Recurrent Meningitis in Hyper IgE Syndrome

Kariyappa, Mallesh; Kommalur, Anitha; Jayaram, Shantala; Javarappa, Prakash; Ambazhagan, Arunkumar

doi:10.1007/s12098-014-1613-2

Cited by 2 publications

(3 citation statements)

References 4 publications

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“…One of our patients (case 2) had two episodes of bacterial meningitis. Although meningitis has been reported in AD HIGE [ 7 , 19 ] recurrent episodes are very rare, with one patient previously reported from India [ 20 ]. However, mutation analysis was not performed in that patient.…”

Section: Discussionmentioning

confidence: 99%

Hyper IgE recurrent infection syndrome in South Asia: is there a different outcome?

Silva

Dasanayake

Senanayake

et al. 2018

Allergy Asthma Clin Immunol

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BackgroundHyper IgE recurrent infection syndrome (HIES) is a rare immune deficiency, characterized by recurrent staphylococcal skin and lung abscesses, pneumonia and increased IgE levels. The majority of autosomal dominant HIES (AD HIGE) is due to hypomorphic mutations in the signal transducer and the activator of transcription 3 (STAT3) gene.Case presentationFive patients were diagnosed with HIES, based on the clinical criteria and scoring system developed at the National Institutes of Health (NIH), USA. The STAT3 gene was sequenced and previously described mutations were identified in all five patients. We compare the clinical features of our patients with those from Asia, Europe and the US.ConclusionEven though the number of patients is limited, there are some clinical differences in patients from South Asia compared to European and even East Asian patients. However, the mutations detected are located at hot spots seen in western and Asian patients with AD HIGE.

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Section: Discussionmentioning

confidence: 99%

Hyper IgE recurrent infection syndrome in South Asia: is there a different outcome?

Silva

Dasanayake

Senanayake

et al. 2018

Allergy Asthma Clin Immunol

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“…Apart from Enteroviruses, E.coli can cause meningitis in MHC class II [ 31 ]. Recurrent Staphylococcal meningitis may be an indicator of Hyper IgE syndrome [ 101 ]. Anticapsular antibody deficiency is encountered in conditions like Wiskott Aldrich syndrome and Di George syndrome leading to increased risk of meningitis by capsulated bacteria [ 102 , 103 ].…”

Section: Spectrum Of Infectionsmentioning

confidence: 99%

“…Recurrent oral thrush is also a troublesome symptom. [ 101 ]. In a series of 25 children diagnosed with CHARGE syndrome, 32% died during infancy two of whom died to complications following infection (rhinovirus pneumonia and overwhelming sepsis) [ 161 ].…”

Section: Thymic Defects With Additional Congenital Anomaliesmentioning

confidence: 99%

Infections in Inborn Errors of Immunity with Combined Immune Deficiency: A Review

George

Govindaraj

2023

Pathogens

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Enhanced susceptibility to microbes, often resulting in severe, intractable and frequent infections due to usually innocuous organisms at uncommon sites, is the most striking feature in individuals with an inborn error of immunity. In this narrative review, based on the International Union of Immunological Societies’ 2022 (IUIS 2022) Update on phenotypic classification of human inborn errors of immunity, the focus is on commonly encountered Combined Immunodeficiency Disorders (CIDs) with susceptibility to infections. Combined immune deficiency disorders are usually commensurate with survival beyond infancy unlike Severe Combined Immune Deficiency (SCID) and are often associated with clinical features of a syndromic nature. Defective humoral and cellular immune responses result in susceptibility to a broad range of microbial infections. Although disease onset is usually in early childhood, mild defects may present in late childhood or even in adulthood. A precise diagnosis is imperative not only for determining management strategies, but also for providing accurate genetic counseling, including prenatal diagnosis, and also in deciding empiric treatment of infections upfront before investigation reports are available.

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Recurrent Meningitis in Hyper IgE Syndrome

Cited by 2 publications

References 4 publications

Hyper IgE recurrent infection syndrome in South Asia: is there a different outcome?

Hyper IgE recurrent infection syndrome in South Asia: is there a different outcome?

Infections in Inborn Errors of Immunity with Combined Immune Deficiency: A Review

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