Paciente varón de tres años con sibilancias y tos crónica / Three-Year-Old Male with Wheezing and Chronic Cough

Se presenta el caso de un niño de 3 años con diagnóstico de asma, rinitis alérgica, características craneofaciales dismórficas e infecciones respiratorias altas y bajas recurrentes, manejado como asma desde un inicio. Como parte del estudio de comorbilidades, se decide realizar una prueba del sudor que sale en rango intermedio y más tarde se encuentra una mutación, donde se obtiene un resultado positivo para una copia que se asocia a fibrosis quística. Se revisará el caso, así como el diagnóstico, clínica y tratamiento del síndrome metabólico relacionado con el regulador de conductancia transmembrana de fibrosis quística (CRMS).

We present the case of a 3-year-old boy with a diagnosis of asthma, allergic rhinitis, dysmorphic craniofacial characteristics and recurrent upper and lower respiratory infections, managed as asthma from the beginning. As part of the study of comorbidi-ties, it was decided to carry out a sweat test that came out in the intermediate range and later one mutation was found, where a positive result was obtained for a copy that is associated with cystic fibrosis. The case will be reviewed, as well as the diagnosis, symptoms and treatment of the metabolic syndrome related to the cystic fibrosis trans-membrane conductance regulator (CRMS).

O uso de métodos contraceptivos hormonais em pacientes com asma: repercussões clínicas / The use of hormonal contraceptives methods in patients with asthma: clinical repercussions

Objetivo: Analisar o uso dos contraceptivos hormonais em mulheres com asma e a escolha desses métodos contraceptivos para essa população, com avaliação de eventuais repercussões sobre novos episódios de asma e sibilos. Métodos: Foram selecionados estudos longitudinais, ensaios clínicos, revisões sistemáticas e metanálises. As plataformas consultadas foram PubMed, Embase, Cochrane e SciELO, com a utilização dos descritores: "contracepção", "contracepção hormonal", "sistema intrauterino liberador de levonorgestrel" e "asma". Resultados: Dois grandes estudos demonstraram que o uso de contraceptivos hormonais esteve associado à redução do risco de novos episódios de asma. Uma revisão sistemática concluiu que os resultados para o uso de contraceptivos hormonais para mulheres com asma foram mistos, com aumento ou redução dos seguintes riscos: novo episódio de asma e aumento da frequência das crises e dos sibilos. O uso da contracepção hormonal em pacientes obesas portadoras de asma é controverso. Conclusão: Os resultados para o uso de contraceptivos hormonais em mulheres com asma são inconsistentes, com relatos de aumento ou de redução do risco de novos episódios. O uso do método contraceptivo deve ser discutido individualmente, levando-se em consideração outros fatores de risco associados e o desejo da mulher. A paciente deverá ser orientada se houver piora dos sintomas clínicos de asma na vigência do uso de qualquer método contraceptivo hormonal.

Objective: To analyze the use of hormonal contraceptives in women with asthma and the choice of this contraceptive method for this population, evaluating possible repercussions on new episodes of asthma and wheezing. Methods: Longitudinal studies, clinical trials, systematic reviews and meta-analyses were selected. Platforms consulted: PubMed, Embase, Cochrane, SciELO, using the descriptors: "contraception", "hormonal contraception", "levonorgestrel-releasing intrauterine system" and "asthma". Results: Two large studies demonstrated that the use of hormonal contraceptives was associated with a reduced risk of new episodes of asthma. A systematic review concluded that the results for the use of hormonal contraceptives for women with asthma were mixed, with increased or decrease in the following risks: new asthma episodes, increased frequency and wheezing. The use of hormonal contraception in obese patients with asthma is controversial. Conclusion: The results for the use of hormonal contraceptives in women with asthma are inconsistent, with reports of increased or reduced risk of new episodes. The use of the contraceptive method should be discussed individually, taking into account other associated risk factors and the woman's desire. The patient will be advised if there is a worsening of the clinical symptoms of asthma while using any hormonal contraceptive method.

Neurofibromatosis laríngea pediátrica: reporte de localización atípica / Pediatric neurofibromatosis of the larynx: report of atypical location

Los neurofibromas laríngeos (NFL) son tumores benignos poco frecuentes de localización principalmente supraglótica. Se manifiestan con síntomas obstructivos de la vía aérea. El tratamiento es la resección completa del tumor mediante abordaje endoscópico; se reserva la cirugía abierta para tumores de gran extensión. Se presenta el caso de un paciente pediátrico con localización atípica de NFL asociado a neurofibromatosis tipo 1 (NF1). Se realizó resección endoscópica del tumor y la anatomía patológica informó neurofibroma plexiforme. Es importante sospechar de esta patología en todo niño con estridor inspiratorio atípico progresivo. Se sugiere seguimiento a largo plazo por la alta probabilidad de recidiva.

Laryngeal neurofibromas (LNFs) are rare benign tumors mainly located in the supraglottis. LNFs occur with airway obstruction symptoms. The treatment is complete resection via an endoscopic technique; the open approach is reserved for large tumors. Here we describe the case of a pediatric patient with LNF of atypical location associated with neurofibromatosis type 1 (NF-1). The tumor was resected with an endoscopic technique, and the pathological study reported a plexiform neurofibroma. It is important to suspect this condition in any child with atypical, progressive inspiratory stridor. Long-term follow-up is recommended due to the high rate of recurrence

Analysis of the clinical features and the risk factors of severe human metapneu movirus-associated community acquired pneumonia in children / 中华儿科杂志

Objective: To investigate the clinical characteristics and the risk factors of severe human metapneumovirus (hMPV)-associated community acquired pneumonia (CAP) in children. Methods: A retrospective case summary was conducted. From December 2020 to March 2022, 721 children who were diagnosed with CAP and tested positive for hMPV nucleic acid by PCR-capillary electrophoresis fragment analysis of nasopharyngeal secretions at the Yuying Children's Hospital, the Second Affiliated Hospital of Wenzhou Medical University were selected as the research objects. The clinical characteristics, epidemiological characteristics and mixed pathogens of the two groups were analyzed. According to CAP diagnostic criteria, the children were divided into the severe group and the mild group. Chi-square test or Mann-Whitney rank and contrast analysis was used for comparison between groups, while multivariate Logistic regression was applied to analyze the risk factors of the severe hMPV-associated CAP. Results: A total of 721 children who were diagnosed with hMPV-associated CAP were included in this study, with 397 males and 324 females. There were 154 cases in the severe group. The age of onset was 1.0 (0.9, 3.0) years, <3 years old 104 cases (67.5%), and the length of hospital stay was 7 (6, 9) days. In the severe group, 67 children (43.5%) were complicated with underlying diseases. In the severe group, 154 cases (100.0%) had cough, 148 cases (96.1%) had shortness of breath and pulmonary moist rales, and 132 cases (85.7%) had fever, 23 cases (14.9%) were complicated with respiratory failure. C-reactive protein (CRP) was elevated in 86 children (55.8%), including CRP≥50 mg/L in 33 children (21.4%). Co-infection was detected in 77 cases (50.0%) and 102 strains of pathogen were detected, 25 strains of rhinovirus, 17 strains of Mycoplasma pneumoniae, 15 strains of Streptococcus pneumoniae, 12 strains of Haemophilus influenzae and 10 strains of respiratory syncytial virus were detected. Six cases (3.9%) received heated and humidified high flow nasal cannula oxygen therapy, 15 cases (9.7%) were admitted to intensive care unit, and 2 cases (1.3%) received mechanical ventilation. In the severe group, 108 children were cured, 42 children were improved, 4 chlidren were discharged automatically without recovery and no death occurred. There were 567 cases in the mild group. The age of onset was 2.7 (1.0, 4.0) years, and the length of hospital stay was 4 (4, 6) days.Compared with the mild group, the proportion of children who age of disease onset <6 months, CRP≥50 mg/L, the proportions of preterm birth, congenital heart disease, malnutrition, congenital airway malformation, neuromuscular disease, mixed respiratory syncytial viruses infection were higher (20 cases (13.0%) vs. 31 cases (5.5%), 32 cases (20.8%) vs. 64 cases (11.3%), 23 cases (14.9%) vs. 44 cases (7.8%), 11 cases (7.1%) vs. 18 cases (3.2%), 9 cases (5.8%) vs. 6 cases (1.1%), 11 cases (7.1%) vs. 12 cases (2.1%), 8 cases (5.2%) vs. 4 cases (0.7%), 10 cases (6.5%) vs. 13 cases (2.3%), χ2=0.42, 9.45, 7.40, 4.94, 11.40, 8.35, 3.52, 6.92, all P<0.05). Multivariate Logistic regression analysis showed that age<6 months (OR=2.51, 95%CI 1.29-4.89), CRP≥50 mg/L (OR=2.20, 95%CI 1.36-3.57), prematurity (OR=2.19, 95%CI 1.26-3.81), malnutrition (OR=6.05, 95%CI 1.89-19.39) were the independent risk factors for severe hMPV-associated CAP. Conclusions: Severe hMPV-associated CAP is most likely to occur in infants under 3 years old and has a higher proportion of underlying diseases and co-infection. The main clinical manifestations are cough, shortness of breath and pulmonary moist rales, fever. The overall prognosis is good. Age<6 months, CRP≥50 mg/L, preterm birth, malnutrition are the independent risk factors for severe hMPV-associated CAP.

Consensus recommendations on the evaluation and treatment of laryngotracheal anomalies in infants and young children / 临床耳鼻咽喉头颈外科杂志

Infants with laryngotracheal anomalies are clinically manifested as stridor or noisy breathing, choking, hoarseness, feeding difficulties, and cyanotic spells, followed by developmental and growth retardation and other health issues; in severe cases, patients may present with severe dyspnea, which is associated with high mortality. A timely diagnosis as well as appropriate strategy for laryngotracheal anomalies is still challenging for pediatric otolaryngologists. This consensus statement, evolved from expert opinion by the members of the Pediatric Otorhinolaryngology Professional Committee of the Pediatrician Branch of the Chinese Medical Doctor Association, provides comprehensive recommendations and standardized guidance for otolaryngologists who manage infants and young children with laryngotracheal anomalies in evaluation and treatment based on symptomatology, physical and laboratory examinations.

Asthma management and asthma control level in children / 中国当代儿科杂志

OBJECTIVES@#To investigate the influencing factors for asthma management and asthma control level in children.@*METHODS@#A total of 202 children with a confirmed diagnosis of asthma were enrolled. The questionnaire of asthma control level and family management was used to investigate the influencing factors for asthma control level and the indicators of family management. The awareness of childhood asthma and its management was analyzed among the parents, as well as the influence on asthma control level in children, and the association between them was analyzed.@*RESULTS@#Compared with the non-complete control group, the complete control group had significantly longer course of asthma and treatment time (P<0.05). The proportions of asthma attacks ≥3 times and aerosol treatment for asthma attacks >3 times in one year in the complete control group were significantly lower than those in the non-complete control group (P<0.05). The complete control group had a significantly lower proportion of children with frequent respiratory infection, wheezing during respiratory infection, or a family history of allergic diseases (P<0.05). The parents in the complete control group had significantly stronger awareness of short-term escalation to asthma medication after respiratory infection and significantly enhanced management of maintenance medication (P<0.05). Compared with the complete control group, the non-complete control group had a significantly higher proportion of children with abnormal pulmonary function at the initial stage (P<0.05). The level of asthma control in children was associated with short-term escalation to asthma medication during respiratory infection and initial lung function (P<0.05).@*CONCLUSIONS@#The level of asthma control in children is closely associated with the severity of asthma and the comprehensive management of childhood asthma. Early treatment and family management, especially escalation to asthma medication during the early stage of respiratory infection, are of great importance in asthma control. Citation:Chinese Journal of Contemporary Pediatrics, 2023, 25(1): 73-79.

Actualización del consenso chileno SOCHINEP-SER para el manejo de las sibilancias recurrentes del preescolar / Update of the chilean consensus SOCHINEP-SER for the management of recurrent wheezing in preschool children

Las sibilancias recurrentes del preescolar son un problema prevalente. 50% de todos los niños tiene al menos un episodio de sibilancias en los primeros 6 años. Sin embargo, solo 4 % de los menores de 4 años tiene diagnóstico de asma. Por este motivo es fundamental realizar una adecuada anamnesis y examen físico tendientes a descartar causas secundarias, lo que debe ser complementado con exámenes de laboratorio de acuerdo con la orientación clínica. En la actualidad se recomienda indicar tratamiento de mantención con corticoides inhalados en aquellos niños que tengan episodios repetidos de obstrucción bronquial y que tengan una alta probabilidad de respuesta favorable a esta terapia. Se ha demostrado que aquellos pacientes que tienen un recuento de eosinófilos en sangre > 300 células por mm3 o aquellos que presentan una prueba cutánea positiva o IgE específicas positivas para alérgenos inhalados, responderán adecuadamente al tratamiento con esteroides inhalados.

Recurrent wheezing in preschoolers has a high prevalence. 50% of all children have at least one wheezing episode in the first six years of life. However, only 4% of children under four years of age are diagnosed with asthma. Therefore, it is essential to carry out an adequate medical history and physical examination to rule out secondary causes, which must be complemented with laboratory tests in accordance with clinical guidance. It is recommended to indicate maintenance treatment with inhaled corticosteroids to those children who have repeated episodes of wheezing and who have a high probability of a good response to this therapy. It has been demonstrated that those patients who have blood eosinophil count > 300 cells per mm3 or those who have a positive skin test or positive specific IgE for inhaled allergens will have a good response to inhaled corticosteroids.

Actualización del consenso chileno SOCHINEP-SER para el manejo de las sibilancias recurrentes del preescolar / Update of the chilean consensus SOCHINEP-SER for the management of recurrent wheezing in preschool children

Las sibilancias recurrentes del preescolar son un problema prevalente. 50% de todos los niños tiene al menos un episodio de sibilancias en los primeros 6 años. Sin embargo, solo 4% de los menores de 4 años tiene diagnóstico de asma. Por este motivo es fundamental realizar una adecuada anamnesis y examen físico tendientes a descartar causas secundarias, lo que debe ser complementado con exámenes de laboratorio de acuerdo con la orientación clínica. En la actualidad se recomienda indicar tratamiento de mantención con corticoides inhalados en aquellos niños que tengan episodios repetidos de obstrucción bronquial y que tengan una alta probabilidad de respuesta favorable a esta terapia. Se ha demostrado que aquellos pacientes que tienen un recuento de eosinófilos en sangre > 300 células por mm3 o aquellos que presentan una prueba cutánea positiva o IgE específicas positivas para alergenos inhalados responderán adecuadamente al tratamiento con esteroides inhalados.

Recurrent wheezing in preschoolers has a high prevalence. 50% of all children have at least one wheezing episode in the first six years of life. However, only 4% of children under four years of age are diagnosed with asthma. Therefore it is essential to carry out an adequate medical history and physical examination to rule out secondary causes, which must be complemented with laboratory tests in accordance with clinical guidance. It is recommended to indicate maintenance treatment with inhaled corticosteroids to those children who have repeated episodes of wheezing and who have a high probability of a good response to this therapy. It has been demonstrated that those patients who have blood eosinophil count > 300 cells per mm3 or those who have a positive skin test or positive specific IgE for inhaled allergens will have a good response to inhaled corticosteroids.

Quiste subglótico adquirido como causa inhabitual de estridor en pediatría. Revisión de la literatura a propósito de un caso / Subglottic cyst as an unusual cause of stridor in pediatric patients. Literature review and case report

El estridor corresponde a un signo altamente frecuente, sin embargo, es heterogéneo e inespecífico. Existen múltiples causas conocidas y manejadas por el otorrinolaringólogo. Los quistes subglóticos constituyen una entidad infrecuente de estridor en pediatría, siendo la población más frecuentemente afectada, niños con antecedentes de prematurez e intubación por períodos prolongados. Su manifestación clínica es variada, desde cuadros asintomáticos a pacientes con riesgo inminente de pérdida de la vía aérea. El diagnóstico suele ser tras largos períodos desde el antecedente de intubación. Su resolución suele ser quirúrgica, teniendo como principal complicación asociada la estenosis subglótica y las recurrencias. Se presenta el caso de una preescolar con un episodio de estridor y distrés respiratorio rápidamente progresivos, cuyo diagnóstico intraoperatorio resultó en quistes subglóticos submucosos bilaterales, que requirieron resolución quirúrgica.

Stridor corresponds to a highly frequent sign; however, it is heterogeneous and nonspecific. There are multiple causes that are widely known and managed by the otorhinolaryngolo-gist. Subglottic cysts are an infrequent entity of stridor in pediatric patients, where the most frequently affected population are childrens with history of prematurity and intubation for long periods. It's clinical manifestations are wide, from asymptomatic cases to patients with imminent risk of airway loss. Their manifestation its often after long periods after the moment of intubation. The management often involves surgery, and the main associated complication is subglottic stenosis and recurrences. We present the case of a preschool girl with an episode of rapidly progressive stridor and respiratory distress; whose intraoperative diagnosis was bilateral subglottic submucosal cysts that required surgical resolution.

Neuropatía laríngea y movimiento paradojal de cuerdas vocales en la edad pediátrica: reporte de un caso / Laryngeal neuropathy and paradoxical vocal fold motion in the pediatric age: case report

Resumen La neuropatía laríngea es una condición de hipersensibilidad, hiperreactividad e hiper-función laríngea secundaria a un desequilibrio entre las aferencias y eferencias laríngeas. La respuesta individual y exagerada frente a diversos gatillantes específicos puede generar síntomas como tos crónica, parestesia laríngea, carraspera, disfonía, estridor, sensación de globus faríngeo, movimiento paradojal de las cuerdas vocales (también conocido como disfunción cordal) y/o laringoespasmo. Existe abundante literatura sobre neuropatía laríngea en adultos, sin embargo, en niños es limitada. El objetivo de este artículo es dar a conocer un caso de neuropatía laríngea en la edad pediátrica y la importancia de su consideración en el enfrentamiento de estos pacientes. Se presenta caso clínico de un paciente de 13 años, con antecedente de cirugía cardiaca reciente, evoluciona con disfonía severa evidenciándose aparente inmovilidad cordal bilateral con resultados discordantes entre nasofibrolaringoscopía y electromiografía laríngea. Posteriormente presenta mejorías en su voz, sin embargo, se agregan otros síntomas laringológicos como carraspera, globus faríngeo y estridor no explicados por causas anatómicas. Se expone la evaluación y abordaje otorrinolaringológico-fonoaudiológico para el caso. Se concluye que el diagnóstico de neuropatía laríngea requiere un alto índice de sospecha clínica ante signos laringológicos sugerentes, debiendo descartarse causas orgánicas y estructurales. El abordaje otorrinolaringológico-fonoaudiológico constituye el pilar terapéutico asociado al uso de neuromoduladores en casos seleccionados.

Abstract Laryngeal neuropathy is a condition of hypersensitivity, hyperresponsiveness and laryngeal hyperfunction secondary to an imbalance between laryngeal afferent and efferent information. The individual and exaggerated response to diverse specific triggers can lead to symptoms such as chronic cough, laryngeal paresthesia, throat clearing, dysphonia, stridor, globus pharyngeus, vocal cord dysfunction, and/or laryngospasm. There is plentiful literature on laryngeal neuropathy in adults, however, in children, it is limited. Here, we present a case report of laryngeal neuropathy in the pediatric age and discuss the importance of its consideration in the approach of these patients. A case of a 13-year-old patient, recently intervened with cardiac surgery that evolves with severe dysphonia is presented. Nasofibrolaryngoscopy shows apparent bilateral vocal fold immobility with discordant results in laryngeal electromyography. Later, his voice improves but other laryngological symptoms appeared, such as throat clearing, globus pharyngeus and stridor, not explained by anatomical causes. The otolaryngological-speech therapy evaluation and approach for the case is exposed. We conclude that for the diagnosis of laryngeal neuropathy, a high index of clinical suspicion is required in the presence of suggestive laryngological symptoms, and organic and structural causes must be previously ruled out. The otorhinolaryngological-logopedic approach constitutes the mainstay of treatment associated with the use of neuromodulators in selected cases.

Factores sociodemográficos, clínicos y asistenciales asociados al impacto de la Covid-19 en la capacidad funcional respiratoria post alta hospitalaria / Sociodemographic, clinical and healthcare factors associated with the impact of Covid-19 on respiratory functional capacity after hospital discharge

La COVID-19 es una enfermedad infecciosa que causa importantes disfunciones respiratorias con repercusión a largo plazo, perdurando después del alta médico. En este estudio, se determinó la asociación entre los factores sociodemográficos, clínicos y asistenciales con el impacto de la COVID-19 en la capacidad funcional respiratoria post alta hospitalaria. Material y métodos: estudio observacional, analítico, transversal. La muestra conformada por 385 pacientes con diagnóstico de COVID-19. Se aplicó una ficha de recolección de datos, se estimó la capacidad funcional respiratoria. Se realizó un análisis bivariado mediante la prueba de chi cuadrado y razón de prevalencias considerando una significancia del 95% (p<0,05). Resultados: De los 385 pacientes, 228 (59,2%) se les percibió capacidad respiratoria normal y, disminuida en 157 (40,8%). Los adultos contemporáneos (55,84%) y masculinos (67,80%) fueron mayormente afectados. La comorbilidad (p= 0,292) y Co-Rads (p= 0,797) no mostraron estar directamente asociadas a la afectación respiratoria; contrario a RALE ≥ 3 en Radiografía de tórax (p=0,000). El tiempo en hospitalización, UCI y ventilación mecánica sugiere estar relacionado con la disminución respiratoria. Sin embargo, por RPc no hubo evidencia estadísticamente significativa. Pero, clínica severa 3.029 [1.611 ­ 5.696] p= 0.001 y RALE ≥3 4.079 [2.248 ­ 7.401] p= 0,000, arrojaron asociación. Conclusión: se identificaron como factores asociados el grado de severidad y RALE≥ 3 en radiografía de tórax. Se sugiere realizar estudios que confirme los hallazgos de esta investigación y se proponga un protocolo de rehabilitación integral dirigido a pacientes post covid-19 que les permita recuperar la normalidad en la capacidad funcional respiratoria(AU)

COVID-19 is an infectious disease that causes significant respiratory dysfunctions with long-term repercussions, lasting after medical discharge. In this study, association between socio-demographic, clinical and healthcare factors with the impact of COVID-19 on post-discharge respiratory functional capacity was determinated. Material and methods: observational study, analytical, cross-sectional study. The sample made up of 385 patients diagnosed with COVID-19. A data collection form was applied; the respiratory functional capacity was estimated. A bivariate analysis was performed using the fe chi square test and prevalence ratio, considering a significance of 95% (p<0.05). Results: of the 385 patients, 228 (59.2%) had normal respiratory capacity, and decreased in 157 (40.8%). Contemporary adults (55.84%) and males (67.80%) were mostly affected. Comorbidity (p= 0,292) and Co-Rads (p= 0,797) were not shown to be directly associated with respiratory involvement; contrary to RALE ≥ 3 in chest X-ray (p=0,000). The time in hospitalization, ICU and mechanical ventilation suggests to be related to the respiratory decrease. However, by PCR there was no statically significant evidence. But, clinical severe 3,029 [1,611 ­ 5,696] p= 0.001 and RALE ≥3 4,079 [2,248 ­ 7,401] p= 0,000, showed association. Conclusion: the degree of severity and RALE ≥ 3 in chest X-ray were identified as associated factors. It is suggest to carry out studies that confirm the findings of this research and propose a comprehensive rehabilitation protocol aimed at post-covid-19 patients that allows them to recover normal respiratory functional capacity(AU)

A propósito de un caso: divertículo de Kommerell / About a case: Kommerell's diverticulum

La presencia de sibilantes refractarios y localizados en una sola área de auscultación en los adultos, sobre todo si están acompañados de anormalidades espirométricas y radiológicas, son a menudo la expresión clínica de una condición que requiere un proceso diagnóstico más profundo, más allá del facilista diagnóstico de asma. Entre estas anomalías se encuentran los anillos vasculares como el divertículo de Kommerel, que es una variante con una prevalencia muy baja y se caracteriza por la presencia de un arco aórtico derecho con una salida de la arteria subclavia izquierda aberrante y que provoca síntomas por la compresión de las estructuras adyacentes a estas. Aportamos en este caso clínico, el proceso diagnóstico de uno de estos hallazgos incidentales: la presencia de sibilantes localizados en una paciente joven, asintomática con una espirometría alterada que podría hacernos sospechar la presencia de broncomalacia, una aspiración de cuerpo extraño, un proceso infeccioso, tumoral o un anillo vascular que pasó desapercibido hasta la edad adulta.

The presence of refractory and localized wheezing in a single area of auscultation in adults, especially if they are accompanied by spirometric and radiological abnormalities, are often the clinical expression of a condition that requires a deeper diagnostic process, beyond the easy diagnosis of asthma. Among these abnormalities are vascular rings such as Kommerell¨s diverticulum, which is a very low prevalence variant and it is characterized by the presence of a right aortic arch with an aberrant exit from the left subclavian artery and that causes symptoms due to compression of the adjacent structures. In this clinical case, we provide the diagnosis process of one of these incidental findings, such as the presence of localized wheezing in a young asyntomatic patient with altered spirometry that could lead us to suspect the presence of bronchomalacia, foreign body aspiration, an infection, tumor or vascular ring that went unnoticed until adulthood.

"Catatrenia": un trastorno del sueño poco conocido: descripción de tres casos clínicos pediátricos / Catathrenia: unknown sleep disorder: report 3 cases in pediatric patients

Catatrenia (gemido nocturno) es una condición rara caracterizada por sonidos irregulares que ocurren durante el sueño. Los comportamientos ocurren intermitentemente durante cualquiera de las dos etapas de sueño, REM o NREM y se caracterizan por gemidos prolongados, a menudo muy fuertes, socialmente perturbadores, durante la expiración. Es poco conocido y espera más definición y estudios terapéuticos. Hay pocos reportes y en su mayoría de pacientes adultos. Se presentan 3 casos en pacientes pediátricos.

Catathrenia (nocturnal groaning) is a rare condition characterized by irregular sounds that occur during sleep. The behaviors occur intermittently during either REM or NREM sleep and are characterized by prolonged, often very loud, socially disruptive groaning sounds during expiration. It is poorly understood and awaits further definition and therapeutic studies. There are few reports mostly adult patients are presented below 3 cases in pediatric patients.

The effect of recombinant human interferon α1b treatment of infants hospitalized with lower respiratory tract infection on subsequent wheezing

Abstract Objective: To investigate the impact of recombinant human interferon α1b (rhIFNα1b) treatment in infants hospitalized with lower respiratory tract infections on subsequent wheezing. Methods: The clinical data of infants (n = 540) with viral pneumonia, wheezy bronchitis, or bronchiolitis hospitalized in 19 Chinese hospitals from June 2009 to June 2015 were retrospectively analyzed. The parameters relevant to wheezing episodes within the last year were collected by telephone and questionnaires. The rhIFNα1b treatment group (n = 253) and control group (n = 287) were compared in terms of wheezing episodes within the last year. Moreover, the wheezing group (95 cases) and non-wheezing group (445 cases) were compared. Results: Out of 540 cases, 95 (17.6%) experienced wheezing episodes, 13.8% (35/253) cases treated with rhIFNα1b, and 20.9% (60/287) cases without rhIFNα1b experienced wheezing episodes within the last year. The rhIFNα1b treatment significantly improved wheezing episodes within the last year, compared with the control peers (p = 0.031). Single-factor regression showed statistically significant differences between the wheezing and non-wheezing groups in terms of age, rhIFNα1b use, childhood and family history of allergy, housing situation, and feeding history (p < 0.05). Binary logistic regression showed a childhood history of allergy (OR = 2.14, p = 0.004), no rhIFNα1b use (OR = 1.70, p = 0.028), and living in a crowded house (OR = 1.92, p = 0.012) might be risk factors of subsequent wheezing. Accordingly, breastfeeding (OR = 0.44, p = 0.008) and hospitalization age of 1-year-old (OR = 0.58, p = 0.024) were protective factors. Conclusions: Early use of rhIFNα1b in infants hospitalized with lower respiratory tract infections and breastfeeding could prevent subsequent wheezing. Living in a crowded house could promote subsequent wheezing.

Associated factors with recurrent wheezing in infants: is there difference between the sexes?

Abstract Objective: Identify associated factors for recurrent wheezing (RW) in male and female infants. Methods: Cross-sectional multicentric study using the standardized questionnaire from the Estudio Internacional sobre Sibilancias en Lactantes (EISL). The questionnaire was applied to parents of 9345 infants aged 12-15 months at the time of immunization/routine visits. Results: One thousand two hundred and sixty-one (13.5%) males and nine hundred sixty-three (10.3%) females have had RW (≥3 episodes), respectively (p10 colds episodes (OR = 3.46; IC 95% 2.35-5.07), air pollution (OR = 1.33; IC 95% 1.12-1.59), molds at home (OR = 1.23; IC 95% 1.03-1.47), Afro-descendants (OR = 1.42; IC 95% 1.20-1.69), bronchopneumonia (OR = 1.41; IC; 1.11-1.78), severe episodes of wheezing in the first year (OR = 1.56; IC 95% 1.29-1.89), treatment with bronchodilators (OR = 1.60; IC 95% 1.22-2,1) and treatment with oral corticosteroids (OR = 1,23; IC 95% 0.99-1,52). Associated factors for RW for females were passive smoking (OR = 1.24; IC 95% 1.01-1,51), parents diagnosed with asthma (OR = 1.32; IC 95% 1,08-1,62), parents with allergic rhinitis (OR = 1.26; IC 95% 1.04-1.53), daycare attendance (OR = 1.48; IC 95% 1.17-1,88), colds in the first 6 months of life (OR = 2.19; IC 95% 1.69-2.82), personal diagnosis of asthma (OR = 1.84; IC 95% 1.39-2.44), emergency room visits (OR = 1.78; IC 95% 1.44-2.21), nighttime symptoms (OR = 2.89; IC 95% 2.34-3.53) and updated immunization (OR = 0.62; IC 95% 0.41-0.96). Conclusion: There are differences in associated factors for RW between genders. Identification of these differences could be useful to the approach and management of RW between boys and girls.

Caracterización clinicoepidemiológica de niños con sibilancia recurrente / Clinical epidemiological characterization of children with recurrent wheezing

Introducción: Las sibilancias recurrentes en lactantes y preescolares son motivo frecuente de consulta en pediatría. Objetivo: Caracterizar a niños con sibilancia recurrente desde los puntos de vista clínico y epidemiológico. Métodos: Se realizó una investigación observacional, longitudinal y prospectiva de 140 niños con sibilancia recurrente, atendidos en el Servicio de Respiratorio del Hospital Infantil Docente Sur Dr. Antonio María Béguez César de Santiago de Cuba, desde de enero de 2016 hasta diciembre de 2018. Resultados: Predominaron el sexo masculino (57,1 %), los pacientes de procedencia urbana (62,1 %) y el área de salud 28 de septiembre como la de mayor incidencia (42,7 %). El promedio de edad fue de 1 año y 5 meses, aproximadamente. Los niños incluidos en el intervalo de 6 a 11 meses y 29 días fueron los más afectados (28,6 %). Entre los municipios, Guamá aportó el mayor número de casos (33,9 %). No existió asociación estadística entre el índice predictivo de asma y el sexo; sin embargo, resultó mayor entre los varones (19,3 %). Conclusiones: Las características clínicas y epidemiológicas de los pacientes con sibilancia recurrente de esta casuística no difieren del contexto epidemiológico mundial, siendo una entidad de observación frecuente en los servicios de pediatría.

Introduction: The recurrent wheezing in infants and preschool children are frequent reason of visit in pediatrics. Objective: To characterize children with recurrent wheezing from the clinical and epidemiologic points of view. Methods: An observational, longitudinal and prospective investigation of 140 children with recurrent wheezing, assisted in the Respiratory Service of Dr. Antonio María Béguez Cesar Southern Teaching Children Hospital was carried out in Santiago de Cuba, from January, 2016 to December, 2018. Results: There was a prevalence of the male sex (57.1 %), the patients of urban origin (62.1 %) and 28 de Septiembre health area as that of more incidence (42.7 %). The average age was of 1 year and 5 months, approximately. The children included in the interval from 6 to 11 months and 29 days were the most affected (28.6 %). Among the municipalities, Guamá contributed the higher number of cases (33.9 %). There was no statistical association between the prediction index of asthma and the sex; however, it was higher among males (19.3 %). Conclusions: The clinical and epidemiologic characteristics of the patients with recurrent wheezing of this case material don't differ from the world epidemiologic context, being an entity of frequent observation in the pediatrics services.

Papel do genoma e do microbioma na patogenia e abordagem terapêutica da asma / Role of the genome and microbiome in the pathogenesis and treatment of asthma

Asma é uma denominação única para um conjunto de disfunções respiratórias que se expressam, clinicamente, por episódios repetidos, com intensidade variável de dispneia, sibilos, tosse e opressão torácica. A variação entre suas formas clínicas é resultante da participação e interação entre fatores genéticos, microbiômicos e ambientais. O progresso na área médica, ao incorporar novos recursos tecnológicos da biociência e bioinformática, vem desvendando a intimidade dos processos genéticos e moleculares envolvidos nos diferentes mecanismos patogênicos presentes na asma. Isso vem levando à identificação de novos alvos terapêuticos e à pesquisa de novos agentes medicamentosos. Ao mesmo tempo, a perspectiva de inserção paulatina desses recursos no cotidiano médico tem promovido mudanças na prática médica, que vem adotando os princípios da medicina de precisão. Possivelmente, estas mudanças melhorarão o horizonte dos asmáticos, uma população ainda desprovida de instrumentos terapêuticos totalmente efetivos.

Asthma is a unique designation for a set of respiratory dysfunctions clinically expressed by repeated episodes of varying intensity of dyspnea, wheezing, cough, and chest oppression. The variation between its clinical forms is the result of the participation and interaction between genetic, microbiomic, and environmental factors. Progress in the medical field, with incorporation of new technological resources from bioscience and bioinformatics, has been unveiling the intimacy of genetic and molecular processes involved in the different pathogenic mechanisms present in asthma. This has led to the identification of new therapeutic targets and the search for new therapeutic agents. At the same time, the perspective of gradual insertion of these resources in daily medical activities has been promoting changes in medical practice, which has been adopting the principles of precision medicine. Possibly, these changes will provide a better future for asthmatic patients, a population still devoid of fully effective therapeutic instruments.

Mepolizumab as a steroid-sparing treatment option in a patient with eosinophilic granulomatosis with polyangiitis / Mepolizumabe como terapêutica poupadora de esteroides num paciente com granulomatose eosinofílica com poliangiite

Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystem disorder characterized by asthma, peripheral blood eosinophilia, and signs of vasculitis. Glucocorticoids are considered the cornerstone of treatment, but most patients remain steroid-dependent and carry a significant burden of adverse effects. We report a case of a patient with steroid-dependent EGPA successfully treated with mepolizumab. A 36-year-old man presented with persistent rhinitis, dyspnea, wheezing, and dry cough poorly controlled with inhaled therapy. Eosinophilia in peripheral blood and bronchoalveolar lavage fluid was seen. Histological findings from nasal mucosa revealed eosinophilic microabscesses and vasculitis without granulomas compatible with EGPA diagnosis. After daily oral prednisolone (PSL) was started, symptoms and eosinophilia improved, but adverse effects emerged. Attempts at tapering off PSL resulted in worsening of symptoms. He started mepolizumab 300 mg monthly, with clinical improvement and sustained disease remission, which allowed reducing the need for PSL. We present a very disabling steroiddependent EGPA. Mepolizumab was able to taper off PSL while maintaining symptomatic control.

Granulomatose eosinofílica com poliangiite (EGPA) é uma doença multissistêmica caracterizada por asma, eosinofilia no sangue periférico e sinais de vasculite. Os corticoides são considerados a base do tratamento, no entanto, a maioria dos pacientes permanece dependente deste tratamento com os seus efeitos adversos associados. Relatamos o caso de um paciente com granulomatose eosinofílica dependente de esteroides com poliangiite (EGPA) tratado com sucesso com mepolizumabe. Um homem de 36 anos apresentou rinite persistente, dispneia, sibilos e tosse seca mal controlada com terapia inalada. Observou-se eosinofilia no sangue periférico e no lavado broncoalveolar. Os achados histológicos da mucosa nasal revelaram microabscessos eosinofílicos e vasculite sem granulomas compatíveis com o diagnóstico de EGPA. Após o início da prednisolona oral diária (PSL), os sintomas e a eosinofilia melhoraram, mas surgiram efeitos adversos. As tentativas de redução gradual da PSL resultaram no agravamento dos sintomas. Iniciou mepolizumabe 300 mg mensalmente, com melhora clínica e remissão sustentada da doença, o que permitiu reduzir a necessidade de PSL. Apresentamos um EGPA dependente de esteroides muito incapacitante. O mepolizumab foi capaz de diminuir o PSL mantendo o controle sintomático sustentado.

Factors associated with recurrent wheezing in the first year of life among premature newborns from Neonatal Intensive Care Units / Fatores associados à sibilância recorrente no primeiro ano de vida entre recémnascidos prematuros provenientes de Unidades de Terapia Intensiva Neonatais

Abstract Objectives: to determine the prevalence and factors associated with recurrent wheezing in the first year of life among premature newborns from Neonatal Intensive Care Units, in the city of Montes Claros, northern Minas Gerais. Methods: cross-sectional study, with data collection from medical records of a follow-up clinic, interviews with mothers and, eventually, search in hospital records. Bivariate analyzes were carried out between sociodemographic and clinical characteristics and recurrent wheezing. Variables associated up to the level of 20% were analyzed by binary logistic regression, and associations defined by the Odds Ratio and respective 95% confidence intervals. Only variables associated with a 5% significance level were maintained in the final model of logistic regression. Results: among 277 infants studied, about one fifth (21.3%) were extremely low birth weight preterm and more than half (60.7%) had birth weight below 1500 grams. The prevalence of recurrent wheezing was 14.4% (CI95% = 10.3-18.4). Mechanical ventilation (OR = 2.12; CI95% = 1.09-4.76; p = 0.030) and oxygen therapy time ≥ 15 days (OR = 2.49; CI95%= 1.12-5.00; p = 0.010) were the risk factors for the event. Conclusions: there is a high prevalence of recurrent wheezing in the evaluated group and the associated variables reiterate the risk of prolonged oxygen therapy and mechanical ventilation for premature newborns.

Resumo Objetivos: determinar a prevalência e fatores associados à sibilância recorrente no primeiro ano de vida entre recém-nascidos prematuros egressos de Unidades de Terapia Intensiva Neonatais, na cidade de Montes Claros, norte de Minas Gerais. Métodos: estudo transversal, com coleta de dados em prontuários de ambulatório de seguimento, entrevistas com mães e, eventualmente, consultas aos prontuários hospitalares. Foram realizadas análises bivariadas entre as características sociodemográficas e clínicas e a sibilância recorrente. As variáveis associadas ao desfecho até um nível de significância de p ≤20% foram analisadas por regressão logística binária e as associações definidas pelas Odds Ratios e respectivos intervalos de confiança de 95%. Somente variáveis associadas a um nível de significância de 5% foram mantidas no modelo final de regressão logística. Resultados: entre 277 crianças estudadas, cerca de um quinto (21,3%) eram prematuros de extremo baixo peso e mais da metade (60,7%) tinha peso de nascimento abaixo de 1500 gramas. A prevalência de sibilância recorrente foi de 14,4% (IC95%=10,3-18,4). Ventilação mecânica (OR=2,12; IC95%= 1,09-4,76; p=0,030) e tempo de oxigenioterapia ≥15 dias (OR=2,49; IC95%=1,12-5,00; p=0,010) foram os fatores de risco para o evento. Conclusão: existe uma elevada prevalência de sibilância recorrente no grupo avaliado e as variáveis associadas reiteram o risco do uso prolongado de oxigenioterapia e da ventilação mecânica para recém-nascidos prematuros.

Diagnostic challenges in paediatric foreign body aspiration: a case report / Desafío diagnóstico en la aspiración de cuerpo extraño en pediatría: reporte de un caso

Abstract Introduction Foreign body aspiration (FBA) is a potentially fatal paediatric emergency. Our objective was to highlight the importance of a multidisciplinary approach to difficult/doubtful diagnosis. Case report 34-month-old girl referred for urgent rigid bronchoscopy after suspected metallic blade ingestion (found chewing on it). She had a previous recurrent history of wheezing. The physical examination revealed face/lip wounds, traces of powder on her teeth but no breathing difficulty. The plain X-Ray revealed radiopaque images of the upper pulmonary field and gastric chamber. In the absence of FBA clinical signs but considering a previous history of bronchial hyperresponsiveness, a direct digital radiographic study was performed. There were no images compatible with foreign bodies: the results were interpreted as artefacts and no bronchoscopy was performed. Conclusions A careful pre-anaesthetic evaluation, a high level of suspicion and excellent multidisciplinary communication led to the recognition of false radiologic findings. A conservative approach was followed and invasive procedures in a remote location, with high anaesthetic risk for the paediatric population were avoided.

Resumen Introducción La aspiración de cuerpo extraño (ACE) es una emergencia pediátrica potencialmente fatal. La intención del presente artículo es resaltar la importancia de un abordaje multidisciplinario en caso de un diagnóstico difícil/dudoso. Reporte de caso Se trata de una paciente de 4 meses de edad remitida para broncoscopia rígida de urgencia, luego de la sospecha de ingestión de una hojilla metálica (se encontró a la bebé mordiéndola). La paciente tenía antecedentes de sibilancia. Al examen físico se encontraron heridas en la cara y los labios, rastros de polvo en los dientes, pero no había dificultad respiratoria. El examen de rayos-x mostraba imágenes radio opacas en el cuadrante superior derecho del pulmón y en la cámara gástrica. En virtud de la ausencia de signos clínicos de ACE en una paciente con antecedentes de hiperresponsividad bronquial, se realizó un estudio radiográfico digital. No hubo imágenes compatibles con cuerpos extraños: los resultados se interpretaron como artefactos y no se realizó la broncoscopia. Conclusiones Un cuidadoso examen pre-anestesia, el alto grado de sospecha y una excelente comunicación multidisciplinaria, permitieron el reconocimiento de hallazgos radiológicos falsos. Se siguió un abordaje conservador, evitando así procedimientos invasivos en lugares remotos con alto grado de riesgo para la población pediátrica.