Health-Related Quality of Life 10 Years after Liver Transplantation: A Longitudinal Retrospective Review.

As survival post-liver transplantation (LTx) improves, it becomes increasingly important to understand how long-term health-related quality of life (HRQOL) is impacted. This was a longitudinal review examining HRQOL measured by Pediatric Liver Transplant Quality of Life (PeLTQL) in children between 8-17 years who underwent LTx (1.4 [0.8-3.3] years) at least one year prior to assessment. Demographic, medical, anthropometric, and HRQOL data (self-reported and parent proxy) were retrospectively collected over four years (2014-2017) at annual LTx clinic visits. The study included 35 patients (18M, 17F) and their parents/guardians. Parent-proxy and child PeLTQL scores (total, subdomain) showed good to excellent agreement (p > 0.05) and did not change over four years (p > 0.05). Younger age (<12 years) and Caucasian ancestry were associated with higher parental and self-reported perceptions of HRQOL, respectively (future health, coping and adjustment, total scores). Parent perceived lower HRQOL in social-emotional sub-domain (p = 0.03) and the child reported lower sub-domain scores related to coping and adjustment (p = 0.04) when the child was noted to have co-morbid conditions related to mental health and neurocognitive development (25.7%). While child-parent perceptions of HRQOL in a multi-ethnic population of pediatric LTx recipients remain unchanged 10 years post-LTx, adolescents of non-Caucasian ancestry remain a population at risk for lower HRQOL.

Diet quality of children post-liver transplantation does not differ from healthy children.

Little has been studied regarding the diets of children following LTX. The study aim was to assess and compare dietary intake and DQ of healthy children and children post-LTX. Children and adolescents (2-18 years) post-LTX (n=27) and healthy children (n=28) were studied. Anthropometric and demographic data and two 24-hour recalls (one weekend; one weekday) were collected. Intake of added sugar, HFCS, fructose, GI, and GL was calculated. DQ was measured using three validated DQ indices: the HEI-C, the DGI-CA, and the DQI-I. Although no differences in weight-for-age z-scores were observed between groups, children post-LTX had lower height-for-age z-scores than healthy children (P<.01). With the exception of vitamin B12, no significant differences in energy and macronutrient (protein, carbohydrate, and fat), added sugar, HFCS, fructose, GI, GL, and micronutrient intakes and DQ indices (HEI-C, DGI-CA, and DQI-I) between groups were observed (P>.05). The majority of children in both groups (>40%) had low DQ scores. No significant interrelationships between dietary intake, anthropometric, and demographic were found (P>.05). Both healthy and children post-LTX consume diets with poor DQ. This has implications for risk of obesity and metabolic dysregulation, particularly in transplant populations on immunosuppressive therapies.

Bone Mineral Density and Growth in Children Having Undergone Liver Transplantation With Corticosteroid-Free Immunosuppressive Protocol.

BACKGROUND: Children post-liver transplantation (post-LTX) are at risk of growth delay and decreased bone mineral density (BMD) secondary to corticosteroid (CS) therapy and suboptimal intake of nutrients important for bone health. The pediatric LTX program at Stollery Children's Hospital introduced a CS-free LTX regimen in 2003. This retrospective study investigated whether the implementation of a CS-free protocol resulted in improvements in BMD (dual x-ray absorptiometry) and growth following LTX. METHODS: A retrospective chart review of all children undergoing LTX was conducted. The parameters included repeated measures of anthropometric (weight, weight z score, height, height z score), BMD/bone mineral content (BMC), laboratory variables, graft function (number/severity of rejection), and CS therapy (dose, duration). RESULTS: A total of 39 patients met study inclusion (20 male; n = 28 on CS; n = 11 CS-free). Mean duration of follow-up was 5.5 ± 3.3 years. The mean weight and height z scores were -0.31 ± 0.14 (CS) and 0.22 ± 0.23 (CS-free; P = .09) and -0.71 ± 0.13 (CS) and 0.23 ± 0.22 (CS-free; P = .002), respectively. Lumbar and whole-body BMD z score less than -2 were present in 15% and 8% of the cohort, respectively. There were no significant differences between CS and CS-free in lumbar BMC (22.2 ± 1.4 and 23.4 ± 2.02 g; P = .165) and lumbar BMD (0.57 ± 0.02 and 0.80 ± 0.22 g/cm2; P = .152), respectively. Lumbar BMC ( r2 = 0.89, P < .05) and whole-body BMC ( r2 = 0.93, P < .05) were inversely related to CS dose >0.2 mg/kg/d and positively related to bone age ( P < .01). CONCLUSION: CS therapy in children post-LTX is associated with reduced BMC and delayed linear growth. Understanding the clinical and nutrition factors influencing bone health is important to optimizing growth and bone health in children post-LTX.

AGY, a Novel Egg Yolk-Derived Anti-gliadin Antibody, Is Safe for Patients with Celiac Disease.

BACKGROUND: Celiac disease (CD) is a gluten-triggered autoimmune disorder of the small intestine. A lifelong gluten-free diet (GFD) is the only approved treatment; however, strict adherence is difficult and many suffer from inadvertent gluten exposure. Oral egg yolk anti-gliadin antibody (AGY) is a novel treatment to neutralize gluten and may improve the efficacy of the GFD. AIMS: To determine the safety, tolerability, and potential efficacy of AGY in patients with CD. METHODS: This 6-week, open-label, single-arm study was conducted in adults with biopsy-proven CD on a GFD. Safety measures included adverse events, physical examination, and clinical laboratory tests. Additional measures included a daily Celiac Symptom Index, Health-Related Quality of life, anti-tissue transglutaminase and anti-gliadin IgA/IgG, and lactulose/mannitol excretion ratio (LMER). A 2-week run-in period to assess questionnaire compliance and acceptability of baseline safety laboratory results was followed by a 4-week treatment period with two AGY capsules taken before meals. RESULTS: Ten patients completed the study (mean age 43.4 years, nine female). All followed a GFD for at least 6 months (mean 5 years). No safety concerns were identified. Most patients had fewer celiac symptoms (especially tiredness, headache, and bloating), improved quality of life, lowered antibodies, and lowered LMER when taking AGY compared to the run-in period. CONCLUSION: In our cohort, AGY was safe and potentially associated with improved CD-related outcome measures in patients on a GFD. A larger study powered for further safety and efficacy evaluation is planned.

Parent readiness for discharge from a tertiary care pediatric cardiology unit.

PURPOSE: This study aimed to measure parents' readiness for discharge from a pediatric cardiology/cardiac surgical inpatient unit. DESIGN AND METHODS: An observational study was conducted at a single tertiary care pediatric cardiac surgical program; parents received teaching from a discharge coordinator, bedside nurse, and, if needed, dietician and pharmacist. We surveyed parents/guardians on the day of discharge and 2 weeks later. RESULTS: We enrolled 181 participants, 53% with children <12 months of age. Length of hospital admission ranged from ≤7 days (54%) to >4 weeks (8%). The most common diagnoses were ventricular septal defect (n = 39), atrial septal defect (n = 28), and coarctation of the aorta (n = 20). Home enteral feeding was required for 21 (12%) children, and 167 (92%) were discharged on medications. Nearly all parents (n = 173, 96%) felt they were ready to take their child home as planned. With respect to medical needs, problems to watch for, who and when to call, what their child was allowed and not allowed to do, and knowledge about follow-up, >90% of respondents rated their knowledge 8+ (range 0-10). Only 68% of respondents rated their knowledge ≥8 regarding services available in their community. Twenty percent experienced challenges at home for which they felt unprepared. These included infection, pain, and gastrointestinal concerns. PRACTICAL IMPLICATIONS: Most parents felt ready for discharge following multidisciplinary teaching. Greater emphasis is needed on teaching families about services available in the community. Further study is required to determine which parents need additional support and education to avoid unanticipated challenges post discharge.

Value of serum cystatin C in estimating renal function in children with non-renal solid organ transplantation.

Children with non-renal solid organ transplants are surviving longer, but outcome is complicated by CKD. Accurate and frequent renal function monitoring is imperative to recognize and institute measures early to reverse, prevent, or arrest progression. This study of 59 children determined the accuracy (P30), bias, sensitivity and specificity between measured renal function by NM-GFR, and estimated GFR by three formulas: Filler (serum cystatin C), mSchwartz (serum creatinine), and CKiD (serum cystatin C, creatinine, urea, and height). Mean GFR by all formulas differed significantly from NM-GFR. Filler and mSchwartz formulas significantly increased the proportion of patients with GFR ≥ 90 mL/min/1.73 m(2) (CKD stage 1) while decreasing those with GFR 60-89 mL/min/1.73 m(2) (CKD stage 2). All formulas overestimated GFR. CKiD showed the highest P30 and lowest bias (79.7%; 6.9 mL/min/1.73 m(2) ) followed by Filler (67.7%; 19.9 mL/min/1.73 m(2) ) and Schwartz (57.6%; 26.8 mL/min/1.73 m(2) ) for all GFR values. All formulas performed best with GFR ≥ 90 mL/min/1.73 m(2) , but CKiD was the only formula to achieve 91.1% accuracy. All formulas showed high sensitivities, but low specificities at NM-GFR cutoff at 90. Thus, GFR estimated by CKiD followed by Filler formula is an adequate method to monitor renal function closely and frequently in these children.

Healthcare transition for youth with heart disease: a clinical trial.

OBJECTIVES: Adolescents with heart disease have complex health needs and require lifelong cardiology follow-up. Interventions to facilitate paediatric to adult healthcare transition are recommended, although outcomes are unknown. We sought to determine the impact of a transition intervention on improving knowledge and self-management skills among this population. METHODS: We conducted a clinical trial of 15-17 year olds with moderate or complex congenital heart disease (CHD) or cardiomyopathy. Participants were systematically allocated to either usual care (controls) or a 1 h nurse-led one-on-one teaching session about their heart. Allocation was determined by week of attendance in the cardiology clinic. The primary outcome was change in Transition Readiness Assessment Questionnaire (TRAQ) score at 6 months, possible scores ranging from 1 (low) to 5 (optimal). Cardiac knowledge (MyHeart score, range 0-100) was a secondary outcome. Analysis was intention to treat. RESULTS: Of 58 participants (48% female), 52 had CHD and 6 had cardiomyopathy. 27 were allocated to the intervention group; 3 declined the intervention and received usual care. When comparing the intervention group with the usual care group at 6 months postintervention, the mean self-management TRAQ score was 3.59 (±0.83) vs. 3.16 (±1.05), respectively (p=0.048, adjusted for baseline score); the mean self-advocacy TRAQ score was 4.38 (±0.56) vs. 4.01 (±0.95) (p=0.18) and the mean MyHeart score was 75% (±15) vs. 61% (±25) (p=0.019). CONCLUSIONS: A 1 h nurse-led transition intervention resulted in a significant improvement in self-management and cardiac knowledge scores. An educational intervention should be routine for youth with congenital or acquired heart disease. TRIAL REGISTRATION NUMBER: NCT01286480.

Metformin and exercise in type 2 diabetes: examining treatment modality interactions.

OBJECTIVE: To determine the effect of metformin on the acute metabolic response to submaximal exercise, the effect of exercise on plasma metformin concentrations, and the interaction between metformin and exercise on the subsequent response to a standardized meal. RESEARCH DESIGN AND METHODS: Ten participants with type 2 diabetes were recruited for this randomized crossover study. Metformin or placebo was given for 28 days, followed by the alternate condition for 28 days. On the last 2 days of each condition, participants were assessed during a nonexercise and a subsequent exercise day. Exercise took place in the morning and involved a total of 35 min performed at three different submaximal intensities. RESULTS: Metformin increased heart rate and plasma lactate during exercise (both P≤0.01) but lowered respiratory exchange ratio (P=0.03) without affecting total energy expenditure, which suggests increased fat oxidation. Metformin plasma concentrations were greater at several, but not all, time points on the exercise day compared with the nonexercise day. The glycemic response to a standardized meal was reduced by metformin, but the reduction was attenuated when exercise was added (metformin×exercise interaction, P=0.05). Glucagon levels were highest in the combined exercise and metformin condition. CONCLUSIONS: This study reveals several ways by which metformin and exercise therapies can affect each other. By increasing heart rate, metformin could lead to the prescription of lower exercise workloads. Furthermore, under the tested conditions, exercise interfered with the glucose-lowering effect of metformin.