Feasibility and Acceptability of a mHealth Self-Management Intervention for Pediatric Transplant Families.

Families of pediatric solid organ transplant recipients need ongoing education and support in the first 30 days following hospital discharge for the transplantation. The purpose of this report is to describe the feasibility, acceptability, and preliminary efficacy of a mHealth family-self management intervention, (myFAMI), designed to improve post-discharge outcomes of coping, family quality of life, self-efficacy, family self-management, and utilization of health care resources. We enrolled 46 primary family members. myFAMI was feasible and acceptable; 81% (n=17/21) of family members completed the app at least 24/30 days (goal 80% completion rate). Family members generated 134 trigger alerts and received a nurse response within the goal timeframe of < 2 h 99% of the time. Although there were no significant differences between groups, primary outcomes were in the expected direction. The intervention was well received and is feasible for future post-discharge interventions for families of children who receive an organ transplant.

Phenotypic Differences in Juvenile Polyposis Syndrome With or Without a Disease-causing SMAD4/BMPR1A Variant.

Juvenile polyposis syndrome (JPS) is a clinically diagnosed hamartomatous polyposis syndrome that increases the risk of gastrointestinal cancer. Approximately 40%-50% of JPS is caused by a germline disease-causing variant (DCV) in the SMAD4 or BMPR1A genes. The aim of this study was to characterize the phenotype of DCV-negative JPS and compare it with DCV-positive JPS. Herein, we analyzed a cohort of 145 individuals with JPS from nine institutions, including both pediatric and adult centers. Data analyzed included age at diagnosis, family history, cancer history, need for colectomy/gastrectomy, and polyp number and location. Compared with DCV-positive JPS, DCV-negative JPS was associated with younger age at diagnosis (P < 0.001), lower likelihood of having a family history of JPS (P < 0.001), and a lower risk of colectomy (P = 0.032). None of the DCV-negative individuals had gastric or duodenal polyps, and polyp burden decreased after the first decade compared with DCV-positive JPS. Subgroup analysis between SMAD4 and BMPR1A carriers showed that SMAD4 carriers were more likely to have a family history of JPS and required gastrectomy. Taken together, these data provide the largest phenotypic characterization of individuals with DCV-negative JPS to date, showing that this group has distinct differences compared with JPS due to a SMAD4 or BMPR1A variant. Better understanding of phenotype and cancer risk associated with JPS both with and without a DCV may ultimately allow for individualized management of polyposis and cancer risk.Prevention Relevance: Juvenile Polyposis Syndrome (JPS) is a gastrointestinal cancer predisposition syndrome requiring lifelong surveillance, however there is limited data comparing individuals with and without a germline disease-causing variant in SMAD4 or BMPR1A Herein we show that individuals with JPS without an underlying disease-causing variant have distinct phenotypic differences including lack of upper gastrointestinal polyps and lower rates of a family history of JPS, suggesting that a different approach to management may be appropriate in this population.

Closed and Open Reduction of Displaced Pediatric Lateral Condyle Humeral Fractures, a Study of Short-Term Complications and Postoperative Protocols.

BACKGROUND: Displaced lateral condyle humeral fractures in children are treated operatively to maximize function and growth of the elbow. Traditionally an open approach is used for reduction of the fracture, but recent series have shown promising results with closed reduction. Percutaneous pins are typically used for fixation, no matter the reduction method. This retrospective review compares our experience with early complications after open and closed reduction of these fractures. METHODS: We retrospectively reviewed charts and radiographs of operatively treated lateral condyle fractures. The Song and Jakob classification systems were utilized to determine fracture severity. High-grade displacement was defined as Song stage 4 and 5, or Jakob type 3. Data was analyzed by fracture type (high-grade or low-grade) and by treatment method to look for differences in complication rates and treatment differences. Complications were defined as delayed union and infection. RESULTS: 172 fractures were analyzed, 141 were treated open, and 31 were treated closed. There were no statistically significant differences in pin duration, total cast time, additional procedures, or short term complications between the open and closed treatment groups, or the high and low-grade fracture groups. High-grade fractures were more likely to be treated with open reduction (p<0.0001). Pin duration prior to removal was not associated with increased incidence of infection or delayed union. CONCLUSIONS: Closed reduction and percutaneous pinning of lateral condyle fractures amenable to this treatment does not seem to require any changes in postoperative treatment or alter the incidence of early complications when compared to open procedures. LEVEL OF EVIDENCE: IV.