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Hyperinflation in children with liver disease due to alpha-1-antitrypsin deficiency
M F Hird1, A Greenough, G Mieli-Vergani
1Department of Child Health, King's College Hospital, London, England.
Insights
Alpha-1-antitrypsin deficiency (A1ATD) in children may cause lung hyperinflation, even in those as young as 3-4 years old. This suggests early lung function abnormalities in A1ATD patients with liver disease.
Area of Science:
- Pediatric Pulmonology
- Hepatology
- Genetics
Background:
- Liver disease in children can impact respiratory function.
- Alpha-1-antitrypsin deficiency (A1ATD) is a genetic disorder associated with liver and lung disease.
Purpose of the Study:
- To investigate lung function, specifically functional residual capacity (FRC), in children with liver disease.
- To determine if A1ATD is associated with lung volume abnormalities in pediatric patients.
Main Methods:
- Serial measurements of FRC were taken over at least 6 months in 59 children with liver disease and 10 healthy controls.
- Children with A1ATD and extrahepatic biliary atresia (EHBA) were compared for lung volumes.
- Hyperinflation was defined as FRC >120% predicted and assessed for bronchodilator responsiveness.
Main Results:
- Children with A1ATD tended to have higher lung volumes than those with EHBA.
- Fifteen children exhibited persistent hyperinflation, unresponsive to bronchodilators in five cases (four with A1ATD).
- Two A1ATD children aged 3 and 4 years showed persistent, unresponsive hyperinflation; no healthy children were hyperinflated.
Conclusions:
- A1ATD may be linked to lung function abnormalities, including hyperinflation, in very young children.
- Persistent, unresponsive hyperinflation can occur in young children with A1ATD and liver disease.
- Early detection of lung abnormalities in A1ATD is crucial.
Abstract:
Fifty-nine patients (median age 6.0 years) with liver disease and ten healthy children without liver disease (median age 7.5 years) had serial measurements of functional residual capacity (FRC) over a period of at least 6 months. Twenty-eight children with alpha-1-antitrypsin deficiency (A1ATD) tended to have higher lung volumes than 26 with extrahepatic biliary atresia (EHBA). Fifteen children had persistent hyperinflation (FRC greater than 120% predicted for height; 2 SD above the mean of controls); in five children (four with A1ATD) this was unresponsive to bronchodilator therapy. Two of the children with A1ATD who had persistent unresponsive hyperinflation on three serial measurements were only 3 and 4 years of age. No healthy child without liver disease was hyperinflated. These results suggest that A1ATD may be associated with lung function abnormalities even in very young children.