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Body growth after combined liver-kidney transplantation in children with primary hyperoxaluria type 1
Richard Nissel1, Kay Latta, Marie-France Gagnadoux
1Department of Pediatric Nephrology, Charité Children's Hospital, Berlin, Germany. richard.nissel@med.uni-rostock.de
Insights
Combined liver and kidney transplantation (LKT) in children with primary hyperoxaluria type 1 (PH1) improves height but does not fully correct growth failure. Many patients still experience reduced final height due to pre-existing growth retardation.
Area of Science:
- Pediatric Nephrology
- Metabolic Disorders
- Transplantation Immunology
Background:
- Primary hyperoxaluria type 1 (PH1) causes severe growth failure in children due to chronic kidney disease and oxalate deposition.
- Combined liver and kidney transplantation (LKT) addresses the metabolic defect and restores kidney function in PH1 patients.
Purpose of the Study:
- To evaluate the longitudinal growth and final height of children with PH1 after LKT.
- To identify factors influencing catch-up growth post-LKT.
Main Methods:
- Analysis of growth data from 24 children with PH1 undergoing LKT across nine European centers.
- Mean follow-up duration of 5.7 years, with a mean age of 8.9 years at transplantation.
Main Results:
- Mean standardized height increased from -1.79 SD to -1.47 SD post-LKT.
- Mean adult heights were 167 cm for boys and 158 cm for girls; 7 of 24 patients remained stunted.
- Catch-up growth correlated positively with pre-LKT stunting and negatively with prednisolone dosage (explaining 39% of variability).
Conclusions:
- LKT in PH1 children does not typically result in complete catch-up growth.
- A significant proportion of patients achieve reduced final height due to pre-existing growth deficits at the time of LKT.
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.
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