Survival and predictors of outcome among patients with decompensated liver disease in a non-liver transplant intensive care unit. Pessimism is historical and unjustified.

BACKGROUND: Recent literature emanating from the United Kingdom and United States has reported decreasing mortality rates in patients with decompensated cirrhosis and organ failures presenting to the intensive care unit (ICU). AIM: To determine if there were comparable outcomes in a single-centre non-transplant unit in Australia. METHODS: A retrospective observational study was conducted in a tertiary, non-liver transplant unit in Sydney, Australia. Admission data and mortality outcomes were collected from patients with cirrhosis non-electively admitted to ICU between 2013 and 2017. Liver-specific and general intensive care scoring tools were also assessed for their discriminative ability to predict short-term prognostic outcomes. RESULTS: Sixty-three patients were admitted with decompensated liver disease who fulfilled the inclusion criteria. The overall hospital mortality was 37% (95% CI: 0.26-0.49). There was no difference in survival based on aetiology of liver disease (P = 0.96) but a significant difference was found based on the presenting diagnosis, with greater survival among patients diagnosed with hepatic encephalopathy on ICU admission (P = 0.02). There was 4% mortality in patients with no organ failure and 52% mortality in those with ≥3 organs in failure (P < 0.001). The ICU prognostic Sequential Organ Failure Assessment score was the better discriminative tool in predicting short-term outcomes when compared to liver prognostic scores. CONCLUSION: The outcomes of this single-centre Australian study align with current overseas literature. These results reinforce and expand on limited local evidence, corroborating the former universal prognostic pessimism towards cirrhotic patients with organ failure as unwarranted.

Post-partum catastrophic antiphospholipid syndrome presenting with shock and digital ischaemia - A diagnostic and management challenge.

Catastrophic antiphospholipid syndrome is a rare multisystem autoimmune condition characterised by rapid development of widespread thrombotic disease and subsequent multi-organ failure. It is the most severe complication of antiphospholipid syndrome, carrying significant morbidity and mortality. We report a patient with post-partum catastrophic antiphospholipid syndrome with cardiac, hepatic, renal and cutaneous manifestations. The diagnostic challenges in establishing a definitive diagnosis in catastrophic antiphospholipid syndrome is discussed, along with the difficulties in managing these patients in the intensive care unit.

Klinefelter syndrome with fabry disease--a case of nondisjunction of the X-chromosome with sex-linked recessive mutation.

A 52 year-old male with Klinefelter syndrome presented with chest tightness and rapid atrial fibrillation with hypotension. His echocardiogram demonstrated symmetrical left ventricular hypertrophy with minimal diastolic dysfunction. Subsequent investigations confirmed the diagnosis of Fabry cardiomyopathy. This is the first reported case of Klinefelter syndrome with homozygous sex-linked recessive mutation presenting primarily with cardiac manifestation.