Authors:Réka Bodnár, Klára Holics, Rita Ujhelyi, László Kádár, Lajos Kovács, Katalin Bolbás, Gyöngyi Székely, Kálmán Gyurkovits, Enikő Sólyom, and Ágnes Mészáros
Kovács, L., Veres, G., Csiszér, E.: Guideline about diagnostic and treatment of cysticfibrosis. [Irányelvek a cystás fibrosis diagnosztikájáról és kezeléséről]. 2011.
Authors:S. Flores-Martínez, J. Martínez, M. Machorro-Lazo, A. García-Zapién, L. Salgado-Goytia, E. Cruz-Quevedo, M. Morán-Moguel, and J. Sánchez-Corona
Welsh MJ, Ramsey BW, Accurso F, Cuttina GR (2001): CysticFibrosis. In: The metabolic and molecular bases of inherited disease. Eds Scriver CR, Beaudet AL, Valle D, Sly WS, McGraw-Hill, New York, pp. 5121
Authors:Márta Péntek, György Kosztolányi, Béla Melegh, Adrienn Halász, Gábor Pogány, Petra Baji, Valentin Brodszky, Noémi Vártokné Hevér, Imre Boncz, and László Gulácsi
Infant Care and Pediatrics Professional Body: Clinical guideline on cysticfibrosis by the Ministry of Health. [Csecsemő és Gyermekgyógyászati Szakmai Kollégium: Az Egészségügyi Minisztérium szakmai protokollja cystás
Authors:M. Olguin, M. Jimenez-Reyes, M. Peña-Aguil, and F. Sanchez-Aguirre
This study reports sodium and chlorine values in g/g, as detemined by neutron activation analysis in washed hair and nails from healthy and cystic fibrosis children. The values thus determined in cystic fibrosis tended to be higher than those in controls, however statistical differences were not significant (p>0.01). Additional experiments were carried out for comparison between washed and unwashed samples of the cystic fibrosis and control group and only the differences between washed and unwashed cystic fibrosis nails were significant (p<0.01) in both odium and chlorine values. Analysis of a standard reference milk sample, A-11 from IAEA, for the elements mentioned above gave a good agreement with the certified values.
Authors:M. Mason, J. Morris, B. Derenzy, V. Spate, L. Clarke, L. Hillman, L. Gawenis, T. Horsman, C. Baskett, T. Nichols, and J. Colbert
A genetically engineered “knockout gene” mouse model for human cystic fibrosis (CF) has been utilized to study bone mineralization.
In CF, the so-called cystic fibrosis transmembrane conductance regulator (CFTR) protein, a chloride ion channel, is either
absent or defective. To produce the animal model the murine CFTR gene has been inactivated producing CF symptoms in the homozygotic
progeny. CF results in abnormal intestinal absorption of minerals and nutrients which presumably results in substandard bone
mineralization. The objective of this study was to determine the feasibility of using whole-body thermal and fast neutron
activation analysis to determine mineral and trace-element differences between homozygote controls (+/+) and CF (−/−), murine
siblings. Gender-matched juvenile +/+ and −/− litter mates were lyophilized and placed in a BN capsule to reduce thermal-neutron
activation and irradiated for 10 seconds at φfast ≈ 1·1013 n·cm−2·s−1 using the MURR pneumatic-tube facility. Phosphorus was measured via the31P15(n,α)28Al13 reaction. After several days decay, the whole-body specimens were re-irradiated in the same facility, but without thermal-neutron
shielding, for 5 seconds and the gamma-ray spectrum was recorded at two different decay periods allowing measurement of77mSe,24Na,27Mg,38Cl,42K,49Ca,56Mn,66Cu and80Br from the corresponding radiative-capture reactions.