US2016324788A1PendingUtilityA1

Formulations of 3-(6-(1-(2,2-difluorobenzo[d][1,3]dioxol-5-yl) cyclopropanecarboxamido)-3-methylpyridin-2-yl)benzoic acid

Assignee: VERTEX PHARMAPriority: Jan 25, 2012Filed: Apr 7, 2016Published: Nov 10, 2016
Est. expiryJan 25, 2032(~5.5 yrs left)· nominal 20-yr term from priority
A61P 5/14A61P 9/04A61P 5/18A61P 3/06A61P 3/10A61P 35/00A61P 43/00A61P 7/00A61P 7/10A61P 25/16A61P 25/28A61P 27/02A61P 25/08A61P 25/14A61P 3/00A61P 1/16A61P 1/10A61P 25/00A61P 19/08A61P 21/02A61P 1/18A61P 15/10A61P 21/04A61P 19/10A61P 11/02A61P 11/06A61P 11/00A61K 31/4709A61K 9/2027A61K 9/2077A61K 9/2095A61K 47/38A61K 47/12A61K 31/47A61K 9/1652A61K 9/2866A61K 9/2054A61K 9/1623A61J 3/10A61K 9/28A61K 47/32A61K 2121/00A61K 9/2013A61K 45/06A61K 9/20A61K 31/443A61K 47/10A61K 9/0053A61K 9/16
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Claims

Abstract

A pharmaceutical composition comprising Compound 1, (3-(6-(1-(2,2-difluorobenzo[d][1,3]dioxol-5-yl) cyclopropanecarboxamido)-3-methylpyridin-2-yl)benzoic acid), and at least one excipient selected from: a filler, a disintegrant, a surfactant, a binder, and a lubricant, the composition being suitable for oral administration to a patient in need thereof to treat a CFTR mediated disease such as Cystic Fibrosis. Processes of preparing pharmaceutical compositions comprising Compound 1 are also disclosed.

Claims

exact text as granted — not AI-modified
1 . A tablet for oral administration comprising:
 a. Compound 1, Compound 1 Form I, Compound 1 Form II, and/or Compound 1 HCl Salt Form A;   b. a filler;   c. a disintegrant;   d. a surfactant;   e. a lubricant; and   f. at least one of a binder or a glidant.   
     
     
         2 . The tablet of  claim 1 , wherein Compound 1, Compound 1 Form I, Compound 1 Form II, and/or Compound 1 HCl Salt Form A is present in the tablet in an amount ranging from about 25 mg to about 500 mg. 
     
     
         3 . The tablet of  claim 1 , wherein the amount of Compound 1, Compound 1 Form I, Compound 1 Form II, and/or Compound 1 HCl Salt Form A in the tablet ranges from about 15 wt % to about 75 wt % by weight of the tablet. 
     
     
         4 . The tablet of  claim 1 , wherein the amount of Compound 1, Compound 1 Form I, Compound 1 Form II, and/or Compound 1 HCl Salt Form A in the tablet ranges from about 40 wt % to about 70 wt % by weight of the tablet. 
     
     
         5 . The tablet of  claim 1  having one of the following formulations: 
       
         
           
                 
                 
                 
               
                     
                     
                 
                     
                   Roller Compaction Granule 
                     
                 
                     
                   Blend 
                   (% w/w) 
                 
                     
                     
                 
                     
                   Compound 1 
                   20-40 
                 
                     
                   Microcrystalline cellulose 
                   30-50 
                 
                     
                   Mannitol 
                   10-30 
                 
                     
                   Croscarmellose Sodium 
                   1-5 
                 
                     
                   Sodium Lauryl Sulfate 
                   0.1-2   
                 
                     
                   Colloidal Silica 
                   0.1-1   
                 
                     
                   Magnesium Stearate 
                   1-3 
                 
                     
                     
                 
                     
                   Tablet Composition 
                   (% w/w) 
                 
                     
                     
                 
                     
                   Roller Compaction Granule 
                     99-99.9 
                 
                     
                   Blend 
                 
                     
                   Magnesium Stearate 
                   0.1-1   
                 
                     
                     
                 
                     
                   High Shear Granule Blend 
                   (% w/w) 
                 
                     
                     
                 
                     
                   Compound 1 
                   60-70 
                 
                     
                   Microcrystalline cellulose 
                    5-15 
                 
                     
                   Croscarmellose Sodium 
                   1-5 
                 
                     
                   Sodium Lauryl Sulfate 
                   0.1-2   
                 
                     
                   Polyvinylpyrrolidone 
                   1-5 
                 
                     
                     
                 
                     
                   Tablet Composition 
                   (% w/w) 
                 
                     
                     
                 
                     
                   High Shear Granule Blend 
                   75-89 
                 
                     
                   Microcrystalline cellulose 
                   10-15 
                 
                     
                   Croscarmellose Sodium 
                   1-5 
                 
                     
                   Magnesium Stearate 
                   0.1-5   
                 
                     
                     
                 
                     
                   High Shear Granule Blend 
                   (% w/w) 
                 
                     
                     
                 
                     
                   Compound 1 Form I 
                   60-70 
                 
                     
                   Microcrystalline cellulose 
                    5-15 
                 
                     
                   Croscarmellose Sodium 
                   1-5 
                 
                     
                   Polyvinylpyrrolidone 
                   1-5 
                 
                     
                   Sodium Lauryl Sulfate 
                   0.1-2   
                 
                     
                     
                 
                     
                   Tablet Composition 
                   (% w/w) 
                 
                     
                     
                 
                     
                   High Shear Granule Blend 
                   78-89 
                 
                     
                   Microcrystalline cellulose 
                   10-15 
                 
                     
                   Croscarmellose Sodium 
                   1-5 
                 
                     
                   Magnesium Stearate 
                   0.1-2   
                 
                     
                     
                 
                     
                   Film Coated Tablet 
                   (% w/w) 
                 
                     
                     
                 
                     
                   Core Tablet Composition 
                   95-99 
                 
                     
                   Film Coat 
                   1-5 
                 
                     
                   Wax 
                   Trace 
                 
                     
                     
                 
                     
                   Roller Compaction Granule 
                 
                     
                   Blend 
                   (% w/w) 
                 
                     
                     
                 
                     
                   Compound 1 Form I 
                   30 
                 
                     
                   Microcrystalline cellulose 
                   42.3 
                 
                     
                   Mannitol 
                   21.2 
                 
                     
                   Croscarmellose Sodium 
                   3 
                 
                     
                   Sodium Lauryl Sulfate 
                   1 
                 
                     
                   Colloidal Silica 
                   0.5 
                 
                     
                   Magnesium Stearate 
                   2 
                 
                     
                     
                 
                     
                   Tablet Composition 
                   (% w/w) 
                 
                     
                     
                 
                     
                   Roller Compaction Granule 
                   99.5 
                 
                     
                   Blend 
                   0.5 
                 
                     
                     
                 
                     
                   Magnesium Stearate 
                 
                     
                   High Shear Granule Blend 
                   (% w/w) 
                 
                     
                     
                 
                     
                   Compound 1 Form I 
                   40-80 
                 
                     
                   Microcrystalline cellulose 
                   20-40 
                 
                     
                   Mannitol 
                   10-15 
                 
                     
                   Croscarmellose Sodium 
                   1-5 
                 
                     
                   Polyvinylpyrrolidone 
                    1-10 
                 
                     
                   Sodium Lauryl Sulfate 
                   0.1-2   
                 
                     
                     
                 
                     
                   Tablet Composition 
                   (% w/w) 
                 
                     
                     
                 
                     
                   High Shear Granule Blend 
                   95-99 
                 
                     
                   Croscarmellose Sodium 
                   1-4 
                 
                     
                   Magnesium Stearate 
                   0.1-1   
                 
                     
                     
                 
                     
                   High Shear Granule Blend 
                   (% w/w) 
                 
                     
                     
                 
                     
                   Compound 1 Form I 
                   50 
                 
                     
                   Microcrystalline cellulose 
                   30 
                 
                     
                   Mannitol 
                   13 
                 
                     
                   Croscarmellose Sodium 
                   2 
                 
                     
                   Polyvinylpyrrolidone 
                   4 
                 
                     
                   Sodium Lauryl Sulfate 
                   1 
                 
                     
                     
                 
                     
                   Tablet Composition 
                   (% w/w) 
                 
                     
                     
                 
                     
                   High Shear Granule Blend 
                   97.5 
                 
                     
                   Croscarmellose Sodium 
                   2.0 
                 
                     
                   Magnesium Stearate 
                   0.5 
                 
                     
                     
                 
                     
                   High Shear Granule Blend 
                   (% w/w) 
                 
                     
                     
                 
                     
                   Compound 1 Form I 
                   60 
                 
                     
                   Microcrystalline cellulose 
                   20 
                 
                     
                   Mannitol 
                   13 
                 
                     
                   Croscarmellose Sodium 
                   2 
                 
                     
                   Polyvinylpyrrolidone 
                   4 
                 
                     
                   Sodium Lauryl Sulfate 
                   1 
                 
                     
                     
                 
                     
                   Tablet Composition 
                   (% w/w) 
                 
                     
                     
                 
                     
                   High Shear Granule Blend 
                   97.5 
                 
                     
                   Croscarmellose Sodium 
                   2.0 
                 
                     
                   Magnesium Stearate 
                   0.5 
                 
                     
                     
                 
                     
                   High Shear Granule Blend 
                   (% w/w) 
                 
                     
                     
                 
                     
                   Compound 1 Form I 
                   60 
                 
                     
                   Microcrystalline cellulose 
                   20 
                 
                     
                   Mannitol 
                   13 
                 
                     
                   Croscarmellose Sodium 
                   2 
                 
                     
                   Polyvinylpyrrolidone 
                   4 
                 
                     
                   Sodium Lauryl Sulfate 
                   1 
                 
                     
                     
                 
                     
                   Tablet Composition 
                   (% w/w) 
                 
                     
                     
                 
                     
                   High Shear Granule Blend 
                   83 
                 
                     
                   Microcrystalline cellulose 
                   14 
                 
                     
                   Croscarmellose Sodium 
                   2 
                 
                     
                   Magnesium Stearate 
                   1 
                 
                     
                     
                 
                     
                   Twin Screw Granule Blend 
                   (% w/w) 
                 
                     
                     
                 
                     
                   Compound 1 Form I 
                   60 
                 
                     
                   Microcrystalline cellulose 
                   20 
                 
                     
                   Mannitol 
                   13 
                 
                     
                   Croscarmellose Sodium 
                   2 
                 
                     
                   Polyvinylpyrrolidone 
                   4 
                 
                     
                   Sodium Lauryl Sulfate 
                   1 
                 
                     
                     
                 
                     
                   Tablet Composition 
                   (% w/w) 
                 
                     
                     
                 
                     
                   Twin Screw Granule Blend 
                   83 
                 
                     
                   Microcrystalline cellulose 
                   14 
                 
                     
                   Croscarmellose Sodium 
                   2 
                 
                     
                   Magnesium Stearate 
                   1 
                 
                     
                     
                 
                     
                   Twin Screw Wet Granule Blend 
                   (% w/w) 
                 
                     
                     
                 
                     
                   Compound 1 Form I 
                   80.0 
                 
                     
                   Microcrystalline cellulose 
                   13.6 
                 
                     
                   Croscarmellose Sodium 
                   2.5 
                 
                     
                   Polyvinylpyrrolidone 
                   3.1 
                 
                     
                   Sodium Lauryl Sulfate 
                   0.7 
                 
                     
                     
                 
                     
                   Tablet Composition 
                   (% w/w) 
                 
                     
                     
                 
                     
                   Twin Screw Granule Blend 
                   83 
                 
                     
                   Microcrystalline cellulose 
                   12 
                 
                     
                   Croscarmellose Sodium 
                   4 
                 
                     
                   Magnesium Stearate 
                   1 
                 
                     
                     
                 
                     
                   Twin Screw Granule Blend 
                   (% w/w) 
                 
                     
                     
                 
                     
                   Compound 1 Form I 
                   80.0 
                 
                     
                   Microcrystalline cellulose 
                   13.6 
                 
                     
                   Croscarmellose Sodium 
                   2.5 
                 
                     
                   Polyvinylpyrrolidone 
                   3.1 
                 
                     
                   Sodium Lauryl Sulfate 
                   0.7 
                 
                     
                     
                 
                     
                   Tablet Composition 
                   (% w/w) 
                 
                     
                     
                 
                     
                   Twin Screw Granule Blend 
                   83 
                 
                     
                   Microcrystalline cellulose 
                   12 
                 
                     
                   Croscarmellose Sodium 
                   4 
                 
                     
                   Magnesium Stearate 
                   1 
                 
                     
                     
                 
                     
                   Film Coated Tablet 
                   (% w/w) 
                 
                     
                     
                 
                     
                   Core Tablet Composition 
                   97 
                 
                     
                   Film Coat 
                   3 
                 
                     
                   Wax 
                   Trace 
                 
                     
                     
                 
                     
                   High Shear Granule Blend 
                   mg 
                 
                     
                     
                 
                     
                   Compound 1 Form I 
                   200 
                 
                     
                   Microcrystalline cellulose 
                   66 
                 
                     
                   Mannitol 
                   43 
                 
                     
                   Croscarmellose Sodium 
                   7 
                 
                     
                   Polyvinylpyrrolidone 
                   13 
                 
                     
                   Sodium Lauryl Sulfate 
                   3 
                 
                     
                     
                 
                     
                   Core Tablet Composition 
                 
                     
                   (200 mg dose) 
                   mg 
                 
                     
                     
                 
                     
                   High Shear Granule Blend 
                   332 
                 
                     
                   Microcrystalline cellulose 
                   56 
                 
                     
                   Croscarmellose Sodium 
                   8 
                 
                     
                   Magnesium Stearate 
                   4 
                 
                     
                     
                 
                     
                   Film Coated Tablet 
                 
                     
                   (200 mg dose) 
                   mg 
                 
                     
                     
                 
                     
                   Core Tablet Composition 
                   400 
                 
                     
                   Film Coat 
                   12 
                 
                     
                   Wax 
                   trace 
                 
                     
                     
                 
                     
                   Twin Screw Granule Blend 
                   mg 
                 
                     
                     
                 
                     
                   Compound 1 Form I 
                   200 
                 
                     
                   Microcrystalline cellulose 
                   66 
                 
                     
                   Mannitol 
                   43 
                 
                     
                   Croscarmellose Sodium 
                   7 
                 
                     
                   Polyvinylpyrrolidone 
                   13 
                 
                     
                   Sodium Lauryl Sulfate 
                   3 
                 
                     
                     
                 
                     
                   Core Tablet Composition 
                 
                     
                   (200 mg dose) 
                   mg 
                 
                     
                     
                 
                     
                   Twin Screw Granule Blend 
                   332 
                 
                     
                   Microcrystalline cellulose 
                   56 
                 
                     
                   Croscarmellose Sodium 
                   8 
                 
                     
                   Magnesium Stearate 
                   4 
                 
                     
                     
                 
                     
                   High Shear Granule Blend 
                   mg 
                 
                     
                     
                 
                     
                   Compound 1 Form I 
                   200 
                 
                     
                   Microcrystalline cellulose 
                   67 
                 
                     
                   Mannitol 
                   45 
                 
                     
                   Croscarmellose Sodium 
                   7 
                 
                     
                   Polyvinylpyrrolidone 
                   10.4 
                 
                     
                   Sodium Lauryl Sulfate 
                   2.6 
                 
                     
                     
                 
                     
                   Core Tablet Composition 
                 
                     
                   (200 mg dose) 
                   mg 
                 
                     
                     
                 
                     
                   High Shear Granule Blend 
                   332 
                 
                     
                   Microcrystalline cellulose 
                   56 
                 
                     
                   Croscarmellose Sodium 
                   8 
                 
                     
                   Magnesium Stearate 
                   4 
                 
                     
                     
                 
                     
                   Film Coated Tablet 
                 
                     
                   (200 mg dose) 
                   mg 
                 
                     
                     
                 
                     
                   Core Tablet Composition 
                   400 
                 
                     
                   Film Coat 
                   12 
                 
                     
                   Wax 
                   trace 
                 
                     
                     
                 
                     
                   High Shear Granule Blend 
                   mg 
                 
                     
                     
                 
                     
                   Compound 1 Form I 
                   300 
                 
                     
                   Microcrystalline cellulose 
                   99 
                 
                     
                   Mannitol 
                   64.5 
                 
                     
                   Croscarmellose Sodium 
                   10.5 
                 
                     
                   Polyvinylpyrrolidone 
                   19.5 
                 
                     
                   Sodium Lauryl Sulfate 
                   4.5 
                 
                     
                     
                 
                     
                   Core Tablet Composition 
                 
                     
                   (300 mg dose) 
                   mg 
                 
                     
                     
                 
                     
                   High Shear Granule Blend 
                   498 
                 
                     
                   Microcrystalline cellulose 
                   84 
                 
                     
                   Croscarmellose Sodium 
                   12 
                 
                     
                   Magnesium Stearate 
                   6 
                 
                     
                     
                 
                     
                   Film Coated Tablet 
                 
                     
                   (300 mg dose) 
                   mg 
                 
                     
                     
                 
                     
                   Core Tablet Composition 
                   600 
                 
                     
                   Film Coat 
                   18 
                 
                     
                   Wax 
                   trace 
                 
                     
                     
                 
                     
                   High Shear Granule Blend 
                   mg 
                 
                     
                     
                 
                     
                   Compound 1 Form I 
                   300 
                 
                     
                   Microcrystalline cellulose 
                   100.5 
                 
                     
                   Mannitol 
                   67.5 
                 
                     
                   Croscarmellose Sodium 
                   10.5 
                 
                     
                   Polyvinylpyrrolidone 
                   15.6 
                 
                     
                   Sodium Lauryl Sulfate 
                   3.9 
                 
                     
                     
                 
                     
                   Core Tablet Composition 
                 
                     
                   (300 mg dose) 
                   mg 
                 
                     
                     
                 
                     
                   High Shear Granule Blend 
                   498 
                 
                     
                   Microcrystalline cellulose 
                   84 
                 
                     
                   Croscarmellose Sodium 
                   12 
                 
                     
                   Magnesium Stearate 
                   6 
                 
                     
                     
                 
                     
                   Film Coated Tablet 
                 
                     
                   (300 mg dose) 
                   mg 
                 
                     
                     
                 
                     
                   Core Tablet Composition 
                   600 
                 
                     
                   Film Coat 
                   18 
                 
                     
                   Wax 
                   trace 
                 
                     
                     
                 
                     
                   High Shear Granule Blend 
                   (% w/w) 
                 
                     
                     
                 
                     
                   Compound 1 Form I 
                   70 
                 
                     
                   Microcrystalline cellulose 
                   12 
                 
                     
                   Mannitol 
                   11 
                 
                     
                   Croscarmellose Sodium 
                   2 
                 
                     
                   Polyvinylpyrrolidone 
                   4 
                 
                     
                   Sodium Lauryl Sulfate 
                   1 
                 
                     
                     
                 
                     
                   Tablet Composition 
                   (% w/w) 
                 
                     
                     
                 
                     
                   High Shear Granule Blend 
                   97.5 
                 
                     
                   Croscarmellose Sodium 
                   2.0 
                 
                     
                   Magnesium Stearate 
                   0.5 
                 
                     
                     
                 
                     
                   High Shear Granule Blend 
                   (% w/w) 
                 
                     
                     
                 
                     
                   Compound 1 Form I or Form II 
                   61 
                 
                     
                   Microcrystalline cellulose 
                   20.3 
                 
                     
                   Mannitol 
                   13.2 
                 
                     
                   Croscarmellose Sodium 
                   2 
                 
                     
                   Polyvinylpyrrolidone 
                   2.7 
                 
                     
                   Sodium Lauryl Sulfate 
                   0.7 
                 
                     
                     
                 
                     
                   Tablet Composition 
                   (% w/w) 
                 
                     
                     
                 
                     
                   High Shear Granule Blend 
                   83 
                 
                     
                   Microcrystalline cellulose 
                   14 
                 
                     
                   Croscarmellose Sodium 
                   2 
                 
                     
                   Magnesium Stearate 
                   1 
                 
                     
                     
                 
                     
                   High Shear Granule Blend 
                   mg 
                 
                     
                     
                 
                     
                   Compound 1 Form I or Form II 
                   100 
                 
                     
                   Microcrystalline cellulose 
                   33.3 
                 
                     
                   Mannitol 
                   21.7 
                 
                     
                   Croscarmellose Sodium 
                   3.3 
                 
                     
                   Polyvinylpyrrolidone 
                   4.4 
                 
                     
                   Sodium Lauryl Sulfate 
                   1.1 
                 
                     
                     
                 
                     
                   Core Tablet Composition 
                 
                     
                   (100 mg dose) 
                   mg 
                 
                     
                     
                 
                     
                   High Shear Granule Blend 
                   163.9 
                 
                     
                   Microcrystalline cellulose 
                   27.6 
                 
                     
                   Croscarmellose Sodium 
                   3.9 
                 
                     
                   Magnesium Stearate 
                   2.0 
                 
                     
                     
                 
                     
                   Twin Screw Granule Blend 
                   mg 
                 
                     
                     
                 
                     
                   Compound 1 Form I 
                   200 
                 
                     
                   Microcrystalline cellulose 
                   34.0 
                 
                     
                   Croscarmellose Sodium 
                   6.3 
                 
                     
                   Polyvinylpyrrolidone 
                   7.8 
                 
                     
                   Sodium Lauryl Sulfate 
                   1.8 
                 
                     
                     
                 
                     
                   Core Tablet Composition 
                 
                     
                   (200 mg dose) 
                   mg 
                 
                     
                     
                 
                     
                   Twin Screw Granule Blend 
                   249.9 
                 
                     
                   Microcrystalline cellulose 
                   36.1 
                 
                     
                   Croscarmellose Sodium 
                   12.0 
                 
                     
                   Magnesium Stearate 
                   3.0 
                 
                     
                     
                 
                     
                   Twin Screw Granule Blend 
                   mg 
                 
                     
                     
                 
                     
                   Compound 1 Form I 
                   400 
                 
                     
                   Microcrystalline cellulose 
                   68.0 
                 
                     
                   Croscarmellose Sodium 
                   12.6 
                 
                     
                   Polyvinylpyrrolidone 
                   15.6 
                 
                     
                   Sodium Lauryl Sulfate 
                   3.6 
                 
                     
                     
                 
                     
                   Core Tablet Composition 
                 
                     
                   (400 mg dose) 
                   mg 
                 
                     
                     
                 
                     
                   Twin Screw Granule Blend 
                   499.8 
                 
                     
                   Microcrystalline cellulose 
                   72.2 
                 
                     
                   Croscarmellose Sodium 
                   24.0 
                 
                     
                   Magnesium Stearate 
                   6.0 
                 
                     
                     
                 
                     
                   Twin Screw Granule Blend 
                   mg 
                 
                     
                     
                 
                     
                   Compound 1 Form I 
                   200 
                 
                     
                   Microcrystalline cellulose 
                   34.0 
                 
                     
                   Croscarmellose Sodium 
                   6.3 
                 
                     
                   Polyvinylpyrrolidone 
                   7.8 
                 
                     
                   Sodium Lauryl Sulfate 
                   1.8 
                 
                     
                     
                 
                     
                   Core Tablet Composition 
                 
                     
                   (200 mg dose) 
                   mg 
                 
                     
                     
                 
                     
                   Twin Screw Granule Blend 
                   249.9 
                 
                     
                   Microcrystalline cellulose 
                   36.1 
                 
                     
                   Croscarmellose Sodium 
                   12.0 
                 
                     
                   Magnesium Stearate 
                   3.0 
                 
                     
                     
                 
                     
                   Film Coated Tablet 
                 
                     
                   (200 mg dose, 310 mg total) 
                   mg 
                 
                     
                     
                 
                     
                   Core Tablet Composition 
                   301 
                 
                     
                   Film Coat 
                   9.0 
                 
                     
                   Wax 
                   trace 
                 
                     
                     
                 
                     
                   , and 
                 
                     
                   Twin Screw Granule Blend 
                   mg 
                 
                     
                     
                 
                     
                   Compound 1 Form I 
                   400 
                 
                     
                   Microcrystalline cellulose 
                   68.0 
                 
                     
                   Croscarmellose Sodium 
                   12.6 
                 
                     
                   Polyvinylpyrrolidone 
                   15.6 
                 
                     
                   Sodium Lauryl Sulfate 
                   3.6 
                 
                     
                     
                 
                     
                   Core Tablet Composition 
                 
                     
                   (400 mg dose) 
                   mg 
                 
                     
                   Twin Screw Granule Blend 
                   499.8 
                 
                     
                   Microcrystalline cellulose 
                   72.2 
                 
                     
                   Croscarmellose Sodium 
                   24.0 
                 
                     
                   Magnesium Stearate 
                   6.0 
                 
                     
                     
                 
                     
                   Film Coated Tablet 
                 
                     
                   (400 mg dose, 620 mg total) 
                   mg 
                 
                     
                     
                 
                     
                   Core Tablet Composition 
                   602 
                 
                     
                   Film Coat 
                   18.0 
                 
                     
                   Wax 
                   trace 
                 
                     
                     
                 
             
                
               
               
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
                
               
            
           
         
       
     
     
         6 .- 27 . (canceled) 
     
     
         28 . The tablet of  claim 1 , wherein the tablet further comprises at least one additional therapeutic agent. 
     
     
         29 . The tablet of  claim 28 , wherein the additional therapeutic agent is a CFTR modulator. 
     
     
         30 . (canceled) 
     
     
         31 . The tablet of  claim 29 , wherein the CFTR modulator is N-(5-hydroxy-2,4-ditert-butyl-phenyl)-4-oxo-1H-quinoline-3-carboxamide. 
     
     
         32 . The tablet of  claim 1 , wherein Compound 1 is in Form I characterized by one or more peaks at 15.2 to 15.6 degrees, 16.1 to 16.5 degrees, and 14.3 to 14.7 degrees in an X-ray powder diffraction obtained using Cu K alpha radiation. 
     
     
         33 . The tablet of  claim 32 , wherein Compound 1 Form I is characterized by one or more peaks at 15.4, 16.3, and 14.5 degrees. 
     
     
         34 . The tablet of  claim 1 , wherein Compound 1 is in Form I characterized by a diffraction pattern substantially similar to that of  FIG. 1 . 
     
     
         35 . The tablet of  claim 1 , wherein Compound 1 is in Form I characterized by a diffraction pattern substantially similar to that of  FIG. 2 . 
     
     
         36 . A method of treating or lessening the severity of a disease in a patient comprising administering to the patient the tablet of  claim 1 , wherein the disease is selected from cystic fibrosis, asthma, smoke induced COPD, chronic bronchitis, rhinosinusitis, constipation, pancreatitis, pancreatic insufficiency, male infertility, mild pulmonary disease, idiopathic pancreatitis, allergic bronchopulmonary aspergillosis (ABPA), liver disease, hereditary emphysema, hereditary hemochromatosis, coagulation-fibrinolysis deficiencies, protein C deficiency, Type 1 hereditary angioedema, lipid processing deficiencies, familial hypercholesterolemia, Type 1 chylomicronemia, abetalipoproteinemia, lysosomal storage diseases, I-cell disease/pseudo-Hurler, mucopolysaccharidoses, Sandhof/Tay-Sachs, Crigler-Najjar type II, polyendocrinopathy/hyperinsulemia, Diabetes mellitus, Laron dwarfism, myleoperoxidase deficiency, primary hypoparathyroidism, melanoma, glycanosis CDG type 1, congenital hyperthyroidism, osteogenesis imperfecta, hereditary hypofibrinogenemia, ACT deficiency, Diabetes insipidus (DI), neurophyseal DI, neprogenic DI, Charcot-Marie Tooth syndrome, Perlizaeus-Merzbacher disease, neurodegenerative diseases, Alzheimer's disease, Parkinson's disease, amyotrophic lateral sclerosis, progressive supranuclear plasy, Pick's disease, several polyglutamine neurological disorders, Huntington's, spinocerebullar ataxia type I, spinal and bulbar muscular atrophy, dentatorubal pallidoluysian, myotonic dystrophy, spongiform encephalopathies, hereditary Creutzfeldt-Jakob disease, Fabry disease, Straussler-Scheinker syndrome, COPD, dry-eye disease, Sjogren's disease, Osteoporosis, Osteopenia, Gorham's Syndrome, chloride channelopathies, myotonia congenita, Bartter's syndrome type III, Dent's disease, hyperekplexia, epilepsy, hyperekplexia, lysosomal storage disease, Angelman syndrome, Primary Ciliary Dyskinesia (PCD), inherited disorders of the structure and/or function of cilia, PCD with situs inversus, PCD without situs inversus, or ciliary aplasia. 
     
     
         37 . The method of  claim 36 , wherein the disease is cystic fibrosis, emphysema, COPD, or dry-eye disease. 
     
     
         38 . The method of  claim 36 , wherein the disease is cystic fibrosis wherein the patient has a F508del CFTR mutation. 
     
     
         39 . The method of  claim 38 , wherein the patient is homozygous for F508del. 
     
     
         40 . The method of  claim 38 , wherein the patient is heterozygous for F508del. 
     
     
         41 . The method of  claim 36 , wherein the method comprises administering an additional therapeutic agent. 
     
     
         42 . The method of  claim 41 , wherein the therapeutic agent is selected from a mucolytic agent, bronchodilator, an anti-biotic, an anti-infective agent, an anti-inflammatory agent, a CFTR potentiator, or a nutritional agent. 
     
     
         43 . The method of  claim 41 , wherein the additional therapeutic agent is N-(5-hydroxy-2,4-ditert-butyl-phenyl)-4-oxo-1H-quinoline-3-carboxamide. 
     
     
         44 - 54 . (canceled)

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