A few challenges in mucopolysaccharidosis type I.

Here we describe the current challenges of mucopolysaccharidosis type I: the need for an adequate classification, establishing its relationship to therapeutic indications; an early diagnosis, from neonatal screening, its advantages and barriers, to clinical suspicion of severe and attenuated forms; spinal and eye disease care, from diagnosis to follow-up and treatment; allergic reactions caused by enzyme replacement therapy, their diagnosis and treatment. And lastly, transition to adult care.

[Vascular rings. Series of 28 patients of a Pediatrie Respiratory Endoscopy Department].

Vascular rings account for 1 % of the congenital cardiovascular diseases. They constitute an embryological malformation in which the aortic arch, its branches, or the pulmonary arteries cause pressure on the trachea and/or oesophagus. Anatomically, they are divided into two groups -complete or incomplete- depending on how they surround the trachea and/or the oesophagus.

Paediatric bilateral vocal cord paralysis: our experience.

Unlabelled: 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.

[Cough and hypoxemia as clinical manifestation of pulmonary alveolar proteinosis. Clinical case report].

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.

Primary lung tumors in children: 24 years of experience at a referral center.

Purpose: Primary lung tumors are rare during childhood and encompass a wide variety of histological types. Each has a different biology and a different therapeutic approach. The aim of this article is to review the experience of a pediatric referral center with this kind of tumors during the last 24 years.

[Clinical consensus statement on the care of the child with a tracheostomy].

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.

[Diagnosis and treatment of pediatric subglottic stenosis: experience in a tertiary care center].

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.

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.

[Severe laryngitis associated to gastroesophageal reflux].

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.

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.

[Delayed diagnosis of foreign body in the airway in children: case series].

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.

[Foreign bodies in esophagus in children: case series].

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.

[Right postpneumonectomy syndrome: case report].

Postpneumonectomy syndrome is a rare entity in children. We report a case secondary to pneumonectomy performed due to an intrathoracic tumor. A 4-year-old boy with a history of right pneumonectomy and progressive respiratory distress, with extrinsic airway obstruction due to displaced mediastinal structures.

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.

[Ex utero intrapartum treatment - Management of neonatal congenital high airway obstruction syndrome: Case report].

Unlabelled: In recent years, the ex utero intrapartum treatment (EXIT), that involves extrauterine fetal intubation prior to delivery, has become relevant for the reduction in morbidity and mortality of neonates affected by congenital high airway obstruction syndrome (CHAOS).

Clinical Case: We report the case of the mother of an unborn child at pregnancy week 22, who was diagnosed a congenital pulmonary malformation that precluded intrapartum fetal circulatory deficit and resulted in the conduction of an EXIT technique, with the aim of ensuring fetal blood gas exchange at the time of delivery.

Conclusions: A timely practice of the EXIT technique resulted, by monitoring both maternal and fetal factors that might affect fetoplacental circulation, in the birth of a child whose immediate and long-term outcomes were successful allowing the child live a normal life.

[Surgical treatment of tracheal stenosis in pediatrics].

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.

[Cytomegalovirus detection in blood and bronchoalveolar lavage, and prophylaxis or preemptive treatment with ganciclovir in children with allogeneic stem cell transplantation].

Introduction: Cytomegalovirus disease is one of the complications of allogeneic stem cell transplantation (SCT). The objective of this study was to determine the frequency of CMV infection and CMV disease in children undergoing allogeneic SCT, and in whom antigenemia and bronchoalveolar lavage (BAL) were performed for early detection of CMV infection.

Methods: A total of 59 consecutive children who underwent allogeneic-identical, related SCT were prospectively evaluated.