Possible Gynecologic Manifestations of Keishi-Bukuryo-Gan Syndrome: A Case Report.

BACKGROUND: Keishi-Bukuryo-Gan (KBG) syndrome is a rare genetic disorder that can present with dysmorphic facial features as well as skeletal, neurological, and developmental abnormalities. Little is reported or understood about the gynecologic associations with KBG syndrome. CASE: Monozygotic twin 14-year-old sisters, both with KBG syndrome, presented independently with abdominal pain, for which they both underwent laparoscopic appendectomies. Intraoperatively, there was no evidence of appendiceal pathology. Both patients were noted to have abnormally appearing elongated ovaries, and 1 was also diagnosed with endometriosis. SUMMARY AND CONCLUSION: The aim of this paper was to highlight a possible gynecologic association with KBG syndrome. For providers caring for this unique subset of patients, it could lower the threshold to suspect endometriosis and increase the suspicion for ovarian dysmorphism.

Pemigatinib-induced nail changes - A case report.

INTRODUCTION: Pemigatinib is an FGFR inhibitor that is one of few second-line treatment options for cholangiocarcinoma. Nail toxicities were common in the phase 2 study evaluating the safety and efficacy of pemigatinib. CASE REPORT: We describe a 54-year-old female with a history of stage IV cholangiocarcinoma presenting for a follow-up visit after completing Cycle 4 of pemigatinib. The patient had been having significant nail changes to her fingernails and toenails, which has led to her great toenails falling off.Management and outcome: The patient was prescribed betamethasone dipropionate 0.05% cream to help with her nail changes and instructed to continue vinegar and hot water soaks that she had already been doing. It was discussed that if this did not help with her nail changes, treatment may have to be held for 2 weeks to allow her nails to heal. DISCUSSION: Nail changes are a common side effect with pemigatinib and should be monitored closely for the need for temporary disruption in therapy.

Atezolizumab-induced hemolytic anemia - A case report.

INTRODUCTION: Atezolizumab is currently the only immunotherapy used in conjunction with nab-paclitaxel for locally advanced or triple negative breast cancer. Limited data is available regarding hemolytic anemia as a side effect of atezolizumab. CASE REPORT: We describe a 59-year-old female with a history of triple negative breast cancer with bone metastases presenting for follow up on Cycle 1, Day 15 of atezolizumab and nab-paclitaxel (100 mg/m2). Patient's complete blood count (CBC) showed macrocytic anemia, with further workup significant for autoimmune hemolytic anemia (AIHA) attributed to atezolizumab.Management and outcome: Patient was started on a high dose prednisone taper starting at 80 mg daily for 16 days, folic acid 1 mg three times daily, iron sucrose, and darbepoetin alfa. Patient's counts recovered, and she was able to start Cycle 2 and continued through Cycle 10 without any additional pre-medications. DISCUSSION: Hemolytic anemia induced by atezolizumab is a rare side effect that was successfully treated in this patient with a prednisone taper.

App-guided exposure and response prevention for obsessive compulsive disorder: an open pilot trial.

Although effective treatments for obsessive-compulsive disorder (OCD) exist, there are significant barriers to receiving evidence-based care. Mobile health applications (Apps) offer a promising way of overcoming these barriers by increasing access to treatment. The current study investigated the feasibility, acceptability, and preliminary efficacy of LiveOCDFree, an App designed to help OCD patients conduct exposure and response prevention (ERP). Twenty-one participants with mild to moderate symptoms of OCD were enrolled in a 12-week open trial of App-guided self-help ERP. Self-report assessments of OCD, depression, anxiety, and quality of life were completed at baseline, mid-treatment, and post-treatment. App-guided ERP was a feasible and acceptable self-help intervention for individuals with OCD, with high rates of retention and satisfaction. Participants reported significant improvement in OCD and anxiety symptoms pre- to post-treatment. Findings suggest that LiveOCDFree is a feasible and acceptable self-help intervention for OCD. Preliminary efficacy results are encouraging and point to the potential utility of mobile Apps in expanding the reach of existing empirically supported treatments.

Right service, right place: optimising utilisation of a community nursing service to reduce planned re-presentations to the emergency department.

BACKGROUND: Congruent with international rising emergency department (ED) demand, a focus on strategies and services to reduce burden on EDs and improve patient outcomes is necessary. Planned re-presentations of non-urgent patients at a regional Australian hospital exceeded 1200 visits during the 2013-2014 financial year. Planned re-presentations perpetuate demand and signify a lack of alternative services for non-urgent patients. The Community Nursing Enhanced Connections Service (CoNECS) collaboratively evolved between acute care and community services in 2014 to reduce planned ED re-presentations. OBJECTIVE: This study aimed to investigate the evolution and impact of a community nursing service to reduce planned re-presentations to a regional Australian ED and identify enablers and barriers to interventionist effectiveness. METHODS: A mixed-methods approach evaluated the impact of CoNECS. Data from hospital databases including measured numbers of planned ED re-presentations by month, time of day, age, gender and reason were used to calculate referral rates to CoNECS. These results informed two semistructured focus groups with ED and community nurses. The researchers used a theoretical lens, 'diffusion of innovation', to understand how this service could inform future interventions. RESULTS: Analyses showed that annual ED planned re-presentations decreased by 43% (527 presentations) after implementation. Three themes emerged from the focus groups. These were right service at the right time, nursing uncertainty and system disconnect and medical disengagement. CONCLUSIONS: CoNECS reduced overall ED planned re-presentations and was sustained longer than many complex service-level interventions. Factors supporting the service were endorsement from senior administration and strong leadership to drive responsive quality improvement strategies. This study identified a promising alternative service outside the ED, highlighting possibilities for other hospital emergency services aiming to reduce planned re-presentations.

Changes in children's sleep duration on food intake, weight, and leptin.

OBJECTIVE: To examine the effect of experimental changes in children's sleep duration on self-reported food intake, food reinforcement, appetite-regulating hormones, and measured weight. METHODS: Using a within-subjects, counterbalanced, crossover design, 37 children, 8 to 11 years of age (27% overweight/obese) completed a 3-week study. Children slept their typical amount at home for 1 week and were then randomized to either increase or decrease their time in bed by 1.5 hours per night for 1 week, completing the alternate schedule on the third week. Primary outcomes were dietary intake as assessed by 24-hour dietary recalls, food reinforcement (ie, points earned for a food reward), and fasting leptin and ghrelin. The secondary outcome was child weight. RESULTS: Participants achieved a 2 hour, 21 minute difference in the actigraph defined sleep period time between the increase and decrease sleep conditions (P < .001). Compared with the decrease sleep condition, during the increase condition, children reported consuming an average of 134 kcal/day less (P < .05), and exhibited lower fasting morning leptin values (P < .05). Measured weights were 0.22 kg lower during the increase sleep than the decrease sleep condition (P < .001). There were no differences in food reinforcement or in fasting ghrelin. CONCLUSIONS: Compared with decreased sleep, increased sleep duration in school-age children resulted in lower reported food intake, lower fasting leptin levels, and lower weight. The potential role of sleep duration in pediatric obesity prevention and treatment warrants further study.

Incidence and management of arthralgias in breast cancer patients treated with aromatase inhibitors in an outpatient oncology clinic.

PURPOSE: Aromatase inhibitors (AIs) are routinely used as first-line adjuvant treatment of breast cancer in postmenopausal women with hormone receptor positive tumors. The current recommended length of treatment with an AI is 5 years. Arthralgias have been frequently cited as the primary reason for discontinuation of AI therapy. Various treatment strategies are proposed in literature, but a standardized treatment algorithm has not been established. The initial purpose of this study was to describe the incidence and management of AI-induced arthralgias in patients treated at Kellogg Cancer Center (KCC). Further evaluation led to the development and the implementation of a treatment algorithm and electronic medical record (EMR) documentation tools. METHODS: The retrospective chart review included 206 adult patients with hormone receptor positive breast cancer who were receiving adjuvant therapy with an AI. A multidisciplinary treatment team consisting of pharmacists, collaborative practice nurses, and physicians met to develop a standardized treatment algorithm and corresponding EMR documentation tool. The treatment algorithm and documentation tool were developed after the study to better monitor and proactively treat patients with AI-induced arthralgias. RESULTS/ CONCLUSIONS: The overall incidence of arthralgias at KCC was 48% (n = 98/206). Of these patients, 32% were documented as having arthralgias within the first 6 months of therapy initiation. Patients who reported AI-induced arthralgias were younger than patients who did not report AI-induced arthralgias (61 vs. 65 years, p = 0.002). There was no statistical difference in the incidence of arthralgias in patients with a history of chemotherapy (including taxane therapy) compared to those who did not receive chemotherapy (p = 0.352). Of patients presenting with AI-induced arthralgias, 41% did not have physician-managed treatment documented in the EMR. A standardized treatment algorithm and electronic chart documentation tools were then developed by the multidisciplinary team.

Outcomes of computerized physician order entry in an electronic health record after implementation in an outpatient oncology setting.

PURPOSE: Computerized physician order entry (CPOE) in electronic health records (EHR) has been recognized as an important tool in optimal health care provision that can reduce errors and improve safety. The objective of this study is to describe documentation completeness and user satisfaction of medical charts before and after implementation of an outpatient oncology EHR/ CPOE system in a hospital-based outpatient cancer center within three treatment sites. METHODS: This study is a retrospective chart review of 90 patients who received one of the following regimens between 1999 and 2006: FOLFOX, AC, carboplatin + paclitaxel, ABVD, cisplatin + etoposide, R-CHOP, and clinical trials. Documentation completeness scores were assigned to each chart based on the number of documented data points found out of the total data points assessed. EHR/CPOE documentation completeness was compared with completeness of paper charts orders of the same regimens. A user satisfaction survey of the paper chart and EHR/CPOE system was conducted among the physicians, nurses, and pharmacists who worked with both systems. RESULTS: The mean percentage of identified data points successfully found in the EHR/CPOE charts was 93% versus 67% in the paper charts (P < .001). Regimen complexity did not alter the number of data points found. The survey response rate was 64%, and the results showed that satisfaction was statistically significant in favor of the EHR/CPOE system. CONCLUSION: Using EHR/CPOE systems improves completeness of medical record and chemotherapy order documentation and improves user satisfaction with the medical record system. EHR/CPOE requires constant vigilance and maintenance to optimize patient safety.

Microarray analysis of senescent vascular smooth muscle cells: A link to atherosclerosis and vascular calcification.

Little is known about the senescent phenotype of human vascular smooth muscle cells (VSMCs) and the potential involvement of senescent VSMCs in age-related vascular disease, such as atherosclerosis. As such, VSMCs were grown and characterised in vitro to generate senescent VSMCs needed for microarray analysis (Affymetrix). Comparative analysis of the transcriptome profiles of early (14 CPD) and late (39-42 CPD) passage VSMCs found a total of 327 probesets called as differentially expressed: 149 are up-regulated in senescence and 178 repressed (p-value<0.5%, minimum effect size of at least 2-fold differential regulation, explore data at http://www.madras.cf.ac.uk/vsmc). Data mining shows a differential regulation of genes at senescence associated with the development of atherosclerosis and vascular calcification. These included genes with roles in inflammation (IL1beta, IL8, ICAM1, TNFAP3, ESM1 and CCL2), tissue remodelling (VEGF, VEGFbeta, ADM and MMP14) and vascular calcification (MGP, BMP2, SPP1, OPG and DCN). The microarray data for IL1beta, IL8 and MGP were validated by either, ELISA, Western blot analysis or RT-PCR. These data thus provide the first evidence for a role of VSMC senescence in the development of vascular calcification and provides further support for the involvement of senescent VSMCs in the progression of atherosclerosis.