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1.
Orphanet J Rare Dis ; 19(1): 105, 2024 Mar 07.
Artículo en Inglés | MEDLINE | ID: mdl-38454433

RESUMEN

BACKGROUND: This study aimed to assess medication adherence and demographic, clinical, and psychopathological parameters such as quality of life, depression, and anxiety levels that can affect pediatrics with Wilson's Disease (WD). METHODS: A prospective cohort study was conducted at an outpatient clinic in Turkey among pediatric patients (2 to 18 years) with WD between November 2022 and April 2023. The Medication Adherence Report Scale (MARS-5) as a subjective and Medication Possession Ratio (MPR) as an objective assessment were scored. Physical, genetic and biochemical parameters, the Pediatric Quality of Life Inventory (PedsQL) for both parents and patients, Childhood Depression Inventory, State Trait Anxiety Inventory were also administered. RESULTS: A total of 30 pediatric outpatients who were prescribed D-penicillamine (n = 27) or trientine (n = 3) as chelators and zinc (n = 29) and pyridoxine (n = 19) as supplements were included. Proteinuria (n = 3), skin rash (n = 2), and gastrointestinal upset (n = 2) were observed. When the correlation between MARS-5 and duration of follow-up was examined, a significant negative correlation was found (p = 0.014). According to MPRs, non-adherence rates (missed doses ≥ 20%) were 29.6%, 17.2% and 5.3% for D-penicillamine, zinc and pyridoxine, respectively. PedsQL scores were higher than those of parents, with a positive correlation between them (p < 0.001). Also, there was a significant positive correlation between PedsQL and State Anxiety Inventory (p < 0.001). Comparing the change in urinary copper levels between different levels of treatment knowledge, significant differences were observed between high- and low levels (p = 0.043). CONCLUSIONS: Overall, nonadherence rates were 23.3% based on MARS-5 and 5.3-29.6% based on MPR. It is essential to consider factors such as the duration of follow-up, biochemical parameters, treatment knowledge, quality of life and anxiety as potential influencers of medication adherence.


Asunto(s)
Degeneración Hepatolenticular , Adolescente , Niño , Humanos , Estudios de Cohortes , Degeneración Hepatolenticular/tratamiento farmacológico , Degeneración Hepatolenticular/patología , Penicilamina/uso terapéutico , Estudios Prospectivos , Piridoxina/uso terapéutico , Calidad de Vida , Turquía , Zinc/uso terapéutico , Preescolar
2.
Turk J Gastroenterol ; 32(1): 88-96, 2021 01.
Artículo en Inglés | MEDLINE | ID: mdl-33893771

RESUMEN

BACKGROUND/AIMS: Chronic hepatitis C (CHC) is the only viral infection that can be treated with oral antiviral agents. However, CHC awareness is a major barrier to the World Health Organization's target of eliminating hepatitis C virus (HCV) by 2030. Here, CHC awareness trends were analyzed in Hacettepe University Hospital, Turkey, between January 2000 and December 2017. MATERIALS AND METHODS: Central laboratory data were retrospectively analyzed for HCV test results (anti-HCV, HCV RNA, HCV genotype). After combining 548,141 anti-HCV test results, 395,103 cases were analyzed. The following two parameters were defined for CHC awareness: (1) the presence of HCV RNA results for anti-HCV positives and (2) the presence of a genotype result for HCV RNA positives. RESULTS: Anti-HCV positives were older than negatives (mean age-years ± SD, 59.4 ± 19.0 vs. 44.0 ± 18.9), and the positivity rate was higher in women than in men (1.4% vs. 1.0%). Anti-HCV positivity decreased from 3.1% to 0.6% from 2000 to 2015 and subsequently stabilized. The overall percentage of RNA testing among anti-HCV positives was 53.1% (range, 20%-70%), which stabilized at approximately 50% after 2010. The genotyping rate for RNA positives varied between 40% and 70%. The main genotype identified was genotype 1 (85.7%). CONCLUSION: In an ideal CHC awareness state, all anti-HCV positives should undergo RNA testing, and genotyping should be performed when RNA tests are positive. However, even in our referral center, the combined rate of RNA and genotype testing was only approximately 50% during the last 10 years.


Asunto(s)
Concienciación , Hepatitis C Crónica , Adulto , Anciano , Anciano de 80 o más Años , Actitud Frente a la Salud , Femenino , Genotipo , Hepacivirus/genética , Hepatitis C/diagnóstico , Hepatitis C/tratamiento farmacológico , Hepatitis C/epidemiología , Hepatitis C/psicología , Hepatitis C Crónica/diagnóstico , Hepatitis C Crónica/tratamiento farmacológico , Hepatitis C Crónica/epidemiología , Hepatitis C Crónica/psicología , Humanos , Masculino , Persona de Mediana Edad , Programas Nacionales de Salud , ARN Viral/genética , Estudios Retrospectivos , Turquía/epidemiología , Adulto Joven
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