Steatorrhea and azotorrhea are evaluated, by Van de Kamer and Kjendalh methods, in 24 patients affected by cystic fibrosis in the diagnosis and in their evolution. High values were obtained for steatorrhea in 87.5% of the cases and for azotorrhea in the 79.1% at the moment of diagnosis. Steatorrhea and azotorrhea have been correlated obtaining a high statistic significant for p less than 0.01 in the diagnosis and in patients who followed treatment with the same medication, not being significative for all patients during their evolution. With results obtained, determination of fecal nitrogen, is positively evaluated not only in the diagnosis but also in the evolution of these patients to obtain an optimum nutritious condition.
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J Cyst Fibros
January 2025
Department NEUROFARBA, University of Florence; Paediatric and Liver Unit, Meyer Children's Hospital IRCCS, Florence, Italy.
Background: Elexacaftor-tezacaftor-ivacaftor (ETI) has significantly improved the clinical course of people with cystic fibrosis (pwCF) and eligible CFTR variants. In this study, we prospectively evaluated liver elastography, liver fibrosis indices and liver tests in children with CF aged 6-12 years started on ETI therapy.
Methods: Body mass index, sweat test, percent predicted forced expiratory volume in one second, serum markers of liver injury or portal hypertension, liver fibrosis indices, controlled attenuation parameter and liver stiffness were assessed before starting ETI and three and twelve months post-ETI, according to new international guidelines.
J Cyst Fibros
January 2025
The Lundquist Institute, Harbor-UCLA Medical Center, Torrance 90502 CA, USA. Electronic address:
Background: Cystic Fibrosis-related Bone Disease is an emerging challenge faced by 50 % of adult people with cystic fibrosis (CF). The multifactorial causes of this comorbidity remain elusive. However, congenital bone defects have been observed in animal models with CFTR mutations, suggesting its importance.
View Article and Find Full Text PDFIntroduction: The Lung Clearance Index (LCI) is an established research test, but its role in clinical decision-making is not well defined. This study estimated the proportion of treatment decisions that are changed or supported by the added information provided by LCI.
Methods: A mixed methods prospective observational study was conducted in North America.
Dig Liver Dis
January 2025
Unit of Clinical and Molecular Epidemiology, IRCCS San Raffaele Pisana, 00166 Rome, Italy; Department of Human Sciences and Quality of Life Promotion, San Raffaele University, 00166 Rome, Italy.
Background: In pediatric patients, celiac disease (CD) may influence the health-related quality of life (HRQoL).
Aims: The study aimed to assess HRQoL and further characterise the clinical factors associated with reduced HRQoL, in a large multicenter pediatric cohort with CD.
Methods: The disease-specific questionnaire CD Dutch Questionnaire (CDDUX) and the generic questionnaire Paediatric Quality of Life Inventory (PedsQL) were used to assess the HRQoL.
J Control Release
January 2025
Univ Brest, Inserm, EFS, UMR 1078, GGB, F-29200, Brest, France; CHU de Brest, Service de Génétique Médicale et de Biologie de la Reproduction, F-29200 Brest, France. Electronic address:
Aerosol delivery represents a rapid and non-invasive way to directly reach the lungs while escaping the hepatic first-pass effect. The development of pulmonary drugs for respiratory diseases such as cystic fibrosis, lung infections, pulmonary fibrosis or lung cancer requires an enhanced understanding of the relationships between the natural physiology of the respiratory system and the pathophysiology of these conditions. This knowledge is crucial to better predict and thereby control drug deposition.
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