BEMORII GOSHE

BEMORII GOSHE, bemorii irsi, ki hangomi on dar organizm mubodilai glikosfingolipidho khalal meyobad. Bemori az nomi dermatologi fransavi E. Goshe girifta shudaast. Dar mavridi B. G. sfingolipidi glyukoserebrozid asosan dar sipurz, jigar va magzi ustukhon jam meshavad.
B. G. ba tavri shadid va muzmin padid meoyad. Bemorii shadid dar mohhoi avvali hayoti kudak ba mushohida merasad; khunshorii pust va luobpardaho ba vuqu payvasta, az bini khun meravad, jigar va sipurz kalon meshavad, kudak jisman va ruhan az sabzish memonad. YAke az khususiyathoi marizi ikhtiloli sistemai asabhoi markazi ast.
Bemorii muzmin dar javonon va kalonsolon ba mushohida merasad. Hangomi on jigar va sipurz kalon, tahti pust khunshor meshavad, ustukhonhoi naychashakl meshikanand, az bini khun meravad.
Muolijai bemori vobasta ba alomathoi on surat megirad; dar holathoi vaznin sipurzro burida mepartoyand. Dar asnoi shadid budani bemori mariz badi yak yo du sol mefavtad. Hangomi bemorii muzmin oqibati on, odatan, nek ast.
Dar vaqti bo alomathoi B. G. ba dunyo omadani tifl, homilagii minbadaro hatman boyad zeri nazorat girift. Hangomi dar obi bachadon oshkor sokhtani hujayrahoi Goshe hamlro qat kardan lozim ast.