Repository logo

Browsing by Issue Date, starting with "2018-09"

Filter results by year or month
Now showing 1 - 6 of 6
  • Early Feeding Practices in Infants with Phenylketonuria Across Europe
    Publication . Pinto, A; Adams, S; Ahring, K; Allen, H; Almeida, MF; Garcia-Arenas, D; Arslan, N; Assoun, M; Atik Altınok, Y; Barrio-Carreras, D; Belanger Quintana, A; Bernabei, SM; Bontemps, C; Boyle, F; Bruni, G; Bueno-Delgado, M; Caine, G; Carvalho, R; Chrobot, A; Chyż, K; Cochrane, B; Correia, C; Corthouts, K; Daly, A; De Leo, S; Desloovere, A; De Meyer, A; De Theux, A; Didycz, B; Dijsselhof, ME; Dokoupil, K; Drabik, J; Dunlop, C; Eberle-Pelloth, W; Eftring, K; Ekengren, J; Errekalde, I; Evans, S; Foucart, A; Fokkema, L; François, L; French, M; Forssell, E; Gingell, C; Gonçalves, C; Gökmen Özel, H; Grimsley, A; Gugelmo, G; Gyüre, E; Heller, C; Hensler, R; Jardim, I; Joost, C; Jörg-Streller, M; Jouault, C; Jung, A; Kanthe, M; Koç, N; Kok, I L; Kozanoğlu, T; Kumru, B; Lang, F; Lang, K; Liegeois, I; Liguori, A; Lilje, R; Ļubina, O; Manta-Vogli, P; Mayr, D; Meneses, C; Newby, C; Meyer, U; Mexia, S; Nicol, C; Och, U; Olivas, SM; Pedrón-Giner, C; Pereira, R; Plutowska-Hoffmann, K; Purves, J; Re Dionigi, A; Reinson, K; Robert, M; Robertson, L; Rocha, JC; Rohde, C; Rosenbaum-Fabian, S; Rossi, A; Ruiz, M; Saligova, J; Gutiérrez-Sánchez, A; Schlune, A; Schulpis, K; Serrano-Nieto, J; Skarpalezou, A; Skeath, R; Slabbert, A; Straczek, K; Giżewska, M; Terry, A; Thom, R; Tooke, A; Tuokkola, J; van Dam, E; van den Hurk, TM; van der Ploeg, EC; Vande Kerckhove, K; Van Driessche, M; van Wegberg, AJ; van Wyk, K; Vasconcelos, C; Velez García, V; Wildgoose, J; Winkler, T; Żółkowska, J; Zuvadelli, J; MacDonald, A
    In infants with phenylketonuria (PKU), dietary management is based on lowering and titrating phenylalanine (Phe) intake from breast milk or standard infant formula in combination with a Phe-free infant formula in order to maintain blood Phe levels within target range. Professionals use different methods to feed infants with PKU and our survey aimed to document practices across Europe.
    2018-09Journal article Open access Show more
  • Infeção da Cavidade Oral Associada a Ventilação Não Invasiva
    Publication . Prates, M; Tavares, AT; Pereira, A; Mussá, Y; Martins, J; Fernandes, F
    2018-09Other Open access Show more
  • 2018-09Other Open access Show more
  • 2018-09Other Open access Show more
  • Preoperative Prognostic Nutritional Index Predicts Survival of Patients with Intrahepatic Cholangiocarcinoma After Curative Resection
    Publication . Akgül, Ö; Bagante, F; Olsen, G; Cloyd, JM; Weiss, M; Merath, K; Alexandrescu, S; Pinto Marques, H; Aldrighetti, L; Maithel, SK; Pulitano, C; Bauer, TW; Shen, F; Poultsides, GA; Soubrane, O; Martel, G; Koerkamp, BG; Guglielmi, A; Itaru, E; Pawlik, TM
    Background: Intrahepatic cholangiocarcinoma (ICC) is an aggressive malignancy. We sought to examine the association between preoperative prognostic nutritional index (PNI) and long-term overall survival among patients with ICC who underwent curative-intent resection. Methods: Patients who underwent hepatectomy for ICC between 1990 and 2015 were identified using an international multi-institutional database. Clinic-pathological characteristics and long-term outcomes of patients with PNI ≥ 40 and <40 were compared using univariable and multivariable analyses. Results: Among 637 patients, 53 patients had PNI < 40 (8.3%) and 584 patients had PNI ≥ 40 (91.7%). While there was no difference between PNI groups with regard to tumor size (P = .87), patients with PNI < 40 were more likely to have multifocal disease (PNI < 40, n = 16, 30.2% vs PNI ≥ 40, n = 65, 11.1%; P < 0.001), poorly differentiated or undifferentiated ICC (PNI < 40, n = 13, 25.5% vs PNI ≥ 40, n = 75, 13.1%; P = 0.020) and T2/T3/T4 disease vs patients with PNI ≥ 40 (PNI < 40, n = 38, 71.7% vs PNI ≥ 40, n = 265, 45.4%; P < 0.001). Patients with PNI ≥ 40 had better OS vs patients with PNI < 40 (5-year OS: PNI ≥ 40: 47.5%, 95% CI, 42.2 to 52.6% vs PNI < 40: 24.6%, 95% CI, 12.1 to 39.6%; P < 0.001). On multivariable analysis, PNI < 40 remained associated with increase risk of death (HR, 1.71; 95% CI, 1.15 to 2.53; P = 0.008). Conclusion: A low preoperative PNI was associated with a more aggressive ICC phenotype. After controlling for these factors, PNI remained independently associated with a markedly worse prognosis.
    2018-09Journal article Open access Show more
  • Simultaneous Supratentorial and Infratentorial Pilocytic Astrocytomas in an Adult Patient with Concurrent Neurofibromatosis Type 1 and HIV Infection
    Publication . Forte, D; Nabais, A; Pontinha, C; Mafra, M; Mateus, L
    BACKGROUND: Neurofibromatosis type 1 (NF1) has been identified as a predisposing factor in the development of pilocytic astrocytoma (PA), a common benign central nervous system tumor. Although this is a common association, simultaneous development of multiple lesions is an infrequent finding, especially in nonoptic and hypothalamic locations. CASE DESCRIPTION: A 41-year-old female patient with NF1 and uncontrolled human immunodeficiency virus (HIV) type 1 infection presented with a first generalized seizure and associated headache and ataxia. Imaging studies revealed 2 large intra-axial PAs, nodular-cystic in the supratentorial compartment and solid in the infratentorial compartment. Both lesions were treated by gross total resection in 2 surgeries performed 1 week apart. Despite their different imaging patterns, the tumors were histologically and genetically identical. CONCLUSIONS: We present a unique case involving 2 histologically and genetically identical PAs occurring simultaneously in supratentorial and infratentorial locations. We suggest that an intrinsic predisposition to tumor development in patients with NF1 might have been enhanced by the HIV-related immunosuppression in this case. Strict oncologic surveillance is essential in patients with a tumor predisposition syndrome combined with immunosuppression.
    2018-09Journal article Open access Show more