Современная концепция диагностики и терапии

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  • 1. C .. IV , 27 -2 2013 .., IV , 2013.

2. PEDIATRICS Official Journal of the American Academy of Pediatrics Vol 127 5 may 2011 Clinical Features That Identify Children With Primary Immunodeficiency Diseases .., IV , 2013. 3. Proportion of Children With the 10 Warning Signs Grouped According to PID Type No Defianble PID (N=133), n(%) Definable PID (N = 430), n(%) B Lymhocyte (N = 92), n(%) Positive family history 6(4) 148(34) 24(26) 2 deep-seated infections 15(11) 44(10) 8(9) 2 episodes of pneumonia 34(26) 105(24) 34(37) Abscesses(deep skin or organ) 9(7) 56(13) 2(2) Multiple acute otitis media 47(35) 64(15) 29(31) 2 sinus infections 21(16) 23(5) 14(15) Persistent thrush 5(4) 63(15) 2(2) Intravenous antibiotics 56(42) 241(56) 41(45) 2 mo of oral antibiotics with little effect 3(2) 75(17) 14(15) Failure to thrive 7(3) 135(31) 9(10) .., IV , 2013. 4. Agammaglobulinemia: historical notes Colonel Odgen C. Bruton, 1952 .., IV , 2013. 5. Agammaglobulinemia:historical notes Colonel Bruton, 1952 .., IV , 2013. 6. AGAMMAGLOBULINEMIA By Col. Ogden C. Bruton, M.C., U.S.A. Washinton, D.C. The complete absence of gamma globulin in human serum with a normal total protein asdet determined by electrophoretic analysis does not appear to have as yet been reported in the literature. Stern1 mentions two cases of hypoproteinemia in children who had almost complete absence of gamma globulin and were singularly free of infection. Schick2 reported a similar congenital case without nephrosis with a review of the literature in wich the total protein was low, the gamma globulin fraction low, and edema present. The latter findings in nephrosis are well known. Krebs3 reported a case in wich there was a depression of gammaglobulin in hypoproteinemia due to malnutrition. The present author had the opportunity of following a patient without nephrotic syndrome, with normal nutrition, with complete absence of the gamma globulin fraction and normal total serum protein through several years of many infections including 19 episodes of clinical sepsis in wich some type pneumococcus was recovered by blood culture 10 time. This entity, wich, it was found, could be controlled by supplying gamma globulin as contained in concentrated immune human serum globulin, appears to be unique. Pediatrics, 9, 722-728, 1952 .., IV , 2013. 7. Mancini G, Carbonara AO, Heremans JF. Immunochemical quantitation of antigens by single radial immunodiffusion. Immunochemistry. 1965 Sep;2(3):235-54. .., IV , 2013. 8. Agammaglobulinemia Immunological profile: - Absent/low immunoglobulin serum levels - Absence of circulating B cells (CD19+:Ta c.881 C>T missense mutationmissense mutation in exon 10 of thein exon 10 of the BTK geneBTK gene It wasnIt wasnt madet made Mother and childMother and child have Mutationhave Mutation Nt61delTNt61delT Therapy nowTherapy now Regular replacement therapy with intravenous IgRegular replacement therapy with intravenous Ig Antibacterial therapy in case of infectionAntibacterial therapy in case of infection SymptomaticSymptomatic therapytherapy AgeAge 2929yearsyears 1212yearsyears 1717yearsyears 1010yearsyears .., IV , 2013. 22. ( ) .., IV , 2013. 23. The importance of regular immune globulin replacement in patients with antibody deficiencies was initially attributed to its ability to provide specific antibodies that could not be produced by these patients in particular, antibodies to encapsulated organisms such as Streptococcus pneumoniae or Haemophilus influenzae. .., IV , 2013. 24. : , - , - (www.gpma.ru), ESID, Steering Committee of The J Project : 194100 ., . .22 . : 8 (812) 295 41 75 . : 8 (812) 295 10 72 E-mail: [email protected] . , 8(812)233-48-11 .., IV , 2013. 25. .., IV , 2013.