Search results for "hunter"

PO-0096 Clinical Effectiveness Of Idursulfase In Boys Aged 0–5 Years With Hunter Syndrome: 3-year Data From The Hunter Outcome Survey

2014

Background and aims Symptoms of Hunter syndrome typically become apparent at 2–4 years of age. Previous analyses have demonstrated improvements in certain clinical measures in young patients receiving idursulfase (Shire); however, data on long-term idursulfase use in these patients remain limited. This analysis used data available in the Hunter Outcome Survey (HOS), a global, observational registry sponsored by Shire, to investigate long-term effectiveness of idursulfase in boys with Hunter syndrome aged 0–5 years. Methods As of January 2014, 260/564 males followed prospectively in HOS had received ≥1 idursulfase infusion (excluding those who had received a bone marrow transplant or were en…

Age at symptom onset, diagnosis, and enzyme replacement therapy (ERT) initiation among family members with mucopolysaccharidosis II (Hunter Syndrome)…

2013

Mental health perspectives of Hunter syndrome: Case reports of two biological siblings

2016

Hunter syndrome is a rare X-linked recessive disorder caused by deficiency of the lysosomal enzyme iduronate-2-sulphatase, leading to progressive accumulation of a substance called glycosaminoglycans in nearly all cell types, tissues, and organs. Hunter syndrome presents with facial dysmorphism, airway diseases, skeletal defects, cardiomyopathies, and neuropsychiatric manifestations. Mental subnormality is a cardinal feature in Hunter syndrome. This is a progressive cognitive decline that is not amenable to enzyme replacement therapy. Due to progressive cognitive decline, training the children to improve the adaptive functioning is a challenge that creates immense stress for the caregivers.…

Roots of the Savage Mind. Apophenia and Imagination as Cognitive Process

2009

l'articolo studia il ruolo cognitivo dell'immaginario con particolare riferimento alle culture di caccia e raccolta e al problema della costruzione simbolica del paesaggio

Hunter-GatHerer Game: uno strumento simulativo dei comportamenti di caccia/raccolta nel mondo paleolitico

2013

In this paper we present the Hunter-Gatherer Game, a computer simulation of an environment where the individual and group survival depends on the ability to capture a prey or to find edible vegetables. The instrument is based on the Darwinian Hunter-Gatherer Theory stating that, during the Palaeolithic age, natural selection designed different gender preferences for foraging strategies (hunting in men and gathering in women), which are still sculpted in our modern brains. At present we are using the Hunter-Gatherer Game in order to experimentally test the above-mentioned theory, even though it also encompasses a number of potential applications.

Correction: Corrigendum: Long-term, open-labeled extension study of idursulfase in the treatment of Hunter syndrome

2013

CORRIGENDUM: Long-term, open-labeled extension study of idursulfase in the treatment of Hunter syndrome

Evaluation of the long-term treatment effects of idursulfase using statistical modelling: Data from the Hunter Outcome Survey (HOS)

2019

Treatment for mucopolysaccharidosis type II (MPS II Hunter syndrome) is available in the form of intravenous enzyme replacement therapy (ERT) with idursulfase (Shire, Lexington, MA, USA). This analysis used statistical modelling to evaluate the long-term treatment effects of idursulfase on selected clinical parameters based on data from HOS, a global, observational registry (Shire, Lexington, MA, USA). Mixed modelling was used to analyse data from male patients followed prospectively in HOS who had received idursulfase for 5-8 years and information available for two or more timepoints, of which one was pre-ERT. Data were excluded from patients with only pre-ERT information available, who ha…

219 Incidence Testing of Hunter Syndrome in A Population at Risk - First Results of A Binational Screening Programme

2012

Background Hunter syndrome (Mucopolysaccharidosis type II; X-linked inheritance; prevalence rate in Europe approximately 1:77000 male newborns) is a rare, progressive, multisystemic disease, caused by deficiency of the lysosomal enzyme Iduronate-2-sulfatase. Due to the very heterogeneous phenotype Hunter syndrome is often not diagnosed before pre-school age. This is unfortunate, because patients would significantly benefit from the earliest possible start of treatment containing enzyme replacement therapy. Early screening methods are possible, but due to the rarity of this disease they are too expensive to be performed in all newborns. An at-risk patient population screening provides opport…

Transformacja Polskiego Związku Łowieckiego w świetle ewolucji modelu łowiectwa w Polsce

2020

The Polish Hunting Association is a nationwide organization of Polish hunters and hunting clubs, whose traditions date back to the interwar period. This study presents the genesis and evolution of the Polish Hunting Association, which, in the wake of social and political changes in the country, has been transformed from a voluntary association into a monopolistic and compulsory-membership organization, which is to carry out tasks commissioned by public authorities. Additionally, as a result of further amendments, the Association has been transformed into a governmental agency, organizationally subordinated to the minister of the environment also in the scope of appointing its high-rank staf…

Polska kultura łowiecka jako niematerialne dziedzictwo kulturowe

2021

Pod koniec średniowiecza kultura łowiecka, czerpiąca z etosu rycerskiego, stała się ważnym składnikiem obyczajowości dworskiej, aby w następnych wiekach wpisać się w życie polskiego ziemiaństwa. Mimo wpływów zwłaszcza niemieckich, zachowała odrębność i narodowy charakter, które powodowały, że w czasach zaborów i II Rzeczypospolitej była jednym z czynników kształtujących świadomość narodową. Jej zasadnicze przejawy niszczono w czasach komunizmu, a mimo to przetrwała i została z sukcesem odbudowana w latach 90. XX w., stanowiąc źródło tożsamości wspólnotowej dla ponad 120 tys. myśliwych oraz członków ich rodzin. Polska kultura łowiecka obejmuje zasady etycznego postępowania względem przyrody,…