The invention is directed to novel pharmaceutical compositions comprising chemicals agents that are useful in the treatment and prevention of cystic fibrosis and the prevention of signs and symptoms of this disease. These pharmaceutical compositions are surprisingly successful in the treatment disorders related to cystic fibrosis including disorders of blood production. Many of these compositions of the invention are even more effective when administered to a patient in pulses. Pulse therapy is not a form of discontinuous administration of the same amount of a composition over time, but comprises administration of the same dose of the composition at a reduced frequency or administration of reduced doses.
CROSS-REFERENCE TO RELATED APPLICATION
This Divisional Application claims the benefit under 35 U.S.C. .sctn.120 of U.S. Ser. No. 09/248,260, filed Feb. 11, 1999, now abandoned which claims the benefit under 35 U.S.C. .sctn. 119(e) of U.S. Provisional Application No. 60/074,304, filed Feb. 11, 1998.
Compounds are disclosed having the structure of Formula I: ##STR00001## where R.sup.1, R.sup.3, and R.sup.4 are independently hydrogen or C.sub.1 to C.sub.4 alkyl, and R.sup.2 is: ##STR00002## where R.sup.5 is selected from the group consisting of hydrogen and C.sub.1 to C.sub.4 alkyl, or ##STR00003## where R.sup.6, R.sup.7 and R.sup.8 are independently hydrogen or C.sub.1 to C.sub.4 alkyl; or the esters or pharmacologically acceptable salts thereof. Such compounds may be utilized for the prophylaxis or treatment of various diseases, particularly inflammatory conditions of the GI tract.Methods of treating inflammatory conditions of the GI tract such as inflammatory bowel disease using compounds having the following formula are also disclosed: ##STR00004## where R.sup.9, R.sup.10 and R.sup.11 are independently selected from the group consisting of hydrogen and C.sub.1 to C.sub.4 alkyl, and R.sup.12 is selected from the group consisting of hydrogen and --C(O)R.sup.13, where R.sup.13 is a C.sub.1 to C.sub.6 alkyl or an aryl group.
