HYPERIMMUNOGLOBULIN E SYNDROME: GENETICS, IMMUNOPATHOGENESIS, CLINICAL FINDINGS, AND TREATMENT MODALITIES

Q: How can I download an article?

To download an article from SID, first log in to the site, search for the article title, and click on the 'Download Article' option.

Q: How can I download an ISI article?

To download an ISI article on SID, enter the keyword or article title in the search bar, view the relevant results, click on the desired article, and select the 'Download Article' option.

Q: How can I access the SID database?

To access the SID database, visit SID.ir, create an account, and log in to access scientific resources.

Q: Is downloading articles from SID free?

Some articles on SID are available for free, while others require payment. Details are specified on the article's page.

HASHEMI HASAN; MOHEBBI MASOUMEH; MEHRAVARAN SHIVA; MAZLOUMI MEHDI; JAHANBANI ARDAKANI HAMIDREZA; ABTAHI SEYED HOSSEIN

مرکز اطلاعات علمی Scientific Information Database (SID) - Trusted Source for Research and Academic Resources

Journal Paper

Paper Information

Journal: JOURNAL OF RESEARCH IN MEDICAL SCIENCES (JRMS) Year:2017 | Volume:22 | Issue:4 Page(s): 0-0

Download Full-Text

Persian Verion

View:

645

Download:

247

Cites:

Information Journal Paper

Title

HYPERIMMUNOGLOBULIN E SYNDROME: GENETICS, IMMUNOPATHOGENESIS, CLINICAL FINDINGS, AND TREATMENT MODALITIES

Author(s)

Keywords

AUTOIMMUNE DISEASEQ4

EYEQ4

HYPERIMMUNOGLOBULIN E SYNDROMEQ4

IMMUNODEFICIENCYQ4

OCULARQ4

OMALIZUMABQ4

STAPHYLOCOCCUS AUREUSQ4

Abstract

The HYPERIMMUNOGLOBULIN E SYNDROMEs (HIESs) are very rare IMMUNODEFICIENCY syndromes with multisystem involvement, including immune system, skeleton, connective tissue, and dentition. HIES are characterized by the classic triad of high serum levels of immunoglobulin E (IgE), recurrent staphylococcal cold skin abscess, and recurrent pneumonia with pneumatocele formation.Most cases of HIES are sporadic although can be inherited as autosomal dominant and autosomal recessive traits. A fundamental immunologic defect in HIES is not clearly elucidated but abnormal neutrophil chemotaxis due to decreased production or secretion of interferon γ has main role in the immunopathogenesis of syndrome, also distorted Th1/Th2 cytokine profile toward a Th2 bias contributes to the impaired cellular immunity and a specific pattern of infection susceptibility as well as atopic‑allergic constitution of syndrome. The ophthalmic manifestations of this disorder include conjunctivitis, keratitis, spontaneous corneal perforation, recurrent giant chalazia, extensive xanthelasma, tumors of the EYElid, strabismus, and bilateral keratoconus. The diagnosis of HIES is inconclusive, dependent on the evolution of a constellation of complex multisystemic symptoms and signs which develop over the years. Until time, no treatment modality is curative for basic defect in HIES, in terms of cytokines/chemokines derangement. Of note, bone marrow transplant and a monoclonal anti‑IgE (omalizumab) are hoped to be successful treatment in future.

Cites

عملکرد فارغ التحصیلان رشته های هوشبری و اتاق عمل دانشگاه علوم پزشکی لرستان

References

No record.

Cite

APA: Copy

HASHEMI, HASAN, MOHEBBI, MASOUMEH, MEHRAVARAN, SHIVA, MAZLOUMI, MEHDI, JAHANBANI ARDAKANI, HAMIDREZA, & ABTAHI, SEYED HOSSEIN. (2017). HYPERIMMUNOGLOBULIN E SYNDROME: GENETICS, IMMUNOPATHOGENESIS, CLINICAL FINDINGS, AND TREATMENT MODALITIES. JOURNAL OF RESEARCH IN MEDICAL SCIENCES (JRMS), 22(4), 0-0. SID. https://sid.ir/paper/26693/en

Vancouver: Copy

HASHEMI HASAN, MOHEBBI MASOUMEH, MEHRAVARAN SHIVA, MAZLOUMI MEHDI, JAHANBANI ARDAKANI HAMIDREZA, ABTAHI SEYED HOSSEIN. HYPERIMMUNOGLOBULIN E SYNDROME: GENETICS, IMMUNOPATHOGENESIS, CLINICAL FINDINGS, AND TREATMENT MODALITIES. JOURNAL OF RESEARCH IN MEDICAL SCIENCES (JRMS)[Internet]. 2017;22(4):0-0. Available from: https://sid.ir/paper/26693/en

IEEE: Copy

HASAN HASHEMI, MASOUMEH MOHEBBI, SHIVA MEHRAVARAN, MEHDI MAZLOUMI, HAMIDREZA JAHANBANI ARDAKANI, and SEYED HOSSEIN ABTAHI, “HYPERIMMUNOGLOBULIN E SYNDROME: GENETICS, IMMUNOPATHOGENESIS, CLINICAL FINDINGS, AND TREATMENT MODALITIES,” JOURNAL OF RESEARCH IN MEDICAL SCIENCES (JRMS), vol. 22, no. 4, pp. 0–0, 2017, [Online]. Available: https://sid.ir/paper/26693/en

Related Journal Papers