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A collection of terms from Judaism
Ovu knjigu pripremio je i uredio dugogodišnji vrhovni rabin Jugoslavije (službovao od 1972 do 1998.) Cadik Danon. Ona predstavlja zbir prikaza iz jevrejskog verskog, pisanog i običajnog nasleđa i tradicije. Čine je sledeće teme: jevrejski kalendar, jevrejski praznici, postovi, spomen-dani, teme iz Biblije, Mišne, atmosfera, vreme i delovanje proroka, osnovni pojmovi Talmuda, životni ciklus (berit mila, bar/bat micva, brak, žalobni običaji), osnovi Alefbeta (hebrejskog pisma), pojašnjenja osnovnih jevrejskih simbola i pojmova, pozdravljanja-čestitanja i odavanja poštovanja, i na kraju molitveni dodatak, odnosno osnovne jevrejske molitve.This book was prepared and edited by the longtime Supreme Rabbi of Yugoslavia (served from 1972 to 1998), Cadik Danon. It is a collection of reviews from the Jewish religious, written and common heritage and tradition. It consists of the following topics: Jewish calendar, Jewish holidays, fasts, memorial days, topics from the Bible, Mishnah, atmosphere, time and work of the prophets, basic concepts of the Talmud, life cycle (berit milah, bar/bat mitzvah, marriage, mourning customs), the basics of Alephbet (Hebrew scriptures), explanations of basic Jewish symbols and concepts, greetings-congratulations and giving honours, and finally a prayer supplement, i.e. basic Jewish prayers
Entretien avec Antoine Culioli (recueilli par Laurent Danon-Boileau et Mary-Annick Morel)
Danon-Boileau Laurent, Morel Mary-Annick. Entretien avec Antoine Culioli (recueilli par Laurent Danon-Boileau et Mary-Annick Morel). In: Faits de langues, n°4, Septembre 1994. L'indéfini. pp. 265-271
Entretien avec David Cohen (recueilli par Mary-Annick Morel et Laurent Danon-Boileau)
Morel Mary-Annick, Danon-Boileau Laurent. Entretien avec David Cohen (recueilli par Mary-Annick Morel et Laurent Danon-Boileau). In: Faits de langues, n°3, Mars 1994. La personne. pp. 113-119
Entretien avec Jean-Biaise Grize (recueilli par Laurent Danon-Boileau et Mary-Annick Morel)
Danon-Boileau Laurent, Morel Mary-Annick. Entretien avec Jean-Biaise Grize (recueilli par Laurent Danon-Boileau et Mary-Annick Morel). In: Faits de langues, n°4, Septembre 1994. L'indéfini. pp. 155-159
Entretien avec Jacqueline de la Fontinelle et Michel Aufray (recueilli par Laurent Danon-Boileau et Mary-Annick Morel)
Danon-Boileau Laurent, Morel Mary-Annick. Entretien avec Jacqueline de la Fontinelle et Michel Aufray (recueilli par Laurent Danon-Boileau et Mary-Annick Morel). In: Faits de langues, n°4, Septembre 1994. L'indéfini. pp. 259-264
A Case Study and Literature Review of the Diagnosis of Danon Disease in Patients Presenting Only with Severe Cardiac Symptoms
Yu-Qing Sun,1 Qiang Lv,1 Dong Chen,2 Yuwei Da,3 Xiao-Yan Zhao,4 Jian-Zeng Dong1 1Department of Cardiology, Beijing Anzhen Hospital, Capital Medical University, Beijing Institute of Heart Lung & Blood Vessel Diseases, Beijing, People’s Republic of China; 2Department of Pathology, Beijing Anzhen Hospital, Capital Medical University, Beijing Institute of Heart Lung & Blood Vessel Diseases, Beijing, People’s Republic of China; 3Department of Neurology, Beijing Xuanwu Hospital, Capital Medical University, Beijing, People’s Republic of China; 4Department of Cardiology, The First Affiliated Hospital, Zhengzhou University, Zhengzhou, Henan, People’s Republic of ChinaCorrespondence: Jian-Zeng Dong, Email [email protected]: The clinical manifestations of Danon disease, which result from the primary deficiency of the lysosome-associated membrane protein 2 gene, include cardiomyopathy, skeletal myopathy, and different degrees of intellectual disability that vary greatly among patients. The present study reports on two cases of Danon disease in two patients who only presented cardiac symptoms. Cardiac symptoms usually occur in adolescence and during a patient’s twenties, and most patients die from heart failure. However, the lab results from these cases suggested that other systems were involved, despite no other clinical symptoms. Significantly, the two patients had elevated serum cardiac troponin I, which often manifests in the acute cardiac phase, especially in severely affected patients with rapidly fatal outcomes. Danon disease is a multi-system involvement disease. Therefore, clinicians must be aware of its complexity when evaluating newly diagnosed patients due to its vastly different clinical course and prognosis and the importance of multidisciplinary management.Keywords: Danon disease, cardiomyopathy, serum cardiac troponin I, managemen
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Potential treatments for Danon disease
Danon disease is a congenital X-linked hypertrophic cardiomyopathy that results from mutations in LAMP2, a transmembrane lysosomal protein necessary for autophagy. The disease presents in childhood or adolescence, and life expectancy is 19 for men and 34 for women. Although Danon disease is rare, no specific therapy based on its biology is currently known. Recent in vitro studies using mouse by the Cherqui Lab have shown evidence supporting hematopoetic stem cell transplant as a novel treatment option for cystinosis. Cystinosis is a metabolic disease that arises from mutations in a transmembrane lysosomal protein - cystinosin (CTNS gene). Detailed in vitro and in vivo studies have shown that rescue of the phenotype occurs because wildtype macrophages derived from the transplanted wildtype hematopoietic stem cells can transfer lysosomes containing wildtype cystinosin directly to Ctns-deficient cells in multiple organs of the host Ctns -/- mice. Because Danon disease also arises from mutations in a transmembrane lysosomal protein, LAMP2, a similar approach could be effective in rescuing the phenotype of LAMP2 mutant mice. The Adler Lab has recently developed an in vitro cell model for Danon disease using skin fibroblasts expanded from skin biopsies from two identified patients diagnosed with Danon disease, confirmed by RT-PCR. These in vitro and in vivo models can be used show efficacy of hematopoietic stem cell transplant for Danon disease
Cardiac arrhythmias in patients with Danon disease
Different cardiac arrhythmias have been suggested to be associated with Danon disease, e.g. Wolff–Parkinson–White syndrome. However, a systematic electrophysiological investigation of patients with Danon disease is lacking thus far.Medicin
Avant-propos
Danon-Boileau Laurent. Avant-propos. In: Langages, 19ᵉ année, n°73, 1984. Les Plans d'Énonciation, sous la direction de Laurent Danon-Boileau. pp. 5-6
Avant-propos
Danon-Boileau Laurent. Avant-propos. In: Langages, 19ᵉ année, n°73, 1984. Les Plans d'Énonciation, sous la direction de Laurent Danon-Boileau. pp. 5-6
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