نتایج جستجو برای: lipoid proteinosis

تعداد نتایج: 15341  

2017
Fabiano Reis Eduardo Macedo Marcondes Cavalcanti França Junior Eliane Ingrid Amstalden Simone Appenzeller

1. Universidade Federal de Alagoas (UFAL), Maceió, AL, Brazil. 2. Clínica de Medicina Nuclear e Radiologia de Maceió (MedRadius), Maceió, AL, Brazil. Mailing address: Dra. Christiana Maia Nobre Rocha de Miranda. Clínica de Medicina Nuclear e Radiologia de Maceió (MedRadius). Rua Hugo Corrêa Paes, 104, Farol. Maceió, AL, Brazil, 57050-730. E-mail: maia.christiana@ gmail.com. interlobular and int...

2014
Rufino Mondejar Jose Manuel Garcia-Moreno Rocio Rubio Francisca Solano Mercedes Delgado Begona Garcia-Bravo Juan Jose Rios-Martin Amalia Martinez-Mir Miguel Lucas

BACKGROUND Lipoid proteinosis (LP) is a rare autosomal recessive disorder characterized by a hoarse voice, variable scarring, and infiltration of the skin and mucosa. This disease is associated with mutations of the gene encoding extracellular matrix protein 1 (ECM1). CASE REPORT This was a clinical and molecular study of a new case of LP with a severe phenotype. A 35-year-old female born to ...

Journal: :Biological psychiatry 2012
Benjamin Becker Yoan Mihov Dirk Scheele Keith M Kendrick Justin S Feinstein Andreas Matusch Merve Aydin Harald Reich Horst Urbach Ana-Maria Oros-Peusquens Nadim J Shah Wolfram S Kunz Thomas E Schlaepfer Karl Zilles Wolfgang Maier René Hurlemann

BACKGROUND The human amygdala plays a crucial role in processing social signals, such as face expressions, particularly fearful ones, and facilitates responses to them in face-sensitive cortical regions. This contributes to social competence and individual amygdala size correlates with that of social networks. While rare patients with focal bilateral amygdala lesion typically show impaired reco...

2013
Noritaka Oyama Joseph Merregaert

Extracellular matrix protein 1 (ECM1) is a secreted glycoprotein that plays a pivotal role in the structural and homeostatic biology of the skin, particularly in angiogenesis, reconstitution of basement membrane, proliferation and differentiation of epidermal keratinocytes and dermal fibroblasts, and malignant transformation. This rationale is substantiated by loss-of-function mutations in the ...

پایان نامه :وزارت علوم، تحقیقات و فناوری - دانشگاه رازی - دانشکده علوم 1390

هدف: lipoid proteinosis یک بیماری نادر اتوزومی مغلوب است که از جهش در پروتئین ماتریکس خارج سلولی1 (ecm1) بوجود می آید. ژن ecm1 ، 10 اگزون دارد و در مطالعاتی که تا به امروز صورت گرفته، ثابت شده میزان جهش در 3 اگزون این ژن (6، 7 و8) از سایر اگزون های آن بیشتر بوده است. مطالعات قبلی در مورد این بیماری، بررسی جهش های 3 اگزون 6، 7 و 8 بوده است. مطالعه فعلی ما در مورد 7 اگزون (1، 2، 3، 4، 5، 9 و 10...

2017
David Chardin Guillaume Nivaggioni Philippe Viau Caherine Butori Bernard Padovani Caroline Grangeaon Micheline Razzouk-Cadet

RATIONALE Exogenous lipoid pneumonia is a rare condition due to abnormal presence of oily substances in the lungs. It is a rarely known cause for false positive FDG PET-CT results and can sometimes lead to invasive investigations. Searching and finding the source of the oily substance is one of the keys to the diagnosis. Inhalation of oily drugs during snorting has rarely been described. PATI...

Journal: :Archivos de bronconeumologia 2009
José Antonio Rodríguez Portal Eulogio Rodríguez Becerra Antonio Sánchez Garrido

Pulmonary alveolar proteinosis is a rare disease characterized by the accumulation of lipoproteinaceous material derived from alveolar surfactant in the alveoli, with a consequent deterioration in gas exchange. Pathogenesis is related to impaired phagocytic function of alveolar macrophages. In recent years, a new treatment for pulmonary alveolar proteinosis-consisting of subcutaneous administra...

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