Cystic fibrosis-related

Cheng K, Ashby D, Smyth R. Ursodeoxycholic acid in cystic fibrosis-related liver disease a systematic review. J R Soc Med 1997 90 Suppl 31 6-12. 

Moran A, Phillips J, Milla C. Insulin and glucose excursion following premeal insulin lispro or repaglinide in cystic fibrosis-related diabetes. Diabetes Care 2001 24(10) 1706-1710. 

The effect of nebulized salbutamol 2.5 mg on blood glucose concentration has been assessed in a doubleblind, placebo-controlled study in 19 patients with insulin-dependent diabetes (9 of whom had cystic fibrosis-related diabetes) (1053). There were no significant... 

Wilson DC, Kalnins D, Stewart C, et al. Challenges in the dietary treatment of cystic fibrosis related diabetes mellitus. Clin Nutr 2000 19 87-93. 

Marin, S. andMerkogi, A. (2009) Direct electrochemical stripping detection of cystic-fibrosis-related DNA linked through cadmium sulfide quantum dots. Nanotechnology, 20 (5), 055101. 

Age does not significantly affect plasma concentrations or disposition of ibuprofen however, investigators have determined that the onset of antipyresis and maximum antipyretic effect is greater in children less than one year old as compared to children older than 6 years [43]. The authors hypothesized that this accelerated response was related to the greater relative body surface area of the young child. It should be noted that cystic fibrosis patients do have a higher clearance of ibuprofen [43a]. 

RFLPs are often a reflection of individual genetic diversity and are not related to a clinical phenotype, but occasionally they can be diagnostic of an inherited disease. This technique is relatively new yet, it has been applied to the prenatal detection of sickle cell anemia, thalassemia, phenylketonuria, a,-antitrypsin deficiency, Huntington s chorea, Duchenne muscular dystrophy, hemophilia A and B, cystic fibrosis, and several other, diseases. 

Although therapeutic approaches based upon gene therapy (Chapter 14) may well one day cure cystic fibrosis, current therapeutic intervention focuses upon alleviating cystic fibrosis symptoms, particularly those relating to respiratory function. Improved patient care has increased life expectancy of cystic fibrosis patients to well into their 30s. The major elements of cystic fibrosis management include ... 

The 12-transmembrane-spanning domain topology of the adenylyl cyclase enzymes is similar to that found in the ABC family of transporters (see Ch. 5), which includes the cystic fibrosis transmembrane rectifier and the P-glyco-protein. However, there is currently no convincing evidence of a transporter or channel function for mammalian adenylyl cyclases. The structural similarity may indicate that these functionally divergent protein families are derived in an evolutionary sense from related proteins. 

To take a step closer to defining the function of DNA sequences, the sequences are fluorescence tagged to identify transcriptional activity of genes (but not translation of protein), which then can be related to the disease state or medication. This information allows researchers to hunt for genes that are affected in disease states (e.g., mutation in CFTR gene regulating a chloride channel in cystic fibrosis patients)... 

The causes of human copper deficiency include (1) low intake - malnutrition, total parenteral nutrition (TPN) (2) high loss - cystic fibrosis, nephrotic syndromes and (3) genetic factors — Menkes disease. Copper deficiency may also be associated with chronic malabsorption, a situation which is made much worse in cases of gastric and bowel resection. Several special diets, including powdered milk, liquid protein and standard hospital diets are a means of inducing copper deficiency. The amount of copper in US food has decreased steadily since 1942, and may be related to the rising incidence of coronary artery disease. A copper deficiency may also occur as the result of the use of chelators for other purposes for example, diethyl dithiocarbamate is an in vivo metabolite of ANTABUSE (disulfiram). 

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