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Gene Review

HIES  -  Hyper-IgE syndrome

Homo sapiens

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Disease relevance of HIES

  • The hyper-IgE syndrome (HIES) is a rare primary immunodeficiency characterized by recurrent skin abscesses, pneumonia, and highly elevated levels of serum IgE [1].
  • Therapy for HIES is directed at prevention and management of infections by using sustained systemic antibiotics and antifungals along with topical therapy for eczema and drainage of abscesses [2].
  • In contrast, those with AR-HIES have severe molluscum contagiosum and other viral infections and may develop severe neurological complications [2].
  • CONCLUSION: IVGG is effective in the treatment of severe eczema in patients with HIES and atopic dermatitis [3].
  • METHODS: I initially treated two patients with Kawasaki disease, both of whom also had HIES, with IVGG [3].

Psychiatry related information on HIES


High impact information on HIES


Chemical compound and disease context of HIES


Biological context of HIES

  • By microarray analysis, 38 genes showed over 2-fold differences between the HIES patients and healthy controls in purified CD14+ cells, although only small differences in the gene expression profiles were observed between the two groups in purified CD4+ T cells [8].
  • CONCLUSIONS: These data revealed a severe dysbalance towards a Th2 phenotype in HIES patients which is likely to contribute to the specific pattern of infection susceptibility characteristic to HIES [9].
  • A significantly larger number of lysosome-related genes were up-regulated, and significantly larger number of genes related to cell growth and maintenance were down-regulated in HIES [8].
  • Proximal chromosome 4q therefore is a candidate region for disease genes for both HIES and autism [4].
  • Our results showed a marked PMN chemotaxis defect (p less than 0.01) in the patients with HIES, while in the subjects with AD PMN cellular chemotaxis was normal and there was no correlation between the results of chemotaxis and serum IgE levels [10].

Anatomical context of HIES

  • The reduced TGFbeta expression may involve the indigenous T-cell defects of HIES [11].
  • She had been diagnosed with HIES at the age of 6 when the papillomatous lesions first occurred in oral cavity [12].
  • The HIES is a multisystem disorder that affects especially cutaneous, respiratory, skeletal and the immune system [13].
  • Peyer's patches (PP) in germ-free rats (GF) and in the hyper-IgE syndrome patient (HIES) differ from their conventional rat (C) and healthy human (HH) counterparts in that GF rats contained fewer (two-fold) PP and none was detected in HIES [14].

Associations of HIES with chemical compounds


Regulatory relationships of HIES

  • In contrast, the mean proportion of IL-13 expressing CD4+ cells was increased significantly in patients with HIES in both the resting and the activated state compared to healthy controls [16].

Other interactions of HIES

  • Our studies do not support a role for a defective TLR response in HIES individuals [17].
  • These results suggested that the in vivo activated T cells of HIES did not sufficiently express the IFNgamma and TGFbeta genes, which could affect IL-4-dependent IgE production [11].
  • We conclude that increased expression of IL-13 in CD4+ cells from patients with HIES could account, at least partly, for raised IgE levels in those individuals [16].
  • In summary, although HIES individuals had an IFN-gamma secretion defect, they also produced normal levels of several TLR-regulated proinflammatory cytokines [17].
  • RESULTS: Interferon (IFN)gamma production, in addition to IFNgamma/IL-10 ratios, was 10-30-fold lower in the HIES patients compared with the healthy volunteers [9].

Analytical, diagnostic and therapeutic context of HIES


  1. Genetic linkage of hyper-IgE syndrome to chromosome 4. Grimbacher, B., Schäffer, A.A., Holland, S.M., Davis, J., Gallin, J.I., Malech, H.L., Atkinson, T.P., Belohradsky, B.H., Buckley, R.H., Cossu, F., Español, T., Garty, B.Z., Matamoros, N., Myers, L.A., Nelson, R.P., Ochs, H.D., Renner, E.D., Wellinghausen, N., Puck, J.M. Am. J. Hum. Genet. (1999) [Pubmed]
  2. Hyper-IgE syndromes. Grimbacher, B., Holland, S.M., Puck, J.M. Immunol. Rev. (2005) [Pubmed]
  3. High-dose intravenous gamma-globulin treatment for hyperimmunoglobulinemia E syndrome. Kimata, H. J. Allergy Clin. Immunol. (1995) [Pubmed]
  4. Analphoid marker chromosome in a patient with hyper-IgE syndrome, autism, and mild mental retardation. Grimbacher, B., Dutra, A.S., Holland, S.M., Fischer, R.E., Pao, M., Gallin, J.I., Puck, J.M. Genet. Med. (1999) [Pubmed]
  5. Hyper-IgE syndrome and H2-receptor blockade. Thompson, R.A., Kumararatne, D.S. Lancet (1989) [Pubmed]
  6. Hyper-IgE syndrome associated with an IL-4-producing gamma/delta(+) T-cell clone. Simon, H.U., Seger, R. J. Allergy Clin. Immunol. (2007) [Pubmed]
  7. Oral chloramphenicol therapy for multiple liver abscesses in hyperimmunoglobulinemia E syndrome. Fanconi, S., Seger, R.A., Willi, U., Otto, R., Spiess, H., Kayser, F.H., Hitzig, W.H. Eur. J. Pediatr. (1984) [Pubmed]
  8. Distinct gene expression patterns of peripheral blood cells in hyper-IgE syndrome. Tanaka, T., Takada, H., Nomura, A., Ohga, S., Shibata, R., Hara, T. Clin. Exp. Immunol. (2005) [Pubmed]
  9. Severely impaired IL-12/IL-18/IFNgamma axis in patients with hyper IgE syndrome. Netea, M.G., Kullberg, B.J., van der Meer, J.W. Eur. J. Clin. Invest. (2005) [Pubmed]
  10. IgE levels and PMN chemotaxis in atopic dermatitis. Galli, E., Rossi, P., Fiore, L., Perlini, R., Celiboni, F., Betti, P., Businco, L. Allergologia et immunopathologia. (1983) [Pubmed]
  11. Cytokine imbalance in hyper-IgE syndrome: reduced expression of transforming growth factor beta and interferon gamma genes in circulating activated T cells. Ohga, S., Nomura, A., Ihara, K., Takahata, Y., Suga, N., Akeda, H., Shibata, R., Okamura, J., Kinukawa, N., Hara, T. Br. J. Haematol. (2003) [Pubmed]
  12. OC11 Hyperimmunoglobulin E syndrome presenting with oral premalignant papillomas. Tanyeri, H., Kucukay, S., Dursun, G., Kurklu, E., Aslan, B., Gulluoglu, M., Beka, H., Agacfidan, A. Oral diseases. (2006) [Pubmed]
  13. The clinical and laboratory survey of Iranian patients with Hyper-IgE syndrome. Moin, M., Farhoudi, A., Movahedi, M., Rezaei, N., Pourpak, Z., Yeganeh, M., Gharagozlou, M., Mirsaeid Ghazi, B., Arshi, S., Mansouri, D., Sherkat, R., Kashef, S., Mahmoudi, M., Nabavi, M., Aghamohammadi, A. Scand. J. Infect. Dis. (2006) [Pubmed]
  14. Control of IgE responses. Durkin, H.G., Auci, D.L., Chice, S.M., Smith, M.C., Murali, M.R., Bazin, H., Tarcsay, L., Dukor, P. Clin. Immunol. Immunopathol. (1989) [Pubmed]
  15. Hyper IgE syndrome: four case report. Pigatto, P.D., Polenghi, M.M., Altomare, G.F., Brugo, A.M., Tadini, G.L., Morelli, M. Bollettino dell'Istituto sieroterapico milanese. (1984) [Pubmed]
  16. Increased expression of interleukin-13 but not interleukin-4 in CD4+ cells from patients with the hyper-IgE syndrome. Gudmundsson, K.O., Sigurjonsson, O.E., Gudmundsson, S., Goldblatt, D., Weemaes, C.M., Haraldsson, A. Clin. Exp. Immunol. (2002) [Pubmed]
  17. Hyper-IgE syndrome is not associated with defects in several candidate toll-like receptor pathway genes. Hawn, T.R., Ozinsky, A., Williams, L.M., Rodrigues, S., Clark, A., Pham, U., Hill, H.R., Ochs, H., Aderem, A., Liles, W.C. Hum. Immunol. (2005) [Pubmed]
  18. Keratoconus associated with hyperimmunoglobulin E syndrome. Kim, J., Netto, M.V. Cornea (2004) [Pubmed]
  19. Standardising surveillance of nosocomial infections: the HISS program. Hospital Infection Standardised Surveillance. McLaws, M.L., Murphy, C., Whitby, M. Journal of quality in clinical practice. (2000) [Pubmed]
  20. Clinical aspects of allergic bronchopulmonary aspergillosis. Greenberger, P.A. Front. Biosci. (2003) [Pubmed]
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