“We report a 51-year-old female patient with adult-onset type II citrullinemia (CTLN2) who had a history of pancreatoduodenectomy for duodenal somatostatinoma with metastases to regional lymph nodes at age 49 Gemcitabine price years, paying special attention
to indications for liver transplantation. At age 50 years, she developed hepatic encephalopathy with elevation of plasma ammonia and citrulline levels. A diagnosis of CTLN2 was made by DNA analysis of the SLC25A13 gene and treatment with conservative therapies was begun, including a low-carbohydrate diet and supplementation with arginine and sodium pyruvate. However, despite these treatments, frequent attacks of encephalopathy occurred with markedly elevated plasma ammonia levels.
While we were apprehensive regarding the risk of recurrence of somatostatinoma due to immunosuppressive therapy after liver transplantation, the patient was in a critical condition with CTLN2 and it was decided to perform living-donor liver transplantation using a graft obtained from her son. Her MG-132 manufacturer postoperative clinical course was uneventful and she has had an active life without recurrence of somatostatinoma for 2 years. This is the first case of CTLN2 with somatostatinoma. Acetophenone As the condition of CTLN2 patients with rapidly progressive courses is often intractable by conservative therapies alone, liver transplantation should be considered even after surgery for malignant tumors in cases with neither metastasis nor recurrence. “
“See Articles on Pages 1201 and 1214. Natural killer (NK) cells play an important role in innate immune response and are essential in the host’s first-line defense against viral infections. A major hallmark of NK cells
is their ability to kill infected cells without requiring previous immunization and to produce large amounts of antiviral effector cytokines, including interferon gamma (IFN-γ) and tumor necrosis factor alpha (TNF-α). NK cells also play an important role in the priming and regulation of adaptive immune responses. For example, NK cells can regulate T-cell responses by lysing virus-infected antigen-presenting cells or by cytolytically eliminating activated CD4 T cells that affect CD8 T-cell function and exhaustion, as has been recently demonstrated in the lymphocytic choriomeningitis virus mouse model.1, 2 Accordingly, in that model, NK-cell depletion causes enhanced T-cell immunity that may lead to rapid virus control and prevention of chronic infection.