{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,1]],"date-time":"2025-11-01T05:34:15Z","timestamp":1761975255817},"reference-count":116,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2011,11,29]],"date-time":"2011-11-29T00:00:00Z","timestamp":1322524800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Allergy Immunology"],"published-print":{"date-parts":[[2011,12]]},"abstract":"<jats:sec><jats:label \/><jats:p><jats:bold>To cite this article:<\/jats:bold> Hoernes M, Seger R, Reichenbach J. Modern management of primary B\u2010cell immunodeficiencies. <jats:italic>Pediatr Allergy Immunology<\/jats:italic> 2011: <jats:bold>22<\/jats:bold>: 758\u2013769.<\/jats:p><\/jats:sec><jats:sec><jats:title>Abstract<\/jats:title><jats:p>B\u2010cell defects constitute the majority of primary immunodeficiencies. Although a heterogeneous group of diseases, all are characterized by the reduction in or absence of immunoglobulins and\/or specific antimicrobial antibodies. Substitution of immunoglobulin G (IgG) is therefore the mainstay of treatment. While from the late 1970s, the intravenous route of administration was the most common, in the past decades, subcutaneous immunoglobulin replacement therapy has become more popular among patients and physicians. Independently of the optimal route of administration, dosage and IgG trough level remain subjects of debate. Higher IgG trough levels seem to improve the protection against recurrent infections and thus better prevent complications such as bronchiectasis. Some patients, however, achieve protection with IgG trough levels on the lower IgG limit of healthy persons. Therefore, an individual protective IgG trough level needs to be defined for each patient. Use of additional prophylactic antibiotics and immunosuppressive drugs differs amongst specialized immunodeficiency centres and clearly requires future investigation in multi\u2010centre trials. Haematopoietic stem cell transplantation (HSCT) is to date indicated as curative treatment in certain patients with B\u2010cell defects associated with cell deficiencies, for example in two class\u2010switch recombination defects and in selected severe forms of common variable immunodeficiency.<\/jats:p><\/jats:sec>","DOI":"10.1111\/j.1399-3038.2011.01236.x","type":"journal-article","created":{"date-parts":[[2011,11,30]],"date-time":"2011-11-30T05:19:11Z","timestamp":1322630351000},"page":"758-769","source":"Crossref","is-referenced-by-count":23,"title":["Modern management of primary B\u2010cell immunodeficiencies"],"prefix":"10.1111","volume":"22","author":[{"given":"Miriam","family":"Hoernes","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Reinhard","family":"Seger","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Janine","family":"Reichenbach","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2011,11,29]]},"reference":[{"key":"e_1_2_7_2_2","doi-asserted-by":"publisher","DOI":"10.1007\/978-1-4419-7185-2_13"},{"key":"e_1_2_7_3_2","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.immunol.021908.132649"},{"key":"e_1_2_7_4_2","doi-asserted-by":"publisher","DOI":"10.1067\/mpd.2002.127711"},{"key":"e_1_2_7_5_2","doi-asserted-by":"publisher","DOI":"10.1097\/00005792-198505000-00001"},{"key":"e_1_2_7_6_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197708042970503"},{"key":"e_1_2_7_7_2","doi-asserted-by":"publisher","DOI":"10.1093\/clinids\/9.2.334"},{"key":"e_1_2_7_8_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00441520"},{"key":"e_1_2_7_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(86)90812-0"},{"key":"e_1_2_7_10_2","doi-asserted-by":"publisher","DOI":"10.1099\/00222615-40-3-197"},{"key":"e_1_2_7_11_2","doi-asserted-by":"publisher","DOI":"10.1006\/clim.2000.4932"},{"key":"e_1_2_7_12_2","doi-asserted-by":"crossref","first-page":"3925","DOI":"10.4049\/jimmunol.161.8.3925","article-title":"Functional analysis of peripheral blood B cells in patients with X\u2010linked agammaglobulinemia","volume":"161","author":"Nonoyama S","year":"1998","journal-title":"J Immunol"},{"key":"e_1_2_7_13_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.clim.2005.10.007"},{"key":"e_1_2_7_14_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI0215658"},{"key":"e_1_2_7_15_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.clim.2009.10.003"},{"key":"e_1_2_7_16_2","doi-asserted-by":"publisher","DOI":"10.1517\/14728222.2011.585971"},{"key":"e_1_2_7_17_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2010.08077.x"},{"key":"e_1_2_7_18_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(97)70123-9"},{"key":"e_1_2_7_19_2","doi-asserted-by":"publisher","DOI":"10.1097\/01.md.0000100046.06009.b0"},{"key":"e_1_2_7_20_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2003-06-2014"},{"key":"e_1_2_7_21_2","doi-asserted-by":"publisher","DOI":"10.1006\/clim.1999.4725"},{"key":"e_1_2_7_22_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(08)61199-X"},{"key":"e_1_2_7_23_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.iac.2008.01.001"},{"key":"e_1_2_7_24_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2009.10.013"},{"key":"e_1_2_7_25_2","doi-asserted-by":"publisher","DOI":"10.1006\/clim.1999.4799"},{"key":"e_1_2_7_26_2","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(76)90173-X"},{"key":"e_1_2_7_27_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2007-06-091744"},{"key":"e_1_2_7_28_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2007-11-124545"},{"key":"e_1_2_7_29_2","article-title":"Outcome of allogeneic stem cell transplantation in adults with common variable immunodeficiency","author":"Rizzi M","journal-title":"J Allergy Clin Immunol"},{"key":"e_1_2_7_30_2","doi-asserted-by":"publisher","DOI":"10.1016\/S1081-1206(10)61142-8"},{"key":"e_1_2_7_31_2","doi-asserted-by":"publisher","DOI":"10.1542\/peds.9.6.722"},{"key":"e_1_2_7_32_2","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-93-1-55"},{"key":"e_1_2_7_33_2","doi-asserted-by":"publisher","DOI":"10.1016\/0140-6736(91)90147-H"},{"key":"e_1_2_7_34_2","doi-asserted-by":"publisher","DOI":"10.2165\/00063030-200721020-00005"},{"key":"e_1_2_7_35_2","volume-title":"Klinischer Einsatz von Immunglobulinen","author":"Wahn V","year":"2007"},{"key":"e_1_2_7_36_2","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-101-4-435"},{"key":"e_1_2_7_37_2","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(85)90006-3"},{"key":"e_1_2_7_38_2","doi-asserted-by":"publisher","DOI":"10.1067\/mai.2002.124999"},{"key":"e_1_2_7_39_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.intimp.2004.02.011"},{"key":"e_1_2_7_40_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(99)70246-5"},{"key":"e_1_2_7_41_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.clim.2003.10.007"},{"key":"e_1_2_7_42_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198602273140907"},{"key":"e_1_2_7_43_2","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000068335.01620.9D"},{"key":"e_1_2_7_44_2","doi-asserted-by":"publisher","DOI":"10.1159\/000075091"},{"key":"e_1_2_7_45_2","doi-asserted-by":"publisher","DOI":"10.4065\/75.1.83"},{"key":"e_1_2_7_46_2","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1177\/014107680109400412","article-title":"Pulmonary embolism after intravenous immunoglobulin","volume":"94","author":"Alliot C","year":"2001","journal-title":"J R Soc Med"},{"key":"e_1_2_7_47_2","doi-asserted-by":"crossref","first-page":"3269","DOI":"10.1001\/jama.1988.03720220017011","article-title":"Administration of immune globulin associated with aseptic meningitis","volume":"259","author":"Kato E","year":"1988","journal-title":"JAMA"},{"key":"e_1_2_7_48_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2001.02742.x"},{"key":"e_1_2_7_49_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2011.01.061"},{"key":"e_1_2_7_50_2","article-title":"The role of anti\u2010IgA antibodies in causing adverse reactions to gamma globulin infusion in immunodeficient patients: a comprehensive review of the literature","author":"Rachid R","journal-title":"J Allergy Clin Immunol"},{"key":"e_1_2_7_51_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.clim.2007.06.009"},{"key":"e_1_2_7_52_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.iac.2008.06.005"},{"key":"e_1_2_7_53_2","doi-asserted-by":"publisher","DOI":"10.1023\/A:1006678312925"},{"key":"e_1_2_7_54_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2249.2009.04079.x"},{"key":"e_1_2_7_55_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(95)90346-1"},{"key":"e_1_2_7_56_2","doi-asserted-by":"publisher","DOI":"10.1136\/adc.79.1.48"},{"key":"e_1_2_7_57_2","doi-asserted-by":"publisher","DOI":"10.1007\/s10198-004-0250-5"},{"key":"e_1_2_7_58_2","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-006-9002-x"},{"key":"e_1_2_7_59_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF01541341"},{"key":"e_1_2_7_60_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.clim.2004.02.002"},{"key":"e_1_2_7_61_2","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-006-9021-7"},{"key":"e_1_2_7_62_2","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-008-9231-2"},{"key":"e_1_2_7_63_2","first-page":"1","article-title":"Subcutaneous immunoglobulin replacement therapy in the treatment of patients with primary immunodeficiency disease","volume":"6","author":"Skoda\u2010Smith S","year":"2010","journal-title":"Ther Clin Risk Manag"},{"key":"e_1_2_7_64_2","doi-asserted-by":"publisher","DOI":"10.1016\/0140-6736(93)92798-X"},{"key":"e_1_2_7_65_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0091-6749(03)01781-0"},{"key":"e_1_2_7_66_2","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-010-9423-4"},{"key":"e_1_2_7_67_2","doi-asserted-by":"publisher","DOI":"10.1089\/jpm.2007.0126"},{"key":"e_1_2_7_68_2","doi-asserted-by":"publisher","DOI":"10.1517\/17425247.4.4.427"},{"key":"e_1_2_7_69_2","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-009-9352-2"},{"key":"e_1_2_7_70_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2249.2009.04027.x"},{"key":"e_1_2_7_71_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.tmrv.2009.09.011"},{"key":"e_1_2_7_72_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2249.2008.03620.x"},{"key":"e_1_2_7_73_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2249.2011.04376.x"},{"key":"e_1_2_7_74_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(82)90793-0"},{"key":"e_1_2_7_75_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2648.1995.21050917.x"},{"key":"e_1_2_7_76_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1398-9995.2005.00756.x"},{"key":"e_1_2_7_77_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2010.02.040"},{"key":"e_1_2_7_78_2","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-011-9511-0"},{"key":"e_1_2_7_79_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.clim.2010.06.012"},{"key":"e_1_2_7_80_2","doi-asserted-by":"publisher","DOI":"10.1006\/clim.2002.5241"},{"key":"e_1_2_7_81_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0091-6749(99)70085-0"},{"key":"e_1_2_7_82_2","doi-asserted-by":"publisher","DOI":"10.1159\/000101784"},{"key":"e_1_2_7_83_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2249.2007.03550.x"},{"key":"e_1_2_7_84_2","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.21036"},{"key":"e_1_2_7_85_2","doi-asserted-by":"publisher","DOI":"10.1080\/00365540110076769"},{"key":"e_1_2_7_86_2","doi-asserted-by":"publisher","DOI":"10.2165\/00003495-200363130-00003"},{"key":"e_1_2_7_87_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF01966999"},{"key":"e_1_2_7_88_2","volume-title":"Primary Immunodeficiency Diseases. A Molecular and Genetic Approach","author":"Ochs HD","year":"2007"},{"key":"e_1_2_7_89_2","doi-asserted-by":"publisher","DOI":"10.1159\/000237649"},{"key":"e_1_2_7_90_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.clim.2007.04.011"},{"key":"e_1_2_7_91_2","doi-asserted-by":"publisher","DOI":"10.1128\/CVI.00489-07"},{"key":"e_1_2_7_92_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.clim.2005.03.019"},{"key":"e_1_2_7_93_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-3083.2011.02561.x"},{"key":"e_1_2_7_94_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaut.2005.04.006"},{"key":"e_1_2_7_95_2","doi-asserted-by":"publisher","DOI":"10.1097\/01.md.0000133624.65946.40"},{"key":"e_1_2_7_96_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2009.07669.x"},{"key":"e_1_2_7_97_2","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-008-9182-7"},{"key":"e_1_2_7_98_2","doi-asserted-by":"publisher","DOI":"10.1196\/annals.1361.111"},{"key":"e_1_2_7_99_2","doi-asserted-by":"publisher","DOI":"10.1136\/jcp.2006.041426"},{"key":"e_1_2_7_100_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.20619"},{"key":"e_1_2_7_101_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.20072"},{"key":"e_1_2_7_102_2","doi-asserted-by":"publisher","DOI":"10.1182\/asheducation-2009.1.139"},{"key":"e_1_2_7_103_2","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-127-8_Part_1-199710150-00005"},{"key":"e_1_2_7_104_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2005.10.002"},{"key":"e_1_2_7_105_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2005.08.041"},{"key":"e_1_2_7_106_2","doi-asserted-by":"publisher","DOI":"10.1016\/S1081-1206(10)61228-8"},{"key":"e_1_2_7_107_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2133.2001.04092.x"},{"key":"e_1_2_7_108_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2249.2007.03481.x"},{"key":"e_1_2_7_109_2","doi-asserted-by":"publisher","DOI":"10.1007\/s11882-009-0051-0"},{"key":"e_1_2_7_110_2","doi-asserted-by":"publisher","DOI":"10.1038\/sj.bmt.1705713"},{"key":"e_1_2_7_111_2","doi-asserted-by":"publisher","DOI":"10.1542\/peds.113.2.e122"},{"key":"e_1_2_7_112_2","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-011-9526-6"},{"key":"e_1_2_7_113_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2011.01.052"},{"key":"e_1_2_7_114_2","doi-asserted-by":"publisher","DOI":"10.1038\/nm1086"},{"key":"e_1_2_7_115_2","doi-asserted-by":"publisher","DOI":"10.1038\/3233"},{"key":"e_1_2_7_116_2","doi-asserted-by":"publisher","DOI":"10.1038\/leu.2010.140"},{"key":"e_1_2_7_117_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2008.04.044"}],"container-title":["Pediatric Allergy and Immunology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fj.1399-3038.2011.01236.x","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/j.1399-3038.2011.01236.x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,14]],"date-time":"2023-10-14T14:22:57Z","timestamp":1697293377000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/j.1399-3038.2011.01236.x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2011,11,29]]},"references-count":116,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2011,12]]}},"alternative-id":["10.1111\/j.1399-3038.2011.01236.x"],"URL":"https:\/\/doi.org\/10.1111\/j.1399-3038.2011.01236.x","archive":["Portico"],"relation":{},"ISSN":["0905-6157","1399-3038"],"issn-type":[{"value":"0905-6157","type":"print"},{"value":"1399-3038","type":"electronic"}],"subject":[],"published":{"date-parts":[[2011,11,29]]}}}