Characteristics of Pediatric Emergency Department Presentations of Anaphylaxis in Spain.

Objective: This study aimed to identify the triggers of pediatric anaphylaxis in Spain and to analyze the circumstances of the episode.

Methods: Planned secondary analysis of a prospective observational multicenter study endorsed by the Spanish Society of Pediatric Emergencies including children aged younger than 18 years diagnosed with anaphylaxis in 7 Spanish pediatric emergency departments (EDs) between 2016 and 2018. We analyzed factors related to the anaphylaxis episode and its management.

Importance of Timely Treatment Initiation in Infantile-Onset Pompe Disease, a Single-Centre Experience.

Classic infantile Pompe disease (IPD) is a rare lysosomal storage disorder characterized by severe hypertrophic cardiomyopathy and profound muscle weakness. Without treatment, death occurs within the first 2 years of life. Although enzyme replacement therapy (ERT) with alglucosidase alfa has improved survival, treatment outcome is not good in many cases and is largely dependent on age at initiation.

Urine Phenylacetylglutamine Determination in Patients with Hyperphenylalaninemia.

Phenylketonuria (PKU), an autosomal-recessive inborn error of phenylalanine (Phe) metabolism is the most prevalent disorder of amino acid metabolism. Currently, clinical follow-up relies on frequent monitoring of Phe levels in blood. We hypothesize that the urine level of phenylacetylglutamine (PAG), a phenyl-group marker, could be used as a non-invasive biomarker.

Risk Factors for Severe Anaphylaxis in Children.

Objective: To identify risk factors associated with severe anaphylaxis in children.

Study Design: We carried out a multicenter prospective observational study including children less than 18 years old diagnosed with anaphylaxis in 7 Spanish pediatric emergency departments (EDs) between May 2016 and April 2018. Children were considered to have severe anaphylaxis if they met one or more of the following criteria: requirement for 2 or more doses of epinephrine, clinically important biphasic reaction, endotracheal intubation, intensive care unit admission, and/or death.

Management of anaphylaxis in Spain: pediatric emergency care providers' knowledge.

Background: Acute care providers must diagnose and treat patients with anaphylaxis. The objective was to analyze Spanish pediatric emergency departments' (ED) providers' knowledge of the international recommendations for the management of anaphylaxis.

Methods: A web-based survey including providers (both attending and residents) from seven Spanish pediatric EDs was conducted.

Retroperitoneal fibrosis: role of imaging in diagnosis and follow-up.

Retroperitoneal fibrosis (RPF) encompasses a range of diseases characterized by proliferation of aberrant fibroinflammatory tissue, which usually surrounds the infrarenal portion of the abdominal aorta, inferior vena cava, and iliac vessels. This process may extend to neighboring structures, frequently entrapping and obstructing the ureters and eventually leading to renal failure. The idiopathic form of RPF accounts for more than two-thirds of cases; the rest are secondary to factors such as drug use, malignancies, or infections.