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Patrick Lebecque

Publications and source records attributed to Patrick Lebecque.

2 recordsLinked to original sources

Modified method to measure nasal potential difference.

Nasal potential difference (NPD) measurements have been proposed to assess defective ion transport in cystic fibrosis (CF). Implementing it routinely is, however, difficult. Therefore, a modified method based on nasal instillation in supine position at reduced flow rate was tested to evaluate its ability to discriminate CF from non-CF subjects. Classical and modified methods were compared in nine healthy subjects and there were no statistical differences. Following the new method, 97 tests were performed on 74 subjects divided in three cohorts: 21 CF patients and two control groups consisting of 19 patients with other pulmonary diseases and 34 healthy subjects. Twenty five children were enrolled in this study. Maximal NPD in CF patients (-44.9 +/- 2.5 mV) was significantly different from that obtained in control groups (-18.1 +/- 1.6 and -17.2 +/- 1.1 mV). Depolarization after amiloride also discriminated CF patients (25.9 +/- 1.4 mV) from control groups (10.5 +/- 0.9 and 8.1 +/- 0.7 mV). Marked repolarization following isoprenaline plus amiloride in low chloride solution was seen in control groups (-15.7 +/- 1.1 and -15.3 +/- 1.1 mV). We conclude that the modified method represents a simplified and equally effective approach to discriminate CF patients from non-CF subjects. Moreover, this method presents practical advantages for the patients related to hygiene and convenience, favoring its application in small children.

Adolescent↗

Mutations of the cystic fibrosis gene and intermediate sweat chloride levels in children.

The incidence of mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene in children with intermediate sweat chloride levels is unknown. The results of 2,349 sweat tests performed at two Belgian university hospitals were reviewed. Intermediate chloride concentrations were observed in 98 subjects (4.2%), 68 being younger than 18 years of age. Forty-three children could be traced and their parents agreed to take part in the study. Exhaustive analysis of the CFTR gene disclosed a total of 24 putative mutations (27.9%). Three subjects were found to carry only one CFTR mutation, whereas 10 harbored one mutation on both CFTR genes. These 10 children were investigated in detail. At the time of writing, the mean age (+/-SD) of this group is 8.9 years (+/-4.2 years). Nine children are pancreatic sufficient. Three have been asymptomatic for more than two years, whereas the others display, to different degrees, clinical features suggestive of CF. The sweat chloride concentration is slightly higher in this group (39.4 +/- 5.4 mM) than in subjects without CFTR mutation (35.2 +/- 4.4 mM, p < 0.05). The nasal potential difference was abnormal in five of the nine subjects tested. In this study, 23% of children displaying intermediate sweat chloride levels were found to carry a putative mutation on both CFTR genes.

Adolescent↗