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Clinical Profile and Pulmonary Function of Pediatric Patients with Duchenne Muscular Dystrophy at a Tertiary Government Hospital.
Acta Med Philipp
November 2024
Division of Pediatric Pulmonology, Department of Pediatrics, Philippine General Hospital, University of the Philippines Manila.
Objective: Our study aimed to determine the clinical profile and pulmonary function of pediatric patients with Duchenne Muscular Dystrophy (DMD). We also characterized the stages of progression of the disease and determined their potential association with spirometry variables.
Methods: In this cross-sectional study, we used data obtained from a review of medical records of all pediatric patients (0-18 years old) with DMD seen in a multidisciplinary neuromuscular clinic of a tertiary government hospital from August 2018 until March 2020.
[The repercussions of variations in ambient air pressure on pulmonary airway dystrophy. Applications to scuba diving with mask, commercial air travel and stays in high-altitude settings].
Rev Mal Respir
December 2024
Service de pneumologie, hôpital de la Croix-Rousse, 69004 Lyon, France.
Article Synopsis
- Pulmonary airway dystrophy (PAD) is a rare condition that can be either inherited or developed, with limited research on how air pressure changes during airplane trips or high-altitude situations impact it.
- Studies indicate that all PAD cases have been linked to accidents during exposure to varying air pressures, necessitating careful consideration of each individual case to tailor recommendations based on the disease's origin and severity.
- Establishing a register for cases of barotrauma in individuals with cystic lung disease or pulmonary bullae related to PAD could provide valuable insights and improve patient management.
Comparative lipidomic and metabolomic profiling of mdx and severe mdx-apolipoprotein e-null mice.
Skelet Muscle
December 2024
Department of Physiology and Aging, University of Florida, Gainesville, FL, USA.
Despite its notoriously mild phenotype, the dystrophin-deficient mdx mouse is the most common model of Duchenne muscular dystrophy (DMD). By mimicking a human DMD-associated metabolic comorbidity, hyperlipidemia, in mdx mice by inactivating the apolipoprotein E gene (mdx-ApoE) we previously reported severe myofiber damage exacerbation via histology with large fibro-fatty infiltrates and phenotype humanization with ambulation dysfunction when fed a cholesterol- and triglyceride-rich Western diet (mdx-ApoE). Herein, we performed comparative lipidomic and metabolomic analyses of muscle, liver and serum samples from mdx and mdx-ApoE mice using solution and high-resolution-magic angle spinning (HR-MAS) H-NMR spectroscopy.
View Article and Find Full Text PDF[Nosological aspects of the petrification and ossification processes in the lungs].
Arkh Patol
December 2024
St. Petersburg Research Institute of Phthisiopulmonology, St. Petersburg, Russia.
Microscopic changes in the lung biopsies of 16 adult patients of both sexes without clear clinical symptoms are described. According to X-ray examination data, disseminated process in the lungs was diagnosed in 4 patients, interstitial pneumonia in 4, osteoplastic pneumopathy in 3, as well as tuberculosis, bronchiolitis and a number of others. Microscopic examination showed that the patients had various forms of petrification and ossification of the lungs, diagnosed clinically and radiologically in only 3 cases.
View Article and Find Full Text PDF[Pediatric pneumological aspects in the care of children with neuromuscular diseases with focus on Duchenne muscular dystrophy].
Klin Padiatr
December 2024
Pädiatrische Pneumologie, Klinik für Kinderheilkunde III, Universitätsklinikum Essen, Universität Duisburg-Essen, Essen, Germany.
Article Synopsis
- Duchenne muscular dystrophy is a common genetic disease in children that results in loss of motor skills, heart issues, and respiratory challenges.
- Respiratory care by pediatric pulmonologists is crucial, including lung function monitoring, sleep studies, and managing ventilation support.
- The article highlights the importance of collaboration among specialists to improve the treatment and quality of life for children with neuromuscular diseases like Duchenne muscular dystrophy.
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