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- Armstead, Sumiko I, et al.
(författare)
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A Case of Alport Syndrome with Posttransplant Antiglomerular Basement Membrane Disease despite Negative Antiglomerular Basement Membrane Antibodies by EIA Treated with Plasmapheresis and Intravenous Immunoglobulin.
- 2013
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Ingår i: Case reports in transplantation. - : Hindawi Limited. - 2090-6943 .- 2090-6951. ; 2013:Dec 2
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Tidskriftsartikel (refereegranskat)abstract
- Posttransplant antiglomerular basement membrane (anti-GBM) disease occurs in approximately 5% of Alport patients and usually ends in irreversible graft failure. Recent research has focused on characterizing the structure of the anti-GBM alloepitope. Here we present a case of a 22-year-old male with end-stage renal disease secondary to Alport syndrome, with a previously failed renal allograft, who received a second deceased-donor kidney transplant. Six days after transplantation, he developed acute kidney injury. The serum anti-GBM IgG was negative by enzyme immunoassay (EIA). On biopsy, he had crescentic glomerulonephritis with linear GBM fixation of IgG. With further analysis by western blotting, we were able to detect antibodies to an unidentified protein from the basement membrane. This patient was treated with plasmapheresis twice per week and monthly intravenous immunoglobulin (IVIG) for a total of five months. At the end of treatment, these unknown antibodies were no longer detected. His renal function improved, and he has not required dialysis. We conclude that anti-GBM disease in patients with Alport Syndrome may be caused by circulating antibodies to other components of the basement membrane that are undetectable by routine anti-GBM EIA and may respond to treatment with plasmapheresis and IVIG.
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| 2. |
- Ivanics, Tommy, et al.
(författare)
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Combined Chylothorax and Chylous Ascites Complicating Liver Transplantation: A Report of a Case and Review of the Literature
- 2019
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Ingår i: Case Reports in Transplantation. - : Hindawi Limited. - 2090-6943 .- 2090-6951.
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Tidskriftsartikel (refereegranskat)abstract
- Chyle leaks may occur as a result of surgical intervention. Chyloperitoneum, or chylous ascites after liver transplantation, is rare and the development of chylothorax after abdominal surgery is even more rare. With increasingly aggressive surgical resections, particularly in the retroperitoneum, the incidence of chyle leaks is expected to increase in the future. Here we present a unique case of a combined chylothorax and chyloperitoneum following liver transplantation successfully managed conservatively. Risk factors for chylous ascites include para-aortic manipulation, extensive retroperitoneal dissection, use of a Ligasure device, and early enteral feeding as well as early enteral feeding. The clinical presentation is typically insidious and may include painless abdominal distension. Diagnosis can be made by noting characteristic milky white drainage which on laboratory examination has a total fluid triglyceride level >110 mg/dl, an ascites/serum triglyceride ratio of >1 and a leukocyte count in fluid >1000/uL with a lymphocyte predominance. Chyle leaks may lead to significant morbidity and mortality. Numerous management options exist, with conservative nonoperative measurements leading to the most consistent and successful outcomes. This includes a step-up approach beginning with dietary modifications to a low-fat or medium chain triglyceride diet followed by nil per os with addition of total parenteral nutrition and somatostatin analogues such as octreotide. Rarely do patients require more invasive treatment. Early recognition and appropriate management are imperative to mitigate this complication.
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