Living Donor Liver Transplantation for Budd-Chiari Syndrome: A Propensity Score-Matched Analysis.

INTRODUCTION: There are unique technical and management challenges associated with living donor liver transplantation (LDLT) for Budd-Chiari Syndrome (BCS). The outcomes of LDLT for BCS in comparison to other indications remain unclear and warrant elucidation. METHODS: Data of 24 BCS patients who underwent LDLT between January 2012 and June 2019 were analyzed. There were 20 adults and 4 children. The early and long-term outcomes of adult LDLT BCS patients were compared to a control group of LDLT patients for other indications and matched using propensity scoring methodology. RESULTS: Primary BCS was observed in 18 (90%) patients. Caval replacement was performed in 7 (35%) patients. Early and late hepatic venous outflow tract (HVOT) complications were seen in 1 (5%) and 3 (15%) patients. Preoperative acute kidney injury was identified as a risk factor for mortality in the BCS cohort (p = 0.013). On comparison, BCS recipients were younger with fewer comorbidities, more large volume ascites and higher rates of PVT. They also had longer cold ischemia time, increased blood loss and transfusion requirements, increased hospital stay, and higher late outflow complications. The 1-year and 3-year survivals were similar to non-BCS cohort (84.2% vs. 94% and 71.3% vs. 91.9%, respectively, log rank test p = 0.09). CONCLUSION: LDLT is a good option for symptomatic BCS who have failed non-transplant interventions. The clinical and risk factor profile of BCS recipients is distinct from non-BCS recipients. By following an algorithmic management protocol, we show on propensity-score matched analysis that outcomes of LDLT for BCS are similar to non-BCS indications.

An all-consuming case.

This is a case of a 6-month-old child who presented to the local paediatric department with a relatively short history of developmental regression, hypotonia, weight loss, irritability and hepatosplenomegaly. The child then proceeded to spike temperatures up to 39°C. The case demonstrates the logical investigative process involved in such a case and discusses the differential diagnoses at each stage. It also highlights the importance of multi-specialty work in complex cases and the potential consequence of premature closure of diagnosis.

Invasive Cryptococcus neoformans infection in an asplenic patient.

Individuals who are asplenic or have impaired splenic function are at increased risk of developing life-threatening infections, especially due to encapsulated bacteria. This risk is higher in children, but adults can also develop fulminant infection or "post splenectomy sepsis" (PSS). Cryptococcus neoformans is an encapsulated yeast usually causing infection in immunocompromised patients. In a recent review of cryptococcal infection in HIV-negative patients, splenectomy was reported to be a risk factor for infection in 3% of cases. Detailed case reports are lacking. Here we report a case of disseminated C. neoformans infection in a patient who had a splenectomy performed for warm autoantibody haemolytic anaemia some months before he presented with signs and symptoms of meningitis. This report aims to raise awareness of the possibility of C. neoformans infection in asplenic patients.