Children

Research

10 pages, 398 KiB

Open AccessArticle

Unveiling the Respiratory Muscle Strength in Duchenne Muscular Dystrophy: The Impact of Nutrition and Thoracic Deformities, Beyond Spirometry

by Mine Yuksel Kalyoncu, Yasemin Gokdemir, Cansu Yilmaz Yegit, Muruvvet Yanaz, Aynur Gulieva, Merve Selcuk, Şeyda Karabulut, Neval Metin Çakar, Pinar Ergenekon, Ela Erdem Eralp, Gülten Öztürk, Olcay Unver, Dilsad Turkdogan, Yavuz Sahbat, Ahmet Hamdi Akgülle, Fazilet Karakoç and Bulent Karadag

Children 2024, 11(8), 994; https://doi.org/10.3390/children11080994 - 15 Aug 2024

Viewed by 811

Abstract

Background/Objectives: Duchenne muscular dystrophy (DMD) is the most prevalent progressive muscular dystrophy, and the guidelines recommend the regular assessment of respiratory muscle function. This study aimed to assess the relationship between maximum inspiratory pressure (MIP), maximum expiratory pressure (MEP) and sniff nasal inspiratory [...] Read more.

Background/Objectives: Duchenne muscular dystrophy (DMD) is the most prevalent progressive muscular dystrophy, and the guidelines recommend the regular assessment of respiratory muscle function. This study aimed to assess the relationship between maximum inspiratory pressure (MIP), maximum expiratory pressure (MEP) and sniff nasal inspiratory pressure (SNIP) measurements and upright-supine spirometry parameters in children with DMD, the predictability of upright–supine spirometry in terms of diaphragm involvement, and the impact of nutrition on muscle strength. Methods: This prospective cross-sectional study examined patients with DMD by comparing upright and supine FVC, MIP, MEP, and SNIP measurements. The effects of the ambulatory status, kyphoscoliosis, chest deformity, and low BMI on respiratory parameters were investigated. Results: Forty-four patients were included in the study. The mean patient age was 10.8 ± 2.9 years. Twenty-five patients were ambulatory. A significant decrease in FVC, FEV1, and FEF25–75 values was detected in the supine position in both ambulatory and non-ambulatory patients (p < 0.05). All patients had low MIP, MEP, and SNIP measurements (less than 60 cm H₂O). MIP, MEP, and SNIP values were significantly lower in patients with a low BMI than in those without (p < 0.05). Conclusions: To accurately assess respiratory muscle strength, supine FVC should be combined with upright FVC, MIP, MEP, and SNIP measurements. It is crucial to regularly screen patients for nutrition, as this can significantly affect respiratory muscle function during pulmonology follow-up. Full article

(This article belongs to the Special Issue Updates on Lung Function, Respiratory and Asthma Disease in Children)

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12 pages, 255 KiB

Open AccessArticle

Association of Maternal Air Pollution Exposure and Infant Lung Function Is Modified by Genetic Propensity to Oxidative Stress

by Dwan Vilcins, Wen Ray Lee, Cindy Pham, Sam Tanner, Luke D. Knibbs, David Burgner, Tamara L. Blake, Toby Mansell, Anne-Louise Ponsonby, Peter D. Sly and Barwon Infant Study Investigator Group

Children 2024, 11(8), 937; https://doi.org/10.3390/children11080937 - 31 Jul 2024

Viewed by 924

Abstract

Background and objective: The association between air pollution and poor respiratory health outcomes is well established. Children are particularly at risk from air pollution, especially during the prenatal period as their organs and systems are still undergoing crucial development. This study investigated maternal [...] Read more.

Background and objective: The association between air pollution and poor respiratory health outcomes is well established. Children are particularly at risk from air pollution, especially during the prenatal period as their organs and systems are still undergoing crucial development. This study investigated maternal exposure to air pollution during pregnancy and oxidative stress (OS), inflammation, and infant lung function at 4 weeks of age. Methods: Data from the Barwon Infant Study were available for 314 infants. The exposure to NO₂ and PM_2.5 were estimated. Infant lung function (4 weeks) was measured by multiple-breath washout. Glycoprotein acetyls (GlycA) (36 weeks prenatal), cord blood, and OS biomarkers were measured in maternal urine (28 weeks). A genetic pathway score for OS (gPFSox) was calculated. Linear regression was used and potential modification by the OS genotype was tested. Results: There was no relationship between maternal exposure to air pollution and infant lung function, or with GlycA or OS during pregnancy. We found an association in children with a genetic propensity to OS between NO₂ and a lower functional residual capacity (FRC) (β = −5.3 mls, 95% CI (−9.3, −1.3), p = 0.01) and lung clearance index (LCI) score (β = 0.46 turnovers, (95% CI 0.10, 0.82), p = 0.01). Conclusion: High prenatal exposure to ambient NO₂ is associated with a lower FRC and a higher LCI score in infants with a genetic propensity to oxidative stress. There was no relationship between maternal exposure to air pollution with maternal and cord blood inflammation or OS biomarkers. Full article

(This article belongs to the Special Issue Updates on Lung Function, Respiratory and Asthma Disease in Children)

Review

Jump to: Research, Other

11 pages, 761 KiB

Open AccessReview

Use of Lung Ultrasound in Cystic Fibrosis: Is It a Valuable Tool?

by Alessandra Boni, Luca Cristiani, Fabio Majo, Nicola Ullmann, Marianna Esposito, Maria Chiara Supino, Paolo Tomà, Alberto Villani, Anna Maria Musolino and Renato Cutrera

Children 2024, 11(8), 917; https://doi.org/10.3390/children11080917 - 30 Jul 2024

Viewed by 648

Abstract

Cystic fibrosis (CF) is a multisystem disorder characterized by progressive respiratory deterioration, significantly impacting both quality of life and survival. Over the years, lung ultrasound (LUS) has emerged as a promising tool in pediatric respiratory due to its safety profile and ease at [...] Read more.

Cystic fibrosis (CF) is a multisystem disorder characterized by progressive respiratory deterioration, significantly impacting both quality of life and survival. Over the years, lung ultrasound (LUS) has emerged as a promising tool in pediatric respiratory due to its safety profile and ease at the bedside. In the era of highly effective CF modulator therapies and improved life expectancy, the use of non-ionizing radiation techniques could become an integral part of CF management, particularly in the pediatric population. The present review explores the potential role of LUS in CF management based on available data, analyzing all publications from January 2015 to January 2024, focusing on two key areas: LUS in CF pulmonary exacerbation and its utility in routine clinical management. Nonetheless, LUS exhibits a robust correlation with computed tomography (CT) scans and serves as an additional, user-friendly imaging modality in CF management, demonstrating high specificity and sensitivity in identification, especially in consolidations and atelectasis in the CF population. Due to its ability, LUS could be an instrument to monitor exacerbations with consolidations and to establish therapy duration and monitor atelectasis over time or their evolution after therapeutic bronchoalveolar lavage. On the basis of our analysis, sufficient data emerged showing a good correlation between LUS score and respiratory function tests. Good sensitivity and specificity of the methodology have been found in rare CF pulmonary complications such as effusion and pneumothorax. Regarding its use in follow-up management, the literature reports a moderate correlation between LUS scores and the type, extent, and CT severity score of bronchiectasis. A future validation of ultrasound scores specifically in CF patients could improve the use of LUS to identify pulmonary exacerbations and monitor disease progression. However, further research is needed to comprehensively establish the role of LUS in the CF population, particularly in elucidating its broader utility and long-term impact on patient care. Full article

(This article belongs to the Special Issue Updates on Lung Function, Respiratory and Asthma Disease in Children)

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15 pages, 930 KiB

Open AccessReview

Dysfunctional Breathing in Children: A Literature Review

by Georgia Karkouli, Konstantinos Douros, Dafni Moriki, Paraskevi Moutsatsou, Ioanna Giannopoulou, Eirini Maratou and Despoina Koumpagioti

Children 2024, 11(5), 556; https://doi.org/10.3390/children11050556 - 6 May 2024

Cited by 1 | Viewed by 2012

Abstract

Dysfunctional breathing (DB) describes a respiratory condition that is mainly characterized by abnormal breathing patterns, affecting both children and adults, often leading to intermittent or chronic complaints and influencing physiological, psychological, and social aspects. Some symptoms include breathlessness; dizziness; palpitations; and anxiety, while [...] Read more.

Dysfunctional breathing (DB) describes a respiratory condition that is mainly characterized by abnormal breathing patterns, affecting both children and adults, often leading to intermittent or chronic complaints and influencing physiological, psychological, and social aspects. Some symptoms include breathlessness; dizziness; palpitations; and anxiety, while its classification lies in breathing pattern disorders and upper airway involvement. Its prevalence among the pediatric population varies with a female overrepresentation, while the existence of comorbidities in DB, such as asthma, gastro-esophageal reflux, nasal diseases, and anxiety/depression, frequently leads to misdiagnosis or underdiagnosis and complicates therapeutic approaches. The basic diagnostic tools involve a detailed history, physical examination, and procedures such as structured light plethysmography, cardiopulmonary exercise testing, and laryngoscopy when a laryngeal obstruction is present. The management of DB presumes a multidimensional approach encompassing breathing retraining, disease-specific advice through speech and language therapy in the presence of laryngeal obstruction, psychotherapy for fostering self-efficacy, and surgical therapy in a structural abnormality. The current review was developed to provide a summary of classifications of DB and epidemiological data concerning the pediatric population, comorbidities, diagnostic tools, and therapeutic approaches to enhance the comprehension and management of DB in children. Full article

(This article belongs to the Special Issue Updates on Lung Function, Respiratory and Asthma Disease in Children)

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12 pages, 1755 KiB

Open AccessReview

Recurrent Respiratory Infections in Children with Down Syndrome: A Review

by Michele Ghezzi, Nicolò Garancini, Raffaella De Santis, Laura Gianolio, Salvatore Zirpoli, Anna Mandelli, Andrea Farolfi, Enza D’Auria and Gian Vincenzo Zuccotti

Children 2024, 11(2), 246; https://doi.org/10.3390/children11020246 - 15 Feb 2024

Cited by 1 | Viewed by 3438

Abstract

Down Syndrome (DS) is the most common chromosomal abnormality compatible with life. The life of patients suffering from DS can be strongly impacted by Recurrent Respiratory tract Infections (RRIs), leading to an increased rate of hospitalisation, a higher need for intensive care and [...] Read more.

Down Syndrome (DS) is the most common chromosomal abnormality compatible with life. The life of patients suffering from DS can be strongly impacted by Recurrent Respiratory tract Infections (RRIs), leading to an increased rate of hospitalisation, a higher need for intensive care and fatality. With a literature review, we summarise here the main etiological factors for RRI in this category of patients, particularly focusing on airway malformations such as tracheomalacia, tracheal bronchus and bronchomalacia, comorbidities associated with the syndrome, like congenital heart diseases, dysphagia, gastroesophageal reflux, musculoskeletal involvement and obesity, and immunologic impairments, involving both innate and adaptive immunity. For these patients, a multidisciplinary approach is imperative as well as some preventive strategies, in particular vaccinations in accordance with their national schedule for immunization. Full article

(This article belongs to the Special Issue Updates on Lung Function, Respiratory and Asthma Disease in Children)

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Other

Jump to: Research, Review

12 pages, 707 KiB

Open AccessOpinion

Hematopoietic Stem Cells Transplant (HSCT)-Related Chronic Pulmonary Diseases: An Overview

by Arianna Traunero, Francesca Peri, Laura Badina, Alessandro Amaddeo, Elettra Zuliani, Massimo Maschio, Egidio Barbi and Sergio Ghirardo

Children 2023, 10(9), 1535; https://doi.org/10.3390/children10091535 - 11 Sep 2023

Cited by 1 | Viewed by 1364

Abstract

Recipients of HSCT have a high risk of infective and non-infective pulmonary diseases. Most patients with pulmonary involvement present multiple pathogenetic mechanisms simultaneously with complex interactions. Therefore, it can be difficult to distinguish the contributions of each one and to perform studies on [...] Read more.

Recipients of HSCT have a high risk of infective and non-infective pulmonary diseases. Most patients with pulmonary involvement present multiple pathogenetic mechanisms simultaneously with complex interactions. Therefore, it can be difficult to distinguish the contributions of each one and to perform studies on this subject. In this opinion article, we discuss only chronic pulmonary manifestations, focusing on LONIPCs (late-onset non-infectious pulmonary complications). This term embraces drug-related toxicity, allergies, and chronic pulmonary graft versus host disease (GvHD) in all its recently identified clinical variants. Among LONIPCs, GvHD represents the most critical in terms of morbidity and mortality, despite the rapid development of new treatment options. A recently emerging perspective suggests that pulmonary lung rejection in transplant patients shares striking similarities with the pathogenesis of GvHD. In a pulmonary transplant, the donor organ is damaged by the host immune system, whereas in GvHD, the donor immune system damages the host organs. It constitutes the most significant breakthrough in recent years and is highly promising for both hematologists and thoracic transplant surgeons. The number of patients with LONIPCs is scarce, with heterogenous clinical characteristics often involving several pathogenetic mechanisms, making it challenging to conduct randomized controlled trials. Therefore, the body of evidence in this field is scarce and generally of low quality, leading to jeopardized choices in terms of immunosuppressive treatment. Moreover, it risks being outdated by common practice due to the quick evolution of knowledge about the diagnosis and treatment of LONIPCs. The literature is even more pitiful for children with pulmonary involvement related to HSCT. Full article

(This article belongs to the Special Issue Updates on Lung Function, Respiratory and Asthma Disease in Children)

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14 pages, 577 KiB

Open AccessPerspective

Metabolomics Applied to Pediatric Asthma: What Have We Learnt in the Past 10 Years?

by Valentina Agnese Ferraro, Stefania Zanconato and Silvia Carraro

Children 2023, 10(9), 1452; https://doi.org/10.3390/children10091452 - 25 Aug 2023

Viewed by 1230

Abstract

Background: Asthma is the most common chronic condition in children. It is a complex non-communicable disease resulting from the interaction of genetic and environmental factors and characterized by heterogeneous underlying molecular mechanisms. Metabolomics, as with the other omic sciences, thanks to the [...] Read more.

Background: Asthma is the most common chronic condition in children. It is a complex non-communicable disease resulting from the interaction of genetic and environmental factors and characterized by heterogeneous underlying molecular mechanisms. Metabolomics, as with the other omic sciences, thanks to the joint use of high-throughput technologies and sophisticated multivariate statistical methods, provides an unbiased approach to study the biochemical–metabolic processes underlying asthma. The aim of this narrative review is the analysis of the metabolomic studies in pediatric asthma published in the past 10 years, focusing on the prediction of asthma development, endotype characterization and pharmaco-metabolomics. Methods: A total of 43 relevant published studies were identified searching the MEDLINE/Pubmed database, using the following terms: “asthma” AND “metabolomics”. The following filters were applied: language (English), age of study subjects (0–18 years), and publication date (last 10 years). Results and Conclusions: Several studies were identified within the three areas of interest described in the aim, and some of them likely have the potential to influence our clinical approach in the future. Nonetheless, further studies are needed to validate the findings and to assess the role of the proposed biomarkers as possible diagnostic or prognostic tools to be used in clinical practice. Full article

(This article belongs to the Special Issue Updates on Lung Function, Respiratory and Asthma Disease in Children)

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9 pages, 3536 KiB

Open AccessCase Report

Surgical Treatment of Lung Abscess Due to an Awn Aspiration in a 9-Year-Old Child: A Case Report

by Angelina Vlahova, Zdravka Antonova, Edmond Rangelov, Nikola Kartulev, Velichka Oparanova, Natalia Gabrovska, Albena Spasova, Svetlana Velizarova and Hristo Shivachev

Children 2023, 10(6), 910; https://doi.org/10.3390/children10060910 - 23 May 2023

Cited by 1 | Viewed by 1476

Abstract

Introduction: Foreign body aspiration is a common condition in the child population and is one of the leading causes of accidental deaths in children. The aspiration of an awn (grass inflorescences) is extremely rare. Aim of the study: This study aims to describe [...] Read more.

Introduction: Foreign body aspiration is a common condition in the child population and is one of the leading causes of accidental deaths in children. The aspiration of an awn (grass inflorescences) is extremely rare. Aim of the study: This study aims to describe the symptoms, diagnosis, therapeutic difficulties, and results of the aspiration of grass inflorescence. They are all related to the shape of the awn’s head and its behavior in the tracheobronchial tree. Case description: We present a 9-year-old boy with a history of an awn aspiration and recurrent respiratory infections. After antibiotic and symptomatic treatment, two bronchoscopies were performed, and both showed stenosis and obturation of the segmental and subsegmental bronchi of the left posterior basal segment, but no foreign body was found. After recanalization and continuous medicamentous treatment, a computed tomography (CT) verified the lung abscess. Video-assisted thoracoscopic surgery (VATS) was performed, and an atypical lung resection was conducted. During the surgery, a foreign body—grass inflorescence from the species Hordeum murinum—was found in the resected tissue. The patient recovered uneventfully after the surgery. Conclusions: Grass inflorescence aspiration in the child population is an extremely rare event, and it represents a diagnostic and therapeutic issue. The exact timing of surgery is very important to reduce complications and avoid death. Full article

(This article belongs to the Special Issue Updates on Lung Function, Respiratory and Asthma Disease in Children)

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