Hemangioendotelioma hepático infantil num recém-nascido

Infantile hepatic hemangioendothelioma is the most common benign hepatic vascular tumor in infants younger than six months and may undergo spontaneous regression during the first years of life. Diagnosis of these lesions in preterm newborns can be challenging, with its timing and accuracy relevant to avoid inappropriate therapeutic measures.info:eu-repo/semantics/publishedVersio

Wilson’s Disease: A Prevalence Study in a Portuguese Population

Introduction Wilson's disease (WD) is a rare and underdiagnosed genetic disorder caused by anomalous tissue copper deposition, and for which epidemiological studies, specifically in Portugal, are scarce. Objectives This study aimed to evaluate the prevalence and incidence of WD and provide a description of its main clinical and laboratory features. Methods A retrospective study was carried out, with a search between 1995 and 2015, of all patients with a minimum follow-up of three months and birth confirmed in the northern region of Portugal, with an estimated population of 3,689,682 inhabitants. Database collection was based on the Portuguese National Health Service's clinical coding system, relying on clinical data from 13 northern Portuguese hospitals, liver biopsy histology results, and hospital prescription records. Clinical and biochemical correlations were statistically assessed using chi-square, Mann-Whitney U, Friedman, and Wilcoxon tests. Results Over the 20-year period, a prevalence of 1:37.000 and an incidence of one per million person-year was found. A total of 94 patients were analyzed, with a slight male predominance (53%), the majority with the onset of clinical manifestations in pediatric age (56%), with a median age at diagnosis of 16.6 years (interquartile range of 12.3-20,.8 years). Most patients presented with predominant liver disease (54.8%), with more than a third with cirrhosis; mixed hepatic and neurological manifestations in 17.9%; and mainly neurological symptoms in 10.7% of the patients. Neurological impairment was strongly associated with delayed development of the manifestations of the disease (p = 0.001) and also a higher detection of Kayser-Fleischer rings (p < 0.001), present in 27.0% of the patients. Regarding therapy, penicillamine has been the most widely used, with adverse reactions reported in 24.8%. At six and 12 months after initiation of therapy, a significant decrease in liver enzymes was found (ALT: p = 0.002; AST: p = 0.002, respectively), but no significant reduction was observed in urinary copper excretion. Conclusion This was one of the first studies regarding WD prevalence in a Portuguese population, contributing to a better understanding of the epidemiology, diagnosis, and management of WD in the northern region of Portugal. WD should be considered in any individual with unexplained hepatic or neurological manifestations, and initial symptoms may manifest at an early age, even in children less than five years old. A high percentage of patients were identified in the early stages of the disease by asymptomatic elevation of transaminases. Following copper chelation therapy, cytolysis markers appear to be more sensitive indicators of treatment response.info:eu-repo/semantics/publishedVersio

Consenso Português sobre o Diagnóstico, Tratamento e Abordagem da Anemia na Doença Inflamatória Intestinal Pediátrica

Anemia is a common extraintestinal manifestation of inflammatory bowel disease (IBD), both in pediatric and in adult patients. Iron deficiency is the main cause of anemia in patients with IBD. Anemia is a clinically relevant comorbidity, with impact on patients' quality of life and it should be timely diagnosed and adequately treated. Currently, an active treatment approach is the recommended strategy, with evidence showing efficacy and safety of intravenous iron formulations. However, evidence in pediatric age remains scarce and no clinical recommendations exist for the diagnosis and treatment of this particular age group. The present document represents the first national consensus on the management of anemia in pediatric IBD and is therefore particularly relevant. The authors anticipate that the proposed recommendations will be useful in daily clinical practice for diagnosing and managing iron deficiency and iron-deficiency anemia in the pediatric population with IBD.A anemia é uma manifestação extra-digestiva frequente associada à doença inflamatória intestinal, tanto na população pediátrica como adulta, sendo a anemia por défice de ferro a sua forma mais frequente. Constitui uma comorbilidade clinicamente relevante, com repercussão na qualidade de vida. Deve ser atempadamente diagnosticada e adequadamente tratada. A estratégia terapêutica atualmente aceite preconiza uma atitude interventiva. Neste contexto, a evidência científica atual tem demonstrado a eficácia e segurança da utilização das formulações de ferro endovenoso. Contudo, em idade pediátrica a evidência ainda é insuficiente, não existindo orientações de abordagem diagnóstica ou terapêutica especificamente dirigidas a este grupo etário. Este é o primeiro consenso nacional sobre a abordagem da anemia na doença inflamatória intestinal pediátrica, revestindo-se por isso de particular relevância. Pretendese que este documento tenha utilidade e aplicabilidade na prática clínica na avaliação e seguimento do défice de ferinfo:eu-repo/semantics/publishedVersio

Liver dysfunction and hypoglycemia as presentation of hypopituitarism in a child

In the presence of hypoglycemia and liver dysfunction in the neonatal period and/or during the first months of life, a myriad of etiologies should be considered. We herein report the case of a child with refractory hypoglycemia and severe liver dysfunction, highlighting the importance of a multidisciplinary and rapid investigation to obtain the final diagnosis

Predicción de modelos cinéticos de secado de limoncillo en secadora convectiva

O presente trabalho teve como objetivo avaliar os ajustes aos modelos matemáticos da cinética de secagem de folhas de erva cidreira em secador convectivo nas temperaturas de 40, 55 e 70°C, com velocidades de ar de secagem de 1,0, 1,5 e 2,0 m/s mediante planejamento fatorial 22 + 3 experimentos no ponto central. As folhas in natura foram submetidas as caracterizações químicas, físicas e físico-químicas. A cinética de secagem foi realizada em secador convectivo, com ajustes para quatro modelos matemáticos, sendo considerado como critério de qualidade de ajuste, os coeficientes de determinação (R2) e os desvios quadráticos médios (DQM). Após realização dos ensaios de secagem, pode-se destacar o ensaio três conduzido com temperatura de 40 °C e velocidade de 1,0 m/s, apresentando maior rendimento e menor teor de água. O pó resultante foi submetido as análises de: granulometria, teor de água, atividade de água, cinzas, molhabilidade, higroscopicidade, solubilidade, massa específica, massa específica de compactação, fator de Hausner, índice de compressibilidade, cor, flavonoides, clorofilas a, b e total, carotenoides e taninos. Os modelos apresentaram ajustes satisfatórios à cinética de secagem, com R² > 99,0% e DQM <0,02, destacando-se o modelo de Cavalcanti-Mata. O processo de secagem utilizado foi eficiente resultando em um pó solúvel, pouco higroscópico e fino, com concentrações de bioativos relevantes para seu uso medicinal.The present work aimed to evaluate the adjustments to the mathematical models of the drying kinetics of lemongrass leaves in a convective dryer at 40, 55 and 70 ° C, with drying air velocities of 1.0, 1.5 and 2.0 m / s by factorial design 22 + 3 experiments at the central point. The fresh leaves were subjected to chemical, physical and physicochemical characterization. The drying kinetics was performed in a convective dryer, with adjustments for four mathematical models, being considered as fit quality criterion, the determination coefficients (R2) and the mean square deviations (DQM). After the drying tests, one can highlight the three test conducted at a temperature of 40 ° C and a speed of 1.0 m / s, presenting higher yield and lower water content, the resulting powder was subjected to analysis of: granulometry , water content, water activity, ashes, wettability, hygroscopicity, solubility, specific mass, specific compaction mass, Hausner factor, compressibility index, color, flavonoids, total and b chlorophylls, carotenoids and tannins. The models presented satisfactory adjustments to the drying kinetics, with R²> 99.0% and DQM <0.02, especially the Cavalcanti Mata model. The drying process used was efficient resulting in a soluble, little hygroscopic and fine powder with bioactive concentrations relevant for its medicinal use.El presente trabajo tuvo como objetivo evaluar los ajustes a los modelos matemáticos de la cinética de secado de las hojas de hierba de limón en un secador convectivo a 40, 55 y 70 ° C, con velocidades de aire de secado de 1.0, 1.5 y 2.0 m / s por diseño factorial 22 + 3 experimentos en el punto central. Las hojas frescas se sometieron a caracterización química, física y fisicoquímica. La cinética de secado se realizó en un secador convectivo, con ajustes para cuatro modelos matemáticos, considerándose como criterio de calidad de ajuste, los coeficientes de determinación (R2) y las desviaciones cuadráticas medias (DQM). Después de las pruebas de secado, se pueden resaltar las tres pruebas realizadas a una temperatura de 40 ° C y una velocidad de 1.0 m / s, presentando mayor rendimiento y menor contenido de agua, el polvo resultante se sometió a análisis de: granulometría , contenido de agua, actividad del agua, cenizas, humectabilidad, higroscopicidad, solubilidad, masa específica, masa específica de compactación, factor de Hausner, índice de compresibilidad, color, flavonoides, clorofilas totales y b, carotenoides y taninos. Los modelos presentaron ajustes satisfactorios a la cinética de secado, con R²> 99.0% y DQM <0.02, especialmente el modelo Cavalcanti Mata. El proceso de secado utilizado fue eficiente, dando como resultado un polvo soluble, poco higroscópico y fino, con concentraciones bioactivas relevantes para su uso medicinal