--- a +++ b/data/text/es-S1139-76322016000300013-1.txt @@ -0,0 +1,8 @@ +Infant aged three and a half months, of Maghrebi origin, correctly vaccinated for his age, with no medical history of interest. Pregnancy controlled in Spain, gestational period passed without incident. +Family history: healthy, non-consanguineous parents; two sisters aged three and ten years, healthy, and five brothers who died before the age of one year, all of whom died of a complicated respiratory condition. +The infant had adequate weight gain and no infections since birth. +He was referred to his referral hospital because of a family history of male infants who had died prematurely. +A blood test was performed at our centre that showed decreased absolute and subclass immunoglobulin levels: IgG 153 mg/dl, IgA 23 mg/dl, IgM 17 mg/dl and IgE 10.8 mg/dl. Flow cytometry for the study of lymphocyte subpopulations showed a percentage and absolute decrease in B lymphocytes (1.5%, 109/µL), with a relative predominance of T lymphocytes, confirming the diagnosis of humoral immunodeficiency, suspected Bruton's disease, which was confirmed by genetic study. +Treatment was started with monthly intravenous gamma globulin (400 mg/kg) and complete vaccination (including pneumococcal), with satisfactory evolution. +She is currently six years old, undergoing replacement treatment with monthly gamma globulin and has not presented any complications or intercurrent processes, presenting a good staturoponder curve. +