The hyperimmunoglobulinaemia E and recurrent infections syndrome in an adult

L'Huillier, J P; Thoreux, P H; Delaval, P; Desrues, B; Le Gall, E; Kernec, J; Delambre, J F
September 1990
Thorax;Sep1990, Vol. 45 Issue 9, p707
Academic Journal
No abstract available.


Related Articles

  • Tendonitis in variant hyperimmunoglobulinaemia D and periodic fever syndrome--a rare disease with a new symptom. Armbrust, Sven; Drenth, Joost P. H.; Schröder, Carmen; Domning, Ester; Poeschl, Ernestine; Wiersbitzky, Siegfried K. W.; Schröder, Carmen // European Journal of Pediatrics;Jun2005, Vol. 164 Issue 6, p391 

    Unlabelled: Hyperimmunoglobulinaemia D syndrome (HIDS) is defined as recurrent fever, generalised lymphadenitis, abdominal pain, arthritis and raised polyclonal serum IgD >100 IU/ml. The cause is a mutation in the mevalonate kinase gene. Other periodic fever syndromes are known. We...

  • Increased urinary leukotriene E4 during febrile attacks in the hyperimmunoglobulinaemia D and periodic fever syndrome. Frerikel, J.; Willemsen, M. A. A. P.; Weemaes, C. M. R.; Dorland, L.; Mayatepek, E. // Archives of Disease in Childhood;Aug2001, Vol. 85 Issue 2, p158 

    Background-Thehyperimmunoglobulinaemia D and periodic fever syndrome is a hereditary periodic fever, caused by deficiency of the enzyme mevalonate kinase. It is unclear how this defect leads to recurrent fever episodes. Aim-To assess the involvement of cysteinyl leukotrienes in the pathogenesis...

  • Arthritis as a presenting symptom in a hypogammaglobulinemic patient with thymectomy. Ardeniz, Ömür; Vatansever, Sezgin; Musabak, Uğur; Aksu, Kenan; Sin, Aytül; Kokuludağ, Ali // Rheumatology International;Jan2007, Vol. 27 Issue 3, p263 

    The article discusses implications of common variable immunodeficiency (CVID) and Good's syndrome. It has been noticed that CVID is the most frequent primary immunodeficiency syndrome in adults. It symptoms is presented by recurrent sinopulmonary infections with longer duration. Both CVID and...

  • Hyper IgE syndrome: Report of two cases with moderate elevation of IgE. Muhammed, K. // Indian Journal of Dermatology, Venereology & Leprology;Mar/Apr2005, Vol. 71 Issue 2, p112 

    Hyper IgE syndrome with recurrent infection (Job's syndrome) is a rare idiopathic primary immunodeficiency disease characterized by the triad of elevated serum IgE (>2000 lU/mI), recurrent cutaneous abscesses and recurrent sinopulmonary infections. The bacteria which commonly infect these...

  • Toxic Shock Syndrome: The Emerging Picture. Shands, Kathryn N.; Dan, Bruce B.; Schmid, George P. // Annals of Internal Medicine;Feb81, Vol. 94 Issue 2, p264 

    Editorial. Discusses toxic shock syndrome. Details on the early reports on the syndrome; Information on the recurrences of the syndrome in women; Infections associated with the syndrome.

  • Localized cutaneous acremoniasis in a patient with hyperimmunoglobulin E syndrome. Tambe, S. A.; Jerajani, H. R.; Ghate, S. S.; Lambay, S. A. // European Journal of Pediatric Dermatolgy;Apr2011, Vol. 21 Issue 2, p71 

    Hyperimmunoglobulin E syndrome is a primary immunodeficiency characterized by elevated levels of IgE, recurrent infections and skin, skeletal and dental alterations linked to a deficiency of T lymphocytes. The Acremonium is a saprophytic fungus responsible for serious infections in...

  • Diagnostic value of serum immunoglobulinaemia D level in patients with a clinical suspicion of hyper IgD syndrome. Ammouri, W.; Cuisset, L.; Rouaghe, S.; Rolland, M.-O.; Delpech, M.; Grateau, G.; Ravet, N. // Rheumatology;Oct2007, Vol. 46 Issue 10, p1597 

    Objective. The hyperimmunoglobulinaemia D and periodic fever syndrome (HIDS) was originally defined by the presence of a high serum level of immunoglobulin D associated with recurrent fever. Since the discovery of the mevalonate kinase gene (MVK) gene encoding the mevalonate kinase enzyme, most...

  • PID in Disguise: Molecular Diagnosis of IRAK-4 Deficiency in an Adult Previously Misdiagnosed With Autosomal Dominant Hyper IgE Syndrome. Frans, Glynis; Moens, Leen; Schrijvers, Rik; Wuyts, Greet; Bouckaert, Bernard; Schaballie, Heidi; Dupont, Lieven; Bossuyt, Xavier; Corveleyn, Anniek; Meyts, Isabelle // Journal of Clinical Immunology;Nov2015, Vol. 35 Issue 8, p739 

    Autosomal recessive IL-1R-associated kinase 4 (IRAK-4) deficiency is a rare cause of recurrent pyogenic infections with limited inflammatory responses. We describe an adult female patient with severe lung disease who was phenotypically diagnosed as suffering from autosomal dominant Hyper IgE...

  • Autoinflammatory diseases: an update of clinical and genetic aspects. Q. Yao; D. E. Furst // Rheumatology;Jul2008, Vol. 47 Issue 7, p946 

    To review clinical manifestations and genetic features of the autoinflammatory diseases, a group of rare, genetically defined diseases which have been newly grouped into a coherent whole. We performed a literature review using the keywords ‘periodic fever syndrome’,...

  • Recurrent Infections and Cows-milk Hypersensitivity in a 2-Year-Old Girl with Hyper Immunoglobulin E Syndrome. Prcic, Sonja; Tomić, Jelena; Petrović, Slobodanka; Radulović, Anica; Djuran, Verica; Gajinov, Zorica // Iranian Journal of Allergy, Asthma & Immunology;2011, Vol. 10 Issue 4, p299 

    The hyperimmunoglobulin E syndrome is a rare complex primary immunodeficiency characterized by high serum IgE levels, eczema, and recurrent infections. We present a case of 2-years-old girl with eczema and repeated bacterial skin and lung infections since the period of infancy. The patient also...


Read the Article


Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics