publication . Article . Other literature type . 2016

FAbry STabilization indEX (FASTEX): an innovative tool for the assessment of clinical stabilization in Fabry disease

Renzo Mignani; Federico Pieruzzi; Francesco Berri; Alessandro P. Burlina; Benito Chinea; Maurizio Gallieni; Maurizio Pieroni; Alessandro Salviati; Marco Spada;
Open Access Russian
  • Published: 01 Oct 2016 Journal: Nervno-Myšečnye Bolezni, volume 7, issue 2, pages 37-47 (issn: 2222-8721, eissn: 2413-0443, Copyright policy)
  • Publisher: ABV-press
  • Country: Italy
Abstract
На сегодняшний день предложены 2 системы количественной оценки бремени гликогеноза с дефицитом α-галактозидазы: индекс оценки степени тяжести Майнца (MSSI) и система балльной оценки тяжести болезни Фабри (DS3). Сделана попытка разработать динамическую математическую модель FASTEX (от англ. FAbry STabilization indEX, индекс стабилизации Фабри) для оценки клинической стабильности состояния. Мультидисциплинарная группа экспертов по болезни Фабри впервые предложила новую шкалу оценки тяжести заболевания по предварительной оценке (от англ. raw score, RS), основанную на 3 доменах (домен нервной системы (боль, цереброваскулярные события), почечный домен (протеинурия, с...
Subjects
free text keywords: α-галактозидаза a, дефицит α-галактозидазы a, прогрессирование заболевания, стабилизация заболевания, болезнь фабри, показатели дисфункции органов, Fabry disease; disease progression; disease stability; organ dysfunction scores; α-galactosidase A/α-galactosidase A deficiency, Settore MED/14 - Nefrologia, Management and Education Tools, α-galactosidase A/α-galactosidase A deficiency, disease progression, disease stability, Fabry disease, organ dysfunction scores, lcsh:Neurology. Diseases of the nervous system, lcsh:RC346-429, Surgery, medicine.medical_specialty, medicine, Fabry disease, medicine.disease, Severity of illness, Renal function, Organ Dysfunction Scores, New York Heart Association Class, Cardiology, Raw score, business.industry, business, Internal medicine, Clinical significance, Transplantation
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