title: Does liver transplantation affect growth pattern in Alagille syndrome?
abstract: Alagille syndrome (AGS) is frequently associated with growth failure, which has been attributed to concurrent congenital anomalies, cholestasis, and malabsorption and/or malnutrition. However, the underlying cause of the growth failure is not well understood. Our objective is to analyze the growth pattern in 26 patients with AGS and the possible effect that orthotopic liver transplantation (OLT) may have on this pattern. The standardized height, weight, and growth velocity of 26 pair-matched patients with AGS were compared. Thirteen patients underwent OLT. Repeated-measure ANOVA methods were used for the statistical analysis. The overall mean standardized height (z score) was -2.92 in the OLT group versus -1.88 in the non-OLT group (P =.03). The overall mean standardized weight was -1. 21 in the non-OLT group and -1.67 in the OLT group (P =.23). In 15 patients, birth weight was 2.82 +/- 0.4 kg, for a mean standardized weight of -0.95, and weight at diagnosis was 4.53 +/- 2.12 kg, for a mean standardized weight of -1.56. Bone age was delayed in the 9 patients who underwent bone-age analysis. Growth hormone therapy administered to 2 patients did not improve growth. Patients with AGS had growth failure secondary to other factors in addition to liver disease. Growth failure beginning in the prenatal period supports a genetic basis for this feature. Growth improvement up to normal levels should not be expected as a benefit of OLT in these patients. Growth failure as a primary indication for OLT should be cautiously examined in patients with AGS.
authors.affiliation: Division of Pediatric Gastroenterology and Nutrition, University of California at Los Angeles Medical Center, 90095-1752, USA.
authors.forename: R E
authors.initials: RE
authors.lastname: Quiros-Tejeira
authors.forename: M E
authors.initials: ME
authors.lastname: Ament
authors.forename: M B
authors.initials: MB
authors.lastname: Heyman
authors.forename: M G
authors.initials: MG
authors.lastname: Martin
authors.forename: P
authors.initials: P
authors.lastname: Rosenthal
authors.forename: J A
authors.initials: JA
authors.lastname: Gornbein
authors.forename: S V
authors.initials: SV
authors.lastname: McDiarmid
authors.forename: J H
authors.initials: JH
authors.lastname: Vargas
mesh_terms: D000365:Age Determination by Skeleton; D016738:Alagille Syndrome / Q000473:pathology / Q000503:physiopathology* / Q000601:surgery*; D001827:Body Height; D001835:Body Weight; D001842:Bone and Bones / Q000503:physiopathology; D002648:Child; D002657:Child Development; D002675:Child, Preschool; D005260:Female; D006128:Growth; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D016031:Liver Transplantation; D008297:Male; D016896:Treatment Outcome