Non-cirrhotic portal hypertension (obliterative portal venopathy) is the predominant form of chronic liver disease in cystic fibrosis.

BACKGROUND: Cystic fibrosis (CF)-associated liver disease commonly manifests as portal hypertension and its complications. We investigated the proposal that the pathophysiology is of non-cirrhotic rather than cirrhotic portal hypertension. This distinction may have important implications for treatment. METHODS: We compared liver transplant explants from cystic fibrosis patients with explants from patients with classical cholestatic diseases, primary biliary cholangitis and primary sclerosing cholangitis. Presence of cirrhosis, fibrosis, nodular regenerative hyperplasia, biliary and portal venous pathology were recorded. Quantitation of portal venules in representative section was performed. RESULTS: Nine patients with cystic fibrosis liver disease, 7 primary biliary cholangitis (PBC) and 7 primary sclerosing cholangitis (PSC) were evaluated. Cirrhosis was present in 0/9 of CF patients and 11/14 of the PBC and PSC controls (p < 0.01). Nodular regenerative hyperplasia was present in 8/9 of the CF patients but none of the controls (p < 0.01). Portal venule numbers per 15 mm2 section were significantly lower in the CF patients 52 (20-72) compared to the primary biliary cholangitis 78 (47-110) and primary sclerosing cholangitis patients, 79 (41-134) (p < 0.05). Portal sclerotic nodules were found in all the CF patients but in only one of the controls (9/9 vs 1/14 p < 0.01). CONCLUSIONS: This study demonstrates that non-cirrhotic portal hypertension or obliterative portal venopathy is the predominant hepatic pathophysiology in adult CF patients requiring liver transplantation. It suggests that treatments directed at the hepatic portal venous system may be more effective than current treatment directed at the biliary system in cystic fibrosis.

Generalist vs specialist acute medical admissions - What is the impact of moving towards acute medical subspecialty admissions on efficacy of care provision?

INTRODUCTION: The discussion surrounding generalist versus specialist acute medical admissions continues to stimulate debate and patients with certain conditions benefit from specialist care. AIM: To determine whether a specialty medical admission program would reduce inpatient length of stay (LOS), mortality and readmission rates. DESIGN/METHODS: A prospective cohort study of inpatients admitted under a general internal medicine (GIM) service before and after introduction of a specialty-directing programme. We hypothesized that early transfer of patient care to a specialty suited to their presenting complaint would reduce LOS and a specialty-directing early redistribution of care programme was introduced. Seven of the ten clinical teams participating in the GIM roster adopted the programme. On the morning following a specialty-directing team being on call for all new GIM admissions during a 24-hour period, specialty-directing teams were allocated one patient appropriate to their specialty. RESULTS: 5,144 patient-care episodes were analysed over the two-year study period. LOS increased by greater than 15%, one year after introducing the specialty-directing programme (8.5±8.4 vs 7.3±7.5 days, p < 0.001). LOS did not differ between teams that participated and those who did not (8.4±8.1 vs 8.1±7.9 days, p = 0.298). No differences were found in the proportion of patients who were discharged home, died while an inpatient or re-admitted within 30 days of discharge. The proportion of patients aged greater than 80 years increased significantly also - from 24.7% in 2017 to 27.9% in 2019(p == 0.009). CONCLUSION: Widespread adoption of specialist care may not be beneficial for all medical inpatients and physicians should continue to undergo dual specialist and GIM training.