Transplantation in paediatric patients with MMA requires multidisciplinary approach for achievement of good clinical outcomes.

BACKGROUND: As modern medicine is advancing, younger, small, and more complex children are becoming multi-organ transplant candidates. This brings up new challenges in all aspects of their care. METHODS: We describe the first report of a small child receiving a simultaneous liver and kidney transplant and abdominal rectus sheath fascia transplant on the background of Williams syndrome and methylmalonic acidaemia. At the time of transplantation, the child was 3 years old, weighed 14.0 kg, had chronic kidney disease stage V, and had not yet started any other form of kidney replacement therapy. RESULTS: There were many anaesthetic, medical, metabolic, and surgical challenges to consider in this case. A long general anaesthetic time increased the risk of cardiac complications and metabolic decompensation. Additionally, the small size of the patient and the organ size mis-match meant that primary abdominal closure was not possible. The patient's recovery was further complicated by sepsis, transient CNI toxicity, and de novo DSAs. CONCLUSIONS: Through a multidisciplinary approach between 9 specialties in 4 hospitals across England and Wales, and detailed pre-operative planning, a good outcome was achieved for this child. An hour by hour management protocol was drafted to facilitate transplant and included five domains: 1. management at the time of organ offer; 2. before the admission; 3. at admission and before theatre time; 4. intra-operative management; and 5. post-operative management in the first 24 h. Importantly, gaining a clear and in depth understanding of the metabolic state of the patient pre- and peri-operatively was crucial in avoiding metabolic decompensation. Furthermore, an abdominal rectus sheath fascia transplant was required to achieve abdominal closure, which to our knowledge, had never been done before for this indication. Using our experience of this complex case, as well as our experience in transplanting other children with MMA, and through a literature review, we propose a new perioperative management pathway for this complex cohort of transplant recipients.

Reframing Adolescent Development: Identifying Communications Challenges and Opportunities.

Understanding Americans' deeply held and widely shared assumptions about adolescents and their development can reveal key opportunities and challenges for developmental science communicators. Twenty-nine in-depth interviews were conducted with adolescents and adults about adolescence. We analyzed the cultural models the public use to make meaning about what adolescence is, what development involves, what adolescents need, and how adolescents can be supported. The analysis revealed several cultural models that may impede public engagement around youth issues. These dominant ways of thinking include a strong focus on the vulnerability of adolescence and a narrow understanding of how environments affect adolescent development. The findings have important implications for communicators seeking to expand and deepen public thinking about adolescence.

Associations Between Mother-Adolescent and Father-Adolescent Relationships and Young Adult Health.

Importance: Studies linking the quality of parent-adolescent relationships with young adult health outcomes could inform investments to support these complex relationships. Objective: To evaluate whether consistently measured, modifiable characteristics of parent-adolescent relationships are associated with young adult health across multiple domains. Design, Setting, and Participants: This cohort study used data from waves I (1994-1995; ages 12-17 years) and IV (2008-2009; ages 24-32 years) of the US National Longitudinal Study of Adolescent to Adult Health. Of 20 745 adolescents enrolled in wave I, 15 701 of 19 560 who were eligible completed wave IV (response rate, 80.3%). Data analyses were conducted from February 2019 to November 2020. Exposures: Parental warmth, parent-adolescent communication, time together, relationship and communication satisfaction, academic expectations, and maternal inductive discipline as reported at wave I by adolescent participants. Main Outcomes and Measures: Wave IV participant-reported self-rated health, depression, stress, optimism, nicotine dependence, substance abuse symptoms (alcohol, cannabis, or other drugs), unintended pregnancy, romantic relationship quality, physical violence, and alcohol-related injury. Separate regression models were run for mother-adolescent and father-adolescent relationships while controlling for age, biological sex, race and ethnicity, parental educational level, family structure, and child maltreatment experiences. Results: A total of 10 744 participants (mean [SD] age at wave IV, 28.2 [1.8] years; 52.0% female; 67.3% non-Hispanic White) and 8214 participants (mean [SD] age at wave IV, 28.2 [1.8] years; 50.8% female; 71.9% non-Hispanic White) had valid sampling weights and complete data for mother-adolescent and father-adolescent relationship characteristics, respectively. Adolescents who reported higher levels of mother-adolescent warmth (ß = 0.11 [95% CI, 0.06-0.15]), communication (ß = 0.02 [95% CI, 0.00-0.04]), time together (ß = 0.07 [95% CI, 0.05-0.09]), academic expectations (ß = 0.05 [95% CI, 0.02-0.08]), relationship or communication satisfaction (ß = 0.07 [95% CI, 0.04-0.10]), and inductive discipline (ß = 0.03 [95% CI, 0.01-0.05]) reported significantly higher levels of self-rated general health in young adulthood. Adolescents who reported higher levels of father-adolescent warmth (ß = 0.07 [95% CI, 0.03-0.11]), communication (ß = 0.03 [95% CI, 0.01-0.05]), time together (ß = 0.06 [95% CI, 0.03-0.08]), academic expectations (ß = 0.04 [95% CI, 0.01-0.06]), and relationship satisfaction (ß = 0.07 [95% CI, 0.04-0.10]) also reported significantly higher levels of self-rated general health in young adulthood. Adolescents reporting higher levels of all exposures also reported significantly higher levels of optimism and romantic relationship quality in young adulthood (ß coefficient range, 0.02 [95% CI, 0.00-0.04] to 0.24 [95% CI, 0.15-0.34]) and lower levels of stress and depressive symptoms (ß coefficient range, -0.07 [95% CI, -0.12 to -0.02] to -0.48 [95% CI, -0.61 to -0.35]). Higher levels of parental warmth, time together, and relationship or communication satisfaction were significantly associated with lower levels of nicotine dependence (odds ratio range, 0.78 [95% CI, 0.72-0.85] to 0.89 [95% CI, 0.81-0.98]) and substance abuse symptoms (incidence rate ratio range, 0.60 [95% CI, 0.50-0.73] to 0.94 [95% CI, 0.89-0.99]), as well as lower odds of unintended pregnancy (odds ratio range, 0.81 [95% CI, 0.74-0.88] to 0.93 [95% CI, 0.86-0.99]). Patterns were less consistent for physical violence and alcohol-related injury. Characteristics of mother-adolescent and father-adolescent relationships were similarly associated with young adult outcomes. Conclusions and Relevance: The findings of this cohort study suggest that adolescents' positive perceptions of their relationships with their mothers and fathers are associated with a wide range of favorable outcomes in young adulthood. Investments in improving parent-adolescent relationships may have substantial benefits for young adult population health.

Associations between weight misperception, contextual factors, and weight loss behaviours in young adult men with overweight/obesity.

OBJECTIVE: Young men are less likely to engage in weight loss behaviours than their female counterparts. This may be because of an increased likelihood for young men, particularly young black men, with overweight/obesity to misperceive their weight status. This study examined racial differences in weight status perception accuracy and associations between this perception and weight loss behaviours among young men. Associations between weight loss behaviours and contextual factors were also explored. METHODS: Data from 1417 young adult (YA) men with overweight/obesity from the 2007 to 2014 National Health and Nutrition Examination Survey (NHANES) were analysed. Associations between weight status perception accuracy, contextual factors, and weight loss attempts and behaviours were examined with logistic regression. RESULTS: YA men with overweight/obesity were more likely to report weight loss attempts and behaviours if they perceived themselves as being overweight (OR = 3.10; 95% CI, 2.18-4.41; P < .01; OR = 3.20, 95% CI, 2.16-4.72, P < .01, respectively). Greater education and income were associated with a greater likelihood of reporting weight loss attempts and healthy weight loss behaviours. Greater reported depressive symptoms were associated with reported weight loss attempts but not healthy weight loss behaviours. There were no differences by race for reported weight loss attempts or behaviours. CONCLUSION: Among YA men with overweight/obesity, perceiving oneself as overweight was associated with reporting weight loss attempts and healthy weight loss behaviours. Future research should consider how weight status perception accuracy affects weight loss attempts among YA men and what additional factors may account for racial differences.

Ten-Year Secular Trends in Youth Violence: Results From the Philadelphia Youth Risk Behavior Survey 2003-2013.

BACKGROUND: Youth violence reduction is a public health priority, yet few studies have examined secular trends in violence among urban youth, who may be particularly vulnerable to numerous forms of violence. This study examines 10-year secular trends in the prevalence of violence-related behaviors among Philadelphia high school students. METHODS: Repeated cross-sectional data were analyzed from 5 waves of the Philadelphia Youth Risk Behavior Survey (YRBS) from 2003 to 2013. Sex-specific multivariate regression models were used to examine secular trends in multiple types of violence, accounting for age, race/ethnicity, and sampling strategy. RESULTS: In 2013, the most prevalent violent behavior was physical fighting among boys (38.4%) and girls (32.7%). Among girls, the prevalence of sexual assault and suicide attempts declined between 2003 and 2013 (ß = -0.13, p = .04 and ß = -0.14, p = .007, respectively). Among boys, significant declines in carrying a weapon (ß = -0.31, p < .001), carrying a gun (ß = -0.16, p = .01), and physical fighting (ß = -0.35, p = .001) were observed. CONCLUSIONS: Whereas the prevalence of some forms of violence stabilized or declined among Philadelphia youth during 2003-2013 time span, involvement in violence-related behaviors remains common among this population. Continued surveillance and evidence-based violence reduction strategies are needed to address violence among urban youth.

Impact of online patient reminders to improve asthma care: A randomized controlled trial.

IMPORTANCE: Asthma is one of the most burdensome chronic illnesses in the US. Despite widespread dissemination of evidence-based guidelines, more than half of the adults with asthma have uncontrolled symptoms. OBJECTIVE: To examine the efficacy of an online tool designed to improve asthma control. DESIGN: 12-month single blind randomized controlled trial of the online tool (Intervention condition, IC) versus an active control tool (CC). SETTING: Patients enrolled in an insurance plan. PARTICIPANTS: Participants were 408 adults (21-60 years of age) with persistent asthma. INTERVENTION: At least once each month and before provider visits, participants in the IC answered questions online about their asthma symptoms, asthma medications and asthma care received from providers, such as an asthma management plan. The tool then provided tailored feedback to remind patients 1) to ask health care providers specific questions that may improve asthma control (e.g., additional controller medications) and 2) to consistently perform specific self-care behaviors (e.g., proper inhaler technique). Participants in the CC received similar questions and feedback, yet focused instead on preventive services unrelated to asthma control (e.g., cancer screening). MAIN OUTCOME MEASURES: The main outcome measure was asthma control, as assessed by the 5-question Asthma Control Test (ACT). Secondary outcomes included quality of life, medication use and healthcare utilization (e.g., emergency department visits). RESULTS: After 12 months, 323 participants completed follow-up measures (79.2%). Participants in the IC reported a greater mean improvement in the ACT score than participants in the CC (2.3 vs. 1.2; p = 0.02) and 9 of 11 individual asthma control survey items showed non-significant improvements favoring the IC. No differences were observed in medication adherence, number of asthma controller medications or health care utilization. CONCLUSION AND RELEVANCE: Simple and brief online patient reminders improved asthma control among insured patients. Although future studies are needed to understand the mechanism of the improvement, the magnitude of the effect on asthma control was similar to the addition of an additional controller medication. Given the widespread use of the Internet, simple tools such as this may be useful for improving the control of other chronic diseases as well. TRIAL REGISTRATION: This study is registered at clinicaltrials.gov, NCT00921401, "Improving the Quality of Asthma Care Using the Internet".

The impact of physician weight discussion on weight loss in US adults.

PROBLEM: The increasing prevalence of overweight and obesity in the United States and worldwide is at epidemic levels. Physicians may play a vital role in addressing this epidemic. We aimed to examine the association of a physician's discussion of patients' weight status with self-reported weight loss. We hypothesized that physician discussion of patients' being overweight is associated with increased weight loss in patients with overweight and obesity. METHODS: Data analysis of participants (n = 5054) in the National Health and Nutritional Examination Survey (NHANES) in 2005-2008. The main outcome was rates of self-reported weight loss and the association with physicians' discussion of their patients' weight status. RESULTS: Overweight and obese participants were significantly more likely to report a 5% weight loss in the past year if their doctor had told them they were overweight (adjusted OR (AOR) 1.88; 95% CI 1.45-2.44; AOR 1.79; 95% CI 1.30-2.46, respectively). CONCLUSIONS: Physicians' direct discussion of their patients' weight status is associated with clinically significant patient weight loss and may be a targetable intervention. Further studies are needed to determine if increasing physician discussion of patients' weight status leads to significant weight loss.

Serum galactosyltransferase isoform changes in rheumatoid arthritis.

OBJECTIVE: To investigate glycosylation changes associated with rheumatoid arthritis (RA) by determining whether there are beta1,4-galactosyltransferase (GTase) isoforms specific to or altered in the serum of patients with RA. Methods. Serum GTase isoform profiles were determined using isoelectric focusing (IEF) in patients with active RA (n = 9), disease controls (DC; n = 9), and healthy individuals (HI; n = 10). RESULTS: There was a highly significant difference (p < 0.0001) between the IEF profiles. The RA IEF profile was significantly (p < 0.0001) different from that of the DC or the HI group. There was, however, no significant difference between the DC and HI profiles. Serum GTase samples from 8/9 RA, 9/9 DC, and 9/10 HI resolved into 2 distinct peaks of activity. The RA isoform profile was associated with an acidic shift. There were no significant differences in the pH value of the first peak; the second peak was found to be significantly more acidic in the RA group (mean pH 5.02) compared to the DC and HI group (mean pH 5.20; p < 0.05). The RA associated isoform constituted a significantly greater proportion of total enzymatic activity in the RA sera (16.1%) compared to DC and HI (13.5%; p < 0.05 and 12.6%; p < 0.01, respectively). RA and HI serum GTase desialylation resulted in an alkaline shift of the isoforms into similar pH bands: 5.25-5.50, 5.70-5.85, and 6.20-6.40. GTase was found to be on average 75% more active in its desialylated form than in its sialylated state. CONCLUSION: RA is associated with a differential expression of GTase isoforms. This may be due to increased hypersialylation, which has the potential to adversely affect the catalytic activity of the enzyme, thus providing a possible mechanism for posttranslational regulation of GTase activity in RA.