A pediatric disease to keep in mind: diagnostic tools and management of bronchiectasis in pediatric age.

Ital J Pediatr

Pediatric Unit, Department of Medical and Surgical Sciences, S. Orsola - Malpighi Hospital, University of Bologna, Via Massarenti 11, 40138, Bologna, Italy.

Published: December 2017

Bronchiectasis in pediatric age is a heterogeneous disease associated with significant morbidity.The most common medical conditions leading to bronchial damage are previous pneumonia and recurrent lower airway infections followed by underlying diseases such as immune-deficiencies, congenital airway defects, recurrent aspirations and mucociliary clearance disorders.The most frequent symptom is chronic wet cough. The introduction of high-resolution computed tomography (HRCT) has improved the time of diagnosis allowing earlier treatment.However, the term "bronchiectasis" in pediatric age should be used with caution, since some lesions highlighted with HRCT may improve or regress. The use of chest magnetic resonance imaging (MRI) as a radiation-free technique for the assessment and follow-up of lung abnormalities in non-Cystic Fibrosis chronic lung disease is promising.Non-Cystic Fibrosis Bronchiectasis management needs a multi-disciplinary team. Antibiotics and airway clearance techniques (ACT) represent the pillars of treatment even though guidelines in children are lacking. The Azithromycin thanks to its antinflammatory and direct antimicrobial effect could be a new strategy to prevent exacerbations.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5747121PMC
http://dx.doi.org/10.1186/s13052-017-0434-0DOI Listing

Publication Analysis

Top Keywords

pediatric age
12
bronchiectasis pediatric
8
pediatric
4
pediatric disease
4
disease mind
4
mind diagnostic
4
diagnostic tools
4
tools management
4
management bronchiectasis
4
age bronchiectasis
4

Similar Publications

Purpose: Heterozygous pathogenic variants in SPAST are known to cause Hereditary Spastic Paraplegia 4 (SPG4), the most common form of HSP, characterized by progressive bilateral lower limbs spasticity with frequent sphincter disorders. However, there are very few descriptions in the literature of patients carrying biallelic variants in SPAST.

Methods: Targeted Sanger sequencing, panel sequencing and exome sequencing were used to identify the genetic causes in 9 patients from 6 unrelated families with symptoms of HSP or infantile neurodegenerative disorder.

View Article and Find Full Text PDF

BACKGROUND Arterioportal fistulas (APFs) are abnormal connections between the arterial and portal venous systems, leading to portal hypertension (PH) and symptoms such as gastrointestinal bleeding, splenomegaly, and hepatic pain. Symptoms typically appear by the age of 2 years in about 75% of cases. CASE REPORT A 7-year-old boy with an asymptomatic APF developed life-threatening complications following a Clostridium difficile infection.

View Article and Find Full Text PDF

Impact of enzyme replacement therapy on clinical manifestations in females with Fabry disease.

Orphanet J Rare Dis

December 2024

Department of Internal Medicine D, and Interdisciplinary Fabry Center (IFAZ), University Hospital Muenster, Muenster, Germany.

Background: The aim of our multicenter study was to investigate the implementation of the European Fabry guidelines on therapeutic recommendations in female patients with Fabry disease (FD) and to analyze the impact of enzyme replacement therapy (ERT) in treated and untreated females.

Results: Data from 3 consecutive visits of 159 female FD patients from 6 Fabry centers were retrospectively analyzed. According to their treatment, patients were separated in 3 groups (untreated, n = 71; newly ERT-treated, n = 47; long-term ERT-treated, n = 41).

View Article and Find Full Text PDF

Assessing the impact of the iPeer2Peer program for adolescents with juvenile idiopathic arthritis: a mixed-methods randomized controlled trial.

Pediatr Rheumatol Online J

December 2024

Child Health Evaluative Sciences, Research Institute, The Hospital for Sick Children, 686 Bay Street, Room 06.9715, Toronto, ON, M5G 0A4, Canada.

Background: Juvenile Idiopathic Arthritis (JIA) is a chronic pediatric illness, whereby youth experience physical, emotional and psychosocial challenges that result in reduced health related quality of life (HRQL). Peer mentoring has been shown to improve disease self-management in adults with chronic conditions, with mixed results in younger populations. Building on our pilot work - which supported the feasibility and initial effectiveness of the iPeer2Peer program - the objective of this study was to assess the clinical effectiveness of the program in youth with JIA through a waitlist randomized controlled trial.

View Article and Find Full Text PDF

Familial hypercholesterolemia in Chinese children and adolescents: a multicenter study.

Lipids Health Dis

December 2024

Department of Endocrinology, Children's Hospital of Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, Zhejiang, 310052, China.

Background: Familial hypercholesterolemia (FH) is an inherited disorder mainly marked by increased low-density lipoprotein cholesterol (LDL-C) concentrations and a heightened risk of early-onset arteriosclerotic cardiovascular disease (ASCVD). This study seeks to characterize the genetic spectrum and genotype‒phenotype correlations of FH in Chinese pediatric individuals.

Methods: Data were gathered from individuals diagnosed with FH either clinically or genetically at multiple hospitals across mainland China from January 2016 to June 2024.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!