Propionic acidemia is a rare autosomal recessive inborn error of metabolism. It is relatively common in Middle East. Dilated cardiomyopathy is one of the leading causes of morbidity and mortality for patients with propionic acidemia. Liver transplantation has been used for patient with frequent metabolic decompensations and was shown to be beneficial in propionic acidemia-related dilated cardiomyopathy. Up to our knowledge, there has been one reported case of recurrent dilated cardiomyopathy 3 years after liver transplantation. We report the first case, from Middle East, of recurrent dilated cardiomyopathy, 6 years after liver transplantation.

Objective of the study was to assess subjective global nutritional assessment (SGNA) in children with chronic liver diseases (CLD). Children aged 3 months to 18 years with CLD were prospectively enrolled (January 2016 to October 2018). SGNA was performed as per validated pro forma for children. Nutritional categories were categorised into three groups: A (well-nourished), B (moderately malnourished) and C (severely malnourished). Agreement between SGNA and anthropometric measures, prediction of morbidity and death or liver transplantation (LT) at 1-year post-enrolment by SGNA and inter-observer reliability of SGNA were assessed. Ninety-two subjects were enrolled, median age 23·5 (3–216) months. SGNA classified 47 patients (51·1 %) in group A, 26 (28·3 %) in group B and 19 (20·6 %) in group C. Kendall coefficients disclosed significant association of SGNA with all anthropometric measurements, greatest with weight for age (r = −0·637), height for age (r = −0·581) and mid-arm fat area (r = −0·449). At 12 months follow-up, twenty children died and four received LT. A significantly higher number of children with malnutrition (groups B and C) had poor outcome (OR 6·74 (95 % CI 2·21, 20·55), P = 0·001), increased risk of hospital readmission (OR 12·2 (95 % CI 4·60, 35·88), P = 0·001), higher rate of infectious complications (OR 22·68 (95 % CI 7·29, 70·53), P < 0·0001) and lower median survival with native liver (Log Rank < 0·001) as compared with group A. Inter-observer agreement in assessment of SGNA was good (90·2 %). SGNA, in contrast to anthropometric measures, is a better nutritional assessment tool. It is reliable, comprehensive and predicts poor outcome in childhood CLD.

Liver transplantation for patients with atrial septal defect and pulmonary artery stenosis, causing high right atrium pressure, raises concerns about embolism in systemic vessels during reperfusion of the donor liver graft. Temporal atrial septal defect occlusion by a catheter is a simple and easy method of preventing the complication.

Severe pulmonary hypertension is a contraindication for liver transplantation owing to high mortality. However, decision-making regarding the treatment approach for patients with bilateral peripheral pulmonary artery stenosis, typically complicated by elevated main pulmonary artery and right ventricle pressures, can be challenging. Here, we report successful living donor liver transplantation after bilateral pulmonary artery stent implantation in a patient with Alagille syndrome, severe bilateral peripheral pulmonary artery stenosis, and extremely high main pulmonary artery and right ventricle pressures.

Objectives: Our study addresses the important issue of estimating treatment costs from historical data. It is a problem frequently faced by health technology assessment analysts. We compared four approaches used to estimate current costs when good quality contemporary data are not available using liver transplantation as an example.

Methods: First, the total cost estimates extracted for patients from a cohort study, conducted in the 1990s, were inflated using a published inflation multiplier. Second, resource use estimates from the cohort study were extracted for hepatitis C patients and updated using current unit costs. Third, expert elicitation was carried out to identify changes in clinical practice over time and quantify current resource use. Fourth, routine data on resource use were obtained from National Health Service Blood and Transplant (NHSBT).

Results: The first two methods did not account for changes in clinical practice. Also the first was not specific to hepatitis patients. The use of experts confirmed significant changes in clinical practice. However, the quantification of resource use using experts is challenging as clinical specialists may not have a complete overview of clinical pathway. The NHSBT data are the most accurate reflection of transplantation and posttransplantation phase; however, data were not available for the whole pathway of care. The best estimate of total cost, combining NHSBT data and expert elicitation, is £121,211.

Discussion: Observational data from routine care are potentially the most reliable reflection of current resource use. Efforts should be made to make such data readily available and accessible to researchers. Expert elicitation provided reasonable estimates.

Surgical advances have allowed for the development of split liver transplantation, providing two recipients with the opportunity to potentially benefit from one donated liver by splitting the liver into two usable parts. Although current data suggest that the splitting of livers provides overall benefit to the liver-recipient population, relatively low numbers of livers are actually split in the United Kingdom. This article addresses the question of whether ethical concerns are posing an unnecessary barrier to further increasing the number of life-saving transplantations. Recognizing that an important aspect of exploring these concerns is gaining insight into how transplant staff and patients regard splitting livers, the article presents the findings of a qualitative study examining the views of senior transplant staff and liver transplant patients in the UK and uses these to inform a commentary on the ethical issues relating to split liver transplantation.

Summary

Background and objectives: Positive end-expiratory pressure may alter cardiac function and systemic haemodynamics. As transplanted livers may be sensitive to liver congestion, the aim of our study was to evaluate the effect of positive end-expiratory pressure on the cardiovascular system and in particular on central venous and iliac venous pressure in liver transplanted patients. Patients and methods: Seventy-two liver transplant patients were enrolled in this prospective, interventional study. On admission to our Intensive Care Unit all patients were ventilated in a biphasic positive airway pressure mode. Haemodynamic effects of three randomly set levels of end-expiratory pressures (0, 5 and 10 mbar) were studied in the immediate postoperative period in all patients. Mean arterial pressure, central venous pressure, pulmonary capillary wedge pressure, central iliac venous pressure and cardiac index were recorded and analysed at each of the three end-expiratory pressure levels. Results: The values of central- and wedge-pressure significantly increased with increased end-expiratory pressure. Central venous pressure increased by 24% and wedge pressure showed a 6% increase at 10 mbar in comparison to 0 mbar. The values for cardiac index and mean arterial pressure showed no statistically significant difference at 10 mbar as compared to 0 and 5 mbar. The mean pulmonary arterial and common iliac venous pressure were unaffected by different positive end-expiratory pressure levels. Conclusions: Short-term pressure controlled ventilation with end-expiratory pressure up to 10 mbar does not significantly impair systemic haemodynamics in liver-transplanted patients. Further studies are needed to determine whether these findings could be confirmed with higher pressure levels and/or over a longer period of ventilation time.

Summary

Background and objective: End-stage liver disease is associated with an imbalance in the autonomic nervous system. The purpose of this study was to estimate the effect of liver transplantation on this imbalance. Method: The study involved 10 patients undergoing liver transplantation and 9 patients without liver impairment undergoing liver surgery. The spontaneous baroreflex sensitivity was measured before and 1 month after surgery for the liver surgery group; before and 1, 3, 6, 12 and 18 months after orthotopic liver transplantation. Results: The spontaneous baroreflex slope of patients with end-stage liver disease was decreased before liver transplantation compared to the liver surgery group (3.9 ± 2.5 ms mmHg−1 vs. 9.9 ± 5.0 ms mmHg−1, P = 0.002). The mean slope was significantly increased at 12 and 18 months compared to the pre-transplantation value (3.9 ± 2.5 ms mmHg−1 vs. 8.1 ± 6.6 ms mmHg−1 and 7.4 ± 4.8 ms mmHg−1, respectively; P = 0.042). Nevertheless, further analysis of individual data showed that only four patients exhibited a marked increase in their baroreflex slope 12 months after the liver transplantation whereas it remained decreased in the six others. Conclusions: These results confirm that the baroreflex sensitivity is depressed in end-stage liver disease in line with an autonomic nervous system imbalance. The liver transplantation reverses this disturbance only in some patients.