Health-related Quality of Life After Pediatric Liver Transplant: Single-Center Experience in Chile

IntroductionOrthotopic liver transplantation is the treatment of choice for most terminal liver diseases in children. Currently, the improved survival of these patients is well documented, but their quality of life post-transplant is not described. In Chile, Hospital Luis Calvo Mackenna (HLCM) has performed pediatric liver transplantation in children from around the country since 1994. The aim of this study is to evaluate our patients' and parents' current quality of life.MethodsA cross-sectional study was conducted between July 2010 and June 2011. All liver transplant patients currently in control at HLCM were invited to complete the PedsQL 4.0 report (Pediatric Quality of Life Inventory). For each group, average score was calculated and comparisons were done using Student t and χ² tests.ResultsForty-nine patients were enrolled. One-third of the patients were between 2 and 4 years, one-third between 5 and 12, and the rest were 13 to 18 years old. Half of the patients had their transplants for more than 3 years, 53% were female, 53% lived in cities far from the transplant center, 72.5% had chronic liver disease, 53% had received a liver from cadaveric donor, and 21% had received more than 1 liver transplant. Patients under 4 years referred good health-related quality of life (HRQOL) in all categories. All school-age patients had poor or very poor psychosocial HRQOL.DiscussionOur good results obtained in transplant patients under 4 years may be because the questionnaire was completed by caregivers. The school-age patients were affected in terms of school functioning, as they were not able to participate in all the activities. These findings need to be compared with HRQOL perception in other groups, such as children with other chronic conditions, and evaluated with other broader factors, as reported in international HRQOL publications.     

Autoimmune thyroid diseases and Helicobacter pylori: the correlation is present only in Graves's disease

AIM: To investigate the correlation between autoimmune thyroid diseases (ATDs) and the prevalence of Cag-A positive strains of Helicobacter pylori (H. pylori) in stool samples.METHODS: Authors investigated 112 consecutive Caucasian patients (48 females and 4 males with Graves’ disease and 54 females and 6 males with Hashimoto’s thyroiditis HT), at their first diagnosis of ATDs. Authors tested for H. pylori in stool samples using an amplified enzyme immunoassay and Cag-A in serum samples using an enzyme-linked immunoassay method (ELISA). The results were analyzed using the two-sided Fisher’s exact test and the respective odds ratio (OR) was calculated.RESULTS: A marked correlation was found between the presence of H. pylori (P ≤ 0.0001, OR 6.3) and, in particular, Cag-A positive strains (P ≤ 0.005, OR 5.3) in Graves’ disease, but not in Hashimoto’s thyroiditis, where authors found only a correlation with Cag-A strains (P ≤ 0.005, OR 8.73) but not when H. pylori was present.CONCLUSION: The marked correlation between H. pylori and Cag-A, found in ATDs, could be dependent on the different expression of adhesion molecules in the gastric mucosa.     

Effects of aging and methionine restriction applied at old age on ROS generation and oxidative damage in rat liver mitochondria

It is known that a global decrease in food ingestion (dietary restriction, DR) lowers mitochondrial ROS generation (mitROS) and oxidative stress in young immature rats. This seems to be caused by the decreased methionine ingestion of DR animals. This is interesting since isocaloric methionine restriction in the diet (MetR) also increases, like DR, rodent maximum longevity. However, it is not known if old rats maintain the capacity to lower mitROS generation and oxidative stress in response to MetR similarly to young immature animals, and whether MetR implemented at old age can reverse aging-related variations in oxidative stress. In this investigation the effects of aging and 7 weeks of MetR were investigated in liver mitochondria of Wistar rats. MetR implemented at old age decreased mitROS generation, percent free radical leak at the respiratory chain and mtDNA oxidative damage without changing oxygen consumption. Protein oxidation, lipoxidation and glycoxidation increased with age, and MetR in old rats partially or totally reversed these age-related increases. Aging increased the amount of SIRT1, and MetR decreased SIRT1 and TFAM and increased complex IV. No changes were observed in the protein amounts of PGC1, Nrf2, MnSOD, AIF, complexes I, II and III, and in the extent of genomic DNA methylation. In conclusion, treating old rats with isocaloric short-term MetR lowers mitROS production and free radical leak and oxidative damage to mtDNA, and reverses aging-related increases in protein modification. Aged rats maintain the capacity to lower mitochondrial ROS generation and oxidative stress in response to a short-term exposure to restriction of a single dietary substance: methionine.     

Health related quality of life in Middle Eastern children with beta-thalassemia

ABSTRACT: BACKGROUND: Thalassemia is a common disorder worldwide with a predominant incidence in Mediterranean countries, North Africa, the Middle East, India, Central Asia, and Southeast Asia. Whilst substantial progress has been made towards the improvement of Health related quality of life (HRQoL) in western countries, scarce evidence-based data exists on HRQol of thalassemia children and adolescents living in developing countries. Patients and methods We studied 60 thalassemia children from Middle Eastern countries with a median age of 10 years (range 5 to 17 years). HRQoL was assessed with the Pediatric Quality of Life Inventory (PedsQL) 4.0. The Questionnaire was completed at baseline by all patients and their parents. The agreement between child-self and parent-proxy HRQoL reports and the relationship between HRQoL profiles and socio-demographic and clinical factors were investigated. RESULTS: The scores of parents were generally lower than those of their children for Emotional Functioning (mean 75 vs 85; p = 0.002), Psychosocial Health Summary (mean 70.3 vs 79.1; p = 0.015) and the Total Summary Score (mean 74.3 vs 77.7 p = 0.047). HRQoL was not associated with ferritin levels, hepatomegaly or frequency of transfusions or iron chelation therapy. Multivariate analysis showed that a delayed start of iron chelation had a negative impact on total PedsQL scores of both children (p = 0.046) and their parents (p = 0.007). CONCLUSIONS: The PedsQL 4.0 is a useful tool for the measurement of HRQoL in pediatric thalassemia patients. This study shows that delayed start of iron chelation has a negative impact on children's HRQOL.     

Effects of whole body vibration training on body composition in adolescents with Down syndrome

The present study aimed to determine the effect of 20 weeks of whole body vibration (WBV) on the body composition of adolescents with Down syndrome (DS). Thirty adolescent with DS were divided into two groups: control and WBV. Whole body, upper and lower limbs body fat and lean body mass were measured with dual energy X-ray absorptiometry (DXA) before and after 20 weeks of WBV training. Repeated measures of ANOVA adjusting by height, weight and Tanner stage were used to analyze possible group by time interactions on body composition. The adjusted percentages of change in body composition were also compared between control and WBV groups. No group by time interactions were found for any variable, but the WBV group showed a higher reduction in body fat at the upper limbs (p < 0.05), and a tendency toward higher percent increase in whole body lean body mass. Overall, a 20-week WBV training is not enough by itself for increasing lean body mass in adolescents with DS, but it might be helpful for improving body composition in this population. Its relationship with health and autonomy enhances the importance of these results.     

Auricular rehabilitation by means of bone grafting from the iliac crest in combination with porous extraoral implants: a case report

Background: Maxillofacial defects caused by cancer treatment are a huge problem affecting the quality of life of patients. Some of these deformities are minimized using facial epitheses, which need some additional retention devices like glasses or skin adhesives. The use of extraoral fixtures as bone anchorage was introduced many years ago and since then many patients were rehabilitated with better results. Purpose: Because of poor bone conditions, for example, irradiated bone, the success rate of extraoral implants is less than in the oral cavity, causing difficulties to rehabilitation. One possible cause of fixture failure could be the poor primary stability achieved in some cases, hence, with an increased bone contact implant stability and survival could be improved. The present report discusses possibilities to use extraoral fixtures with a modified surface structure. Materials and Methods: A new porous surfaced Brazilian extraoral implant (MasterExtra®, Conexão, Sistema de Próteses, São Paulo, Brazil) was used. A bone transplant from the iliac crest was taken to make it possible to insert at least three extraoral implants for an auricle epithesis. Clinical evaluation and resonance frequency analysis (RFA) measurements were performed during the course of the treatment. Results: Eight months after grafting, four fixtures were inserted. Three fixtures were used for connection of an auricular epithesis. RFA measurements did show high initial values and the values remained stable during the course of the treatment and at later checkups. Conclusion: Porous fixture is a good option in areas where the bone is compromised. RFA is a good tool also in the clinical setting to evaluate immediate and long‐term stability of extraoral fixtures.