Portal pressure is of significant prognostic value in primary biliary cholangitis.

BACKGROUND AND AIMS: In other forms of chronic liver disease, measurement of portal pressure is of prognostic value, but this has not yet been established in primary biliary cholangitis (PBC). The aim of the study is to determine the prognostic value of hepatic venous pressure gradient (HVPG) in relation to liver-related survival outcomes, as well as to the development of hepatic decompensation, oesophageal varices and variceal bleeding. METHODS: Baseline HVPG and liver biopsies were obtained in 86 patients followed for 10 years in a controlled trial of colchicine treatment, and subsequently in a long-term observational cohort study for a further 30 years. RESULTS: There were 49 Hepatic deaths in addition to 10 Liver Transplants (Hepatic death/transplant; n = 59). Some of these were associated with a significant variceal bleed within 3 months of death or transplant (Portal hypertension-associated death or transplant; n = 19). There were 63 deaths from all causes. During follow-up, oesophageal varices developed in 26 patients, whilst 17 bled from varices and 32 developed hepatic decompensation over a median follow-up of 18.1 years (1.9-28.5). Baseline HVPG was highly predictive of all 6 clinical outcomes and contributed significant predictive information additional to that provided by Mayo score and Ludwig stage. CONCLUSION: Measurement of baseline portal pressure is of significant prognostic value in primary biliary cholangitis.

Portal hypertension in primary biliary cholangitis: prevalence, natural history and histological correlates.

OBJECTIVES: The histopathological mechanisms underlying portal hypertension in primary biliary cholangitis (PBC) are poorly understood, as is its natural history. We have therefore determined the prevalence, severity and progression of portal hypertension in PBC and investigated whether its presence is related to specific histological lesions. METHODS: Hepatic venous pressure gradient (HVPG) was measured in 86 patients, with 186 assessments over up to 7 years of follow-up and the results correlated with a semiquantitative grading of 8 histological features and nodular regenerative hyperplasia (NRH). RESULTS: Portal hypertension (HVPG >5 mmHg) was present in 88% of all assessments (86% at baseline), and in 45% of patients at baseline was >12 mmHg (high-risk portal hypertension). The rise in portal pressure occurs early in the disease, since 45% of patients with normal serum bilirubin had a raised HVPG, as did 72% of patients with early (Ludwig stages 1 and 2) disease. After baseline, there was a small increase in HVPG over the next 5 years in most patients. In patients with precirrhotic PBC, 82% had portal hypertension and in 34% this was >12 mmHg. Portal pressure correlated significantly with a semiquantitative grading of cholestasis, interface hepatitis and portal tract and sinusoidal fibrosis. NRH was present in only 20% of wedge biopsies. CONCLUSIONS: Portal hypertension commences in the early stages of PBC, long preceding both rises in serum bilirubin and the development of cirrhosis. Around 34% of precirrhotic PBC patients have 'high-risk' portal hypertension, which is associated with lesions in the portal tracts and sinusoids rather than with NRH.

Assessment of three methods of pH probe positioning in preterm infants.

BACKGROUND: In the assessment of gastroesophageal reflux, correct placement of the pH catheter is crucial. This is particularly so in very low birth weight infants where a small error in positioning could give rise to a potentially large error in results. Accepted modes of assessing correct positioning can be problematic in this population of infants and alternative methods were investigated. METHODS: A total of 26 preterm infants (<35 weeks gestation) were enrolled in this study. All infants were suspected of having GOR and pH monitoring was performed. Probe position was assessed using Strobel's formula, manometry and acid-alkali interface and confirmed by chest x-ray. RESULTS: There was a highly significant positive correlation between the calculation of the pH probe position using Strobel's formula and the position on x-ray and a significant positive correlation between the acid/alkali interface and x-ray position. There was no correlation between manometry and x-ray position and this method was also shown to be problematic in its' application. CONCLUSION: The data suggests that it is appropriate to use Strobel's formula as a method of accurate positioning of pH probes in preterm infants, thus avoiding the need for additional x-rays. The acid/alkali interface, when obtainable, is a secondary, satisfactory method to confirm the position calculated by Strobel's formula.