title: Primary hypogammaglobulinemia: The impact of early diagnosis in lung complications.
abstract: To describe clinical features, tomographic findings and pulmonary function in pediatric patients with primary hypogammaglobulinemia (PH).
  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.
  We evaluated 30 patients with PH. After the start of IVIG replacement, there was a decline in the frequency of pneumonia (p<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<0.001) and FVC (r=-0.773; p=0.002).
  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.
authors.affiliation: Unit of Allergy and Immunology, Department of Pediatrics, Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo (HC-FMUSP), São Paulo, SP, Brazil.
authors.forename: Mayra de Barros
authors.initials: MB
authors.lastname: Dorna
authors.affiliation: Unit of Allergy and Immunology, Department of Pediatrics, Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo (HC-FMUSP), São Paulo, SP, Brazil.
authors.forename: Cristiane de Jesus Nunes Dos
authors.initials: CJ
authors.lastname: Santos
authors.affiliation: Unit of Allergy and Immunology, Department of Pediatrics, Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo (HC-FMUSP), São Paulo, SP, Brazil.
authors.forename: Ana Paula Beltran Moschione
authors.initials: AP
authors.lastname: Castro
authors.affiliation: Diagnostic Imaging Service, Instituto da Criança, HC-FMUSP, São Paulo, SP, Brazil.
authors.forename: Luiz Antônio Nunes de
authors.initials: LA
authors.lastname: Oliveira
authors.affiliation: Diagnostic Imaging Service, Instituto da Criança, HC-FMUSP, São Paulo, SP, Brazil.
authors.forename: Lisa
authors.initials: L
authors.lastname: Suzuki
authors.affiliation: Diagnostic Imaging Service, Instituto da Criança, HC-FMUSP, São Paulo, SP, Brazil.
authors.forename: Andrea Langone
authors.initials: AL
authors.lastname: Ferme
authors.affiliation: Unit of Allergy and Immunology, Department of Pediatrics, Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo (HC-FMUSP), São Paulo, SP, Brazil.
authors.forename: Magda Maria Sales
authors.initials: MM
authors.lastname: Carneiro-Sampaio
authors.affiliation: Unit of Allergy and Immunology, Department of Pediatrics, Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo (HC-FMUSP), São Paulo, SP, Brazil.
authors.forename: Antonio Carlos
authors.initials: AC
authors.lastname: Pastorino
mesh_terms: D000293:Adolescent; D000361:Agammaglobulinemia / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D001987:Bronchiectasis / Q000175:diagnosis* / Q000209:etiology; D002648:Child; D015331:Cohort Studies; D042241:Early Diagnosis; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage; D008297:Male; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D055815:Young Adult
chemical_list: D016756:Immunoglobulins, Intravenous