Kidney Transplantation in Abernethy Malformation: A Case Report |
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| Affiliation: | 1. Clinical Department of General, Oncological, Gastroenterological Surgery and Transplantation, University Hospital in Krakow, Poland;2. Clinical Department of Nephrology, Dialysis and Transplantation, University Hospital in Krakow, Poland;1. Departments of Ophthalmology, Transplantology, and Internal Diseases, Medical University of Gdansk, Gdansk, Poland;2. Departments of Nephrology, Transplantology, and Internal Diseases, Medical University of Gdansk, Gdansk, Poland;1. Department of Cardiac, Vascular and Endovascular Surgery and Transplantology, Silesian Center for Heart Diseases in Zabrze, Medical University of Silesia, Katowice, Poland;2. Department of General, Vascular and Transplant Surgery, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland;1. Department of Nephrology, Dialysis and Internal Diseases, Medical University of Warsaw, Warsaw;2. Department of Anaesthesiology and Intensive Care, Medical University of Warsaw, Warsaw;3. Department of General, Transplant and Liver Surgery, Medical University of Warsaw, Warsaw;4. Department of Medical Informatics and Telemedicine, Medical University of Warsaw, Warsaw, Poland;1. Department of Cardiac, Vascular and Endovascular Surgery and Transplantology, Medical University of Silesia, Silesian centre for Heart Diseases, Zabrze, Poland;2. Department of Cardiac Surgery, Transplantology and Mechanical Circulatory Support in Children, Silesian Center for Heart Diseases, Zabrze, Poland;3. Faculty of Medical Sciences in Zabrze, Medical University of Silesia, Katowice, Poland;1. Department of Biophysics, Division of Laboratory Medicine, School of Pharmacy, Medical University of Silesia in Katowice,Sosnowiec, Poland;2. Laboratory of Transplant Immunology, Silesian Centre for Heart Diseases, Zabrze, Poland;3. Department of Cardiac, Vascular and Endovascular Surgery and Transplantology, Medical University of Silesia in Katowice, Silesian Centre for Heart Diseases, Zabrze, Poland |
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| Abstract: | Abernethy malformation is a rare disorder defined by congenital portosystemic shunt. Advances in clinical imaging have led to increased identification of this anomaly, which has proven to be more common and more clinically diverse than previously assumed. Late presentations are not uncommon. We present a 35-year-old patient with type Ia Abernethy malformation and biopsy-confirmed mesangiocapillary glomerulonephritis who was referred for deceased donor kidney transplantation. After the diagnosis was confirmed, the patient remained stable and asymptomatic on a supervised low-protein, high-carbohydrate diet. The patient received the kidney transplant from a brain-dead donor with standard characteristics. The procedure was uneventful; no vascular or vesical abnormalities could be identified at the surgical site. Recovery was uneventful with excellent graft function. Unique issues with immunosuppression were identified. Pharmacologic adjustments accounting for congenital complete portosystemic shunting affecting liver first pass effect as well as multiple drug interactions were necessary and sufficient. Abernethy malformation may follow indolent course into adulthood and may be an unrelated finding in a patient with chronic kidney disease. Kidney transplantation proved to be feasible and safe in this young male with apparently efficient compensatory mechanisms. |
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