Object's details: Two-year follow-up of Sanfilippo Disease patients treated with a genistein-rich isoflavone extract: Assessment of effects on cognitive functions and general status of patients

Provider:Repozytorium Instytutu Biochemii i Biofizyki Polskiej Akademii Nauk

Description

Title:
Two-year follow-up of Sanfilippo Disease patients treated with a genistein-rich isoflavone extract: Assessment of effects on cognitive functions and general status of patients
Creator:
Piotrowska, Ewa;
Jakóbkiewicz-Banecka, Joanna;
Maryniak, Agnieszka;
Tylki-Szymańska, Anna;
Puk, Ewa;
Liberek, Anna;
Węgrzyn, Alicja;
Czartoryska, Barbara;
Słomińska-Wojewódzka, Monika;
Węgrzyn, Grzegorz
Description:
Mucopolysaccharidoses (MPS) are inherited metabolic disorders caused by deficiencies in enzymes involved in degradation of glycosaminoglycans. MPS type III (Sanfilippo disease) is clinically characterized mainly by progressive and severe behavioral disturbances and cognitive dysfunction. Recent 1-year experimental treatment of 10 patients with a genistein (4’, 5, 7-trihydroxyisoflavone)-rich extract resulted in improvement of tested parameters, including cognitive and behavioral functions.Eight pediatric patients with Sanfilippo disease were enrolled into the study. The modified version of the Brief Assessment Examination was used to assess cognitive functions. Moreover, 18 different parameters concerning changes in conditions of patients were assessed by their parents.During the first year of the treatment, an improvement of cognitive functions in 7 patients and stabilization in 1 patient were assessed, while after the third year (2-year follow-up) further improvement was observed in 2 patients, stabilization in 3 patients and some deterioration in 3 patients. Monitoring of general and behavioral symptoms revealed improvement in all patients after the first year of the treatment, further improvement in 5 patients, and deterioration in 3 patients during the next 2 years. We conclude that the treatment of Sanfilippo patients with a genistein-rich soy isoflavone extract(called gene expression-targeted isoflavone therapy[GET IT]) may be effective in either inhibition (in some patients) or slowing down (in other patients) of behavioral and cognitive problems over a longer period. An increased dose of genistein may improve the efficacy of the treatment.
Object availability:
free access
Date:
2011
Type:
Article;
PeerReviewed
Language:
English
Format:
application/pdf
Relation: http://eprints.ibb.waw.pl/250
Subject:
QH301 Biology;
QH426 Genetics
Identifier: http://eprints.ibb.waw.pl/250/1/Med_Sci_Monit_2011.pdf ;
Piotrowska, Ewa and Jakóbkiewicz-Banecka, Joanna and Maryniak, Agnieszka and Tylki-Szymańska, Anna and Puk, Ewa and Liberek, Anna and Węgrzyn, Alicja and Czartoryska, Barbara and Słomińska-Wojewódzka, Monika and Węgrzyn, Grzegorz (2011) Two-year follow-up of Sanfilippo Disease patients treated with a genistein-rich isoflavone extract: Assessment of effects on cognitive functions and general status of patients. Medical Science Monitor , 17 . pp. 196-202.
Data provider:
Repozytorium Instytutu Biochemii i Biofizyki Polskiej Akademii Nauk

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Object's details: Two-year follow-up of Sanfilippo Disease patients treated with a genistein-rich isoflavone extract: Assessment of effects on cognitive functions and general status of patients

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