Search results for "gauche"
showing 10 items of 62 documents
Gošē slimības skrīnings pacientiem ar splenomegāliju, hepatomegāliju un trombocitopēniju
2018
Ievads. Gošē slimība ir viena no visbiežāk sastopamajām lizosomālās uzkrāšanās slimībām. Tās manifestēšanās vecuma un klīniskās ainas dažādības dēļ ir apgrūtināta slimības laicīga diagnostika. Mērķtiecīga slimības diagnostika ļautu laicīgi atpazīt skartos indivīdus un uzsākt nepieciešamo ārstēšanu, izvairoties no komplikācijām. Mērķis. Skrīnēt pacientus ar splenomegāliju, hepatomegāliju un/vai trombocitopēniju lai atklātu Gošē slimību un noteiktu tās izplatību Latvijas populācijā. Metodes. Darba ietvaros tika veikts prospektīvs pētījums, kura laikā RAKUS stacionāra LIC nodaļās tika atlasīti 100 pacienti ar splenomegāliju, hepatomegāliju un/vai trombocitopēniju. Pacientu nodotajos sausā asin…
Monitorización e individualización posológica de glucocerebrosidasa en pacientes con enfermedad de Gaucher Tipo I
2018
La enfermedad de Gaucher tipo I (EG1) es una enfermedad de depósito lisosomal causada por una deficiencia de la enzima glucocerebrosidasa, cuyo tratamiento consiste en la reposición mediante la administración exógena de la enzima deficiente, denominada terapia enzimática sustitutiva(TES). Actualmente, existe una gran controversia y una elevada variabilidad en la dosificación de la TES y en los regímenes utilizados en la práctica clínica debido a la amplia variabilidad observada en la respuesta a la TES; además las modificaciones de dosis para objetivar respuesta al tratamiento se alargan entre 12-24 meses, en una terapia con un elevado impacto económico al sistema nacional de salud. Sin emb…
De l’État de droit libéral à l’État de droit social. Critique et transformation de l’État de droit chez Hermann Heller
2019
This article analyzes the genesis and meaning of the concept of a “social rule of law” (sozialer Rechtsstaat) which Heller put forward in the particular context of The Weimar Republic. Heller was at this time one of the jurists who criticized the liberal ideal of law. He instead promoted a social conception that would take socio-economic inequalities into consideration. However, this did not imply eliminating the rule of law; it was rather a question of extending it or elevating it to a higher point – which he called “social democracy” and from 1929, “social rule of law”. In the context of the crisis of democracy, this kind of statement clearly had a political dimension.
Intraventrikuläre Obstruktionen bei der Dobutamin-Streßechokardiographie: Determinanten ihrer Entstehung und klinische Folgen (Dynamic intraventricul…
1997
Die Dobutamin-Stresechokardiographie (DSE) fuhrt zu einer starken Hyperkontraktilitat des Myokards bei Tachykardie und Vesodilatation. Diese Effekte konnen zu einer unphysiologischen Abnahme des endsystolischen Volumenindex (ESVI) mit Obstruktion des linksventrikularen Ausflustraktes (LVOT) fuhren. Bei 100 Patienten (Pat.), die sich einer DSE (5–40) μg/kg/min) unterzogen, wurde die kontinuierliche Dopplerregistrierung (DW-Doppler) vor Infusion und bei maximaler Herzfrequenz durchgefuhrt. Eine dynamische, spat-systolische, Flusakzeleration auf mehr als 2 Meter/Sekunde (m/s) unter maximaler Stimulation wurde als systolische Obstruktion des LVOT interpretiert. Manifestationsfaktoren systolisch…
Critical assessment of chitotriosidase analysis in the rational laboratory diagnosis of children with Gaucher disease and Niemann-Pick disease type A…
2006
Laboratory diagnosis of lysosomal storage disorders, especially sphingomyelinase deficiency (Niemann–Pick disease type A/B) and Niemann–Pick disease type C (NPC) can be challenging. We therefore aimed to analyse the feasibility of first-step screening with specific chitotriosidase cut-off values in children ≤ 10 years of age with visceral organomegaly (hepatomegaly, splenomegaly, or hepatosplenomegaly) in whom a storage disorder was suspected. We conducted a retrospective, cross-sectional, referral, single-centre study to assess diagnostic test properties in 106 individuals. Median chitotriosidase activity was 12 655 nmol/h per ml (interquartile range 4693–20982) in Gaucher disease (GD); 78…
Retrospective Analysis of Whole-Body Magnetic Resonance Imaging of Bone Manifestations in Long-Term Treated Patients with Gaucher Disease Type 1
2018
We retrospectively assessed bone and visceral manifestations in patients with Gaucher disease type 1 (GD1) with whole-body magnetic resonance imaging (WB-MRI) to determine the effects of different timing in initiating long-term enzyme replacement therapy.In 17 patients with GD1, we performed 2 WB-MRI examinations at a median interval of 13 months. Patients had received enzyme replacement therapy with alglucerase/imiglucerase for a median of 13 years prior to the first examination. MRI results were retrospectively stratified based on treatment initiation into 2 groups: "early" (age ≤12 years, median 5 years) and "late" (during adulthood, median 32 years). We evaluated occurrence of irreversi…
Les sociétés secrètes sous la Monarchie constitutionnelle
2004
Les gauches, l'antifascisme et le pacifisme dans les années 1930
2004
Alain Damasio, Les furtifs
2019
Clamart, La Volte, 2019, 704 p.; International audience