Résumé
Hepatopulmonary syndrome [HPS] is a rare but severe complication in children with chronic liver disease [CLD] that may necessitate "urgent" liver transplantation. This study aimed at studying the feasibility and usefulness of pulmonary function testing [PFT] in the diagnosis of lung affections in a group of Egyptian children with chronic liver disease [CLD] in order to diagnose HPS in them. Twenty five Egyptian children [aged 10-16 years with a mean of 12.4 +/- 1.8 years] with different causes of CLD and 8 age-matched, healthy controls were subjected to arterial blood gas analysis, PFT and measurement of mean pulmonary artery pressure [MPAP]. Arterial oxygen pressure [Pa0[2]] less than 80 mmHg was present in 10 patients, forced expiratory volume in I second [FEV[1]] less than 80% predicted in 15, forced vital capacity [FVC] less than 80% predicted in 15 and the ratio FEV[1]:FVC less than 65% predicted in 20. Carbon monoxide diffusion capacity [DL[CO]] was less than 80% predicted in 15 patients and 4 controls. Hypoxemia occurred in 60% of patients with decreased DL[co] but none of controls. MPAP was significantly lower in patients than controls, it increased with exercise but not significantly. Neither portal hypertension nor sclerotherapy showed a significant relation to hypoxemia, or low MPAP. Also the duration of CLD, its histological severity and grading of fibrosis were not related to any of the pulmonary function tests. Arterial hypoxemia is present in CLD. PFT was rather "tedious" but non-invasive investigation in children with CLD. It did demonstrate obstructive airway as well as diffusion defects in these children. Therefore we may recommend PFT in the work-up of children with CLD, particularly if liver transplantation is contemplated