Body growth after combined liver-kidney transplantation in children with primary hyperoxaluria type 1.
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.
Nissel
Richard
R
Department of Pediatric Nephrology, Charité Children's Hospital, Berlin, Germany. richard.nissel@med.uni-rostock.de
Latta
Kay
K
Gagnadoux
Marie-France
MF
Kelly
Deirdre
D
Hulton
Sally
S
Kemper
Markus J
MJ
Ruder
Hans
H
Söderdahl
Gunnar
G
Otte
Jean B
JB
Cochat
Pierre
P
Roquet
Olga
O
Jamieson
Neville V
NV
Haffner
Dieter
D
D001827:Body Height; D002648:Child; D005260:Female; D006130:Growth Disorders / Q000175:diagnosis*; D006801:Humans; D006960:Hyperoxaluria, Primary / Q000601:surgery*; D016030:Kidney Transplantation; D016031:Liver Transplantation; D008297:Male; D010070:Oxalates / Q000652:urine
D010070:Oxalates