The appearance of the pancreas in 17 adult patients with cystic fibrosis was evaluated with magnetic resonance (MR) imaging. The pancreas was abnormal in 15 patients. Three patterns were observed: (a) lobulated enlarged pancreas with complete replacement by fatty tissue (n = 9), (b) small atrophic pancreas with partial replacement by fat tissue (n = 5), and (c) diffuse atrophy of the pancreas without fatty replacement (n = 1). Replacement of the pancreas by fat tissue was seen on T1-weighted images with, characteristically, a very high signal intensity. The sensitivity of MR imaging in depicting pancreatic abnormality in cystic fibrosis is 94%, which is comparable to that of computed tomography.
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http://dx.doi.org/10.1148/radiology.179.1.2006275 | DOI Listing |
Pediatr Pulmonol
January 2025
Beatrix Children's Hospital Department of Pediatric Pulmonology and Pediatric Allergy, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.
Introduction: Lumacaftor/ivacaftor (lum/iva) was introduced in the Netherlands in 2017. We investigated 1-year efficacy of lum/iva on lung function and small airway and structural lung disease evaluated by multiple breath nitrogen washout and CT scan. Additionally, we investigated effects of lum/iva on exacerbations, anthropometry, sweat chloride and safety in children with CF in the Netherlands.
View Article and Find Full Text PDFPediatr Pulmonol
January 2025
Stead Family Department of Pediatrics, University of Iowa Carver College of Medicine, Iowa City, Iowa, USA.
Background: People with cystic fibrosis (CF) may not expectorate sputum at young ages or after they receive CFTR modulators. While oropharyngeal swabs are commonly used to test for lower airway pathogens, it is unknown whether Staphylococcus aureus from the oropharynx matches the strain(s) infecting the lungs. Our goal was to determine whether oropharyngeal and sputum isolates of S.
View Article and Find Full Text PDFMonaldi Arch Chest Dis
January 2025
Postgraduate Program in Medical Sciences, State University of Rio de Janeiro; Piquet Carneiro Polyclinic, State University of Rio de Janeiro; Rehabilitation Sciences Postgraduate Program, Augusto Motta University Centre, Rio de Janeiro.
With the increasing use of highly effective modulator therapy (HEMT) in adults with cystic fibrosis (awCF), it is necessary to determine the evolution of the most dynamic physiological markers of this disease, such as the 6-minute walk test (6MWT) and the Glittre-activities of daily living test (TGlittre). The present study aimed to evaluate the 1-year changes in the 6- minute walking distance (6MWD), TGlittre time, and quality of life (QoL) in awCF before the initiation of HEMT and to determine the impact of habitual physical activity (HPA) and chest physiotherapy (CP). This longitudinal study enrolled 24 awCF who completed the 6MWT and TGlittre.
View Article and Find Full Text PDFExpert Rev Respir Med
January 2025
Department of Surgery, Dentistry, Pediatrics and Gynaecology, Pediatric Division, University of Verona, Verona, Italy.
Introduction: Sickle cell disease (SCD) is an inherited hemoglobinopathy characterized by the production of sickle hemoglobin, leading to red blood cells sickling and hemolysis in hypoxic conditions. The resulting acute and chronic endothelial inflammation leads to chronic organ damage. Respiratory manifestations in SCD usually start from childhood and represent the leading causes of morbidity and mortality.
View Article and Find Full Text PDFSci Diabetes Self Manag Care
January 2025
Division of Endocrinology and Diabetes, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
Purpose: The purpose of the study was to identify the most common reasons for and timing of continuous glucose monitoring (CGM) attrition in youth with type 1 diabetes (T1DM).
Methods: This single center retrospective chart review included youth with T1DM <22 years seen between November 1, 2021, and October 31, 2022. Data were gathered from CGM cloud-based software and the electronic medical record.
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