نتایج جستجو برای: hyperimmunoglobulin e syndrome

تعداد نتایج: 1604051  

Journal: :Japanese journal of infectious diseases 2011
Ayhan Kubar Mustafa Haciomeroglu Aykut Ozkul Umit Bagriacik Esragul Akinci Kenan Sener Hurrem Bodur

Crimean-Congo hemorrhagic fever virus (CCHFV), a member of the genus Nairovirus of the family Bunyaviridae, causes a severe disease in humans with high mortality rates. In Turkey, the number of patients with CCHF has increased since 2002. Here, we aimed to treat CCHF patients with CCHFV hyperimmunoglobulin. We prepared a CCHFV hyperimmunoglobulin product from 22 individuals who survived CCHF in...

Journal: :JAAD case reports 2015
Toshiki Ito Fumihide Iwata

E osinophilia, defined as greater than 450 eosinophils/ L, can occur in various disease processes, including infectious, allergic, neoplastic, and primary hematologic disorders. Marked eosinophilia ([1500 eosinophils/ L) can be a diagnostic dilemma because blood eosinophilia rarely exceeds 1500/ L in atopic/allergic diseases. Hyperimmunoglobulinemia E is seen in allergic, parasitic, and primary...

Journal: :Blood 2014
Scott M Steward-Tharp Arian Laurence Yuka Kanno Alex Kotlyar Alejandro V Villarino Giuseppe Sciume Stefan Kuchen Wolfgang Resch Elizabeth A Wohlfert Kan Jiang Kiyoshi Hirahara Golnaz Vahedi Hong-Wei Sun Lionel Feigenbaum Joshua D Milner Steven M Holland Rafael Casellas Fiona Powrie John J O'Shea

Mutations of STAT3 underlie the autosomal dominant form of hyperimmunoglobulin E syndrome (HIES). STAT3 has critical roles in immune cells and thus, hematopoietic stem cell transplantation (HSCT), might be a reasonable therapeutic strategy in this disease. However, STAT3 also has critical functions in nonhematopoietic cells and dissecting the protean roles of STAT3 is limited by the lethality a...

Journal: :Journal of investigational allergology & clinical immunology 2013
H Zeng Y Tao X Chen P Zeng B Wang R Wei C Yao Y Xie F Li Y Tang Y Cui G Sun

OBJECTIVES We analyzed the clinical features of 138 patients with primary immunodeficiency (PID) and performed genetic testing on a subset of patients in order to complete gaps in research on PID in South China and thus improve pediatricians' ability to recognize and diagnose PID. METHODS We performed a retrospective analysis based on the medical records of PID patients hospitalized in our in...

2011
J Santos JI Arostégui MJ Brito C Neves M Conde

Background Hyperimmunoglobulin D Syndrome (HIDS is a rare autosomal recessive condition caused by mutations in the Mevalonate Kinase (MVK) gene that codes for MVK, an essential enzyme in the isoprenoid pathway. It shows a wide phenotypic spectrum probably related with the residual activity of this enzyme (1–20% of normal). So far, about 63 mutations have been described and the four most prevale...

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