Does liver transplantation affect growth pattern in alagille syndrome?

Ruben E. Quiros-Tejeira, Marvin E. Ament, Melvin B. Heyman, Martin G. Martin, Philip Rosenthal, Jeffrey A. Gornbein, Sue V. McDiarmid, Jorge H. Vargas

Research output: Contribution to journalArticle

26 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)582-587
Number of pages6
JournalLiver Transplantation
Volume6
Issue number5
DOIs
StatePublished - Jan 1 2000

Fingerprint

Alagille Syndrome
Liver Transplantation
Growth
Weights and Measures
Pyridinolcarbamate
Bone and Bones
Cholestasis
Birth Weight
Malnutrition
Growth Hormone
Liver Diseases
Analysis of Variance

ASJC Scopus subject areas

  • Surgery
  • Hepatology
  • Transplantation

Cite this

Quiros-Tejeira, R. E., Ament, M. E., Heyman, M. B., Martin, M. G., Rosenthal, P., Gornbein, J. A., ... Vargas, J. H. (2000). Does liver transplantation affect growth pattern in alagille syndrome? Liver Transplantation, 6(5), 582-587. https://doi.org/10.1053/jlts.2000.9739

Does liver transplantation affect growth pattern in alagille syndrome? / Quiros-Tejeira, Ruben E.; Ament, Marvin E.; Heyman, Melvin B.; Martin, Martin G.; Rosenthal, Philip; Gornbein, Jeffrey A.; McDiarmid, Sue V.; Vargas, Jorge H.

In: Liver Transplantation, Vol. 6, No. 5, 01.01.2000, p. 582-587.

Research output: Contribution to journalArticle

Quiros-Tejeira, RE, Ament, ME, Heyman, MB, Martin, MG, Rosenthal, P, Gornbein, JA, McDiarmid, SV & Vargas, JH 2000, 'Does liver transplantation affect growth pattern in alagille syndrome?', Liver Transplantation, vol. 6, no. 5, pp. 582-587. https://doi.org/10.1053/jlts.2000.9739
Quiros-Tejeira, Ruben E. ; Ament, Marvin E. ; Heyman, Melvin B. ; Martin, Martin G. ; Rosenthal, Philip ; Gornbein, Jeffrey A. ; McDiarmid, Sue V. ; Vargas, Jorge H. / Does liver transplantation affect growth pattern in alagille syndrome?. In: Liver Transplantation. 2000 ; Vol. 6, No. 5. pp. 582-587.
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