Disseminated visceral Kaposi´s sarcoma in a pediatric bilateral lung transplant recipient.

Cancer is a major adverse outcome of solid organ transplantation, and risks are especially high for malignancies caused by viral infections. HHV-8 is the etiologic agent of Kaposi´s sarcoma (KS). We report a case of visceral KS occurring in a 15-year-old patient after lung transplantation. The evolution was dramatically fast and interestingly, KS lesions were diffusely observed, but not in the skin. The autopsy showed the presence of numerous tumoral lesions in many organs. Microscopically, they all had very similar features, regardless of the organ affected. KS presented without cutaneous involvement. The girl was not tested for HHV-8 prior to transplantation as it was not part of our protocol. The donor was negative. The aim of the report is to alert other teams, especially those working in pediatrics, about this rare but potential complication in the setting of solid organ transplantation.

Paediatric bilateral vocal cord paralysis: our experience. / Parálisis bilateral de cuerdas vocales en pediatría: nuestra experiencia.

Bilateral vocal cord paralysis (BVCP) is the second most common cause of neonatal stridor. The aim of this study was to describe the demographic features, aetiology, comorbidities, and management of our patients with BVCP. MATERIAL AND METHODS: We conducted a retrospective review of the clinical charts of all patients diagnosed with BVCP seen at the Department of Respiratory Endoscopy between 2011 and 2015. RESULTS: 47 patients were included. Mean age at diagnosis was 1 month and male sex predominated (63%). The aetiology was congenital in 59% and acquired in 41% of the infants. The cause was most frequently idiopathic in the former group and secondary to postoperative injury in the latter. Overall, 42 patients (89%) required tracheostomy, without statistically significant differences between the causes. Of all the patients, 39% regained vocal-cord mobility; 44% of those with congenital BVCP, 31% of those with acquired BVCP and 62.5% with idiopathic BVCP. In five patients a laryngotracheoplasty was performed with a posterior costal cartilage graft and one underwent posterior cordectomy. All were decannulated. In one patient vocal-cord lateralization was performed, avoiding tracheostomy. CONCLUSION: BVCP was most commonly of congenital cause and was mainly idiopathic within this group of patients, with a slight male preponderance. A high percentage of patients required tracheostomy. A higher recovery rate of vocal-cord mobility was observed in idiopathic BVCP, which allowed for successful decannulation. In this series, decannulation was possible in all patients that underwent surgery; however, further studies with comparison of techniques and objective assessment of swallowing and phonation are necessary.

[Cough and hypoxemia as clinical manifestation of pulmonary alveolar proteinosis. Clinical case report]. / Tos e hipoxemia como manifestación clínica de la proteinosis alveolar. Reporte de caso clínico.

Alveolar proteinosis is a rare chronic lung disease, especially in children, characterized by abnormal accumulation of lipoproteins and derived surfactant in the intra-alveolar space that generates a severe reduction of gas exchange. Idiopathic presentation form constitutes over 90% of cases, a phenomenon associated with production of autoimmune antibodies directed at the receptor for granulocyte-macrophage colony-stimulating factor. A case of a girl of 5 years of age treated because of atypical pneumonia with unfavorable evolution due to persistent hypoxemia is presented. The diagnosis is obtained through pathologic examination of lung biopsy by thoracotomy, as treatment is carried out by 17bronchopulmonary bronchoscopy lavages and the patient evidences marked clinical improvement.

La proteinosis alveolar es una enfermedad pulmonar crónica poco frecuente, especialmente en pediatría, caracterizada por la acumulación anormal de lipoproteínas y derivados del surfactante en el espacio intraalveolar, que genera una grave reducción del intercambio gaseoso. La forma de presentación idiopática constituye más del 90% de los casos relacionados con un fenómeno de autoinmunidad, con producción de anticuerpos dirigidos contra el receptor del factor estimulante de colonias de granulocitos y macrófagos. Se presenta un caso clínico de una niña de 4 años de edad tratada por neumonía atípica con evolución desfavorable por hipoxemia persistente. El diagnóstico se obtuvo a través del estudio anatomopatológico de la biopsia pulmonar por toracotomía. Se llevaron a cabo 17 lavados broncopulmonares mediante endoscopía respiratoria y la paciente evidenció franca mejoría clínica.

Complications in children from foreign bodies in the airway.

INTRODUCTION AND OBJECTIVES: Foreign body aspiration in childhood is a common and potentially serious problem. Complications may be the result of the aspiration episode itself, delayed diagnosis or treatment. We describe our experience in a paediatric hospital in Argentina. METHODS: We retrospectively evaluated 56 patients with complications due to foreign body aspiration recorded in the Susy Safe Project between January 2010 and November 2013. The clinical variables analysed were sex, age at time of aspiration, foreign body location and type, time elapsed from the event until object removal, extraction technique, complications, need for hospitalisation and circumstances of the event. RESULTS: 58.9% of the cases described occurred in males, with high presence of adults (76.8%) at the time of aspiration. The incidence was slightly higher in children older than 3 years. In 37 cases (66.1%), the foreign body was located in bronchus; sunflower seeds and ballpoint caps were the most common foreign objects. Only in 10 cases (17.9%) was the object extracted within 24h of the event. The most common complications were pneumonia (18 cases), granuloma (15 cases) and mucosal erosion (9 cases). Hospitalisation was necessary for 41 patients. CONCLUSION: Early diagnosis and immediate control through specialised teams are essential to ensure proper treatment, usually endoscopic, without risk of complications.

[Clinical consensus statement on the care of the child with a tracheostomy]. / Consenso sobre el cuidado del niño con traqueostomía.

The care of the child with a tracheostomy deserves special attention because of the potential devastating airway compromise and because of the need of competent care by caregivers and professionals. The recommendations on tracheostomy care published are few and approaches are inconsistent among different institutions. This clinical consensus statement aims to improve care for children with tracheostomies. A literature search was conducted, reviewed and revised by this group of experts, who concurred with these statements, based on the best evidence available and taking into account the local context.

El cuidado del niño con traqueostomía merece especial atención por el potencial riesgo para la vida que podría ocasionarse con el compromiso súbito de la vía aérea y por la necesidad de contar con cuidadores y profesionales competentes para su cuidado. Los objetivos principales de este consenso son unificar criterios, promover prácticas seguras, fomentar el uso racional de los recursos y contribuir a optimizar la calidad de vida de los niños con traqueostomías y sus familias. Para poder cumplir con estos propósitos, se realizó una búsqueda exhaustiva de la literatura científica; se seleccionó, sobre la base del análisis crítico, la mejor evidencia disponible; y se formularon, por consenso interdisciplinario, una serie de recomendaciones prácticas y adaptables al contexto local.

[Diagnosis and treatment of pediatric subglottic stenosis: experience in a tertiary care center]. / Diagnóstico y tratamiento de las estenosis subglóticas en pediatría: experiencia en un hospital de alta complejidad.

Subglottic stenosis is among the most common causes of airway obstruction in children, 90% of which resulting from endotracheal intubation. The diagnosis is based on the patient's clinical, radiologic evaluation, flexible laryngoscopy and rigid airway endoscopy under general anesthesia. It must be suspected in children with respiratory distress after extubation. The therapeutic approach depends on the severity of the subglottic stenosis and the patient's symptoms. We describe our experience with the subglottic stenosis etiologies, diagnosis, treatment and outcome of patients with this condition.

Metabolomics of plant saponins: bioprospecting triterpene glycoside diversity with respect to mammalian cell targets.

One of the goals of cancer chemotherapy and prevention is the discovery of compounds that are relatively selective to tumor cells and, therefore, have reduced effects on normal cell growth. In previously published studies, it was shown that certain triterpene saponins (called avicins) from a desert tree, Acacia victoriae, are selectively toxic to tumor cells at very low doses (IC(50): 0.2 microg/mL for Jurkat cells). To extend this research to human clinical studies, we needed to find a reliable supply of avicins and have developed a transformed "hairy root" culture as a means of biomass production. Protocols were optimized for A. victoriae micropropagation; after a boiling water treatment, A. victoriae seeds were maintained under in vitro conditions on defined media. Embryo-axis explants from shoot tips were removed and infected with Agrobacterium rhizogenes Conn (R 1000) for hairy root induction. Plasmid integration was confirmed by PCR analysis with a primer set for a segment of the rol B gene. Culture conditions have been optimized for root biomass production, and various inducers have been investigated for enhancement of avicin production. Hairy root cultures were compared with intact pod tissue from field-grown sources for avicin content following partial purification of triterpene glycosides and HPLC separation of the secondary metabolites. From bioassays of the collected HPLC fractions, we have identified putative triterpene "metabolic clusters" with enhanced activity against tumor cells. This now provides a system for both production of clinical trial lots of active samples, but also a means to correlate structure of individual triterpene glycosides with specific cellular target activity in mammalian cells.

[Severe laryngitis associated to gastroesophageal reflux]. / Laringitis graves asociadas a reflujo gastroesofágico.

There is a strong association between gastroesophageal reflux and pharyngolaryngeal reflux as factors leading to respiratory disease, manifested as dysphonia, wheezing, coughing, recurrent laryngitis, bronchial obstruction, laryngospasm and apparent life-threatening events (ALTEs). These manifestations can be mild or severe and may sometimes put the patient's life at risk. We present two cases of patients with severe laryngitis who required endotracheal intubation, one of which underwent tracheostomy. The diagnostic methods and their limitations and the patients outcomes are described.

[Foreign bodies in esophagus in children: case series]. / Cuerpos extraños en el esófago en los niños: Serie de casos.

Ingestion of foreign bodies is an avoidable accident that is seen mainly in children under 3 years-old. Most of them pass through the digestive tract without causing clinical manifestations or complications, but a significant percentage is impacted in the esophagus causing vomiting, sore throat, dysphagia and drooling. The most common foreign bodies are coins. Complications usually occur when there is a delay in diagnosis or with large, sharp or potentially toxic objects, as the button battery. It is essential to make differential diagnosis between coin and button battery, since the latter requires urgent removal due to the earliness of the injury caused. We report 115 cases of foreign bodies in the esophagus, and we alert the pediatrician in recognizing and preventing this problem.

[Delayed diagnosis of foreign body in the airway in children: case series]. / Demora en el diagnóstico de un cuerpo extraño en la vía aérea en los niños: Serie de casos.

Aspiration of foreign bodies is an important and preventable cause of morbidity and mortality in childhood. The early diagnosis and treatment are essential for risk of mortality in the acute and complications arising from the continuance of a foreign body in the airway. The clinical presentation may mimic different diseases, delaying the correct diagnosis. Pediatricians should be aware of the possibility of foreign body in children with persistent respiratory symptoms, even in the absence of a history of choking. Bronchoscopy is indicated in all patients with suspected aspiration, even when the physical and radiological examination is inconclusive. We evaluate in 90 cases the time between the aspiration of foreign body and the removal, and emphasize the need for preventive measures and greater dissemination of knowledge in the community and health professionals about this problem.

Post-intubation subglottic stenosis in children. Diagnosis, treatment and prevention of moderate and severe stenosis.

INTRODUCTION AND OBJECTIVES: Subglottic stenosis is one of the most common causes of upper airway obstruction. Almost 90% of them result from endotracheal intubation. Therapy depends on the degree of stenosis, among other factors. Therapeutic approaches range from watchful waiting, in mild stenosis, to complex surgery for severe cases. We report our experience on the surgical management of post-intubation subglottic stenosis in children, emphasising the need for recognition and prevention of predisposing factors of post-intubation stenosis. METHODS: We retrospectively evaluated 71 patients with moderate to severe post-intubation subglottic stenosis, operated in the Respiratory Endoscopy Service in a period of eight years. The clinical variables analysed were age at surgery, degree of stenosis, surgical technique, complications and outcome. RESULTS: In 84.5% of patients, only 1 surgical approach was required to achieve decannulation. Three surgical techniques were implemented as therapy: laryngotracheal reconstruction, partial cricotracheal resection and anterior cricoid split. Decannulation was achieved in 70 cases. In 71.8%, ventilation, swallowing and voice qualities were good; 23.9% presented dysphonia; and 2.8% presented a mild respiratory distress. One patient died. CONCLUSION: In patients with subglottic stenosis, selection of the most accurate treatment is the key to success, reducing the number of surgeries and preventing complications.

Management of foreign bodies in the airway and oesophagus.

BACKGROUND: Ingestion and/or aspiration of foreign bodies (FB) are avoidable incidents. Children between 1 and 3 years are common victims for many reasons: exploration of the environment through the mouth, lack of molars which decreases their ability to properly chew food, lack of cognitive capacity to distinguish between edible and inedible objects, and tendency to distraction and to perform other activities, like playing, whilst eating. Most FBs are expelled spontaneously, but a significant percentage impacts the upper aerodigestive tract. Approximately 80% of children's choking episodes are evaluated by pediatricians. The symptoms of aspiration or ingestion of FBs can simulate different paediatric diseases such as asthma, croup or pneumonia, delaying the correct diagnosis. SYMPTOMS: There are three clinical phases both in aspiration and in ingestion of FBs: initial stage (first stage or impaction or FB) shows choking, gagging and paroxysms of coughing, obstruction of the airway (AW), occurring at the time of aspiration or ingestion. These signs calm down when the FB lodges and the reflexes grow weary (second stage or asymptomatic phase). Complications occur in the third stage (also defined as complications' phase), when the obstruction, erosion or infection cause pneumonia, atelectasis, abscess or fever (FB in AW), or dysphagia, mediastinum abscess, perforation or erosion and oesophagus (FB in the oesophagus). The first symptoms to receive medical care may actually represent a complication of impaction of FB. LOCATIONS AND MANAGEMENT: Determining the site of obstruction is important in managing the problem. The location of the FB depends on its characteristics and also on the position of the person at the time of aspiration. Determining the site of obstruction is important in managing the problem. Larynx and trachea have the lowest prevalence, except in children under 1year. They are linked with the most dangerous outcomes, complete obstruction or rupture. Bronchus is the preferred location in 80-90% of AW's cases. Esophageal FBs are twice more common than bronchial FBs, although most of these migrate to the stomach and do not require endoscopic removal. Diagnosis of FB proceeds following the traditional steps, with a particular stress on history and radiological findings as goal standards for the FB retrieval. The treatment of choice for AW's and esophageal FBs is endoscopic removal. Endoscopy should be carried out whenever the trained personnel are available, the instruments are checked, and when the techniques have been tested. The delay in the removal of FBs is potentially harmful. The communication between the endoscopist and the anaesthesiologist is essential before the procedure to establish the plan of action; full cooperation is important and improves the outcome of endoscopy. CONCLUSIONS: Ingestion and or aspiration of FB in children are multifactorial in their aetiology, in their broad spectrum of different resolutions for the same FB and in the response of each patient to the treatment. Prevention remains the best treatment, implying an increased education of parents on age-appropriate foods and household items, and strict industry standards regarding the dimensions of toy parts and their secure containers.

Tos e hipoxemia como manifestación clínica de la proteinosis alveolar: Reporte de caso clínico / Cough and hypoxemia as clinical manifestation of pulmonary alveolar proteinosis: Clinical case report

La proteinosis alveolar es una enfermedad pulmonar crónica poco frecuente, especialmente en pediatría, caracterizada por la acumulación anormal de lipoproteínas y derivados del surfactante en el espacio intraalveolar, que genera una grave reducción del intercambio gaseoso. La forma de presentación idiopática constituye más del 90% de los casos relacionados con un fenómeno de autoinmunidad, con producción de anticuerpos dirigidos contra el receptor del factor estimulante de colonias de granulocitos y macrófagos. Se presenta un caso clínico de una niña de 4 años de edad tratada por neumonía atípica con evolución desfavorable por hipoxemia persistente. El diagnóstico se obtuvo a través del estudio anatomopatológico de la biopsia pulmonar por toracotomía. Se llevaron a cabo 17 lavados broncopulmonares mediante endoscopía respiratoria y la paciente evidenció franca mejoría clínica.

Alveolar proteinosis is a rare chronic lung disease, especially in children, characterized by abnormal accumulation of lipoproteins and derived surfactant in the intra-alveolar space that generates a severe reduction of gas exchange. Idiopathic presentation form constitutes over 90% of cases, a phenomenon associated with production of autoimmune antibodies directed at the receptor for granulocyte-macrophage colony-stimulating factor. A case of a girl of 5 years of age treated because of atypical pneumonia with unfavorable evolution due to persistent hypoxemia is presented. The diagnosis is obtained through pathologic examination of lung biopsy by thoracotomy, as treatment is carried out by 17bronchopulmonary bronchoscopy lavages and the patient evidences marked clinical improvement.

[Surgical treatment of tracheal stenosis in pediatrics]. / Tratamiento quirúrgico de la estenosis traqueal en pediatría.

Twenty patients with tracheal stenosis were surgically treated between July 2005 and May 2008; ten patients had a congenital stenosis and ten an acquired one. Global survival was 85%. Three patients died: 1 with acquired stenosis and 2 with congenital stenosis. From 17 alive patients, 15 remain asymptomatic; 2 patients operated on due to congenital stenosis, are under continuous follow-up (both with stents placed in the trachea). Surgical treatment of acquired stenosis presents less severe complications, requires less ventilatory support and hospital stay. Conversely, congenital stenosis often requires more than one procedure to control the disease, and presents a higher mortality rate.

Laringitis graves asociadas a reflujo gastroesofágico / Severe laryngitis associated to gastroesophageal reflux

Está demostrado que hay una fuerte asociación entre el reflujo gastroesofágico y el reflujo faringolaríngeo como causantes de enfermedad respiratoria, que puede manifestarse como disfonía, estridor, tos, laringitis recurrente, obstrucción bronquial, laringoespasmo y eventos de aparente amenaza para la vida (ALTE). Estas manifestaciones pueden ser leves o graves y potencialmente mortales. Se presentan los casos de dos pacientes con laringitis grave que requirieron intubación endotraqueal, a uno de los cuales se le realizó una traqueotomía. Se describen los métodos diagnósticos, sus limitaciones y la evolución presentada por los pacientes

There is a strong association between gastroesophageal reflux and pharyngolaryngeal reflux as factors leading to respiratory disease, manifested as dysphonia, wheezing, coughing, recurrent laryngitis, bronchial obstruction, laryngospasm and apparent life-threatening events (ALTEs). These manifestations can be mild or severe and may sometimes put the patient’s life at risk. We present two cases of patients with severe laryngitis who required endotracheal intubation, one of which underwent tracheostomy. The diagnostic methods and their limitations and the patients outcomes are described.

Cuerpos extraños en el esófago en los niños: Serie de casos / Foreign bodies in esophagus in children: Case series

La ingestión de cuerpos extraños es un accidente evitable que se observa principalmente en niños menores de 3 años. La mayoría de ellos atraviesan el tubo digestivo sin ocasionar manifestaciones clínicas ni complicaciones; sin embargo, un porcentaje significativo se impacta en el esófago y causa vómitos, odinofagia, disfagia y sialorrea. Los cuerpos extraños más frecuentes son las monedas. Las complicaciones ocurren por lo general cuando se demora el diagnóstico o se trata de objetos grandes, afilados o potencialmente tóxicos, como la pila botón. Es fundamental realizar el diagnóstico diferencial entre una moneda y una pila botón, ya que esta última requiere su extracción urgente debido a la precocidad de las lesiones que produce. Se describen 115 casos de cuerpos extraños en el esófago. Se subraya la importancia de realizar un diagnóstico y tratamiento oportunos, así como de asesorar a los padres para la prevención.

Ingestion of foreign bodies is an avoidable accident that is seen mainly in children under 3 years-old. Most of them pass through the digestive tract without causing clinical manifestations or complications, but a significant percentage is impacted in the esophagus causing vomiting, sore throat, dysphagia and drooling. The most common foreign bodies are coins. Complications usually occur when there is a delay in diagnosis or with large, sharp or potentially toxic objects, as the button battery. It is essential to make differential diagnosis between coin and button battery, since the latter requires urgent removal due to the earliness of the injury caused. We report 115 cases of foreign bodies in the esophagus, and we alert the pediatrician in recognizing and preventing this problem.

Demora en el diagnóstico de un cuerpo extraño en la vía aérea en los niños: Serie de casos / Delayed diagnosis of foreign body in the airway in children: Case series

La aspiración de un cuerpo extraño es una causa importante y prevenible de morbimortalidad en la infancia. El diagnóstico y el tratamiento tempranos son esenciales por el riesgo de mortalidad en el episodio agudo y por las complicaciones derivadas de la permanencia de un cuerpo extraño en la vía aérea. La presentación clínica puede simular diferentes enfermedades y retrasar el diagnóstico correcto. Los pediatras deben ser conscientes de la posibilidad de la presencia de un cuerpo extraño en los niños con sintomatología respiratoria persistente, aun si no hay historia de asfixia. La broncoscopia está indicada en todo paciente con sospecha de aspiración, incluso cuando el examen físico y el radiológico no sean concluyentes. Evaluamos en 90 casos el tiempo transcurrido entre la aspiración del cuerpo extraño y su extracción, y enfatizamos la necesidad de medidas preventivas y de difusión de un mayor conocimiento en la comunidad y en los profesionales de la salud acerca de esta problemática.

Aspiration of foreign bodies is an important and preventable cause of morbidity and mortality in childhood. The early diagnosis and treatment are essential for risk of mortality in the acute and complications arising from the continuance of a foreign body in the airway. The clinical presentation may mimic different diseases, delaying the correct diagnosis. Pediatricians should be aware of the possibility of foreign body in children with persistent respiratory symptoms, even in the absence of a history of choking. Bronchoscopy is indicated in all patients with suspected aspiration, even when the physical and radiological examination is inconclusive. We evaluate in 90 cases the time between the aspiration of foreign body and the removal, and emphasize the need for preventive measures and greater dissemination of knowledge in the community and health professionals about this problem.

Tratamiento quirúrgico de la estenosis traqueal en pediatría / Surgical treatment of tracheal stenosis in pediatrics

Veinte pacientes con estenosis de tráquea fueron intervenidos quirúrgicamente entre julio de 2005y mayo de 2008, diez con estenosis congénita y diez de origen adquirido.La sobrevida global fue de 85 por ciento. Fallecieron tres pacientes, uno con estenosis adquirida y dos conestenosis congénita.De los diecisiete sobrevivientes, quince se encuentran asintomáticos; dos pacientes operados por estenosis congénita requieren controlesperiódicos (ambos con tutores endoluminales [stents] colocados).La estenosis traqueal adquirida presenta menos complicaciones, requiere menos asistencia respiratoria y menor estadía hospitalaria en el posoperatorio. Los pacientes con estenosis traquealcongénita necesitan, generalmente, másde un procedimiento terapéutico y presentan mayor mortalidad.