نتایج جستجو برای: pudlak syndrome

تعداد نتایج: 621943  

2017
M. Anthony Sofia Atsushi Sakuraba David T. Rubin

Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessive disorder characterized by oculocutaneous albinism and a lack of dense granules in platelets. HPS types 1 and 4 are associated with a granulomatous enterocolitis that is phenotypically indistinguishable from Crohn's disease. We present two cases of HPS-associated Crohn's disease phenotype in which the patients were refractory to stand...

Journal: :Journal of the Japan Society of the Reticuloendothelial System 1990

2001
Boris Lvov Hasan Al-Nashash

A 3-D model for the His Purkinje System (HPS) of the heart is used to solve the direct problem and simulate the HPS surface electrogram. The same model is also used to solve the inverse problem. Results from normal and pathological models are described.

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