Journal of clinical immunology · 2014
Cartilage-hair hypoplasia: follow-up of immunodeficiency in two patients.
Клинический случайСтатьяНазвание и аннотация: Перевод в очереди
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Kainulainen Leena, Lassila Olli, Ruuskanen Olli
Аннотация
PURPOSE: To study the changes in the immunological status in 2 children with cartilage hair hypoplasia (CHH).
METHODS: A 4-6 year immunological follow-up from infancy.
RESULTS: In infancy the children presented a combined T cell and B cell immunodeficiency which partly resolved in time. Mitogen-induced T cell proliferation values fluctuated but lymphopenia has remained constant. Both patients had no recent thymic emigrants (TREC). Both children have suffered from a prolonged viral infection. Hypogammaglobulinemia normalized during the first years of life but both children have a specific antibody deficiency (SAD).
CONCLUSIONS: The changes in the immunological status in CHH patients emphasize the importance of a regular follow-up. SAD should be searched for in CHH. The absence of TRECs supports combined immunodeficiency and possible need of hematopoietic stem cell transplantation.
METHODS: A 4-6 year immunological follow-up from infancy.
RESULTS: In infancy the children presented a combined T cell and B cell immunodeficiency which partly resolved in time. Mitogen-induced T cell proliferation values fluctuated but lymphopenia has remained constant. Both patients had no recent thymic emigrants (TREC). Both children have suffered from a prolonged viral infection. Hypogammaglobulinemia normalized during the first years of life but both children have a specific antibody deficiency (SAD).
CONCLUSIONS: The changes in the immunological status in CHH patients emphasize the importance of a regular follow-up. SAD should be searched for in CHH. The absence of TRECs supports combined immunodeficiency and possible need of hematopoietic stem cell transplantation.