Body growth after combined liver-kidney transplantation in children with primary hyperoxaluria type 1.
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Body growth after combined liver-kidney transplantation in children with primary hyperoxaluria type 1. / Nissel, Richard; Latta, Kay; Gagnadoux, Marie-France; Kelly, Deirdre; Hulton, Sally; Kemper, Markus J.; Ruder, Hans; Söderdahl, Gunnar; Otte, Jean B; Cochat, Pierre; Roquet, Olga; Jamieson, Neville V; Haffner, Dieter.
in: TRANSPLANTATION, Jahrgang 82, Nr. 1, 1, 2006, S. 48-54.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - Body growth after combined liver-kidney transplantation in children with primary hyperoxaluria type 1.
AU - Nissel, Richard
AU - Latta, Kay
AU - Gagnadoux, Marie-France
AU - Kelly, Deirdre
AU - Hulton, Sally
AU - Kemper, Markus J.
AU - Ruder, Hans
AU - Söderdahl, Gunnar
AU - Otte, Jean B
AU - Cochat, Pierre
AU - Roquet, Olga
AU - Jamieson, Neville V
AU - Haffner, Dieter
PY - 2006
Y1 - 2006
N2 - BACKGROUND: Children with primary hyperoxaluria type 1 (PH1) often develop severe growth failure, which is related to metabolic and endocrine consequences of chronic renal failure, and/or oxalate deposition in bone and cartilage. Combined liver and kidney transplantation (LKT) corrects the underlying metabolic defect and restores renal function in these children. METHODS: We therefore analyzed longitudinal growth of 24 children with PH1 who underwent LKT at nine European centers. Mean age at LKT was 8.9 years, and mean duration of follow-up was 5.7 years. RESULTS: After LKT mean standardized height tended to increase from -1.79 SD to -1.47 SD until last observation. Mean adult height amounted to 167 cm and 158 cm in boys and girls, respectively. At last observation, seven out of 24 patients were stunted. Within the whole study population, the degree of catch-up growth after LKT was positively associated with degree of stunting at the time of LKT and negatively associated with prednisolone dosage explaining together 39% of the overall variability. CONCLUSIONS: Combined LKT does not induce true catch-up growth in the majority of children with PH1. Due to the preexisting growth retardation at the time of LKT, one third of patients end up with a reduced final height.
AB - BACKGROUND: Children with primary hyperoxaluria type 1 (PH1) often develop severe growth failure, which is related to metabolic and endocrine consequences of chronic renal failure, and/or oxalate deposition in bone and cartilage. Combined liver and kidney transplantation (LKT) corrects the underlying metabolic defect and restores renal function in these children. METHODS: We therefore analyzed longitudinal growth of 24 children with PH1 who underwent LKT at nine European centers. Mean age at LKT was 8.9 years, and mean duration of follow-up was 5.7 years. RESULTS: After LKT mean standardized height tended to increase from -1.79 SD to -1.47 SD until last observation. Mean adult height amounted to 167 cm and 158 cm in boys and girls, respectively. At last observation, seven out of 24 patients were stunted. Within the whole study population, the degree of catch-up growth after LKT was positively associated with degree of stunting at the time of LKT and negatively associated with prednisolone dosage explaining together 39% of the overall variability. CONCLUSIONS: Combined LKT does not induce true catch-up growth in the majority of children with PH1. Due to the preexisting growth retardation at the time of LKT, one third of patients end up with a reduced final height.
M3 - SCORING: Zeitschriftenaufsatz
VL - 82
SP - 48
EP - 54
JO - TRANSPLANTATION
JF - TRANSPLANTATION
SN - 0041-1337
IS - 1
M1 - 1
ER -
