between rats with anaphylaxis and SNP, suggesting the absence of anaphylactic reaction in hypothalamus. CONCLUSION: Edema was detected using the MRI technique in the larynx during rat anaphylaxis, but not in the brain.