Hyper-IgM syndrome with early liver involvement].

Hyper-IgM syndrome is an innate error of immunity in which there is a defect in change of isotype of immunoglobulins, with decreased values of IgG, IgA, and IgE, but normal or increased level of IgM. This predisposes to infectious processes at the respiratory and gastrointestinal levels, as well as autoimmune diseases and neoplasm. A 5 year 7-month-old boy with a history of 2 pneumonias, one of them severe, and chronic diarrhea since he was 2 years old. Persistent moderate neutropenia decreased IgG and elevated IgM. Cytometry flow confirmed absence of CD40L. Clinical evolution with early hepatic involvement. Hyper-IgM syndrome predisposes to liver damage, so a complete evaluation is required as well as early diagnosis. Active anti-infective treatment and control of the inflammatory response are key to the treatment of liver damage.

Citation

Kareli Guadalupe Coronado-Hernández, Héctor Hugo Campos-Téllez, Rosa María Cortés-Grimaldo, Ana Paola Macías-Robles, Carlos David Estrada-García, Britza Barrios-Díaz, Adriana Ramírez Nepomuceno, Marlén Barreto-Alcalá, David Esparza-Amaya, Hilda Lilian Carvajal-Alonso, Laura Berrón-Ruiz. Hyper-IgM syndrome with early liver involvement]. Revista alergia Mexico (Tecamachalco, Puebla, Mexico : 1993). 2023 Apr 19;69(4):214-219

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PMID: 37218048

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