Progressive Familial Intrahepatic Cholestasis Type 2 and Recurrence After Liver Transplantation: A Case Report.

Progressive familial intrahepatic cholestasis type 2 (PFIC2) is a rare autosomal recessive disorder caused by mutations in the ABCB11 gene. Clinical manifestations include cholestasis with low γ-glutamyltransferase (GGT), hepatosplenomegaly, and severe pruritus. Liver transplantation is required for individuals with progressive liver disease or failure of the bypass procedure and has been considered curative. However, in the case of PFIC2, although bile salt excretory pump (BSEP) deficiency is a liver-specific condition rather than a systemic disease, evidence of recurrent BSEP disease has been shown in a small proportion of allografts. We describe an unusual case of a 21-year-old individual with PFIC2 and evidence of recurrent BSEP disease after liver transplantation, with clinical and laboratory improvement after pulse therapy with methylprednisolone for 3 days and adjustment of oral immunosuppression. This case report highlights the recurrence of PFIC2 in patients post liver transplant. It also emphasizes the importance of clinical suspicion, which should be considered in cases of posttransplant cholestasis in PFIC2 patients, especially those with low γ-glutamyltransferase (GGT) and without signs of acute graft rejection. Having knowledge of the condition favors a targeted diagnostic approach and contributes to early therapeutic management and a higher success rate.

Liver Transplant and Active Ulcerative Colitis: A Case Report.

BACKGROUND: The association between ulcerative colitis (UC) and primary sclerosing cholangitis has been described for several years and can be classified as having a distinct disease phenotype from inflammatory bowel diseases (IBD). The simultaneous occurrence of decompensated liver disease requiring liver transplant and active IBD is a management challenge, considering that these patients may be at increased risk of infections, thromboembolic events, bleeding, and drug hepatotoxicity. CASE PRESENTATION: We describe a case of a 37-year-old patient with UC and sclerosing cholangitis presenting with severe decompensated rectocolitis complicated with thromboembolic phenomena and severe liver dysfunction who underwent liver transplant while using biological therapy to control bowel disease. CONCLUSIONS: This case highlights the evolution of sclerosing cholangitis to liver transplant in patients with decompensated UC. Despite the risk of recurrence, primary sclerosing cholangitis has excellent results after liver transplant. Despite the use of immunosuppression after liver transplant, biological therapy may be necessary to control IBD.

Liver Transplantation After Acute Portal Vein Thrombosis: Case Report.

BACKGROUND: Portal vein thrombosis is a relatively frequent complication in patients with liver cirrhosis. Its detection and management are essential to avoid worsening portal hypertension or liver function complications. This complication can also negatively impact or even preclude liver transplant. CASE PRESENTATION: We report the case of a patient who presented with acute portal vein thrombosis, which allowed the diagnosis of liver cirrhosis and hepatocarcinoma within the Milan criteria. Chemical thrombolysis was performed with a mechanical aspiration of the thrombus, and in a second moment, the patient was submitted to a liver transplant. CONCLUSIONS: Advances in the therapeutic approach to portal vein thrombosis and surgical techniques have allowed the condition to no longer be an absolute contraindication to liver transplantation. Diagnosis in the acute phase is associated with greater therapeutic success, aiming to avoid the extension of thrombosis and achieve portal vein recanalization.