1. A method of treating a human patient with acid lysosomal lipase (CFL) deficiency, comprising administering a therapeutically effective amount of recombinant human CFL to said human patient in which administration is sufficient to improve liver function. The method according to claim 1, characterized in that said liver function is determined by the levels of serum liver transaminases. The method according to claim 2, characterized in that said liver transaminases are selected from the group consisting of serum aspartate aminotransferase (ACT) and alanine aminotransferase (ALT). The method according to claim 3, characterized in that said liver transaminase is ACT.5. The method according to claim 3, characterized in that said liver transaminase is ALT. 6. The method according to claim 1, characterized in that said administration is sufficient to improve hematomegaly. The method according to claim 1, characterized in that the introduction is sufficient to increase the level of serum hemoglobin. The method according to claim 1, characterized in that the introduction is sufficient to reduce the size of the liver. The method according to claim 1, characterized in that the introduction is sufficient to reduce the level of serum ferritin. The method of. 1, characterized in that said therapeutically effective amount of recombinant CFL is from about 0.1 mg to about 50 mg per kilogram of body weight of said human patient. The method of. 1, characterized in that said therapeutically effective amount of recombinant CFL is at least 0.1 mg per kilogram of body weight of said human patient. The method of. 1, characterized in that the indicated recombinant human CFL is introduced about�1. Способ лечения пациента-человека с дефицитом кислой лизосомной липазы (КЛЛ), включающий введение терапевтически эффективного количества рекомбинантной КЛЛ человека указанному пациенту-человеку, в котором введение является достаточным для улучшения функции печени.2. Способ по п.1, отличающийся тем
