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 hyper igM syndrome  

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is there an Ig A deficiency and susceptibility to sinopulmonary and GIT infections, esp giardia lamblia in x-linked hyperIgM syndrome?


X-linked immunodeficiency with hyper–immunoglobulin M is a rare form of primary immunodeficiency disease caused by mutations in the gene that codes for CD40 ligand. CD40 ligand is expressed on activated T lymphocytes and is necessary for T cells to induce B cells to undergo immunoglobulin (Ig) class-switching from immunoglobulin M (IgM) to immunoglobulin G (IgG), immunoglobulin A (IgA), and immunoglobulin E (IgE). Thus, patients with XHIGM have markedly reduced levels of IgG, IgA, and IgE but have normal or elevated levels of IgM.
  • Among all infections, pneumonia is the most common, occurring in more than 80% of patients. Other infections frequently observed in patients with XHIGM include sinusitis (43%), otitis (43%), recurrent and/or protracted diarrhea (34%), CNS infections (14%), sepsis (13%), hepatitis (9%), and sclerosing cholangitis (6%). Other, less common, infections include cellulites, subcutaneous abscesses, herpes stomatitis, oral candidiasis, parvovirus B19 infection, molluscum contagiosum, warts, and Candida esophagitis.
  • Microbial pathogens that cause pneumonia include P carinii (59%), cytomegalovirus (CMV) (3%), adenovirus (2%), Pseudomonas species (3%), herpesvirus type 1 (2%), respiratory syncytial virus (2%), histoplasmosis (2%), Pneumococcus species (2%), Staphylococcus species (2%), Haemophilus influenzae type b (2%), and other unknown pathogens (27%). Infections with Mycobacterium bovis or atypical Mycobacterium species have been reported.
  • Pathogens that cause diarrhea include Cryptosporidium species (21%), Giardia lamblia (8%), rotavirus (8%), Clostridium difficile (4%), Yersinia enterocolitica (4%), and other unknown pathogens (63%).
  • Causes of CNS infection include echovirus (27%), Cryptococcus species (9%), Pneumococcus species (9%), and other unknown causes (55%).
  • According to the US XHIGM Registry, hepatitis occurred in a significant number of patients (7 of 79 patients); causative agents included hepatitis C virus, echovirus, histoplasmosis, and Bartonella species.
  • Cryptosporidium infection was the etiology of sclerosing cholangitis in 80% of patients.


thank you, dear.
i had a q - immunity against giardia, it is IgA, and susceptible individuals are those who have igA deficiency.
And they mentioned two syndromes - Bruton's agammaglobulinemia and common variable im-deficiency.
i was thinking that selective igA def and x-linked hyper igM should be added to the list

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