This article sets out to evaluate the prevalence and factors associated with adherence to enzyme replacement therapy among adolescents with cystic fibrosis. It is a cross-sectional, descriptive and observational study. Sociodemographic and clinical data were collected. The instruments used to assess adherence were: the Morisky-Green questionnaire and medication dispensation at the pharmacy, and interviews with structured questionnaires for the associated factors. Forty-four adolescents were interviewed. According to the method of the pharmacy medication dispensation analysis and the Morisky-Green questionnaire, the adherence of 45.5% and 11.4% was found, respectively. The higher adherence was observed in those with early diagnosis and the lowest in older adolescents and girls. The factors with the highest prevalence of non-adherence were: not taking enzymes when eating out of the home; only taking enzymes with major meals; normal lung function; with severe and very severe obstruction. The prevalence of adhesion to enzymes was low. Information related to the disease and treatment should be improved, especially among older adolescents and with impairment of lung function, with the creation of strategies and longitudinal studies to identify factors that interfere with adherence.
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http://dx.doi.org/10.1590/1413-812320182412.31622017 | DOI Listing |
J Helminthol
January 2025
Hacettepe University, Faculty of Medicine, Department of Radiology, Ankara, Turkiye.
Cystic Echinococcosis (CE) is a zoonotic disease caused by sensu lato. Diagnosing CE primarily relies on imaging techniques, and there is a crucial need for an objective laboratory test to enhance the diagnostic process. Today, cell-free DNAs (cfDNAs) have gained importance regarding their biomarker potential.
View Article and Find Full Text PDFDig 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.
Pediatr Surg Int
January 2025
Department of Pediatric Surgery, Nagoya University Graduate School of Medicine, 65 Tsurumai-Cho, Showa, Nagoya, Aichi, 466-8550, Japan.
Purpose: To analyze the frequency and predictive factors of the development of postoperative pectus excavatum and scoliosis in children who underwent surgery for cystic lung disease.
Methods: This study examined patients who underwent surgery for cystic lung disease (open and thoracoscopic) between July 2000 and December 2018 with a > 3-year follow-up period. Lesion size, surgical outcomes, and subsequent musculoskeletal complications were compared between the open surgery and thoracoscopic surgery groups.
Taiwan J Obstet Gynecol
January 2025
Department of Obstetrics and Gynecology, Taipei Chang Gung Memorial Hospital, Taipei, Taiwan; School of Medicine, Chang Gung University, Taoyuan, Taiwan. Electronic address:
Objective: To describe a rare case of a retroperitoneal Müllerian cyst in a teenage girl with a protruding uterus and associated urogenital anomalies, and to discuss the challenges faced in differential diagnosis and management of such cases.
Case Report: We present the case of a 14-year-old girl presented with a protruding uterus for several weeks, with a history of twin-twin transfusion syndrome at birth. Initial ultrasonography identified a large pelvic cystic tumor.
Ann Thorac Surg Short Rep
December 2024
Department of Pathology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India.
Synovial sarcoma is a rare malignant mesenchymal neoplasm primarily affecting adolescents and young adults, and it typically arises from deep soft tissues near large joints. Although commonly found in extremities, it can occur in various anatomic locations. We present a rare case of a 29-year-old man with primary pulmonary synovial sarcoma manifesting as a cystic mass masquerading as a lung hydatid cyst.
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