[Liver transplantation in an adolescent with cystic fibrosis].
BACKGROUND
Orthotopic liver transplantation (OLT) is an effective treatment for patients with cystic fibrosis end stage liver disease, especially those with only mild pulmonary involvement. Long-term follow-up in such transplanted patients is still lacking.


METHODS
A 15-year-old girl with cystic fibrosis received an OLT because of severe decompensated cirrhosis. She had been colonized by Pseudomonas aeruginosa for 3 years and had pancreatic insufficiency; she also had mild glucose intolerance. Postoperatively she developed diabetes mellitus requiring insulin therapy for 9 months. Oral cyclosporin was poorly absorbed so that she was given a new emulsion of cyclosporin (Neoral) that was better absorbed. A rapid pubertal catch-up was obtained but the patient remained colonized by Pseudomonas aeruginosa.


CONCLUSIONS
This 3-year postoperative follow-up confirms that OLT can represent a good alternative in those patients with severe liver disease and mild pulmonary involvement.
Thevenot
P
P
Service de pédiatrie, CHRU, hôpital Claude-Huriez, Lille, France.
Gottrand
F
F
Tassin
E
E
Launay
V
V
Loeuille
G A
GA
Razemon
M
M
Turck
D
D
Bonnevalle
M
M
Pruvot
F R
FR
Hue
V
V
Farriaux
J P
JP
D000293:Adolescent; D001824:Body Constitution; D003550:Cystic Fibrosis / Q000150:complications / Q000628:therapy*; D005260:Female; D006801:Humans; D008103:Liver Cirrhosis / Q000150:complications / Q000601:surgery; D016031:Liver Transplantation; D011184:Postoperative Period