HYPER IgE SYNDROME WITH UMBILICAL HERNIA

Hyper IgE Syndrome (HIES) is a rare multi system genetic immunodeficiency disorder, with immunological and non-immunological features. Immunolgical features are 1) Recurrent cutaneous abscesses, 2) Atopic dermatitis like lesions, 3) Sino pulmonary infections, 4) Elevated serum IgE levels and 5) Abno...

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Detalles Bibliográficos
Autores principales: Hiremath, N C, Madan Mohan, N T, Srinivas, C, Sangolli, Prabhakar M, Srinivas, K, Soumya, N
Formato: Texto
Lenguaje:English
Publicado: Medknow Publications 2009
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2807719/
https://www.ncbi.nlm.nih.gov/pubmed/20101344
http://dx.doi.org/10.4103/0019-5154.57619
Descripción
Sumario:Hyper IgE Syndrome (HIES) is a rare multi system genetic immunodeficiency disorder, with immunological and non-immunological features. Immunolgical features are 1) Recurrent cutaneous abscesses, 2) Atopic dermatitis like lesions, 3) Sino pulmonary infections, 4) Elevated serum IgE levels and 5) Abnormal neutrophil chemotaxis. Non immunological features include cranio facial and skeletal abnormalities. We are reporting a girl with classical features of HIES with umbilical hernia with her younger brother suffering from right sided inguinal hernia, as both herniae are hitherto unreported in patients with HIES.

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