Orthotopic liver transplantation in a patient with carbamyl phosphate synthetase deficiency and cystic fibrosis
| Autor: | Urs P. Steinbrecher, S Sirrs, Eric M. Yoshida, S. R. Erb, Stephen W. C. Chung, C Hartnett, Ltk Wong, Agf Davidson, Yolanda Lillquist, C. H. Scudamore |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2003 |
| Předmět: |
medicine.medical_specialty
Pathology business.industry medicine.medical_treatment Case Report Liver transplantation medicine.disease Gastroenterology Cystic fibrosis Pulmonary function testing Transplantation Quality of life Inborn error of metabolism Internal medicine Diabetes mellitus Metabolic control analysis Pediatrics Perinatology and Child Health medicine business |
| Popis: | A 15-year-old female with carbamyl phosphate synthetase deficiency, cystic fibrosis, and cystic fibrosis-related diabetes underwent orthotopic cadaveric liver transplantation. Metabolic control was maintained during the procedure with nutritional support and the use of intravenous sodium phenylacetate and benzoate. Her postoperative course was complicated by seizures and a transient decline in her pulmonary function tests, which returned to preoperative levels within one year of the transplant. Now, four years post-transplant, her quality of life has dramatically improved. There are only four Canadian centres with paediatric liver transplantation programs. However, expert medical care for adults with inborn error of metabolism is even more limited, suggesting that access to adult medical care is one of the many factors to be considered when liver transplantation is contemplated for patients with metabolically unstable conditions. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|