Simultaneous liver-kidney transplantation for glycogen storage disease type IA (von Gierke's disease)
| Autor: | M. Miggino, G. Bottino, Tomasz Jarzembowski, Fabrizio Panaro, M. Bertocchi, L Bonifazio, D Ghinolfi, S DiDomenico, Umberto Valente, G. Basile, Nicola Morelli, Iris Fontana, Enzo Andorno, L Saltalamacchia |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2004 |
| Předmět: |
Adult
medicine.medical_specialty Adenoma Glucose 6 Phosphatase Glycogen Storage Disease Type Ia Liver Kidney Transplantation Disease Glycogen Storage Disease Type I Gastroenterology Glycogen storage disease type Ia Renal Dialysis Internal medicine medicine Hepatectomy Humans Stage (cooking) business.industry medicine.disease Kidney Transplantation Surgery Liver Transplantation medicine.anatomical_structure Treatment Outcome Lactic acidosis Female business Kidney disease |
| Popis: | Introduction Glycogen storage disease type Ia (GSDIa) is due to the deficiency of glucose-6-phosphatase activity in the liver, kidney, and intestine. Although significant progress has been achieved in the management of patients with GSDIa, complications still emerge. The potential for development of liver adenomatosis and kidney failure makes these patients candidates for simultaneous liver-kidney transplantation (SLKT). Herein, we describe such a transplantation in a patient affected by this rare storage disease. Methods A 25-year-old female patient with GSDIa developed hepatic adenoma and kidney failure despite dietary therapy. The patient underwent an SLKT from a cadaveric donor. Results The operative time was 8 hours without hemotransfusion. Only a transitory lactic acidosis was observed. Laboratory results normalized on postoperative day 7. The patient was discharged on postoperative day 9. After 4 months, the patient is in good condition with well-functioning kidney and liver allografts. Conclusion Patients with end-stage renal disease secondary to GSDIa should be considered for SLKT, especially when the disease is in an early stage. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|