publication . Article . 2017

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
  • Published: 01 Sep 2017
  • Publisher: Oxford University Press (OUP)
  • Country: Italy
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...
free text keywords: Transplantation, Nephrology, Management and Education Tools, α-galactosidase A/α-galactosidase A deficiency, disease progression, disease stability, Fabry disease, organ dysfunction scores, Fabry disease; disease progression; disease stability; organ dysfunction scores; α-galactosidase A/α-galactosidase A deficiency, Settore MED/14 - Nefrologia, α-галактозидаза a, дефицит α-галактозидазы a, прогрессирование заболевания, стабилизация заболевания, болезнь фабри, показатели дисфункции органов, Neurology. Diseases of the nervous system, RC346-429, Clinical Neurology, Neurology, Medicine, business.industry, business, Surgery, medicine.medical_specialty, Clinical significance, Transplantation, Renal function, Raw score, Severity of illness, Cardiology, New York Heart Association Class, Internal medicine, Organ Dysfunction Scores, Fabry disease, medicine.disease
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