Review article: maximising quality of life while aspiring for quantity of life in end-stage liver disease.

BACKGROUND: With recent advances in the management of chronic liver disease and its complications, the long-term survival in cirrhosis has improved. Therefore, the number of individuals who will spend a significant proportion of their life with end-stage liver disease (ESLD) may continue to rise. Thus, more attention to quality of life (QOL) and its integration with traditional clinical endpoints is needed. AIMS: Recently, there have been many studies looking at treatment outcomes and their impact on the QOL in patients with ESLD. The aim of this review was to summarise and compare the insights gained from these intervention studies and to make concise recommendations to further promote and improve QOL in this patient population. METHODS: A literature search was conducted using PubMed and Web of Science. Search terms "Quality of life" "Cirrhosis" and "end-stage liver disease" were used as MeSH terms or searched in the title of the article. RESULTS: These studies uniformly show significant improvement in health-related QOL (HRQOL) with management of malnutrition, hepatic encephalopathy and ascites. Thus, early recognition and management of these complications are keys to better serve our patients. Early involvement of palliative care also leads to improved quality of end-of-life care. CONCLUSIONS: Complications of cirrhosis including malnutrition, encephalopathy, ascites and variceal bleeding lead to a decrease in HRQOL. Assessment of HRQOL has an important implication for the patient. The findings of this review illuminate the importance of using consistent tools to accurately assess QOL in patients with ESLD.

Systematic review: recurrent autoimmune liver diseases after liver transplantation.

BACKGROUND: Autoimmune liver diseases (AILD) constitute the third most common indication for liver transplantation (LT) worldwide. Outcomes post LT are generally good but recurrent disease is frequently observed. AIMS: To describe the frequency and risk factors associated with recurrent AILD post-LT and provide recommendations to reduce the incidence of recurrence based on levels of evidence. METHODS: A systematic review was performed for full-text papers published in English-language journals, using the keywords 'autoimmune hepatitis (AIH)', 'primary biliary cholangitis and/or cirrhosis (PBC)', 'primary sclerosing cholangitis (PSC)', 'liver transplantation' and 'recurrent disease'. Management strategies to reduce recurrence after LT were classified according to grade and level of evidence. RESULTS: Survival rates post-LT are approximately 90% and 70% at 1 and 5 years and recurrent disease occurs in a range of 10-50% of patients with AILD. Recurrent AIH is associated with elevated liver enzymes and IgG before LT, lymphoplasmacytic infiltrates in the explants and lack of steroids after LT (Grade B). Tacrolimus use is associated with increased risk; use of ciclosporin and preventive ursodeoxycholic acid with reduced risk of PBC recurrence (all Grade B). Intact colon, active ulcerative colitis and early cholestasis are associated with recurrent PSC (Grade B). CONCLUSIONS: Recommendations based on grade A level of evidence are lacking. The need for further study and management includes active immunosuppression before liver transplantation and steroid use after liver transplantation in autoimmune hepatitis; selective immunosuppression with ciclosporin and preventive ursodeoxycholic acid treatment for primary biliary cholangitis; and improved control of inflammatory bowel disease or even colectomy in primary sclerosing cholangitis.

Profile of children with congenital adrenal hyperplasia--a hospital study.

OBJECTIVE: To collect baseline information on congenital adrenal hyperplasia (CAH) and to identify relevant issues specific to this disease in Pakistan. METHODS: A retrospective analysis of medical records of pediatric patients registered for serum 17 hydroxyprogesterone (17-OHP) measurement and documented to have CAH in the period 1987 to 1998 was carried out at The Aga Khan University, Karachi (AKU). The clinical notes were reviewed for documentation of CAH as the diagnosis. RESULTS: Of the 152 children registered for 17-OHP testing, sixty-three were diagnosed with CAH. Salt wasting, simple virilization and non-classical CAH was found in 40 (63%), 18 (29.0%) and 5 (8.0%) patients respectively. Twenty-one (33.9%) patients were incorrectly assigned sex and of these, 20 (32.2%) patients were females who were either considered males or just not assigned gender. Parental consanguinity was found in 33 (52.3%) cases. No case had a history of similar features in either parent but in 19 (30.6%) cases similar features were present in siblings. Sixteen cases (25.4%) had a history of sibling death in the neonatal period and 7 had a history of sibling death in infancy. Maternal obstetric histories identified 3 (4.8%) cases with a history of still birth(s) and 4 (6.4%) with a history of abortion(s). CONCLUSION: Children with CAH should be diagnosed early as a rational and judicious choice of sex assignment is a critical aspect of treatment. The high rate of consanguinity emphasized the need to establish the true incidence of the defect in Pakistani population.

Effects of displacement resulting from ethnic/religious conflict on the growth and body composition of Fulani children in northern Nigeria.

This study, which was carried out in the Jos Plateau in Nigeria, analysed the changes in growth and body composition that occurred among 17 male and 13 female Fulani children (aged 4-13 years) in the 7-month period which followed their displacement from their homes into a temporary camp due to ethnic/religious violence. The heights and weights of the children, as well as their fat, fat-free mass, and phase angle were determined 3 weeks before the crisis and 7 months post-crisis using standard anthropometric methods and bioelectrical impedance analysis. In terms of mean values and relative to growth curves established during the tranquil period immediately preceding the crisis, all but one of the girls grew taller and gained more weight than predicted; two-thirds of the weight gained by the girls was due to fat. With regard to the male subjects, on average, while they grew taller, they gained 30 per cent less in height than predicted. However, the boys did gain 50 per cent more weight than predicted. Unexpectedly, fat accounted for one-half or more of the weight gain in both the boys and girls. In general, the boys did less well than the girls in the months following the crisis. The phase angle of all subjects did not decline significantly during the pre- and post-crisis interval. In general, from the nutritional perspective, the Fulani children coped relatively well during the 7-month period of displacement. The fact that neither the growth nor body composition of the Fulani children deteriorated significantly following the crisis was attributed to the fact that during that period they were receiving adequate and continuous supplies of food. Furthermore, the displacement camp into which the children and their families migrated was located in a secure region of the country and one that was controlled by people whose culture and ethnicity were similar to theirs. Finally, at no time during their 7 months as a displaced population were the children separated from their mothers. In conclusion, this study shows that displacement in general may not necessarily lead to deleterious effects on the growth of children.

Survey of the growth characteristics and body composition of Fulani children in a rural hamlet in northern Nigeria.

This paper reports the results of a cross-sectional study of the growth of Fulani children, aged 1-16 years, living in the Jos Plateau of northern Nigeria. This particular population of Fulani are semi-nomadic pastoralists whose economy and culture are centered on cattle. We measured the heights and weights of 176 girls and 164 boys and determined their body composition parameters (fat content, fat-free mass, and phase angle) using bioelectrical impedance analysis. The body mass index values for the boys and girls were 14.9 and 15.0 kg/m2, respectively. When the heights and weights of the Fulani children were compared against World Health Organization standards, the incidence of stunting and underweight was high: 46 per cent of the girls and 57 per cent of the boys, respectively, had weight Z-scores below -1.0, and 42 per cent and 57 per cent had height Z-scores below -1.0. Even when weight was adjusted for height, the boys and girls fell well below their age- and gender-matched standards. The percentage fat content of the children declined with age such that by age 16 years the fat content of the boys was 10 per cent and that of the girls 20 per cent. Although the Fulani children were significantly shorter and lighter than the international standards, their phase angle value (determined by bioelectrical impedance analysis), which is a measure of body cell mass and the overall vitality and health of tissue membranes, was comparable to those of similarly aged healthy children in the United States. These results indicate that although a large proportion of the Fulani children who inhabit the Jos Plateau are stunted and underweight, the bioelectrical properties of their tissue membranes suggest that they are relatively healthy. It is not known if the slow growth of the Fulani children has a genetic basis or if it is the result of nutritional shortcomings.