Protein-losing enteropathy after orthotopic liver transplantation: a case series of five patients
(2017) 45th Edition BVK/SBP Congress — Location: Anvers, Belgique (23.March.2017)
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- Introduction Protein-losing enteropathy (PLE) is an uncommon cause of diarrhea and hypoalbuminemia, due to abnormally increased intestinal loss of proteins. Loss of albumin can reach 60% of its turnover, while normally it does not exceed 10%. Concomitantly there can be malabsorption of long chain triglycerides (LCT) and fat-soluble vitamins (FSV). Symptoms are unspecific: diarrhea, edema, ascites, pleural and pericardial effusion (because of (b/o) hypoproteinemia), recurrent infections (b/o hypogammaglobulinemia and T-lymphocyte leakage), thrombo-embolic events (b/o deficiency in anticoagulant proteins), weight gain (b/o fluid retention) or weight loss (b/o malabsorption) and growth retardation. Many etiologies can cause PLE, it can also occur after orthotopic liver transplantation (OLT). Aim To review the causes and treatment of PLE after OLT. Methods At Cliniques universitaires Saint-Luc in Brussels, PLE was diagnosed in five children following liver transplantation (over a total of 1032 OLT). Based on Braamskamp et al., we established a systematic approach of PLE. When PLE is clinically and biologically suspected, fecal α-1-antitrypsin (A1AT) loss is increased and can be measured by 24-hours stool collection. Clearance above 24ml/24h, in absence of diarrhea, or 56ml/24h, in presence of diarrhea, is suggestive of PLE. Alternatively, scintigraphy confirms PLE by showing an increased fecal excretion of radio-isotopes (e.g. Tc-99m Human Serum Albumin, In-111 transferrin) and the site of leakage. Further investigations are necessary to find the underlying etiology. Results Child 1 with progressive familial intrahepatic cholestasis type 1 (PFIC1) developed one month (mo) after OLT (performed at 3years(yrs)3mo) severe diarrhea and dehydration, with partial benefit of cholestyramine. The child needed repeatedly albumin and plasma infusions and FSV supplementation. Its situation remained unchanged for 2yrs. Child 2 with PFIC1 presented 3mo post OLT (performed at 2yrs10mo) with diarrhea and vomiting, dehydration and ionic disorders. Three children underwent OLT at 1yr5mo, 2yrs9mo and 9mo for decompensated cirrhosis secondary to biliary atresia (BA) after failure of Kasaï procedure. Child 3 developed diarrhea, hypoalbuminemia and iron deficiency anaemia 4yrs2mo post OLT. Child 4 presented after 3yrs3mo with diarrhea, facial and scrotal edema. Child 5 suffered 3mo later of diarrhea and intermittent fever. Blood samples showed hypoproteinemia. Levels of gammaglobulins, vitamin A and D were low, except for child 5, for whom gammaglobulins were normal, vitamins A and D were not measured. Transferrin-In111 scintigraphy proved PLE (not done for child 5). Liver biopsies showed moderate fibrosis. Child 1 underwent external, followed by internal biliary diversion which normalized PLE. For child 2 PLE was controlled following ursodeoxycholic acid (UDCA) treatment, with cholestyramine, iron and FSV supplementation. Food allergies were thought to cause PLE in child 3, as only radioallergosorbent tests appeared pathologic, but PLE persisted for 4yrs, despite a restricted diet. A sus-hepatic stenosis was subsequently demonstrated, and treated by balloon venoplasty with resolution of PLE. For child 4, intestinal lymphangiectasia (IL) were seen by enteroscopy. Hepatic vein catheterization was normal. The child was on a restrictive diet with supplementation of MCT, calcium and vitamin D and received repeatedly LCT (b/o skin problems), albumin and gammaglobulins infusions. Six years after OLT, symptoms were still present and no mechanical, vascular or immunological explanation was found. There was a moderate improvement under Somatostatin. External biliary diversion was then performed, with a current improvement of his PLE, and marked decrease of albumin infusion requirements. For child 5, intestinal biopsies were suggestive of post-transplant lymphoproliferative disease (PTLD), with positive EBV PCR. It was treated by methylprednisolone, albumin infusions and Rituximab, and PLE resolved concomitantly to its PTLD Conclusions PLE is an uncommon complication after OLT. PFIC1 patients might be prone to this complication, attributed to restoration of normal bile flow in a bowel unable to process bile acid transport. Biliary diversion corrected PLE in one PFIC1 and one BA patient. A second PFIC patient responded to UDCA and cholestyramine. Other etiologies included sus-hepatic vein stenosis and PTLD, and in both cases, PLE resolved after treatment of the underlying condition.
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Perdaens, O., Bessalah, L., Varma, S., & Sokal, E. (2017). Protein-losing enteropathy after orthotopic liver transplantation: a case series of five patients. 45th Edition BVK/SBP Congress, Anvers, Belgique. https://hdl.handle.net/2078.5/87271