{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,8]],"date-time":"2026-05-08T15:25:49Z","timestamp":1778253949080,"version":"3.51.4"},"reference-count":36,"publisher":"FapUNIFESP (SciELO)","issue":"6","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Rev. Assoc. Med. Bras."],"published-print":{"date-parts":[[2016,9]]},"abstract":"<jats:p>Summary  Objective: To describe clinical features, tomographic findings and pulmonary function in pediatric patients with primary hypogammaglobulinemia (PH).  Method: A retrospective cohort study of children with PH who received intravenous immunoglobulin (IVIG) and prophylactic antibiotics between 2005 and 2010. Epidemiological and clinical features, computed tomography (CT) findings, and spirometric data were compared, assuming a 5% significance level.  Results: We evaluated 30 patients with PH. After the start of IVIG replacement, there was a decline in the frequency of pneumonia (p&lt;0.001). The 11 patients with bronchiectasis in their first CT scan were older at diagnosis (p=0.001) and had greater diagnostic delay (p=0.001) compared to patients without bronchiectasis. At the end of the study, 18 patients had bronchiectasis and 27 also had other lung disorders, alone or in combination. The Bhalla score was applied to the last CT scan of 16 patients, with a median score of 11 (range 7-21), with a positive correlation between the score and the number of pneumonias after the start of treatment (r=0.561; p=0.024). The score was also correlated with forced expiratory volume in one second (FEV1) and forced vital capacity (FVC) values in 13\/16 patients, with negative correlation to FEV1 previously to bronchodilator (r=-0.778; p=0.002) and after bronchodilator (r =-0.837; p&lt;0.001) and FVC (r=-0.773; p=0.002).  Conclusion: Pulmonary complications were common in this cohort, despite the decrease in the frequency of pneumonia with treatment. Early investigation of patients with recurrent infections for primary immunodeficiencies can reduce the frequency of these complications. The monitoring of changes in spirometry may indicate the need to carry out radiological investigation.<\/jats:p>","DOI":"10.1590\/1806-9282.62.06.530","type":"journal-article","created":{"date-parts":[[2016,11,9]],"date-time":"2016-11-09T21:08:03Z","timestamp":1478725683000},"page":"530-536","source":"Crossref","is-referenced-by-count":3,"title":["Primary hypogammaglobulinemia: The impact of early diagnosis in lung complications"],"prefix":"10.1590","volume":"62","author":[{"given":"Mayra de Barros","family":"Dorna","sequence":"first","affiliation":[{"name":"Universidade de S\u00e3o Paulo,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cristiane de Jesus Nunes dos","family":"Santos","sequence":"additional","affiliation":[{"name":"Universidade de S\u00e3o Paulo,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ana Paula Beltran Moschione","family":"Castro","sequence":"additional","affiliation":[{"name":"Universidade de S\u00e3o Paulo,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luiz Ant\u00f4nio Nunes de","family":"Oliveira","sequence":"additional","affiliation":[{"name":"HC-FMUSP,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lisa","family":"Suzuki","sequence":"additional","affiliation":[{"name":"HC-FMUSP,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrea Langone","family":"Ferme","sequence":"additional","affiliation":[{"name":"HC-FMUSP,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Magda Maria Sales","family":"Carneiro-Sampaio","sequence":"additional","affiliation":[{"name":"Universidade de S\u00e3o Paulo,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Antonio Carlos","family":"Pastorino","sequence":"additional","affiliation":[{"name":"Universidade de S\u00e3o Paulo,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"530","reference":[{"issue":"3","key":"ref1","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1097\/00005792-198505000-00001","article-title":"X-linked agammaglobulinemia: an analysis of 96 patients","volume":"64","author":"Lederman HM","year":"1985","journal-title":"Medicine"},{"issue":"1","key":"ref2","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1006\/clim.1999.4725","article-title":"Common variable immunodeficiency: clinical and immunological features of 248 patients","volume":"92","author":"Cunningham-Rundles C","year":"1999","journal-title":"Clin Immunol"},{"issue":"3","key":"ref3","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1006\/clim.2002.5241","article-title":"Clinical, immunological, and molecular analysis in a large cohort of patients with X-linked agammaglobulinemia: an Italian multicenter study","volume":"104","author":"Plebani A","year":"2002","journal-title":"Clin Immunol"},{"issue":"6","key":"ref4","doi-asserted-by":"crossref","first-page":"373","DOI":"10.1097\/01.md.0000100046.06009.b0","article-title":"The X-linked hyper-IgM syndrome: clinical and immunologic features of 79 patients","volume":"82","author":"Winkelstein JA","year":"2003","journal-title":"Medicine"},{"issue":"4","key":"ref5","doi-asserted-by":"crossref","first-page":"193","DOI":"10.1097\/01.md.0000229482.27398.ad","article-title":"X-linked agammaglobulinemia: report on a United States registry of 201 patients","volume":"85","author":"Winkelstein JA","year":"2006","journal-title":"Medicine"},{"issue":"1","key":"ref6","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1007\/s10875-008-9227-y","article-title":"Clinical and molecular analysis of 49 patients with X-linked agammaglobulinemia from a single center in Argentina","volume":"29","author":"Basile N","year":"2009","journal-title":"J Clin Immunol"},{"issue":"6","key":"ref7","doi-asserted-by":"crossref","first-page":"340","DOI":"10.1016\/j.jfma.2012.07.005","article-title":"Clinical characteristics and outcome of primary antibody deficiency: a 20-year follow-up study","volume":"113","author":"Chan HY","year":"2014","journal-title":"J Formos Med Assoc"},{"issue":"8541","key":"ref8","doi-asserted-by":"crossref","first-page":"1075","DOI":"10.1016\/S0140-6736(87)90494-6","article-title":"High-dose versus low-dose intravenous immunoglobulin in hypogammaglobulinaemia and chronic lung disease","volume":"1","author":"Roifman CM","year":"1987","journal-title":"Lancet"},{"issue":"6","key":"ref9","doi-asserted-by":"crossref","first-page":"527","DOI":"10.1136\/adc.74.6.527","article-title":"Treatment of hypogammaglobulinaemia with intravenous immunoglobulin, 1973-93","volume":"74","author":"Skull S","year":"1996","journal-title":"Arch Dis Child"},{"issue":"2","key":"ref10","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1016\/S0928-8244(03)00304-3","article-title":"Efficacy of intravenous immunoglobulin on the prevention of pneumonia in patients with agammaglobulinemia","volume":"40","author":"Aghamohammadi A","year":"2004","journal-title":"FEMS Immunol Med Microbiol"},{"issue":"1","key":"ref11","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1016\/j.clim.2003.10.007","article-title":"Clinical, immunologic and genetic analysis of 29 patients with autosomal recessive hyper-IgM syndrome due to Activation-Induced Cytidine Deaminase deficiency","volume":"110","author":"Quartier P","year":"2004","journal-title":"Clin Immunol"},{"issue":"2","key":"ref12","first-page":"114","article-title":"Effect of regular intravenous immunoglobulin therapy on prevention of pneumonia in patients with common variable immunodeficiency","volume":"39","author":"Pourpak Z","year":"2006","journal-title":"J Microbiol Immunol Infect"},{"issue":"9","key":"ref13","doi-asserted-by":"crossref","first-page":"833","DOI":"10.1001\/archpedi.1983.02140350011004","article-title":"Common variable hypogammaglobulinemia in children. Clinical and immunologic observations in 30 patients","volume":"137","author":"Hausser C","year":"1983","journal-title":"Am J Dis Child"},{"issue":"1","key":"ref14","doi-asserted-by":"crossref","first-page":"32","DOI":"10.1093\/tropej\/fml077","article-title":"Mortality and morbidity in common variable immunodeficiency","volume":"53","author":"Aghamohammadi A","year":"2007","journal-title":"J Trop Pediatr"},{"issue":"3","key":"ref15","doi-asserted-by":"crossref","first-page":"128","DOI":"10.1016\/j.aller.2010.12.003","article-title":"Pulmonary complications in patients with antibody deficiency","volume":"39","author":"Costa-Carvalho BT","year":"2011","journal-title":"Allergol Immunopathol (Madr)"},{"issue":"10","key":"ref16","doi-asserted-by":"crossref","first-page":"655","DOI":"10.1093\/qjmed\/95.10.655","article-title":"Common variable immune deficiency: respiratory manifestations, pulmonary function and high-resolution CT scan findings","volume":"95","author":"Thickett KM","year":"2002","journal-title":"QJM"},{"issue":"2-3","key":"ref17","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1016\/j.clim.2005.11.002","article-title":"The health status and quality of life of adults with X-linked agammaglobulinemia","volume":"118","author":"Howard V","year":"2006","journal-title":"Clin Immunol"},{"issue":"2","key":"ref18","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1182\/blood-2007-11-124545","article-title":"Common variable immunodeficiency disorders: division into distinct clinical phenotypes","volume":"112","author":"Chapel H","year":"2008","journal-title":"Blood"},{"issue":"3","key":"ref19","first-page":"190","article-title":"Diagnostic criteria for primary immunodeficiencies. Representing PAGID (Pan-American Group for Immunodeficiency) and ESID (European Society for Immunodeficiencies)","volume":"93","author":"Conley ME","year":"1999","journal-title":"Clin Immunol"},{"issue":"3","key":"ref20","doi-asserted-by":"crossref","first-page":"783","DOI":"10.1148\/radiology.179.3.2027992","article-title":"Cystic fibrosis: scoring system with thin-section CT","volume":"179","author":"Bhalla M","year":"1991","journal-title":"Radiology"},{"issue":"3","key":"ref21","first-page":"S1","article-title":"Diretrizes para Testes de Fun\u00e7\u00e3o Pulmonar","volume":"28","year":"2002","journal-title":"J Pneumol"},{"issue":"2","key":"ref22","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1183\/09031936.05.00034805","article-title":"Standardisation of spirometry","volume":"26","author":"Miller MR","year":"2005","journal-title":"Eur Respir J"},{"key":"ref23","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1016\/j.clim.2010.06.012","article-title":"Impact of trough IgG on pneumonia incidence in primary immunodeficiency: a meta-analysis of clinical studies","volume":"137","author":"Orange JS","year":"2010","journal-title":"Clin Immunol"},{"issue":"5","key":"ref24","doi-asserted-by":"crossref","first-page":"1031","DOI":"10.1016\/S0091-6749(99)70085-0","article-title":"Pulmonary abnormalities in patients with primary hypogammaglobulinemia","volume":"104","author":"Kainulainen L","year":"1999","journal-title":"J Allergy Clin Immunol"},{"issue":"3","key":"ref25","doi-asserted-by":"crossref","first-page":"308","DOI":"10.1007\/s10875-007-9075-1","article-title":"Long-term follow-up and outcome of a large cohort of patients with common variable immunodeficiency","volume":"27","author":"Quinti I","year":"2007","journal-title":"J Clin Immunol"},{"issue":"10","key":"ref26","doi-asserted-by":"crossref","first-page":"1547","DOI":"10.1086\/587669","article-title":"Infections in 252 patients with common variable immunodeficiency","volume":"46","author":"Oksenhendler E","year":"2008","journal-title":"Clin Infect Dis"},{"issue":"3","key":"ref27","first-page":"222","article-title":"Clinical and immunological analysis of 23 adult patients with common variable immunodeficiency","volume":"20","author":"Ardeniz O","year":"2010","journal-title":"J Investig Allergol Clin Immunol"},{"issue":"3","key":"ref28","doi-asserted-by":"crossref","first-page":"315","DOI":"10.1007\/s10875-011-9511-0","article-title":"Effectiveness of immunoglobulin replacement therapy on clinical outcome in patients with primary antibody deficiencies: results from a multicenter prospective cohort study","volume":"31","author":"Quinti I","year":"2011","journal-title":"J Clin Immunol"},{"issue":"5","key":"ref29","doi-asserted-by":"crossref","first-page":"907","DOI":"10.1007\/s10875-012-9671-6","article-title":"The spectrum of disease manifestations in patients with common variable immunodeficiency disorders and partial antibody deficiency in a university hospital","volume":"32","author":"Maarschalk-Ellerbroek LJ","year":"2012","journal-title":"J Clin Immunol"},{"issue":"7","key":"ref30","first-page":"825","article-title":"Clinical and immunological features of 65 Iranian patients with common variable immunodeficiency","volume":"12","author":"Aghamohammadi A","year":"2005","journal-title":"Clin Diagn Lab Immunol"},{"issue":"5","key":"ref31","doi-asserted-by":"crossref","first-page":"589","DOI":"10.1016\/S0022-3476(99)70246-5","article-title":"Early and prolonged intravenous immunoglobulin replacement therapy in childhood agammaglobulinemia: a retrospective survey of 31 patients","volume":"134","author":"Quartier P","year":"1999","journal-title":"J Pediatr"},{"issue":"3","key":"ref32","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1053\/rmed.2000.1020","article-title":"Respiratory disorders in common variable immunodeficiency","volume":"95","author":"Mart\u00ednez Garc\u00eda MA","year":"2001","journal-title":"Respir Med"},{"issue":"7","key":"ref33","first-page":"514","article-title":"Efficacy of intravenous immunoglobulin treatment in children with common variable immunodeficiency","volume":"21","author":"Baris S","year":"2011","journal-title":"J Investig Allergol Clin Immunol"},{"key":"ref34","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1111\/j.1749-6632.2011.06206.x","article-title":"Ten warning signs of primary immunodeficiency: a new paradigm is needed for the 21st century","volume":"1238","author":"Arkwright PD","year":"2011","journal-title":"Ann N Y Acad Sci"},{"issue":"2","key":"ref35","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1378\/chest.09-2398","article-title":"A CT scan score for the assessment of lung disease in children with common variable immunodeficiency disorders","volume":"138","author":"van de Ven AA","year":"2010","journal-title":"Chest"},{"issue":"2","key":"ref36","first-page":"69","article-title":"Pulmonary complications in primary hypogammaglobulinemia: a survey by high resolution CT scan","volume":"65","author":"Gharagozlou M","year":"2006","journal-title":"Monaldi Arch Chest Dis"}],"container-title":["Revista da Associa\u00e7\u00e3o M\u00e9dica Brasileira"],"original-title":[],"link":[{"URL":"http:\/\/www.scielo.br\/pdf\/ramb\/v62n6\/0104-4230-ramb-62-6-0530.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2017,5,24]],"date-time":"2017-05-24T01:32:17Z","timestamp":1495589537000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.scielo.br\/scielo.php?script=sci_arttext&pid=S0104-42302016000600530&lng=en&tlng=en"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,9]]},"references-count":36,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2016,9]]}},"alternative-id":["S0104-42302016000600530"],"URL":"https:\/\/doi.org\/10.1590\/1806-9282.62.06.530","relation":{},"ISSN":["0104-4230"],"issn-type":[{"value":"0104-4230","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,9]]}}}