Introduction. Thiopurines, such as azathioprine (AZA) and 6-mercaptopurine (6-MP), are immunomodulatory agents, used for the maintenance of remission in children with inflammatory bowel disease (IBD)—Crohn’s disease (CD) and ulcerative colitis (UC), as well as with autoimmunological hepatitis (AIH). Measurements of thiopurine metabolites may allow identifying patients at risk for toxicity and nonadherence. It can also provide an explanation for the ineffectiveness of the treatment, observed in some patients. Patients and Methods. A retrospective analysis was carried out of sixty-eight patients (thirty-six patients with CD, eighteen with UC, and fourteen with AIH), treated with AZA. Thiopurine metabolites, 6-thioguanine nucleotide (6-TGN) and 6-methylmercaptopurine (6-MMP), were assayed by high-performance liquid chromatography (HPLC), and the AZA dose was adjusted when 6-TGN concentration was known. Result. Only twenty-five (41%) children had therapeutic 6-TGN concentrations, ten (16%) subjects had suboptimal 6-TGN concentrations, and twenty-six subjects (43%) had 6-TGN concentrations above the recommended therapeutic range. 6-MMP was not above the therapeutic range in any case. Seven subjects revealed undetectable 6-TGN and 6-MMP levels, indicating nonadherence. The mean AZA dose after the 6-TGN concentration-related adjustment did not differ, in comparison to the initial dose, either in IBD or AIH groups. The mean AZA dose was lower in AIH than in IBD. The subjects with an optimal 6-TGN level presented with a higher ratio of remission (88%) than the under- or overdosed patients (60% and 69%), respectively ( Chi − square test = 3.87 , p < 0.05 ). Conclusion. Timely measurements of thiopurine metabolites can be a useful tool to identify nonadherent patients before a decision is taken to switch to another drug. We may also spot the patients who receive either too low or too high doses, compensating dose deviations in an appropriate way. The patients with optimal 6-TGN levels presented a higher percentage of remission than the under- or overdosed patients. In most patients, both initial and adjusted AZA doses, lower than suggested in guidelines, appeared to be sufficient to maintain remission.
Przewlekłe zaparcia są jedną z najczęstszych dolegliwości w populacji dziecięcej. Stanowią przyczynę około 3% wizyt w poradniach pediatrycznych. Problem, niejednokrotnie bagatelizowany, stanowi przykrą, uporczywą dolegliwość, zarówno dla pacjenta, jak i całej jego rodziny. Zaparcia mogą wynikać z przyczyn organicznych lub nieorganicznych. Wśród przyczyn nieorganicznych kluczowa wydaje się przewlekła retencja stolca, wynikająca ze złych nawyków higienicznych, niewłaściwej diety oraz zbyt niskiej aktywności fizycznej. Zaparcia często pojawiają się wraz ze zmianą otoczenia dziecka, np. po rozpoczęciu nauki w przedszkolu czy szkole, w których pacjent z różnych przyczyn powstrzymuje się od oddania stolca. W celu ustalenia ostatecznego rozpoznania należy wykluczyć przyczyny organiczne zaparć, które wymagają leczenia przyczynowego. Terapia najczęściej występujących zaparć o charakterze czynnościowym obejmuje zastosowanie kompleksowych metod farmakologicznych i niefarmakologicznych; zwykle jest długotrwała. Ma ona na celu utrwalenie takiego modelu defekacji, w którym pacjent regularnie oddaje miękki stolec bez przykrych dolegliwości bólowych. Niezwykle istotne jest wsparcie psychologiczne całej rodziny, niejednokrotnie zaangażowanej w trudny problem dziecka. Sukces terapeutyczny wymaga przede wszystkim cierpliwej i sumiennej współpracy pomiędzy lekarzem a pacjentem i jego rodziną, gdyż pozytywne i trwałe efekty leczenia osiągane są po stosunkowo długim czasie.
Background: Among the extraintestinal manifestations of inflammatory bowel disease (IBD), those involving the lungs are relatively rare and often overlooked. There are only scarce data on the prevalence of IBD-associated lung involvement in children. Objectives: The aim of our study was to assess pulmonary function in IBD children by different methods and to evaluate the influence of immunosuppressive therapy on disease severity. Methods: Seventy-two children with IBD (mean age of 14.45 ± 2.27 years) and 40 age-matched healthy controls (mean age of 14.17 ± 2.82) were included in the study. Pulmonary function tests (PFTs) were carried out by means of spirometry, oscillometry (IOS) and fractional exhaled nitric oxide (FeNO) to assess the pulmonary involvement. Results: Certain differences were observed between the study group and the control group, regarding the spirometric and oscillometry parameters. The fractions of exhaled nitric oxide did not differ between the group with IBD patients and the control group with regards to disease activity, the duration of illness and the administered immunosuppressive treatment. Conclusions: The mean spirometry results were significantly different in the study group compared to the controls, although they were still within the normal limits. The pulmonary function abnormalities did not depend on either the disease activity or the immunosuppressive therapy. Oscillometry could be a supplementary method to assess pulmonary resistance. In turn, FeNO does not appear to be useful either in screening IBD children for pulmonary involvement or for the evaluation of disease activity. It appears then that only general screening of asymptomatic patients is a suitable method and a necessary recommendation in this population, prompting a revision of the current diagnostic approach.
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