3141 Congenital Agamaglobulinemia

Tuesday, 6 December 2011
Poster Hall (Cancún Center)

Cinthia Perez Ortiz, MD , universidad nacional de asuncion, san lorenzo, Paraguay

Background: A 6 year  old male patient coming from San Lorenzo, Paraguay with 4 years of clinical evolution of recurrent infections. At 6 years showed Pseudomonas Aeroginosa sepsis treated with antibiotics ATB, were hospitalized for 20 days.  Past medical History Repeated infections. At 6 months: Bronchiolitis, with fever, treated with ATB, Amoxidal and mists. At 1 year 8 months: pseudomonas sepsis with severe neutropenia treated with Cipro and amikacina, admitted in a hospital for 14 days. At 1 year 11 months:  sepsis was not isolated germ, treated with CEFTA and Vance in a hospital for 17 year. Other three episodies of fever and diarrhea . Family Background: 1 brother died at 4 years Aeroginosa Pseudomonas sepsis, without antecedents of previous infections On the physical examination the patient  not presented the positive findings . Work up CBC:Hb=13,5; hto = 40; plaq = 289000 . Leuco = 9900 ( bast=99, seg=4158, eos=99, linfo=5247, mono=297) Cultures: Blood Pseudomona Aeroginosa. IgA=2, IgG=3, IgM =9mg/dl CD3=3994 CD4=1117 CD8=3338 CD19=8/mm3 NK = 160 . Anion superóxido produccion = normal . Ac of tétano =  (<0,0078) BTK gen mutation Other: Febrile Antigens, hepatitis A, B, C, VDRL, RK 39,CMV, rubella, IFI for Chagas, ELISA for HIV, IgM for EBV, Gastric washings for TB 3 samples, all probed to be negative. Dx:  Agamaglobulinemia Congenita .

Methods: presentation of clinical case

Results:  Evolution of the patient: since 6 años received gamaglobulina 500 mg - kp. Normal development, no presented new infectious processes.


This is an uncommon disease but all the doctors especially allergists, infectologist and pediatricians must have present in some cases like this child