Cystic fibrosis 528 INDEX

Blood plasma of children with cystic fibrosis was found to have decreased TAC (by 16%) in spite of increased concentrations of ascorbic acid, uric acid, and thiol groups (L4). In another study TAC of children with cystic fibrosis was normal, but these children received vitamin supplementation in doses prescribed in international guidelines (a-tocopherol <10 years, 100 mg daily, and >10 years, 200 mg daily retinol 2.5 mg daily ascorbic acid 100-200 mg daily) (M2). Other authors found TAC values for nonhospitalized patients (1.40 0.20 mM) not different from laboratory control values (1.35 0.11 mM), but greater than values for hospitalized patients (1.09 0.17 mM). TAC in CF children correlated positively with anthropometric values (height, weight, body mass index) and pulmonary function (forced expiratory volume in 1 sec), but not with age (L3). 

Muller F, Dommergues M, Simon-Bouy B, Ferec C, Oury JF, Aubry MC, et al. Cystic fibrosis screening a fetus with hyperechogenic bowel may be the index case. 1 Med Genet 1998 35 657 -60. 

Perhaps more importantly, it is freqnently unclear what the significance of a good penetration index is. For a drug like pentamidine, good peripheral deposition is obviously desirable, as its site of action is the alveoli. For drugs such as steroids and antibiotics in cystic fibrosis, their site of action is likely to be the... 

Death A fatal intoxication was reported in a 2-year-old female child, witii prolapsed rectum and cystic fibrosis, when phenol was used as a sclerotherapeutic agent. The authors question the use of phenol for this purpose due to its narrow therapeutic index and the availability of safer alternatives [83 ]. 

Water can bind to mucous glycoprotein (MGP) macromolecules and influence viscosity. Viscosity can be increased by dehydration of the mucus, as can adhesion of mucus-epithelium. In purulent sputum, the correlation between viscosity and dry weight of solids is poor and may explain why mucous glycoprotein content is a poor index of viscoelasticity in chronic bronchitis, bronchiectasis, and cystic fibrosis. Mucus s viscoelasticity increases with acidic pH (3), causing reduced mucociliary clearance. Its viscoelasticity is also dependent on the content of low molecular weight electrolytes. These properties reflect the polyelectrolyte nature of mucins (4-6). 

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