28-03-2013 дата публикации
Номер: US20130079367A1
The present invention relates to solid state forms of N-[2,4-bis(1,1-dimethylethyl)-5-hydroxyphenyl]-1,4-dihydro-4-oxoquinoline-3-carboxamide (Compound 1), pharmaceutical compositions thereof and methods therewith. 126-. (canceled)27. A method of treating or lessening the severity of a disease in a patient , wherein said disease is selected from cystic fibrosis , asthma , smoke induced COPD , chronic bronchitis , rhinosinusitis , constipation , pancreatitis , pancreatic insufficiency , male infertility caused by congenital bilateral absence of the vas deferens (CBAVD) , mild pulmonary disease , idiopathic pancreatitis , allergic bronchopulmonary aspergillosis (ABPA) , liver disease , hereditary emphysema , hereditary hemochromatosis , coagulation-fibrinolysis deficiencies , such as protein C deficiency , Type 1 hereditary angioedema , lipid processing deficiencies , such as familial hypercholesterolemia , Type 1 chylomicronemia , abetalipoproteinemia , lysosomal storage diseases , such as 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 such as Alzheimer's disease , Parkinson's disease , amyotrophic lateral sclerosis , progressive supranuclear plasy , Pick's disease , several polyglutamine neurological disorders such as Huntington's , spinocerebullar ataxia type I , spinal and bulbar muscular atrophy , dentatorubal pallidoluysian , and myotonic dystrophy , as well as spongiform encephalopathies , such as hereditary Creutzfeldt-Jakob disease (due to prion protein processing defect) , Fabry disease , ...
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