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Information Journal Paper

Title

First Case of X-linked Recessive Anhidrotic Ectodermal Dysplasia with Immunodeficiency from Iran with Unusual Aspergillus Infection

Pages

  57-63

Keywords

essential modulator essential modulator (NEMO) 

Abstract

 NEMO (NF-κ B essential modulator) is a regulatory factor involved in signaling pathways of innate and adaptive immune system. Hypomorphic mutation of IKBKG gene on X chromosome leads to X-linked recessive anhidrotic ectodermal dysplasia with immunodeficiency. The affected boys presented developmental phenotype with hypotrichosis, hypohidrosis; hypodontia with conical incisors; and susceptibility to pyogenic bacteria, mycobacteria, and viruses. Most of them have impaired antibody response to polysaccharide antigens. Here, we presented the case report of 7 years old Iranian boy with NEMO-deficiency and unusual Aspergillus infection.

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    APA: Copy

    MOMEN, TOOBA, ALBORZI, ABDOLVAHAB, ESLAMIAN, MOHAMMAD HOSSEIN, & CASANOVA, JEAN LAURENT. (2019). First Case of X-linked Recessive Anhidrotic Ectodermal Dysplasia with Immunodeficiency from Iran with Unusual Aspergillus Infection. IMMUNOLOGY AND GENETICS JOURNA, 2(4), 57-63. SID. https://sid.ir/paper/754014/en

    Vancouver: Copy

    MOMEN TOOBA, ALBORZI ABDOLVAHAB, ESLAMIAN MOHAMMAD HOSSEIN, CASANOVA JEAN LAURENT. First Case of X-linked Recessive Anhidrotic Ectodermal Dysplasia with Immunodeficiency from Iran with Unusual Aspergillus Infection. IMMUNOLOGY AND GENETICS JOURNA[Internet]. 2019;2(4):57-63. Available from: https://sid.ir/paper/754014/en

    IEEE: Copy

    TOOBA MOMEN, ABDOLVAHAB ALBORZI, MOHAMMAD HOSSEIN ESLAMIAN, and JEAN LAURENT CASANOVA, “First Case of X-linked Recessive Anhidrotic Ectodermal Dysplasia with Immunodeficiency from Iran with Unusual Aspergillus Infection,” IMMUNOLOGY AND GENETICS JOURNA, vol. 2, no. 4, pp. 57–63, 2019, [Online]. Available: https://sid.ir/paper/754014/en

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