RESUMO
OBJECTIVE: The aim of this study was to evaluate pulmonary complications in patients with primary antibody deficiency (X-linked agammaglobulinaemia [XLA] and common variable immunodeficiency [CVID]). METHODS: Thirty patients over six years of age regularly followed in a reference out-patient clinic on primary immunodeficiency were studied. All of them have been treated with intravenous immunoglobulin (IVIG) replacement therapy. Pulmonary complications were evaluated analysing clinical data (medical records review), lung function test (spirometry) and pulmonary imaging (chest computed tomography [CCT]). RESULTS: Patients with normal CCT (N = 14) and those with abnormal CCT (N = 16) have shown no differences regarding the age at onset of symptoms, age of diagnosis, and duration of IVIG treatment. The mean number of pneumonia episodes before IVIG replacement was significantly higher among patients with abnormal CCT (4 vs 7 episodes, p = 0.008). CCT abnormalities observed in 16 patients were: bronchiectasis (12/16); peribronchial thickening (3/16); air trapping (5/16); lung volume reduction (4/16); atelectasis (2/16), follicular bronchiolitis and ground-glass abnormality (2/16) and parenchyma nodule (1/16). Lung function tests showed ventilatory disturbance in 18/30: obstructive pattern in 38.8%, restrictive pattern in 44.4%, and mix pattern in 16.7%. There were no significant differences in lung function between those with and without CCT abnormalities. Negative significant correlations were observed between lung function and number of episodes of pneumonia. Chronic persistent cough was associated with a reduction in lung function. CONCLUSIONS: Pulmonary complications are not rare in patients with antibody deficiencies and they must be monitored.
Assuntos
Agamaglobulinemia/complicações , Imunodeficiência de Variável Comum/complicações , Doenças Genéticas Ligadas ao Cromossomo X/complicações , Pneumopatias/epidemiologia , Pneumopatias/etiologia , Adolescente , Adulto , Agamaglobulinemia/tratamento farmacológico , Criança , Imunodeficiência de Variável Comum/tratamento farmacológico , Feminino , Doenças Genéticas Ligadas ao Cromossomo X/tratamento farmacológico , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Masculino , Testes de Função Respiratória , Tomografia Computadorizada por Raios X , Adulto JovemRESUMO
The bifrontal craniotomy approach used to be associated with a high percentage of olfactory tract damage. We present our experience with this technique, that was used with excellent results in a series of 11 patients that underwent the surgical approach described in this paper. We support the idea that bilateral subfrontal craniotomy allows a wide operative exposure as well as the complete anatomic and functional preservation of the olfactory tracts bilaterally.
Assuntos
Neoplasias Encefálicas/cirurgia , Craniotomia/métodos , Condutos Olfatórios/fisiologia , Adulto , Neoplasias Encefálicas/diagnóstico , Pré-Escolar , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Base do CrânioRESUMO
The bifrontal craniotomy approach used to be associated with a high percentage of olfactory tract damage. We present our experience with this technique, that was used with excellent results in a series of 11 patients that underwent the surgical approach described in this paper. We support the idea that bilateral subfrontal craniotomy allows a wide operative exposure as well as the complete anatomic and functional preservation of the olfactory tracts bilaterally
Assuntos
Humanos , Masculino , Feminino , Pré-Escolar , Adulto , Pessoa de Meia-Idade , Neoplasias Encefálicas , Craniotomia , Condutos Olfatórios , Neoplasias Encefálicas , Imageamento por Ressonância Magnética , Base do CrânioRESUMO
Objetivo: Avaliar a função pulmonar de pacientes com hipogamaglobulinemia (HG) primária. Pacientes e método: Participaram deste estudo 24 pacientes com HG acompanhados em ambulatório especializado durante o período de janeiro a dezembro de 2005. Foram incluidos pacientes em reposição regular de gamaglobulina intravenosa (IGIV) e maiores de seis anos de idade. As provas de função pulmonar (FP) foram realizadas pela manhã, conforme o padronizado. Resultados: A idade dos pacientes variou entre 7 e 42 anos (média 18,6 anos) e houve retardo médio no diagnóstico definitivo de HG de 6,8 anos (0,5 a 7,5 anos). Imunodeficiência Comum Variável e Agamaglobulinemia ligada ao X foram as doenças mais comuns. As infecções do trato respiratório foram as mais prevalentes antes e após início da reposição de IGIV. Tosse persistente foi o sintoma respiratório crônico mais relatado no último ano (n=17). Todos os pacientes realizaram provas de FP aceitáveis e 13 (54